Abstract

Background:The scleroderma is a complex autoimmune collagen disorder that can affect many organs simultaneously, as it occurs in the systemic sclerosis (SS), or only the skin, as it occurs in the localized scleroderma (LS). The neurological presentation is extremely uncommon, and even more uncommon are the symptoms of the scleroderma in the cerebellum.

Case Description:We report the case of a 56-year-old male with cerebellar lesions mimicking a brain abscess. After surgical excision, the histopathological diagnosis deposed for an ischemic necrosis caused by a vasculopathy. All the bacteriological and viral exams were negative, whereas the rheumatologic tests were compatible with the scleroderma pattern.

Conclusion:Up to now, the literature has described only 5 cases of scleroderma in the posterior cranial fossa. The authors report a case of SS causing colliquative necrosis in the cerebellum. Pathogenetic mechanisms, clinical aspects, and radiological features are discussed along with the pertinent literature.

Keywords: Cerebellum, colliquative necrosis, posterior cranial fossa, scleroderma

INTRODUCTION

The term “scleroderma” literally means “hard skin,”[ 18 ] however generally, this term includes disease processes that can affect only the skin in the localized scleroderma (LS) or can develop progressive multiple organ disorders in the systemic sclerosis (SS).[ 8 ] The annual incidence of scleroderma is 6–12 patients per million population with a female predominance.[ 15 ]

In general, the neurological presentation is extremely rare.[ 15 ] This is probably due to the lack of adventitial fibrous tissues in the central nervous system (CNS).[ 8 ] Only a few case reports have described the potential symptoms of scleroderma in the cerebellum, and in no one of them, surgery has been reported to be necessary.

The authors report the first case of ischemic necrosis of the cerebellum in a patient with SS mimicking a brain abscess. Clinical aspects, radiological features, surgical treatment, and operative findings are discussed along with a review of the pertinent literature.

CASE REPORT

A 56-year-old male was admitted to our Institute with a sudden headache and dizziness accompanied by nausea and vomiting. The patient had been in good health until this event, except for a history of skin thickening, a Raynaud's phenomenon with acrocyanosis to the fingers from 1 year and arthralgia from 3 years. Other sclerotic lesions were observed on the knees and elbows.

On admission, his blood pressure, pulse, body temperature, and respiration were normal. The neurological examination showed dysmetria, nystagmus, and dizziness. There were neither sensory nor motor deficits and cranial nerves abnormality. The results of the routine hematologic tests showed only neutrophilic leukocytosis with relative lymphocytopenia. The initial brain computerized tomography (CT) scan and the subsequent magnetic resonance imaging (MRI) showed a lesion with fluid collection, measuring 1.5 × 3.5 cm in the right cerebellar hemisphere [ Figure 1 ]. The CT of the total body did not detect lesions of the internal organs. A right suboccipital craniotomy was performed, the fluid collection was free-hand evacuated, and the wall of the lesion was resected [ Figure 2 ]. The bacteriological exams of the fluid collection and of the blood cultures were negative. The echocardiogram showed no pathological lesions. The histopathological diagnosis of the wall of the lesion deposed for ischemic necrosis caused by a vasculitis [ Figure 3 ]. The post-operative MRI showed no residual lesions [ Figure 4 ].

Figure 1

Preoperative magnetic resonance image in axial, coronal, and sagittal view showing a lesion with fluid collection, measuring 1.5 × 3.5 cm. localized in the right cerebellar hemisphere (a-c)

Figure 2

After suboccipital craniotomy, the intraoperative visualization showed a subcortical colliquative necrosis (a and b). The wall of the lesion was resected (c) and the fluid collection was completely free-hand evacuated (d)

Figure 3

Imaging in hematoxylin and eosin and original magnification ×20 and ×40 showed cerebellar necrosis associated with microparenchymal hemorrhage, leukocytoclastic vasculitis, and swollen endothelial cells (a and b). Immunohistochemistry imaging (CD 68 +) showed massive macrophages infiltration in the cerebellar parenchyma (c and d)

Figure 4

Postoperative magnetic resonance in axial, coronal, and sagittal view showing the complete resection of the lesion (a-c)

Further investigations tested positive for antinuclear antibody (ANA) >1:160 and Scl-70 (16.3 UA/mL), whereas other tests for rheumatology and infectious diseases tested negative (antiphospholipid anticentromere and anticardiolipin antibodies, Lupus-like anticoagulant, anti-DNA autoantibodies, perinuclear antineutrophil antibodies (P-ANCA), cytoplasmic antineutrophil antibodies (C-ANCA), Anti-Smith antibodies (Anti-Sm), Anti-Sjögren’s-syndrome-related antigen A/B (Anti-SSA/B), Antibodies against antigen Ro52 (Anti-Ro52), antibodies histidyl-tRNA synthetase Jo-1 (Anti-Jo1), major centromere autoantigen B (CENP-B), Nucleosomes, Ribosomal P- Protein, Anti-Ribonuclear Protein (Anti-U1RNP), Histones, Protein S Factor V Leiden, Hepatitis B surface antigen (HBs-Ag), Hepatitis C Core antibodies (HCV-Ab), antibodies Human Immunodeficiency Virus (HIV-Ab), Veneral Disease Research Laboratory). In addition to this, it was found an increase of circulating immune complexes (38 μg/ml), α1-acid-glycoprotein (208 mg/dl) and C-reactive protein (0.80 mg/dl). The capillaroscopy in the periungual area showed slight disorganization of the capillary architecture, numerous giant capillaries and microhemorrhages, moderate capillary loss, and few ramified capillaries with initial neoangiogenesis. The skin biopsy confirmed a focal extension of dermal collagen in the subcutaneous tissue. All these conditions were compatible with a scleroderma pattern. The patient was sent to our rheumatology reference center to start a specific therapy. At 1-year follow-up there were no other complications.

DISCUSSION

In general, the scleroderma represents a complex autoimmune collagen disorder.[ 10 ] The etiology remains obscure and the course is unpredictable.[ 18 ] It can be a multisystemic disease affecting the skin, lungs, kidneys, vascular system, myocardium, nervous system, and gastrointestinal tract,[ 12 ] or affecting only the skin.[ 8 ] In both the SS and LS, the CNS lesions are considered an uncommon symptom. Even more uncommon are the lesions of the scleroderma localized only in the cerebellum.[ 8 12 ]

Currently, only 5 cases have been described in literature.[ 1 3 9 13 15 ] The average age of the reported patients is 35 years, however, in one case, the patient was 5 years old. There is a female prevalence (4:1). In most of these cases, the cerebellar lesions are secondary to the vascular effects of the scleroderma. In 3 cases, the lesions were caused by the SS, whereas in 2 cases, they were caused by the LS. Raynaud's phenomenon was present only in the SS. The presence of the Anti-Scl-70 and Anti-U1RNP antibody was not always reported. Hypertension was reported in only 2 cases of SS. Surgical treatment was not considered to be necessary, except for our case. We have not found previous reports of cerebellar ischemia with colliquative necrosis associated with the SS [ Table 1 ].

Table 1

Review of the literature on cerebellar lesions in patients with scleroderma

In literature, no studies have correlated the posterior circulation and cerebellum with SS, whereas several theories have been proposed to explain the mechanism of a vascular damage caused by the SS in CNS. Some authors think that the pathogenic mechanism is caused both by the cell-mediated activity and autoantibody production responsible for a chronic inflammation. This determines a progressive fibrosis of the visceral organs and vascular damage, including the intracranial vessels.[ 2 ]

Other studies have shown that the vasculopathy in the SS is caused by an obliterative vasculopathy rather than by the classical atherosclerosis.[ 17 ] In addition, in the early stages of SS, an onset of endothelial dysfunction has been demonstrated, progressively followed by more severe endothelial damages, such as necrosis and devascularization.[ 2 14 ] The endothelial dysfunction has been correlated with an increased risk of acute ischemic stroke.[ 2 16 ] Another possible pathogenetic mechanism is an increased vasospasm in patients with SS.[ 2 6 ]

In general, the cerebral angiopathy in the scleroderma is associated with the presence of malignant hypertension or renal disease,[ 4 5 ] however, in our case, as well as in a few cases present in literature,[ 4 5 12 ] these are not present.

Other rare CNS abnormalities described include encephalopathy, subarachnoid hemorrhage, psychosis, anxiety, and trigeminal neuropathy.[ 4 5 12 ]

There are few reports on the possible associations of different autoantibodies with neurological manifestations of scleroderma.[ 7 ] According to Hietarinta et al.,[ 7 ] the positivity for anti-U1RNP and anti-Scl-70 antibodies in patients with SS could be related with the pathogenesis of the microangiopathy. Furthermore, on the basis of their study, these patients are more prone to developing neurological symptoms.[ 7 ]

We think that the involvement of the CNS is unpredictable, and according to Mohammed et al.,[ 11 ] the use of the MRI might allow an early detection of CNS involvement in patients with SS.

CONCLUSION

We believe that in our case the cerebellar lesion was caused by microangiophaty of the posterior circulation secondary to a high activity of the SS. The pathogenetic mechanism that causes the colliquative necrosis might be generated by microglia and macrophages surrounding the cerebellar vessels. In response to inflammatory stimuli, secondary to the scleroderma effect on the microcirculation, the microglia rapidly transform into an activated phenotype, elaborating both neurotoxic and neurotrophic factors responsible for the formation of a colliquative collection.

In our case, the first manifestation of the scleroderma is cerebellar, and therefore, in the presence of a brain lesion similar to an abscess, it is recommended to include autoimmune diseases such as the scleroderma among the diagnostic hypotheses.

Consent

Written informed consent was obtained from the patient for publication of this case report and any accompanying images.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

Acknowledgements

The authors thank Maria Silvia Marottoli for her assistance in the translation.

References

1. Allmendinger AM, Ricci JA, Desai NS, Viswanadhan N, Rodriguez D. Atypical Neuroimaging Manifestations of Linear Scleroderma “en coup de sabre”. Iran J Child Neurol. 2015. 9: 62-8

2. Chiang CH, Liu CJ, Huang CC, Chan WL, Huang PH, Chen TJ. Systemic sclerosis and risk of ischaemic stroke: A nationwide cohort study. Rheumatology. 2013. 52: 161-5

3. Choi EJ, Lee DW, Park CW, Lee SH. A case of linear scleroderma involving cerebellum with vertigo. Korean J Audiol. 2012. 16: 87-90

4. Das CP, Prabhakar S, Lal V, Kharbanda PS. Scleroderma, stroke, optic neuropathy: A rare association. Neurol India. 2002. 50: 504-7

5. Estey E, Lieberman A, Pinto R, Meltzer M, Ransohoff J. Cerebral arteritis in scleroderma. Stroke. 1979. 10: 595-7

6. Héron E, Fornes P, Rance A, Emmerich J, Bayle O, Fiessinger JN. Brain involvement in scleroderma: Two autopsy cases. Stroke. 1998. 29: 719-21

7. Hietarinta M, Lassila O, Hietaharju A. Association of anti-U1RNP- and anti-Scl-70-antibodies with neurological manifestations in systemic sclerosis (scleroderma). Scand J Rheumatol. 1994. 23: 64-7

8. Kanzato N, Matsuzaki T, Komine Y, Saito M, Saito A, Yoshio T, Suehara M. Localized scleroderma associated with progressing ischemic stroke. Neurol Sci. 1999. 163: 86-9

9. Kawano H, Hayashi M, Handa Y, Miyazaki S. A case of progressive systemic sclerosis associated with a hemorrhagic infarction of the cerebellum. No To Shinkei. 1990. 42: 189-91

10. Mohamed RH, Nassef AA. Brain magnetic resonance imaging findings in patients with systemic sclerosis. Int J Rheum Dis. 2010. 13: 61-7

11. Mohammed RH, Sabry YY, Nasef AA. Brain MRI screening showing evidences of early central nervous system involvement in patients with systemic sclerosis. Rheumatol Int. 2011. 31: 667-71

12. Pathak R, Gabor AJ. Scleroderma and central nervous system vasculitis. Stroke. 1991. 22: 410-3

13. Pinheiro L, Freitas J, Lucas M, Victorino RM. Cerebellar atrophy in systemic sclerosis. J R Soc Med. 2004. 97: 537-8

14. Prescott RJ, Freemont AJ, Jones CJ, Hoyland J, Fielding P. Sequential dermal microvascular and perivascular changes in the development of scleroderma. J Pathol. 1992. 166: 255-6

15. Poursadegh Fard M, Karami Magham S. Cerebral sinus thrombosis in scleroderma: A case report. Acta Med Iran. 2012. 50: 288-91

16. Roquer J, Segura T, Serena J, Castillo J. Endothelial dysfunction, vascular disease and stroke: The ARTICO study. Cerebrovasc Dis. 2009. 27: 25-37

17. Soltész P, Kerekes G, Dér H, Szücs G, Szántó S, Kiss E. Comparative assessment of vascular function in autoimmune rheumatic diseases: Considerations of prevention and treatment. Autoimmun Rev. 2011. 10: 416-25

18. Tuffanelli DL, Winkelmann RK. Systemic scleroderma, a clinical study of 727 cases. Arch Dermatol. 1961. 84: 359-71

Abstract

Background:Remote cerebellar hemorrhage (RCH) is a rare complication after supratentorial craniotomies, which usually presents as linear hemorrhages on the surface of the cerebellum; the exact mechanism of it is not established yet.

Case Description:In case one, a 57-year-old patient demonstrated hemorrhage in the cerebellar sulci in favor of RCH 2 days after craniotomy for sphenoidal wing meningioma resection. He was asymptomatic and showed good prognosis after conservative treatment. However, in the second case, a 21-year-old man presented with symptomatic RCH just after the surgery for resection of huge intraaxial parietooccipital lesion. He had a poor prognosis despite the treatment and died ultimately.

Conclusion:Although some studies reported the good prognosis for this type of hemorrhage, it can cause neurological and clinical deterioration and result to patient death.

Keywords: Postoperative complication, remote cerebellar hemorrhage, supratentorial craniotomy

BACKGROUND

Remote cerebellar hemorrhage (RCH) after supratentorial surgery is rare, ranging between 0.08% and 0.6% in the literature, with poorly-understood underlying mechanisms.[ 2 ] Here, we report two cases of RCH after supratentorial craniotomy with two different presentations and outcomes.

CASE DESCRIPTION

Case 1

A 57-year-old male was presented with headaches, gait problem, and dizziness. Physical examination showed no significant deficits. The brain imaging demonstrated an enhancing extra-axial mass in the left frontal lobe adjacent to the left carotid artery bifurcation in favor of inner third sphenoidal wing meningioma [ Figure 1a ]. Preoperative lab tests, including coagulopathy tests, were normal.

Figure 1

Case one: (a) axial T1weighted magnetic resonance imaging defines anatomic location of the left sphenoidal wing meningioma, (b) early postoperative axial brain CT scan lacks any sign in favor of RCH, (c) late postoperative scan unmask RCH and (d) the scan just before patient discharge depicted resolution of hematoma

Under general anesthesia and in supine position, the patient's head was positioned in a slight extension and rotation to the right while fixed on Mayfield head fixation. Adopting pterional and transsylvian approaches, the sphenoid wing meningioma was resected completely with no significant intraoperative complication. Postoperation, the patient, alert and oriented, was transferred to the neurosurgical intensive care unit (ICU), with no new neurological deficit. Postoperative brain computed tomography (CT) scan showed a moderate volume of pneumocephalus bifrontally, little epidural hematoma, small contusions, and hematoma in the surgical field [ Figure 1b ].

The patient was stable neurologically and hemodynamically, with mean blood pressure between 90 and 110 mmHg. All lab tests, including prothrombin time (PT), partial thromboplastin time (PTT) and international normalized ratio (INR), were normal. Minimal blood was observed in vacuum drainage container. He was transferred to the ward on the second day of the surgery, with no more associated problems. On the third day after surgery, we noticed a large volume of bloody fluid (approximately 300 cc) in vacuum drainage container while the patient had no new complaint or neurologic deficit. Control CT scan showed cerebellar linear hemorrhage (zebra sign) and an intracerebellar hemorrhage of about 35 × 8 mm² with no mass effect on the fourth ventricle [ Figure 1c ].

The patient was taken back to the ICU for close observation, and coagulation profile (PT, PTT, and INR, bleeding and clotting times) showed no abnormality. The patient remained stable hemodynamically and intact neurologically. Follow-up CT scans showed no changes toward hydrocephalus, and the patient was treated conservatively. Brain magnetic resonance angiography (MRA) and magnetic resonance venography (MRV) showed no significant vascular findings. The hematoma resolved after approximately 10 days and the patient was discharged with satisfactory recovery [ Figure 1d ].

Case 2

A 21-year-old man presented with hearing problem. Physical exam was normal; whereas the brain imaging demonstrated a huge intraaxial mass in the parietooccipital lobe originating from lateral ventricle associated with calcification [Figure 2a and b ]. Preoperative lab tests, including coagulopathy tests, were normal.

Figure 2

Case two: (a and b) preoperative CTscan demonstrated posterior fossa and supratentorial tumor, (c and d) the first scan postoperation showed linear hemorrhage in posterior fossa in favor of RCH and blood in surgical field and (e) the second scan postoperation demonstrated the exacerbated hemorrhage

The patient underwent craniotomy in the semilateral position. The head was fixed on Mayfield head fixation, and after craniotomy, total resection of tumor was done without any intraoperative complication. Postoperation, the patient was extubated and transmitted to the recovery room and then to neuro ICU. In ICU, generalized tonic–clonic seizure occurred suddenly and progressed to status epileticus. Despite administration antiepileptic agents, as seizure continued, the patient was intubated again after taking anesthesia. The postoperative CT scan showed the hemorrhage in the surgical field and in the cerebellar sulci in favor of RCH [Figure 2c and d ]. The control CT scan showed a raised amount of hematoma in cerebellar sulci [ Figure 2e ]. All lab tests, including PT, PTT, and INR, were normal. The conservative treatment did not respond for the patient. He deteriorated during the following days and later he expired. The histopathologic finding of the tumor was compatible with ependymoma.

DISCUSSION

RCH is a rare complication after supratentorial surgeries with an incidence rate between 0.08% and 0.6% in literature and poorly-understood underlying mechanisms,[ 2 ] some of which are as follows. The cerebellar sag, as a result of cerebrospinal fluid (CSF) hypovolemia and transient occlusion of superior bridging veins, results in hemorrhagic infarction.[ 1 ] Postoperative suction drainage could result in transtentorial pressure gradient and cerebellar hemorrhage from venous bleeding.[ 4 ] Intraoperative and postoperative CSF loss might lead to parenchymal shifts or a critical increase of transmural venous pressure with subsequent vascular disruption and hemorrhage.[ 5 ] In addition, intracranial hypotension and coagulation disorders have been described as possible risk factors.[ 5 ] Despite the unclear pathomechanism of this phenomenon, most authors agree that RCH is a result of intra- and postoperative loss of CSF, and has a venous origin.

It has been reported that RCH is a benign entity with a good prognosis,[ 1 3 4 ] however, in some patients it can be a life-threatening complication and might even result in death.[ 2 5 ]

Amini et al. suggested that the most common symptom of RCH is loss of consciousness. Therefore, the patient might show motor deficit, gait ataxia, and prolong anesthesia, or be asymptomatic.[ 1 ]

In our cases, a moderate amount of CSF was lost during the operation while opening the cisterns. Blood pressure, platelets count, and coagulation profiles were normal.

In the first patient, a large amount of CSF drainage from vacuum drain might have played an important role in the occurrence of RCH, while in the second patient, RCH occurred just after the surgery and the vacuum drain cannot be the reason. The first patient was asymptomatic and had a good prognosis, however, the second one was symptomatic and demonstrated deterioration and had a poor prognosis.

CONCLUSION

RCH after supratentorial craniotomy is a very rare complication and can be a life threatening and result to death. It is considered to avoid rapid loss of CSF intraoperative, avoiding excessive vacuum drainage, conservative treatment and serial CT scan.

Financial support and sponsorship

Nil.

Conflicts of interest

The authors declare that they have no conflict of interest, financial or otherwise with any organization.

References

1. Amini A, Osborn AG, McCall TD, Couldwell WT. Remote Cerebellar hemorrhage. AJNR Am J Neuroradiol. 2006. 27: 387-90

2. Brisman MH, Bederson JB, Sen CN, Germano IM, Moore F, Post KD. Intracerebral hemorrhage occurringre mote from the craniotomy site. Neurosurgery. 1996. 39: 1114-21

3. Brockmann MA, Nowak G, Reusche E, Russlies M, Petersen D. Zebra sign: Cerebellar bleeding pattern characteristic of cerebrospinal fluid loss. Case report. J Neurosurg. 2005. 102: 1159-62

4. Honegger J, Zentner J, Spreer J, Carmona H, Schulze-Bonhage A. Cerebellar hemorrhage arising postoperatively as a complication of supratentorial surgery: A retrospective study. J Neurosurg. 2002. 96: 248-54

5. Koller M, Ortler M, Langmayr J, Twerdy K. Posterior-fossa haemorrhage after supratentorial surgery- report of three cases and review of the literature. Acta Neurochir. 1999. 141: 587-92

Abstract

Background:Currarino syndrome (CS) is a rare genetic condition that presents with the defining triad of anorectal malformations, sacral bone deformations, and presacral masses, which may include teratoma. Neurosurgeons are involved in the surgical treatment of anterior meningoceles, which are often associated with this condition. The accepted surgical treatment is a staged anterior-posterior resection of the presacral mass and obliteration of the anterior meningocele.

Case Description:This case involved a 36-year-old female who presented with late onset of symptoms attributed to CS (e.g., presacral mass, anterior sacral meningocele, and sacral agenesis). She successfully underwent multidisciplinary single-stage approach for treatment of the anterior sacral meningocele and resection of the presacral mass. This required obliteration of the meningocele and closure of the dural defect. One year later, her meningocele had fully resolved.

Conclusion:While late presentations with CS are rare, early detection and multidisciplinary treatment including single-state anterior may be successful for managing these patients.

Keywords: Currarino syndrome, meningocele, sacral, teratoma

INTRODUCTION

Currarino syndrome (CS) is an autosomal dominant syndrome. The classical presentation is characterized by sacral agenesis, anorectal malformations, and a presacral mass. CS is rare in adults, but should be recognized early to avoid life threatening complications, e.g. meningitis, rectal fistulas, and an approximately 1% risk for malignant transformation.

There are multiple surgical approaches utilized to treat anterior sacral meningoceles (ASM) and presacral masses. The most common is a two-staged anterior-posterior approach that carries an increased risk of anorectal perforation leading to infection along with greater morbidity attributed to increases blood loss, operating time, and length of hospital stay. Here, we offer a single-staged approach wherein the anterior sacral meningocele and presacral mass were both treated in one sitting.[ 1 3 ]

CASE REPORT

A 36-year-old female presented with intermittent constipation and bilateral lower extremity radiculopathy in the L4 distribution on the right and L5 distribution on the left. Family history was significant for the diagnosis of CS in both her children. The magnetic resonance imaging (MRI) and computed tomography (CT) scans revealed an incidental anterior sacral meningocele, a perirectal mass, with sacral agenesis, as well as right hemisacral hypoplasia, and an 8.0 cm × 8.0 cm × 6.0 cm meningocele emerging from a ventral sacral defect [Figures 1 – 3 ]. A 7.5 cm × 5.0 cm cystic heterogeneously enhancing mass was also noted immediately inferior to the meningocele, adjacent to the sigmoid colon. The remainder of the neuraxis appeared normal.

Figure 1

Sagittal MRI, preoperative. (A) Anterior sacral meningocele; (B) teratoma

Figure 2

Axial MRI, preoperative. (A) Anterior sacral meningocele

Figure 3

Axial MRI, preoperative. (B) Teratoma

Surgery

Surgical treatment of the meningocele required a complete S3-4 laminectomy with transdural closure and obliteration of the meningocele pedicle. The laminectomy, along with an extended incision to the anal sphincter, provided excellent exposure of the presacral mass. With the assistance of general surgery, the presacral mass was grossly excised without complications. Pathologically, this proved to be a mature teratoma. One year later, the myelomeningocele had fully resolved [ Figure 4 ].

Figure 4

Sagittal MRI, postoperative

DISCUSSION

CS is a caudal regression syndrome typically associated with the classic triad of sacral malformations (sickle shaped sacrum, sacral agenesis below S2 [ Figure 5 ], and rarely complex malformations), anorectal malformations (anal atresia, low and high anal imperforation/rectal fistulae), Hirschprungs disease, and presacral masses (anterior myelomeningocele, malignant or benign teratoma, dermoid cyst, epidermoid cyst, leomyomasarcoma, lipoma, or a combination thereof).[ 2 5 7 ]

Figure 5

Coronal XR, preoperative, showing sacral agenesis

Genetic discussion

This phenotype has been linked to loss of function mutations that disrupt the HLXB9 homeobox gene located on chromosome 7q36, and which encodes the HB9 nuclear protein. Although it is most commonly associated with nonsense mutations, a variety of mutations have been described including missense, splice site, and frameshift.[ 4 5 ] The disease process itself follows an autosomal dominant inheritance pattern with variable penetrance and weak phenotype-genotype correlation.[ 4 8 ]

Clinical diagnoses

More than 80% of the patients with the classic triad are diagnosed within the first decade of life.[ 2 ] This patient presented at the age of 36 with radiculopathy, and was older than those typically diagnosed with CS. A review of 205 patients by Lynch et al. showed that renal/urinary symptoms were among the three common defining features of CS.[ 8 ] Furthermore, one-third of patients were asymptomatic at the time of diagnosis (incidental finding). A very common presenting symptom was chronic constipation in childhood attributed to the ventral meningocele and/or pre sacral masses, dysganglionosis due to malformations of the enteric nervous system (i.e., Hirschprung’s), tethered cords, or errors in migration of the pluripotent cells of the caudal eminence after primary neurulation.[ 2 6 8 ]

Surgical approaches

Classic CS is routinely treated in a two-staged approach with the anorectal malformation and presacral mass dealt separately from the obliteration of the anterior meningocele.[ 2 ] Here, we opted for a single-stage operation completing simultaneous excision of the mass and ligation of the ASM, thereby avoiding infection and a need for a second operation. In addition, this single staged posterior approach reduced the need for a colostomy, which further reduced the risk of infection.

CONCLUSION

While late presentation of CS is rare, early detection and multidisciplinary treatment including surgical resection is critical for the successful management of these patients because these lesions may undergo malignant transformation. Depending on the lesions present, a single-stage approach minimizes surgery and reduces its inherent risks including those of postoperative infection.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1. Aranda-Narvaez JM, Gonzalez-Sanchez AJ, Montiel-Casado C, Sanchez-Perez B, Jimenez-Mazure C, Valle-Carbajo M. Posterior approach (Kraske procedure) for surgical treatment of presacral tumors. World J Gastrointest Surg. 2012. 4: 126-30

2. Baltogiannis N, Mavridis G, Soutis M, Keramidas D. Currarino triad associated with Hirschsprung's disease. J Pediatr Surg. 2003. 38: 1086-9

3. Fitzpatrick MO, Taylor WA. Anterior sacral meningocele associated with a rectal fistula. Case report and review of the literature. J Neurosurg. 1999. 91: 124-7

4. Hagan DM, Ross AJ, Strachan T, Lynch SA, Ruiz-Perez V, Wang YM. Mutation analysis and embryonic expression of the HLXB9 Currarino syndrome gene. Am J Human Genet. 2000. 66: 1504-15

5. Lynch SA, Wang Y, Strachan T, Burn J, Lindsay S. Autosomal dominant sacral agenesis: Currarino syndrome. J Med Genet. 2000. 37: 561-6

6. Martucciello G, Torre M, Belloni E, Lerone M, Pini Prato A, Cama A. Currarino syndrome: Proposal of a diagnostic and therapeutic protocol. J Pediatr Surg. 2004. 39: 1305-11

7. Shoji M, Nojima N, Yoshikawa A, Fukushima W, Kadoya N, Hirosawa H. Currarino syndrome in an adult presenting with a presacral abscess: A case report. J Med Case Rep. 2014. 8: 77-

8. Yates VD, Wilroy RS, Whitington GL, Simmons JC. Anterior sacral defects: An autosomal dominantly inherited condition. J Pediatr. 1983. 102: 239-42

Abstract

Background:Neuroendocrine tumors (NET) originate from the diffuse neuroendocrine system. These can arise in almost every organ of the body, although they are most commonly found in the gastrointestinal tract and respiratory system. The skull base and sellar region are extremely rare sites for neuroendocrine carcinoma. Consequently, in this case, both diagnosis and definition of surgical goals, as well as further treatment strategies were challenging.

Case Description:A 65-year-old woman was admitted to our Neurosurgery Department with a rapidly progressive visus reduction, drowsiness, polyuria, and polydipsia. Neuroimaging showed a sellar/suprasellar mass (diameter of 2 cm) with a heterogeneous signal compressing the optic chiasm and extending laterally toward the cavernous sinus. Differential diagnosis based on imaging included pituitary macroadenoma or metastasis. The patient underwent endoscopic endonasal transsphenoidal surgery. A total resection of the mass was impossible because of the infiltration of the optic chiasm and the intraoperative histological diagnosis of malignant epithelial neoplasm. Further histological evaluation revealed that the lesion was a NET with no other primary or metastatic sites detectable. Subsequently, the patient was successfully treated with fractioned stereotactic radiotherapy and polychemotherapy. Four years after the surgery, follow-up magnetic resonance imaging showed stability of the residual disease. Neurologic examination revealed a complete visual recovery.

Conclusions:Primary pituitary NET, though rare, should be included in the differential diagnosis of sellar lesions. A multimodality treatment approach is needed. Finally, the present case highlights, that in the case of a pituitary lesion infiltrating the optic chiasm, including NET, the endoscopic endonasal transsphenoidal subtotal resection followed by fractioned stereotactic radiotherapy and chemotherapy may represent an effective and safe choice of treatment.

Keywords: Endoscopic endonasal transsphenoidal approach, neuroendocrine tumor, pituitary, sellar region tumors

INTRODUCTION

This case report describes a neuroendocrine tumor (NET) arising from enterochromaffin cells that underwent neoplastic transformation.[ 2 ] These tumors can arise in almost every organ of the body although they are most commonly found in the gastrointestinal tract and respiratory system.[ 8 ] The skull base and sellar region are extremely rare sites for NET.[ 3 ] To date, only 6 cases of primary intracranial NET have been reported in the literature.[ 1 2 3 4 5 6 7 8 ] In this scenario, both diagnosis and definition of surgical goals, as well as further treatment strategies are challenging.

We present here a case of a pathologically diagnosed isolated pituitary NET, which underwent endoscopic endonasal transsphenoidal subtotal resection. Serial prospective and extensive metastatic workup revealed no other primary lesions during a 4-year follow-up period, indicating this to be the primary site of involvement. Finally, clinical and pathological aspects, imaging findings, surgical strategies, and the outcome of NET are discussed in the light of the pertinent literature.

CASE DESCRIPTION

A 65-year-old woman was admitted to our department following a 2-week history of rapidly progressive visual reduction, drowsiness, polyuria, and polydipsia. Her medical history included a temporal arachnoid cyst, symptomatic with seizures, and VIII cranial nerve schwannoma treated with gamma-knife radiosurgery 2 years before. Her clinical examination revealed no neurological deficit other than bitemporal hemianopsia; there was no diarrhea or flushing. Magnetic resonance imaging (MRI) showed a sellar/suprasellar mass (diameter of 2 cm) with a heterogeneous signal compressing the optic chiasm and extending laterally toward the left cavernous sinus. The lesion was predominantly isointense to the gray matter on T1-weighted images (WI) and hyperintense on T2-WI [ Figure 1a ]. The images showed diffuse homogeneous enhancement following contrast administration [Figure 1b and c ]. Interestingly, an MRI examination performed 2 years before, for follow-up after radiosurgery for vestibular schwannoma and frontal arachnoid cyst, demonstrated a normal pituitary gland [Figure 1d – f ]. Differential diagnosis based on imaging included pituitary macroadenoma or metastasis. Endocrinological evaluation showed the following levels – thyroid-stimulating hormone 1.31 mU/ml, free thyroxine 3.9 pg/ml, free triiodothyronine 2.4 pg/ml, and prolactin (PRL) 77 ng/ml. The patient underwent endoscopic endonasal transsphenoidal surgery with the aid of neuronavigation. On initial inspection, the tumor resembled a pituitary adenoma. However, on entering the lesion, it had a firm and solid consistency and was not removable with suction. Therefore, the main part was dissected with curettes and removed with punch forceps. Moreover, the upper part of the lesion presented clear infiltration of the optic chiasm without a safe plane of cleavage [ Figure 2a ]. Because the intraoperative histological diagnosis was consistent with malignant epithelial neoplasm, and the lesion was firmly adherent to the optic chiasm, total removal was not possible, and after decompression of the optic chiasm, a small residual mass of tumor was left in place.

Figure 1

(a) Preoperative magnetic resonance (MRI) coronal T2-weighted imaging demonstrating sellar/suprasellar mass (diameter of 2 cm) with a heterogeneous signal, mainly hyperintense, compressing the optic chiasm and extended laterally toward the left cavernous sinus. (b and c) Preoperative magnetic resonance coronal and sagittal contrast-enhanced T1-weighted imaging showing an intense and diffuse enhancement. (d-f) MRI imaging performed 2 years before showing normal pituitary/sellar signal (white arrow), the frontal arachnoid cyst and left vestibular schwannoma

Figure 2

(a) Intraoperative endoscopic picture depicting that the upper part of the lesion (black arrow) presented clear infiltration of the optic chiasm (asterisk) without a safe plane of cleavage. (b) Hematoxylin-eosin (H and E) staining demonstrates cells with scant cytoplasm and nuclei with speckled chromatin. Mitotic activity was present and vigorous (magnification ×20). (c and d) Cells are positive for (c) cytokeratin Cam 5.2. (magnification, ×20) and (d) synaptophysin (magnification, ×20)

Histological and immunohistochemical analysis of the pituitary biopsy showed a neuroendocrine neoplasm of at least intermediate grade. The pituitary mass was characterized by cells with scant cytoplasm and nuclei with speckled chromatin. Mitotic activity was present and vigorous (up to 9–10 mitoses per high power field). There was no evidence of necrosis [ Figure 2b ]. In the background, multiple fragments of anterior pituitary tissue were noted. Immunohistochemical stains were performed on paraffin-embedded tissue using antibodies to synaptophysin, cytokeratin Cam 5.2, chromogranin, thyroid transcription factor (TTF-1), CDX2, Ki-67, p53 protein, and pituitary hormones. Tumor cells stained with antibodies to cytokeratin Cam 5.2. [ Figure 2c ], synaptophysin [ Figure 2d ], and chromogranin. The results for staining with TTF-1 and CDX2 were negative, and p53 protein was overexpressed in over 5% of tumor cells. The Ki-67 labeling index was approximately 25%. These findings support the diagnosis of neuroendocrine neoplasm.

The postoperative course was uneventful, and neurological examination revealed a complete visual recovery. Before the patient was discharged, an extensive search for a primary carcinoid tumor or other metastases was undertaken. Images from CT of the thorax and abdomen were normal. Both 11C-5^- hydroxytryptophan PET and Somatostatin Receptor scintigraphy using Indium 111Pentetreotide [111In-DTPA-D-Phe-] (Octreoscan) showed no additional area of abnormal uptake; therefore, primary pituitary NET was confirmed.

Subsequent postoperative CT and MRI [ Figure 3a ] demonstrated the presence of an abnormal signal toward the cavernous sinus and suprasellar area, which was consistent with residual disease. Then, after consultation with the neuro-oncology group, the patient was successfully treated with fractioned stereotactic radiotherapy and polychemotherapy, (using a combination of cisplatinum, ifosfamide, and etoposide) with good tolerance.

Figure 3

(a-d) Postoperative and follow-up at 12, 24 and 48 months, coronal MRI pictures depicting the stability of residual disease

Fractioned stereotactic radiotherapy (SRT), employing image integration techniques and a frame that could be relocated to facilitate a fractioned dosing scheme, was carried out under a plan for reducing the treatment risk of the optic apparatus. Postoperative CT and MRI imaging were employed for greater precision in identifying the target. The relevant anatomy and tumor residual volume were then outlined. Because the tumor was close to critical normal tissue such as the optic chiasm, optic nerves, and cavernous sinus, the rotational angles were planned to reduce the doses to these critical regions. The treatment consisted of 8 daily fractions of 3.5 Gy to the 65% isodose line and a total dose of 43.1 Gy. Thus, the optic chiasm was exposed to a mean total dose of 208.6 cGy. No deterioration of visual acuity or pituitary function occurred after SRT.

Follow-up MRI studies at 12, 24, and 48 months were performed [Figure 3b – d ] showing the stability of the residual mass in the sellar region. Results from the neurological examination were negative.

DISCUSSION

We describe the treatment of a NET arising from enterochromaffin cells that had undergone neoplastic transformation.[ 2 ] Enterochromaffin cells are located in the crypts of Lieberkuhn of the intestinal lumen and in the bronchopulmonary tract. However, these epithelial cells or their progenitors are present in nearly all organs.[ 2 8 ] Therefore, NET can arise in almost every organ of the body although these tumors are most commonly found in the gastrointestinal tract and respiratory system.[ 1 2 3 5 8 ]

Metastatic carcinoid tumors in the central nervous system are an unusual but well-documented finding in several case reports.[ 4 6 8 ] In the series by Patchell and Posner, intracranial metastases were the second most common neurological complication arising from systemic disease.[ 6 ] In addition, a case of a carcinoid tumor metastasizing to the dura mater and simulating a meningioma has been previously reported, however, this patient had a known gastric primary tumor.[ 1 ] The skull base and sellar region are extremely rare sites for NET,[ 3 ] and to date, only 6 cases of primary intracranial NET are reported in the literature.[ 1 2 3 4 5 6 7 8 ]

Patients with NET commonly present with no specific clinical features, including focal neurological deficit and intracranial hypertension. Functional tumors secreting one or more hormones would result in the presence of some endocrinal symptoms, and nonfunctional tumors may affect pituitary function and lead to hypopituitarism.[ 4 ] In our reported case, lesions invaded the suprasellar region with optic chiasm compression and subsequent bitemporal hemianopsia.

Producing images of NET is usually challenging, requiring a combination of functional and anatomical techniques.[ 1 5 7 ] Scans by CT and MRI examination are nonspecific, and in the skull base and sellar region, as in our case, NET presented MRI findings similar to more common pathologies such as pituitary adenoma, meningioma, and metastasis.[ 7 ] Because most cases of NET express somatostatin receptor subtype 2 and 5, somatostatin receptor (SR) scintigraphy represents the primary imaging method for diagnosis, staging, and monitoring of this tumor.[ 4 5 ] Somatostatin receptor scintigraphy study using Indium 111 Pentetreotide (which has a high affinity for SR), can achieve a sensitivity that has been reported to be between 80% and 100%.[ 3 ] An MIBG (131I-metaiodobenzylguanidine) scan uses an analogue of a biogenic amine precursor, 131I-metaiodobenzylguanidine. This analogue is taken up by chromaffin cells and stored in the neurosecretory granules. The MIBG scan presented a sensitivity a little lower than that of the 111In-pentetreotide scintigraphy.[ 4 5 ] Positron emission tomography (PET) scanning using 18F-labeled fluorodeoxyglucose (18FDG) is widely used as a powerful imaging technique in clinical oncology. Unfortunately, monitoring increased FDG uptake in NET is limited because of the low cellular proliferative activity and high differentiation rate.[ 3 ] Therefore, several tracers directed toward the specific characteristics of carcinoid tumors were developed for PET imaging in these tumors, for example, 6-[18F] fluorodopamine (18F-dopa) and 11C-5-hydroxytryptophan.[ 4 ] A PET scan using 11C-5-hydroxytryptophan has been shown to be a sensitive method for the imaging of small NETs, and in most cases yields a significantly higher detection rate than somatostatin receptor scintigraphy.[ 5 6 7 8 ]

Histopathological diagnosis is based on morphological and immunohistochemical examination. The neoplasm comprises cells of different sizes with uniform nuclei and scant cytoplasm; in addition, nidulant, basophil granulocytes are found. Irregular mitosis is present based on the degree of differentiation. The diagnosis of carcinoid tumor was favored based on its immunohistochemical profile; CAM 5.2, a marker for low molecular weight cytokeratins, synaptophysin, and chromogranin are markers for cells of neuroendocrine origin, and supported the diagnosis of NET.[ 4 5 6 7 8 ] Glial fibrillary acidic protein (GFAP), carcinoembryonic antigen (CEA), S100, and calcitonin are negative in NET, contrary to other cerebral tumors. For NET in the sellar region, as in the present case, it is necessary to differentiate it from esthesioneuroblastoma.[ 4 ] Esthesioneuroblastoma comprises neuroblasts with uniform circular nuclei and longitudinal cerebromedullary tubes in the dendrites. However, NET originates from the epithelium, lacking neural features.[ 1 2 3 4 5 6 ]

To date, only 6 cases of primary intracranial NET have been reported in the literature [ Table 1 ]. Therefore, the diagnosis, prognosis, and definition of treatment strategies are challenging. With the exception of the case presented by Deshaies et al.,[ 1 ] describing a frontal convexity mass with a prominent dural-based tail, the other five cases together with our case are located at the skull base, which appears to be the most frequent site of primary intracranial NET.[ 3 4 5 7 ] Porter et al.[ 7 ] reported a 62-year-old male patient who showed signs of elevated intracranial pressure, secondary to a tumor in the right cerebellopontine angle. The patient was treated with surgical subtotal resection of an extra-axial tumor by a retromastoid, retrosigmoid approach leaving a small remnant of tumor on the tentorial edge. The operative impression was of a tentorial edge meningioma. No primary tumor was found and no adjuvant therapy was administered. The follow-up for 5 years did not show any relapse.

Table 1

Summary of clinical, radiological, and surgical findings of patients with neuroendocrine tumor reported in the literature; adjuvant therapy and outcome were also reported

Ibrahim et al.[ 4 ] described another NET located at the skull base in a 29-year-old woman who presented with multiple cranial nerve palsies. The tumor was located at the foramen jugulare extending into the cerebellomedullary angle cistern. A biopsy was performed that led to the diagnosis of NET. The patient was treated with monthly somatostatin injections only and did not receive any postoperative radiation therapy or complete resection; she was in stable condition 1 year after surgical removal. More recently, Hood et al.[ 3 ] illustrated the case of a 61-year-old woman with multimorbidity who presented with transient memory loss and a left cavernous sinus mass extending into the infratemporal fossa. A biopsy was performed, and the patient then underwent subtotal tumor resection, and finally received postoperative radiotherapy. Because the tumor was firm and not easily removable with suction, the final portion off the cavernous carotid artery was left in place. The pathology report indicated a low-grade NET.

Finally, Liu et al.[ 5 ] reported two cases of primary NET: the first located in the sellar/suprasellar area and the second in the anterior cranial fossa with extension into the paranasal sinus. The first patient underwent a gross removal of tumor during surgery with a single nostril transsphenoidal approach. Pathological diagnosis was high-grade small-cell NET, and the patient died of extensive metastases after approximately 3 months. For the second patient, surgery achieved a gross removal through a right frontal craniotomy. The pathological diagnosis was low-grade NET. Follow-up and other treatments were not reported.

Our case was the only one successfully treated with a multimodality approach. After a subtotal resection, the patient underwent to fractioned stereotactic radiotherapy (total irradiation dose 43.1 Gy, fractioned in 8 daily sessions), followed by polychemotherapy protocol (using a combination of cisplatinum, ifosfamide, and etoposide), which was well tolerated. Four years after the treatment, a follow-up MRI showed a complete stability of the residual disease and results from neurological examination were negative. Other particular features of our case were the location of NET and the use of an endoscopic technique. In fact, to the best of our knowledge, we present the second case of primary sellar NET. In contrast to the sellar NET presented by Liu et al.,[ 5 ] our case was preoperatively indistinguishable from non-functioning pituitary macroadenoma, both clinically and radiologically. However, because of the better visualization granted by the endoscopic technique (moving the lens and light source closer to the pathology), we were able to intraoperatively distinguish the lesion from normal macroadenoma. In appearance, it had a firm and solid consistency, and it was not removable with suction; in addition, the upper part of the lesion presented clear infiltration of the optic chiasm without a safe plane of cleavage. Therefore, the surgical strategy was immediately changed, and we proceeded with decompression of locoregional neurovascular structures, leaving a small residue of tumor infiltrating the optic chiasm.

CONCLUSIONS

Primary pituitary NET, though rare, should be included in the differential diagnosis of sellar lesions. A multimodality treatment approach is needed. Finally, the present case highlights that, in the case of a pituitary lesion infiltrating the optic chiasm, including NET, the endoscopic endonasal transsphenoidal subtotal resection followed by fractioned stereotactic radiotherapy and chemotherapy may represent an effective and safe choice of treatment.

Compliance with ethical standards.

Funding

No funding was received for this research.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

Informed consent

Informed consent was obtained from all individual participants included in the study.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1. Deshaies EM, Adamo MA, Qian J, DiRisio DA. A carcinoid tumor mimicking an isolated intracranial meningioma. Case report. J Neurosurg. 2004. 101: 858-60

2. Faggiano A, Mansueto G, Ferolla P, Milone F, del Basso de Caro ML, Lombardi G. Diagnosis and prognostic implication of the World Health Organization classification of neuroendocrine tumors. Endocrinol Invest. 2008. 31: 216-23

3. Hood B, Bray E, Bregy A, Norenberg M, Weed D, Morcos JJ. Primary carcinoid tumor of the cavernous sinus. World Neurosurg. 2014. 81: 202.e9-13

4. Ibrahim M, Yousef M, Bohnen N, Eisbruch A, Parmar H. Primary carcinoid tumor of the skull base: Case report and review of the literature. J Neuroimaging. 2010. 20: 390-2

5. Liu H, Wang H, Qi X, Yu C. Primary intracranial neuroendocrine tumor: Two case reports. World J Surg Oncol. 2016. 14: 138-

6. Patchell R, Posner J. Neurologic complications of carcinoid. Neurology. 1986. 36: 745-9

7. Porter DG, Chakrabarty A, McEvoy A, Bradford R. Intracranial carcinoid without evidence of extracranial disease. Neuropathol Appl Neurobiol. 2000. 26: 298-300

8. Sirsath NT, Babu KG, Das U, Premlatha CS. Paranasal sinus neuroendocrine carcinoma: A case report and review of the literature. Case Rep Oncol Med 2013. 2013. p.

Abstract

Background:Posterior reversible encephalopathy syndrome (PRES) is characterized by reversible edematous lesions on radiological examinations as well as symptoms of altered consciousness and seizures. To date, the underlying mechanism remains largely unknown.

Case Descriptions:Case 1 is a 72-year-old man with a history of hypertension presented with a subarachnoid hemorrhage. Fourteen days after the successful clipping of a ruptured aneurysm; he experienced inadvertent overdrainage via the intraventricular drain. Nine hours later, he started to have seizures followed by disturbances in consciousness. An emergency magnetic resonance imaging showed multiple high-intensity lesions in the frontal, temporal, parietal, and occipital lobes, basal ganglia, brainstem, and cerebellar hemispheres bilaterally, which are compatible with typical magnetic resonance findings in PRES patients. He was treated conservatively and recovered well. Case 2 is a 68-year-old woman with a mild history of hypertension and a ventriculo-peritoneal shunt for obstructive hydrocephalus, who underwent a cysto-peritoneal shunt placement because of an enlarging symptomatic arachnoid cyst. Immediately following surgery, she experienced disturbances in consciousness and developed status epilepticus. Radiological examinations revealed remarkable shrinkage of the arachnoid cyst and multiple edematous lesions, which led us to strongly suspect PRES. With conservative treatment, her symptoms and the radiological abnormalities disappeared.

Conclusion:Based on the previous literature and our cases, we believe that the association between rapid reduction of intracranial pressure (ICP) and the development of PRES should be recognized because most neurosurgical procedures such as craniotomy or cerebrospinal fluid diversion present a potential risk of rapid reduction of ICP.

Keywords: Cerebrospinal fluid, intracranial pressure, lumbar puncture, posterior reversible encephalopathy syndrome

INTRODUCTION

Posterior reversible encephalopathy syndrome (PRES) was proposed by Hinchey et al. in 1996 to describe characteristic radiological changes indicating brain edema, especially distributed in the posterior circulatory area, which is the most apparent on fluid-attenuated inversion recovery (FLAIR) images.[ 14 ] These changes are also accompanied by symptoms, including altered mental status, seizure, cortical blindness and other visual abnormalities, headache, nausea, and vomiting.[ 14 ] These clinical and radiological presentations usually resolve within several weeks.

As the condition gained recognition, additional characteristics have been reported.[ 9 ] For example, radiological changes are not necessarily limited to the posterior lobes. Also, some irreversible symptoms may occasionally lead to death. In addition, we postulate an association between PRES and hypertension, immunosuppressive drugs, chemotherapy, renal insufficiency, autoimmune disease, eclampsia, preeclampsia, and sepsis. There is currently no single theory that can account for the mechanism of this condition.

Here, we present two patients who developed PRES immediately after a rapid reduction in intracranial pressure (ICP). We also reviewed the previous literature and propose that a reduction in ICP may be associated with PRES.

CASE PRESENTATIONS

Case 1

A 72-year-old man on regular medication for hypertension was transported to our hospital complaining of sudden headache followed by disturbed consciousness. Upon arrival, he showed an altered mental status with decreased alertness. He could follow commands but could not speak. He had no other focal deficits, such as hemiparesis or cranial nerve palsy. Computed tomography (CT) showed a subarachnoid hemorrhage (SAH) [ Figure 1a ]. Emergency angiography revealed a small aneurysm at the bifurcation of the left internal carotid artery and the posterior communicating artery [ Figure 1b ]. The aneurysm was clipped successfully on the day of admission, and his neurological conditions showed remarkable improvement. Because the angiogram obtained on day 6 showed a sign of localized mild spasm in the A1 portion of the left anterior cerebral artery, hypervolemic and permissive hypertensive therapy with a continuous ventricular drainage to control the ICP was implemented to prevent ischemic complications. His neurological status remained stable with no findings of ischemia on serial CT examinations; however, he became restless over time and started becoming agitated, likely because of the long period of bed rest. His blood pressure (BP) hovered around 170–190 over 80–100 mmHg. On day 14, he repeatedly got up and lied down on the bed, which caused an inadvertent overdrainage of cerebrospinal fluid (CSF) via the intraventricular drain. The amount of CSF drainage was as high as approximately 200 ml in 2 h. His BP was 199/91 mmHg and no new neurological deficit was observed at this time. Emergency CT obtained 6 h after this episode revealed a ventricular narrowing compared to the previous CT images obtained before 2 days; in addition, a new, small, low-density area in the left occipital lobe was observed [ Figure 1c ]. Three hours after the CT examination, the patient had a tonic-clonic seizure followed by coma. Magnetic resonance (MR) images taken 9 h after the overdrainage showed diffuse hyper-intensity areas on FLAIR in the bilateral frontal, temporal, parietal, and occipital lobes, and basal ganglia, brainstem, and cerebellum [Figure 1d – f ] without an apparent spasm on MR angiography [ Figure 1g ]. Based on the radiological findings, we made a diagnosis of PRES and started him on anticonvulsant and antihypertensive therapies. His neurological status made a slow but steady recovery over the following 9 days. MR imaging performed on day 30 demonstrated a complete disappearance of edema delineated as FLAIR high lesions [ Figure 1h ]. After ventriculo-peritoneal (VP) shunt placement for hydrocephalus and 3 months of rehabilitation, the patient was discharged home with no neurological deficits.

Figure 1

(a) Computed tomography (CT) scan upon admission showing a hematoma in the basal cistern. (b) Anteroposterior view of the left internal carotid angiogram revealing an aneurysm at the bifurcation of the internal carotid artery and the posterior communicating artery. (c) CT on day 14 showing ventricular narrowing and low-density area on the left occipital lobe (arrowhead). (d-f) Magnetic resonance (MR) imaging showed fluid-attenuated inversion recovery (FLAIR) images depicting diffuse high-intensity lesions in the bilateral frontal, temporal, parietal, and occipital lobes, and basal ganglia, brainstem, and cerebellum. (g) MR angiography showing no significant vasospasm. (h) MR imaging obtained on day 30 showing a complete resolution of high-intensity lesions.

Case 2

A 68-year-old woman presented at our outpatient clinic complaining of left hemiparesis. She had a history of well-controlled hypertension and had a VP shunt placed 18 years ago for obstructive hydrocephalus due to midbrain cavernous malformation. CT images showed an enlargement of the right frontotemporal arachnoid cyst [ Figure 2a ], which we considered the cause of her left hemiparesis. A cysto-peritoneal shunt placement using a y-shaped connector was performed. Although there were no hemodynamic changes (her BP remained around 100/40 mmHg) or complications during the operation, the patient did not wake up after the anesthesia was completely terminated. Before the surgery, we had increased the valve pressure to 20 cmH₂O because we were concerned about a rapid reduction in ICP; however, postoperative CT demonstrated remarkable shrinkage of the cyst with a small amount of bleeding [ Figure 2b ]. Following surgery, the patient experienced tonic–clonic seizures and developed status epilepticus on the following day. The emergency CT images showed low-density areas in the bilateral occipital lobes [ Figure 2c ], and MR imaging on the fourth day following surgery revealed diffuse hyper-intensity areas on FLAIR imaging in the temporal, parietal, and occipital lobes on both sides, and the right frontal lobe [ Figure 2d ]. Because we strongly suspected PRES, we initiated anticonvulsant therapy. Although the patient had been comatose and unresponsive for >10 days, she gradually started to respond 12 days after surgery and showed neurological improvement. MR images taken 27 days after surgery demonstrated an almost complete disappearance of edematous areas [ Figure 2e ]. The patient required rehabilitation for another few months, but recovered to a normal status in 6 months.

Figure 2

(a) CT scan demonstrating a large arachnoid cyst on the right frontotemporal region. (b) Immediate postoperative CT revealing a remarkable shrinkage of the cyst. (c) CT obtained 1 day after surgery showing low-density areas in the bilateral occipital lobes. (d) MR FLAIR image on the fourth day after surgery demonstrating diffuse high-intensity areas in the temporal, parietal, and occipital lobes on both sides, and in the right frontal lobe. (e) MR FLAIR image 27 days after surgery showing an almost complete disappearance of lesions

DISCUSSION

Despite its name containing the term “posterior,” PRES is believed to occur in the posterior circulatory areas as well as in the temporal lobes or frontal lobes,[ 9 20 ] as also is seen in the cases described here. PRES is associated with a variety of clinical conditions, including hypertensive status, immunosuppression, chemotherapy, renal failure, autoimmune disease, eclampsia, preeclampsia, and sepsis.[ 9 ] Among these, previous studies have emphasized chronic hypertensive status as a predisposing factor for PRES because it is thought to injure the autoregulation of cerebral vessels and cause vasogenic edema. Although there was a history of hypertension in Case 2, the patient's condition was mild and well controlled; Case 1 had high BP because he was in a state of post-SAH vasospasm, which could have induced vascular autoregulation dysfunction. It is well known that PRES can occur during SAH treatment. In 12 reports of 15 cases of PRES in patients with SAH reviewed, permissive or intensive hypertension employed during the acute phase of SAH to prevent the symptomatic vasospasm was presumed to be a cause of PRES.[ 1 3 5 10 17 18 19 21 24 30 35 36 ]

Although ICP was not directly measured, a rapid reduction in ICP appeared to occur in both of our cases. In Case 1, radiological clinical signs of PRES presented approximately 6 h after the inadvertent massive CSF drainage, indicating that the rapid decrease in ICP could have been the main trigger of PRES. Similarly, the postoperative CT in Case 2 showed substantial shrinkage of the cyst, suggesting a possible abrupt event that decreased ICP during surgery. Ho et al. proposed that reduction in ICP decreases ventricular size, resulting in mechanical stress to vessels and causing vasoconstriction and vasogenic edema.[ 15 ] Some reports that measured ICP during the clinical manifestations of PRES found that the values were all within the normal range,[ 4 7 15 28 ] possibly because of the timing of ICP measurement; autoregulation system dysregulation may recover quickly enough to adjust ICP values within a few hours before clinical symptoms become evident.

Hammad et al. recently reported a case of PRES secondary to CSF leak and intracranial hypotension, and also reviewed 10 cases of PRES that developed after spinal or epidural tap.[ 13 ] They speculated that increased cerebral perfusion pressure due to an elevation of mean arterial pressure or a decrease in ICP resulted in hyperperfusion followed by vasogenic edema. On the basis of this proposal, we further expanded the literature search for reports on possible reductions of ICP because of invasive treatment or surgical procedure. A summary of 19 cases, including 10 cases in Hammad's series, is shown in Table 1 . Twelve cases were associated with a spinal tap or epidural anesthesia.[ 6 12 13 15 16 22 25 27 29 31 32 33 34 ] Although not all of the above-mentioned reports directly described a relationship between ICP and PRES, these cases indicate a strong association between PRES and a rapid reduction in ICP. In fact, cases with a high risk of severe CSF leak, such as in epidural catheter migration,[ 27 ] continuous lumbar drainage after spinal surgery,[ 13 ] and lumbar drainage for incisional effusion after spinal surgery,[ 32 ] were reported to antedate PRES, which is similar to situation described here in Case 1. In addition, the emergence of PRES after surgeries, such as VP shunt,[ 12 ] lumbo-peritoneal shunt,[ 8 ] and posterior fossa tumors with obstructed hydrocephalus,[ 2 11 23 26 33 ] have also been reported.

Table 1

Previous reports of posterior reversible encephalopathy syndrome with a possible relevance to reduction of intracranial pressure

In Case 2, the cysto-peritoneal shunt for the large arachnoid cyst might have caused an acute drop in ICP that resulted in PRES, a novel situation that has not been reported in the literature, to the best of our knowledge. Theoretically, several neurosurgical procedures are at high risk for unintentional hyperperfusions. Although rare, surgeons should be aware of the possibility of PRES in patients with severe headache, seizure, and disturbances in consciousness that cannot be explained by other medical conditions.

The prognosis of PRES is generally good and almost all patients make full recoveries or return to a normal life with minor deficits.[ 13 ] Therefore, we believe that a greater recognition of PRES and careful conservative management, such as avoidance of excessive CSF drainage and early antiepileptic treatment, are critical to minimize complications from this serious but treatable condition.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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24. Muhammad S, Güresir Ã, Greschus S, Scorzin J, Vatter H, Güresir E. Posterior Reversible Encephalopathy Syndrome as an Overlooked Complication of Induced Hypertension for Cerebral Vasospasm: Systematic Review and Illustrative Case. Stroke. 2016. 47: 519-22

25. Orehek EK, Burns JD, Koyfman F, Azocar RJ, Holsapple JW, Green DM. Postpartum trifecta: Simultaneous eclamptic intracerebral hemorrhage, PRES, and herniation due to intracranial hypotension. Neurocrit Care. 2012. 17: 434-8

26. Patel AJ, Fox BD, Fulkerson DH, Yallampalli S, Illner A, Whitehead WE. Posterior reversible encephalopathy syndrome during posterior fossa tumor resection in a child. J Neurosurg Pediatr. 2010. 6: 377-80

27. Pradhan A, Jairam A, Kumar RSV, Srivastava A, Sreevastava D, Dutta A. Posterior reversible encephalopathy syndrome posttransplantation: A case report of possible association with cerebrospinal fluid leak after epidural catheterization. Transplant Proc. 2009. 41: 1957-60

28. Prout RE, Tuckey JP, Giffen NJ. Reversible posterior leucoencephalopathy syndrome in a peripartum patient. Int J Obstet Anesth. 2007. 16: 74-6

29. Pugliese S, Finocchi V, Borgia ML, Nania C, Vella Della B, Pierallini A. Intracranial hypotension and PRES: Case report. J Headache Pain. 2010. 11: 437-40

30. Sanelli PC, Jacobs MA, Ougorets I, Mifsud MJ. Posterior reversible encephalopathy syndrome on computed tomography perfusion in a patient on “Triple H” therapy. Neurocrit Care. 2005. 3: 46-50

31. Shah R, Kubisz-Pudelko A, Reid J. Posterior reversible encephalopathy syndrome following an inadvertent dural puncture during an emergency laparotomy for ischemic colitis-a case report. Local Reg Anesth. 2014. 7: 1-4

32. Shields LBE, Johnson JR, Shields CB. Posterior reversible encephalopathy syndrome following a thoracic discectomy-induced dural leak: Case report. J Neurosurg Spine. 2016. 25: 586-90

33. Sorour M, Sayama C, Couldwell WT. Posterior Reversible Encephalopathy Syndrome after Surgical Resection of a Giant Vestibular Schwannoma: Case Report and Literature Review. J Neurol Surg A Cent Eur Neurosurg. 2016. 77: 274-9

34. Torrillo TM, Bronster DJ, Beilin Y. Delayed diagnosis of posterior reversible encephalopathy syndrome (PRES) in a parturient with preeclampsia after inadvertent dural puncture. Int J Obstet Anesth. 2007. 16: 171-4

35. Voetsch B, Tarlov N, Nguyen TN, DeFusco C, Barest GD, Norbash A. Asymmetric posterior reversible encephalopathy syndrome complicating hemodynamic augmentation for subarachnoid hemorrhage-associated cerebral vasospasm. Neurocrit Care. 2011. 15: 542-6

36. Wartenberg KE, Parra A. CT and CT-perfusion findings of reversible leukoencephalopathy during triple-H therapy for symptomatic subarachnoid hemorrhage-related vasospasm. J Neuroimaging. 2006. 16: 170-5

Abstract

Background:Intracerebral ring enhancing lesions can be the presentation of a variety of pathologies, including neoplasia, inflammation, and autoimmune demyelination. Use of a precise diagnostic algorithm is imperative in correctly treating these lesions and minimizing potential adverse treatment effects.

Case Description:A 55-year-old patient presented to the hospital with complaints of a post-concussive syndrome and a non-focal neurologic exam. Imaging revealed a lesion with an open ring enhancement pattern, minimal surrounding vasogenic edema, and minimal mass effect. Given the minimal mass effect, small size of the lesion, and nonfocal neurological exam, we elected to pursue a comprehensive noninvasive neurologic workup because our differential ranged from inflammatory/infectious to neoplasm. Over the next 8 weeks, the patient's condition worsened, and repeat imaging showed marked enlargement of the lesion with a now closed ring pattern of enhancement with satellite lesions and a magnetic resonance (MR) spectroscopy and perfusion signature suggestive of neoplasm. The patient was taken to surgery for biopsy and debulking of the lesion. Surgical neuropathology examination revealed glioblastoma multiforme.

Conclusion:The unique open ring enhancement pattern of this lesion on initial imaging is highly specific for a demyelinating process, however, high-grade glial neoplasms can also present with complex and irregular ring enhancement including an open ring sign. Therefore, other imaging modalities should be used, and close follow-up is warranted when the open ring sign is encountered.

Keywords: Glioblastoma, incomplete peripheral rim enhancement, open-ring sign, radiographic image enhancement, tumefactive demyelination, tumefactive demyelinating lesion

INTRODUCTION

Intracerebral ring enhancing lesions can be a presentation of neoplasia (including metastases, lymphoma, and gliomas), infections, or autoimmune demyelination mimicking neoplasia (tumefactive demyelination), thus posing a diagnostic and management dilemma.[ 1 ] While neoplastic lesions require surgical intervention for diagnosis and sometimes resection, tumefactive demyelination responds well to corticosteroids without the need for surgery. However, use of corticosteroids can mask lymphomas and should be avoided when a tissue diagnosis is required. Incomplete peripheral enhancement, also referred to as an “open-ring” enhancement is thought to be highly specific for tumefactive demyelinating lesions (TDLs), yet can also represent malignancy – we present such a case and underscore the need for close follow-up of patients presenting with open-ring lesions.

CASE HISTORY

A 55-year-old man presented to an outside institution for treatment of a concussion which he sustained after a fall from a ladder while working in his yard. Initial neurologic examination and magnetic resonance imaging (MRI) of the brain were normal. The patient continued to have concussive symptoms and headache 4 months later. Neurologic exam was still normal, however, repeat MRI revealed a right posterior temporal cystic lesion measuring 1.5 × 1.5 cm with incomplete peripheral enhancement characteristic of an open ring sign [ Figure 1a ], with mild surrounding vasogenic edema [ Figure 1b ], minimal mass effect, no midline shift, and no restriction on diffusion. Because the differential diagnosis included infectious/inflammatory process, cavernoma, and neoplasm, the decision was made to follow the lesion with a comprehensive, noninvasive neurologic workup. Two months later, the patient began having acute, intermittent confusional episodes, culminating in a witnessed generalized seizure. Repeat MRI showed a 5.3 × 4.2 cm lesion with significant mass effect and surrounding vasogenic edema [ Figure 2a ]. The mass had a more defined central cystic component, did not show restriction on diffusion and a closed ring pattern of peripheral enhancement, as well as satellite lesions along its posterior aspect [ Figure 2b ]. MR spectroscopy was performed and showed an increased choline-to-creatine ratio with a decreased NAA signature suggestive of a neoplasm [ Figure 3 ]. MR perfusion showed increased relative blood volume compared to contralateral tissue, strongly suggesting neoplasia [ Figure 4 ].

Figure 1

Axial cut MR showing right, posterior temporal cystic lesion measuring 1.5 × 1.5 cm with incomplete peripheral enhancement on T1 contrast-enhanced image (a) with mild surrounding vasogenic edema on T2 FLAIR (b)

Figure 2

Axial cut MR showing increased size of lesion, measuring 5.3 × 4.2 cm with significant mass effect and surrounding vasogenic edema on T2 FLAIR (a) and a now closed ring pattern of peripheral enhancement on T1 contrast-enchanced image (b). Central cystic component now well-defined and satellite lesions are visible along posterior aspect

Figure 3

Sagittal, coronal and axial cuts of lesion on T2-weighted MRI above, below MR spectroscopy shows an increased choline to creatine ratio with a decreased NAA signature

Figure 4

Axial cut MR perfusion scan shows increased relative blood volume compared to contralateral tissue

The patient was taken to surgery for a right-sided temporal craniotomy for resection of the mass. Intraoperatively, the lesion was noted to be hemorrhagic with areas of necrosis and thrombosed vessels suggestive of a malignant glial neoplasm. Surgical neuropathology examination showed the lesion to be an infiltrating glial neoplasm with a fibrillary background. Tumor cells had elongated, pleomorphic, vesicular nuclei with scant cytoplasm [Figure 5a and b ]. Psuedopallisading, necrosis, vascular proliferation, and abundant cellular mitoses and apoptosis were present, confirming the diagnosis of gliobastoma multiforme.

Figure 5

H and E sections from the neoplasm show pleomorphic process-forming astrocytic cells around an area of necrosis (pseudopalisading necrosis) (a). Higher power (b) shows the atypical, pleomorphic astrocytic cells characteristic of high-grade astrocytic tumors

The patient began treatment with standard radiation and temozolomide and was well at the last follow-up 3 months after his diagnosis.

DISCUSSION

Ring-enhancing lesions are usually subcortical or deep. When presenting with associated vasogenic edema and mass effect, these lesions usually favor primary central nervous system neoplasm or abscess. However, demyelinating lesions seen in multiple sclerosis or tumefactive demyelination during the “active phase” of inflammation can also enhance, though the enhancement pattern of these lesions can be faint, with minimal perilesional vasogenic edema, and the enhancing rim may be incomplete.[ 1 3 ] The “open-ring” sign has been presented in many case studies in which lesions demonstrating incomplete, peripheral rim enhancement have been proven to be TDLs upon histological examination and/or resolution after steroid administration.[ 4 6 8 9 13 14 ] Unfortunately, some of these cases were preemptively treated as high-grade gliomas, and the patients underwent resection surgery only to be diagnosed with atypical demyelinating disease on histopathological examination.[ 8 10 11 ] The risks and potential comorbidities resulting from such misdiagnosis and treatment merit serious consideration when managing lesions exhibiting an open-ring enhancement pattern. Masdeu et al. reviewed imaging from 32 reported pathology-proven TDL cases showing complete or incomplete ring-enhancement and compared them to the same number of cases of neoplasm and brain abscess.[ 7 ] They reported a specificity of 84.4–93.8% of the “open-ring” sign for TDL diagnosis, with a likelihood ratio of 5.2 for demyelination versus neoplasm for a lesion with open-ring enhancement.[ 7 ] Although 70% of the reported TDL cases presented exhibited incomplete peripheral enhancement, the authors highlight the increased incidence of neoplasia as compared to atypical demyelination to keep neoplasm as a possible diagnosis.

This case is a rare example of an open-ring enhancing lesion which evolved from initial presentation to a complete ring-enhancing lesion, which was later confirmed to be glioblastoma multiforme. When trying to identify MR and CT features that distinguish TDLs from glioma or lymphoma, Kim et al. found 4 out of the 13 tumor patients exhibiting focal rim enhancement to have incomplete rings on MR.[ 5 ] They were able to distinguish these patients from TDL with corresponding CT grading of the enhancing portions for each pathology, but underscored that incomplete ring enhancement combined with lack of mass effect alone significantly suggests atypical demyelination.[ 5 ] In this case, there was no head CT done concurrently with the MR demonstrating open-ring enhancement to correlate. Another study investigating MR findings of primary CNS lymphoma patients reported 2 out of 26 studied cases demonstrating open-ring enhancement.[ 15 ] They note thick and non-uniform quality of the rings when compared to the primarily thin and uniform open-ring sign in TDLs.[ 15 ] Nonetheless, the present case shows a thin, uniform pattern of incomplete rim enhancement [ Figure 1 ].

When presented with open-ring enhancing lesions, there is necessity to correlate with other features suggestive of TDL to rule out neoplasm. Such features include T2-weighted iso- and hyperintensity of enhanced regions, absence of mass effect, low relative perfusion, absence of cortical involvement, and CT hypoattenuation of MR enhanced regions.[ 5 ] MR imaging of TDLs may also show necrosis and cystic degeneration.[ 2 ] In this case, MR spectroscopy demonstrated the characteristic glioma spectrum consisting of elevated choline with suppressed levels of NAA, however, these can also be mimicked by TDLs.[ 12 ] The presence of other lesions typical of demyelination or oligoclonal bands in the cerebrospinal fluid are also suggestive of demyelination rather than neoplasia, but not absolute and were absent in our case. Patients with features suggestive of TDL may be managed acutely with a short course of high dose steroids and if a good clinical and radiographic response was observed a diagnosis of a demyelinating process would be supported.[ 6 ] However, because all of these features can be seen in neoplasia, close follow up is required.

CONCLUSION

Open-ring pattern of enhancement is reported to be highly specific for demyelinating lesions, rather than neoplasia. However, high grade glial neoplasms can also present with complex and irregular ring enhancement including an open ring sign. Therefore, other imaging modalities should be used and close follow-up is warranted when the open ring sign is encountered.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

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2. Fallah A, Banglawa S, Ebrahim S, Paulseth JE, Jha NK. Tumefactive demyelinating lesions: A diagnostic challenge. Can J Surg. 2010. 53: 69-70

3. Given CA, Stevens BS, Lee C. The MRI Appearance of Tumefactive Demyelinating Lesions. Am J Roentgenol. 2004. 182: 1959-

4. Javalkar V, Manix M, Wilson J, Nanda A. Open ring enhancement in atypical brain demyelination. J Clin Neurosci. 2012. 19: 910-2

5. Kim DS, Na DG, Kim KH, Kim JH, Kim E. Distinguishing tumefactive demyelinating lesions from glioma or central nervous system lymphoma: Added value of unenhanced CT compared with conventional contrast-enhanced MR imaging. Radiology. 2009. 251: 467-75

6. Kimura N, Kumamoto T, Hanaoka T, Hasama Y, Nakamura K, Okazaki T. Monofocal large inflammatory demyelinating lesion, mimicking brain glioma. Clin Neurol Neurosurg. 2009. 111: 296-9

7. Masdeu JC, Quinto C, Olivera C, Tenner M, Leslie D, Visintainer P. Open-ring imaging sign: Highly specific for atypical brain demyelination. Neurology. 2000. 54: 1427-33

8. McAdam LC, Blaser SI, Banwell BL. Pediatric tumefactive demyelination: Case series and review of the literature. Pediatr Neurol. 2002. 26: 18-25

9. Medeiros FC de, Albuquerque LAF de, Pittella JEH, Souza RB de, Gomes Neto AP, Christo PP. Open-Ring Enhancement in Pseudotumoral Multiple Sclerosis: Important Radiological Aspect. Case Rep Neurol Med 2014. 2014. p. 1-5

10. Report C, Akimoto J, Fukuhara H, Suda T, Nagai K, Hashimoto R. Disseminated cerebellar hemangioblastoma in two patients without von Hippel – Lindau disease. Surg Neurol Int. 2014. 5: 145-

11. Riva D, Chiapparini L, Pollo B, Balestrini MR, Massimino M, Milani N. A Case of Child Neurology A Case of Pediatric Tumefactive. J Child Neurol. 2008. 23: 944-7

12. Saindane AM, Cha S, Law M, Xue X, Knopp EA, Zagzag D. Proton MR spectroscopy of tumefactive demyelinating lesions. Am. J. Neuroradiol. 2002. 23: 1378-86

13. Siddiqui A, Sahni A, Khadilkar S. The open-ring sign. Neurol India. 2005. 53: 253-4

14. Sinha M, Garg R, Bhatt M, Chandra A. Tumefactive demyelinating lesion: Experience with two unusual patients. J Postgrad Med. 2010. 56: 146-

15. Zhang D, Hu LB, Henning TD, Ravarani EM, Zou LG, Feng XY. MRI Findings of Primary CNS Lymphoma in 26 Immunocompetent Patients. Korean J Radiol. 2010. 11: 269-

Abstract

Background:Trigeminal neuralgia (TN) is the most common type of facial neuralgia with incidence of 26.8/100,000 person year. In general, this scenario is characterized by a lancinating, unilateral, paroxysmal pain in the area of the fifth cranial nerve. Several treatment methods, including the injection of ethyl alcohol or butyl alcohol into the ganglion, the glycerol injection into the trigeminal cistern, peripheral nerve divisions, the radiofrequency thermocoagulation of the preganglionic fibers, and radiosurgery has been used for TN.

Case Description:A case of a 74-year-old woman patient who undergone a treatment of TN through a compression of Meckel cave and developed a transient abducent palsy is presented. Complication regarding to a palsy of abducent nerve is discussed as well as the analysis of presumable evolving physiopathology. A critical review of literature was performed.

Conclusions:Among the procedures, we mean that percutaneous microballoon compression (PMC) is the best choice for elderly frail patients, because it had a very low associated mortality-morbidity rate and does not damage permanent the Gasserian ganglion.

Keywords: Abducent palsy, balloon compression, diplopia, Gasserian ganglion, percutaneous procedures, trigeminal neuralgia

INTRODUCTION

Trigeminal neuralgia (TN) is the most common type of facial neuralgia with incidence of 26.8/100,000 person year according to Devor et al.[ 10 ] In general, this scenario is characterized by a lancinating, unilateral, paroxysmal pain in the area of the fifth cranial nerve.[ 7 10 11 ]

The incidence rate of TN according to the gender is 2.5 and 5.7 per 100,000 per year respectively.[ 7 10 11 ]

Several treatment methods, including the injection of ethyl alcohol or butyl alcohol into the ganglion, the glycerol injection into the trigeminal cistern, peripheral nerve divisions, the radiofrequency thermocoagulation of the preganglionic fibers, radiosurgery has been used for TN,[ 13 15 16 21 26 ] from which the patients may obtain relief from pain.

Percutaneous microballoon compression (PMC) was first reported by Mullan et al.[ 18 ] Mullan and Lichtor[ 19 ] whose earlier work compared a transformed open cordotomy with a percutaneous injury, developed the innovative percutaneous compressive technique by using a Fogarty embolectomy catheter to compress the ganglion and retrogasserian nerve.[ 19 ] This is a modification of Shelden's technique,[ 22 23 ] and after improvement has been used widely. The technique is more physiological without provoking a loss of facial sensory due to a minimal microinvasion. Usage of balloon compression of the pathways behind the trigeminal ganglion for the treatment of TN has become a popular method among functional neurosurgeons. In another hand, this method has neurological complications, including double vision caused by trochlear as well as abducent palsy. Although this complication being rare, diplopia may cause significant disability.[ 1 12 27 ] The authors discuss the physiopathology of abducent palsy.

CASE REPORT

A 74-year-old woman patient presented with classic TN, mainly V1, V2 at the right side. First, she was treated clinically with carbamazepine 1200 mg per day, without any result. Due to the worsening of pain she was submitted to an injection of dexamethasone and bupivacaine in the extracranial course of maxillary division of trigeminal nerve with partial result. It was administrated gabapentin 600 mg per day also without results. The pre operative angio MRI did not show any important vascular looping over the right fifth nerve, and no lesions were identified in MRI. After 6 months of clinical treatment she underwent a PMC using the traditional technique: a #4 Fogarty balloon catheter with cannula and obturator, radiopaque contrast, and C-arm fluoroscopy unit were employed as the technique described by Burchiel.[ 7 ]

The patient is placed in supine position, sedated with propofol to describe better the exact place of pain (awake patient). As described above, the foramen ovale was cannulated and the balloon catheter was inserted with radiological guide into the Meckel's cave [ Figure 1 ]. The catheter was inflated till 1 ml, in a desired pressure, and shape and dye was injected to confirm position. A small amount of Omnipaque ® was slowly injected to inflate the balloon and compress the trigeminal ganglion and insufflation of balloon for 2 minutes, guided by fluoroscopy and Omnipaque ® as contrast. She presented during the procedure bradycardia about 25 beats/minute and total recovery after deflation of balloon. After the procedure, she developed severe diplopia, with paresis of abducent at right side and minimal effect in the pain. The paresis of abducent disappeared; however, the diplopia took 5 weeks to became better and disappear. She had undergone a control MRI, CT scan without any changing in comparison with the preoperative images.

Figure 1

Radiological view shows that the foramen ovale was cannulated and the balloon catheter was inserted tenderly with radiological guide into the Meckel's cave. The catheter was inflated till 1 ml, in a desired pressure, and shape and dye was injected to confirm position

DISCUSSION

Initial pain relief through the compression of Gasserian ganglion with balloon is obtained in literature in 93% of the cases and recurrence in about 23% at 3 years as reported by Brown et al.[ 4 6 ] Skirving and Dan[ 25 ] reported a recurrence rate of 19.2% within 5 years and 31.9% over the entire follow-up period. Correa and Teixeira, 1998, presented 187 patients, in which series there were no deaths or serious complications after PMC, and the pain was relieved in all the patients.[ 9 ] Liu et al.[ 14 ] showed a recurrence of 5.2%.

Carbamazepine is beyond any doubt the first choice for TN and for the patients who had drug resistance or intolerance, the percutaneous procedures are necessary.[ 2 3 4 5 6 7 8 9 ] The percutaneous procedures are radiofrequency rhizotomy, glycerol rhizotomy, and balloon compression.[ 2 3 4 5 6 7 8 9 12 13 14 15 18 19 20 22 23 24 26 ]

Among the procedures, we mean that PMC is the best choice for elderly frail patients, because it had a very low associated mortality-morbidity rate and does not damage the Gasserian ganglion. Several surgical methods must be considered, if percutaneous procedures fail, it may include partial sensory trigeminal rhizotomy, peripheral neurectomy, and stereotactic radiosurgery.[ 13 15 16 17 21 23 26 27 ] Even with the awareness that all of them can damage the nerves and result in considerable severe complications.

PMC may cause hypoesthesia, dysesthesia, masseter muscle weakness, anesthesia dolorosa, corneal anesthesia and absent corneal reflex, aseptic meningitis, transient sixth nerve palsy, otalgia, trochlear nerve palsy, and increased olfactory.[ 7 9 14 20 22 23 24 27 28 ] PMC may injure the myelinated fibers causing mediate the “trigger” to the lancinating pain of TN. As the corneal reflex is mediated by unmyelinated fibers, selective monitored compression of the myelinated fibers can preserve the corneal reflex when first divisive pain is present.[ 18 19 20 ]

Monitoring the compressive pressure and reducing the duration of compression, it is possible to diminish the incidence of dysesthesia, hypoesthesia, severe numbness, and masseter motor paresia after PMC without interference in the rate of recurrence of trigeminal pain.[ 22 23 25 26 ]

Fogarty balloon catheter was navigated into the Meckel's cave tenderly, for 1 minute of inflation, but in literature we found groups that have been used to inflate for 3– 0 minutes with good results.[ 18 19 ] Mullan et al.[ 18 ] and Mullan and Lichtor[ 19 ] observed an increased incidence of dysesthesia and numbness, with longer compression time, and after the sixth procedure, they began using only 1 minute of compression.

The experiments at laboratory suggest that balloon compression relieves trigeminal pain by injuring the myelinated axons which are involved in the sensory trigger to the pain and they try to correlate with the lancinating pain in patients suffering from TN.[ 12 14 21 23 ]

Monitoring the heart rate and blood pressure during PMC due to trigeminal cardiac reflex show that the heart rate can fell abruptly to 60 or even less, and the mean arterial blood pressure can decrease transiently in one third.[ 4 7 ] Medication as atropine or nitroprusside sodium may be useful and a temporary pacemaker may be necessary in severe cases of cardiac diseases.

Sixth nerve palsy was described before in functional rhizotomy for TN by radiofrequency. Penetration of the foramen ovale by the proper trajectory and avoidance of excessive penetration (5 mm beyond the profile of the clivus) were described as factors to eliminate the ocular nerve complications. Tew et al.,[ 27 ] described that the abducens was the most frequent injured nerve, and it would be possible to avoid any damage to this nerve, if the electrode trajectory would be maintained between the 5 mm and 10 mm planes and the electrodes does not penetrate more than 10 mm deep into the clival line. They conclude that oculomotor and trochlear palsies rarely occur unless the needle penetrates the cavernous sinus.[ 27 ] They found a 2% (seven cases) of transient diplopia in 400 cases, all of whom related to neuralgia of ophthalmic division. Abducent palsy was observed in five cases and trochlear in two cases. The period of recovery was from few weeks to 4 months.[ 27 ] Five weeks was the period of recovery observed in our patient.

Brown et al.[ 2 ] studying 141 consecutive patients treated by PMC observed one case with temporary sixth nerve palsy that spontaneously resolved. They thought that this was because of balloon over inflation and cavernous sinus compression, and concluded that this occurred before pressure monitoring was routinely done.

Bergenheim and Linderoth[ 1 ] reviewed their joint consecutive series of 193 patients with TN treated with balloon compression. After a meticulous analysis of records and the intraoperative x-ray images, they identified six patients with double vision postoperatively. In reviewing these occurrences, they found that the balloon was inflated outside Meckel's cave in four patients, the balloon was initially inflated too deeply in one patient, and the anatomy of Meckel's cave was probably aberrant in one patient. In five of the six patients, the symptoms disappeared within 5 months.[ 1 ]

Kefalopoulou et al.,[ 12 ] studied 79 patients who underwent a PMC and found two cases with sixth nerve palsy, both presenting a transient palsy, and concluded after reviewing all radiological records that the anatomic position and the pear shape of the inflated balloon, concluding that all represent key factors in determining the procedure's benefit or risk of complications. In our case, the abducent nerve palsy was because the shape was not pear, and by the penetrate above the clival line.

By meticulous surgical technique with close attention to the anatomic position and the shape of the inflated balloon, most cases of postcompression diplopia should be avoided.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1. Bergenheim AT, Linderoth B. Diplopia after balloon compression of retrogasserian ganglion rootlets for trigeminal neuralgia: Technical case report. Neurosurgery. 2008. 62: E533-4

2. Brown JA, Chittum CJ, Sabol D, Gouda JJ. Percutaneous balloon compression of the trigeminal nerve for treatment of trigeminal neuralgia. Neurosurg Focus. 1996. 1: 1-4

3. Brown JA, Gouda JJ. Percutaneous balloon compression of the trigeminal nerve. Neurosurg Clin N Am. 1997. 8: 53-62

4. Brown JA, McDaniel MD, Weaver MT. Percutaneous trigeminal nerve compression for treatment of trigeminal neuralgia: Results in 50 patients. Neurosurgery. 1993. 32: 570-3

5. Brown JA, Pilitsis JG. Percutaneous balloon compression for the treatment of trigeminal neuralgia: Results in 56 patients based on balloon compression pressure monitoring. Neurosurg Focus. 2005. 18: E10-

6. Brown JA, Preul MC. Trigeminal depressor response during percutaneous microcompression of the trigeminal ganglion for trigeminal neuralgia. Neurosurgery. 1988. 23: 745-8

7. Burchiel KJ, Sindou M.editors. Neurosurgery for trigeminal neuralgia. Practical Handbook of Neurosurgery from Leading Neurosurgeons. New York: Springer; 2009. p. 349-64

8. Campos WK, Linhares MN. A prospective study of 39 patients with trigeminal neuralgia treated with percutaneous balloon compression. Arq Neuropsiquiatr. 2011. 69: 221-6

9. Correa CF, Teixeira MJ. Balloon compression of the Gasserian ganglion for the treatment of trigeminal neuralgia. Stereotact Funct Neurosurg. 1998. 71: 83-9

10. Devor M, Amir R, Rappaport ZH. Pathophysiology of trigeminal neuralgia: The ignition hypothesis. Clin J Pain. 2002. 18: 4-13

11. Edlich R, Winters KL, Britt LD, Long IWB. Trigeminal neuralgia. J Long Term Eff Med Implants. 2006. 16: 185-92

12. Kefalopoulou Z, Markaki E, Constantoyannis C. Avoiding abducens nerve palsy during the percutaneous balloon compression procedure. Stereotact Funct Neurosurg. 2009. 87: 101-4

13. Kondziolka D, Lunsford LD, Bissonette DJ. Long-term results after glycerol rhizotomy for multiple sclerosis-related trigeminal neuralgia. Can J Neurol Sci. 1994. 21: 137-40

14. Liu HB, Ma Y, Zou JJ, Li XG. Percutaneous microballoon compression for trigeminal neuralgia. Chin Med J. 2007. 120: 228-30

15. Lopez BC, Hamlyn PJ, Zakrzewska JM. Systematic review of ablative neurosurgical techniques for the treatment of trigeminal neuralgia. Neurosurgery. 2004. 54: 973-82

16. Lunsford LD, Bennett MH. Percutaneous retrogasserian glycerol rhizotomy for tic douloureux: Part 1. Technique and results in 112 patients. Neurosurgery. 1984. 14: 424-30

17. Maher CO, Pollock BE. Radiation induced vascular injury after stereotactic radiosurgery for trigeminal neuralgia: Case report. Surg Neurol. 2000. 54: 189-93

18. Mullan S, Duda EE, Patronas NJ. Some examples of balloon technology in neurosurgery. J Neurosurg. 1980. 52: 321-9

19. Mullan S, Lichtor T. Percutaneous microcompression of the trigeminal ganglion for trigeminal neuralgia. J Neurosurg. 1983. 59: 1007-12

20. Natarajan M. Percutaneous trigeminal ganglion balloon compression: Experience in 40 patients. Neurol India. 2000. 48: 330-2

21. Perkin GD. Trigeminal neuralgia. Curr Treat Options Neurol. 1999. 1: 458-65

22. Shelden CH, Crue BL, Coulter JA. Surgical treatment of trigeminal neuralgia and discussion of compression operation. Postgrad Med. 1960. 27: 595-601

23. Shelden CH, Pudenz RH, Freshwater DB, Crue BL. Compression rather than decompression for trigeminal neuralgia. J Neurosurg. 1955. 12: 123-6

24. Siqueira SR, Nobrega JC, Teixeira MJ, Siqueira JT. Olfactory threshold increase in trigeminal neuralgia after balloon compression. Clin Neurol Neurosurg. 2006. 108: 721-5

25. Skirving DJ, Dan NG. A 20-year review of percutaneous balloon compression of the trigeminal ganglion. J Neurosurg. 2001. 94: 913-7

26. Taha JM, Tew JM. Comparison of surgical treatments for trigeminal neuralgia: Reevaluation of radiofrequency rhizotomy. Neurosurgery. 1996. 38: 865-71

27. Tew JM, Keller JT, Williams DS, Rasmussen T, Marino R.editors. Functional surgery of the trigeminal nerve: Treatment of trigeminal neuralgia. Functional Neurosurgery. New York: Raven Press; 1979. p. 129-42

28. Urculo E, Alfaro R, Arrazola M, Astudillo E, Rejas G. Trochlear nerve palsy after repeated percutaneous balloon compression for recurrent trigeminal neuralgia: Case report and pathogenic considerations. Neurosurgery. 2004. 54: 505-8

Abstract

Background:Cranioplasty (CP) is a widespread surgical procedure aimed to restore skull integrity and physiological cerebral hemodynamics, to improve neurological functions and to protect the underlying brain after a life-saving decompressive craniectomy (DC). Nevertheless, CP is still burdened by surgical complications, among which early or late graft infections are the most common outcome-threatening ones.

Case Description:We report the case of 48-year-old man admitted to our neurosurgical unit because of a painful right frontal swelling and 1-week purulent discharge from a cutaneous fistula. He had been undergone frontal CP because of severe traumatic brain injury (TBI) when he was 9-year-old. Since then, his medical history has been being unremarkable without any surgical or infective complication of the graft for 39 years, until he was accidentally stung by a hornet in the frontal region. After the CT scan and laboratory findings had evidenced a probable infection of the graft, the patient was treated by vancomycin and cefepime before he underwent surgical revision of its former CP, with the removal of the graft and the debridement of the surgical field. Subsequent bacteriological tests revealed Staphylococcus aureus as causal agent of that infection.

Conclusion:This case illustrates an anecdotal example of very late CP infection, due to an unpredictable accident. Due to lack of consensus on risk factors and on conservative or surgical strategy in case of graft infection, we aimed to share our surgical experience.

Keywords: Cranioplasty, late infection management, risk factors, surgical complications

INTRODUCTION

Decompressive craniectomy (DC) has become a widespread procedure for treating life-threatening conditions that lead to a higher intracranial pressure (ICP).[ 3 ] DC, ether “classic” or “internal” (sinuses cranialization in megasinus subdural empyema patients) is performed in the management of neurological emergencies, such as hemispheric stroke, dural sinus thrombosis, subarachnoid hemorrhage, or peri-cephalic and brain infections.[ 15 28 29 32 ] Otherwise to be noted that craniectomy versus burr hole in subdural empynema is still matter of debate.[ 21 ]

Nevertheless, severe traumatic brain injury (TBI) still remains the most common indication for DC in many clinical series.[ 3 14 25 ] Even if DC is a common neurosurgical procedure that might gain the control of resistant intracranial hypertension, it is often related to many side effects, such as the “trephined syndrome” and mostly the need to repair the bone defect. Thus, once resolved the cerebral swelling and restored the ICP, a cranioplasty (CP) is required in surviving patient in order to restore the physiological cerebral hemodynamics,[ 14 15 ] to protect the brain and to reconstruct bone defect.[ 3 11 12 13 ] Despite the clear advantages on clinical and neurological conditions of the patient due to CP, this procedure still entail a high morbidity and overall risk of adverse events ranging from 10.9% to 36.5%.[ 14 15 ] Among those, surgical site infection is one of the most common complications described in literature.[ 25 ] Their rate ranges from 5.8% to 16%,[ 15 16 33 ] and in most cases it implicates additional surgical procedures and the revision of the former CP.[ 14 26 ] Although their high incidence, the majority of surgical site infections is seen between 3 months and 10 months after CP,[ 2 5 20 ] instead late infections are extremely rare.[ 8 22 ] We report an exceptional case of graft infection seen 39 years after primary CP in a male patient with an unremarkable clinical history and uneventful graft permanence.

CASE DESCRIPTION

A 48-year-old male was admitted to our neurosurgical unit because of a frontal swelling with purulent discharge from a cutaneous fistula. In his clinical history, he reported a previous frontal TBI when he was 9-year-old. For this reason, he had undergone a frontal craniectomy with subsequent unspecified CP. Since that, his clinical history has been unremarkable for 39 years, without any surgical or infective complications related to the cranial surgical procedure. A week before his admission he had been stung by a hornet while he was working in the countryside, with a progressive swelling of frontal soft tissues, temperature raising, and local pain with inflammatory state. During that week, a progressive purulent discharge has onset from the site of the sting, so he referred to the emergency department. At the admission, he was awake and aware [Glasgow Coma Scale 15 (GSC 15)], his temperature was 37.8°C and his neurological examination was negative. Initial laboratory studies revealed a pathological increased value of white blood cell (WBC) count (22 × 10⁹/L with 82% neutrophils, 14% lymphocytes, and 7% monocytes) and of serum C-reactive protein level (57 mg/dL with a normal range 0.08–1.5 mg/dL). A head CT scan revealed an irregular subcutaneous fluid collection, adherent to the graft's anterior face, a peripheral soft tissues edema, and a frontal hypodensity in brain parenchyma contiguous to the graft. The administration of contrast medium revealed a moderate peripheral enhancement of the collection. Because of the radiological evidence of the graft infection with subcutaneous abscess, the patient was then transferred to our neurosurgical unit. A swab test of the purulent discharge from the cutaneous fistula was performed, and, after a multidisciplinary consult, a polychemotherapy was started (1 g of vancomycin and 2 g of cefepime every 12 h). Thirty-six hours after his admission, the patient underwent surgical toilet and removal of the graft. This appeared as a porous acrylic graft, with several fibrotic bands firmly tied with the skin and the underlying synthetic dural substitute. All the samples were harvested for microbiological exam, while the surgical site was washed with iodine solution, peroxide, isotonic saline, and rifampicine. At the end, a spongy layer of dural substitute with two overlaying patch of fibrin sealant (Tachosil©) and some fibrin glue (Vivostat©) was then used to ensure dural seal and to contribute in protecting the brain parenchyma under the bone defect [Figure 1a – d ].[ 9 10 18 19 ]

Figure 1

(a) Fronto-orbital swelling with purulent discharging fistula. (b and c) Intraoperative debridement and exposition of infected bone graft. (d) Closure with the apposition of dural substitute, Tachosil® and Vivostat®

Nor titanium mesh or other allograft was applied on the former craniotomy in order to prevent the rejection, waiting for the resolution of the infection in analogy with other surgical procedures.[ 30 ]

Further bacteriological tests revealed Staphylococcus aureus as causal agent of that graft infection. Due to this, the patient was discharged 7 days after surgical procedure under an antibiotic therapy with linezolid and ceftazidime and he is still in follow-up for further CP after the infection resolution and inflammatory markers normalization.

DISCUSSION

CP is a common surgical procedure requisite to restore skull integrity in such cases where it has been compromised. Moreover, it has been demonstrated that CP can be effective in preventing seizures or cerebral atrophy after a DC so avoiding the “trephined syndrome,”[ 27 ] in improving neurological functions due to the re-establishment of normal cerebral hemodynamics, and in restoring the skull shape.[ 14 15 ] Although, necessary for the above-mentioned reasons, CP is a surgical procedure as common as dangerous, carrying a significant rate of complications.[ 3 4 6 7 17 ] It has been estimated that this rate ranges from 15% to 36.5% and further surgical procedures may be necessary up to 76% of the cases.[ 14 ]

CP failure may be attributable to autologous bone flap resorption (when used) or mostly to graft infections,[ 3 11 ] which negatively conditions postoperative prognosis, requiring antibiotic therapy, graft removal, and further surgical procedures. Graft infection rate ranges from 5.8% to 16%,[ 15 16 33 ] and in most cases, it implicates additional surgical procedures with the revision of the former CP.[ 14 26 ]

Even if CP is a relatively old procedure, there still are no solid evidences on the risk factors for graft infections. The first and most controversial issue is the time interval between DC and CP. Many clinical series state that early CP ensures better outcomes, whereas many others advocate late surgery to prevent graft infections, avoiding the risk of performing surgical procedure on a contaminated wound.[ 1 3 14 15 23 27 ] Other risk factors described in literature take account of the use of allograft instead of autologous bone flap, the size of skull defect,[ 14 15 25 31 33 ] and also the etiology of primary DC; Kim et al. reported a higher rate of graft infections in those case of CP performed after DC in severe TBI than in non-TBI ones.[ 14 ] Other possible predictors of CP failure include: Motor deficit, Glasgow Outcome Score (GOS) 14 24 ] previous surgical site infection, or wound dehiscence.[ 25 ] Identifying those risk factors involved in CP procedure in order to reduce the rate of infection remain a challenge for the surgeon; but unfortunately, despite the good clinical and surgical practice not every risk could be avoided and some factors may be even unpredictable, especially forward in time. To the best of our knowledge, the present case of 48-year-old male patient represents the most tardive CP infection reported in literature. Formerly, Gürbüz et al. reported a case of purulent discharge from the surgical site of a patient 22 years after he had undergone DC and subsequent CP because of temporal TBI.[ 8 ] CP infections usually onset within 10 months after the surgical procedure, but the unpredictable happens: The long-term outcome of an accurate surgical procedure is sometimes irremediably influenced by the fate. In particular, in our reported case the correct engraftment and the 39-years long uneventful permanence of the synthetic CP had been foiled by an accidental hornet sting, which led to the graft infection. This complication might be related to the introduction of a foreign body, such as the hornet stinger and delivering bacteria into its surface.[ 27 ] The most of CP infections has been attributed to the colonization by the bacterial skin flora, in particular, Propionebacteria, Klebsiella, Enterobacter, Pseudomonas Aeruginosa also if Staphylococcus aureus results as the most common causal agent with a prevalence of 75%.[ 3 11 15 25 34 ] The execution of a swab test of the purulent discharge and the harvest of some intraoperative specimens are strictly recommended for further bacteriological studies, even if in a small percentage of the whole cases they can be inconclusive, showing no bacterial overgrowth.[ 26 ] In our case, the bacterial culture was positive for Staphylococcus aureus, both on the swab test and on the intraoperative specimens, in accordance with the literature data.[ 15 ] Due to variety of bacterial agents in such this kind of infection, our patient begun a medical therapy with vancomycin and cefepime before he underwent surgical operation and microbiological exam of intraoperative specimens. There is still not a consensus on the timing and the surgical strategy in treating infected CP.[ 26 ] In this challenging scenario, as it is still not possible to find either definite risk factors of earlier CP infections or reliable predictors of the later ones, which often are even unpredictable, a graft infection has to be considered as a diagnostic option, in case of a congruity between patient's clinical presentation, laboratory findings, and neuroimaging, also after several decades.

CONCLUSION

CP is still necessary in surviving patients after life-saving DC. Despite its widespread employment and the wide variety of techniques and material, postoperative complications still threat patients’ clinical outcome. Among these, graft infections are the most common, even many years after the CP have been performed, and they are often due to skin bacterial flora. Nowadays, there is still no consensus on reliable risk factors for early graft infections, moreover late ones are often unpredictable and totally accidental, as we reported. Due to lack of similar cases of very late CP infections in literature, we aimed to share our surgical experience about the most tardive one that we treated, highlighting its extraordinary fortuity.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

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3. Brommeland T, Rydning PN, Pripp AH, Helseth E. Cranioplasty complications and risk factors associated with bone flap resorption. Scand J Trauma Resusc Emerg Med. 2015. 23: 75-

4. Broughton E, pobereskin L, whitfield PC. Seven years of cranioplasty in a regional neurosurgical centre. Br J Neurosurg. 2014. 28: 34-9

5. Cheng YK, Weng HH, Yang JT, Lee MH, Wang TC, Chang CN. Factors affecting graft infection after cranioplasty. J Clin Neurosci. 2008. 15: 1115-9

6. Coulter IC, Pesic-Smith JD, Cato-Addison WB, Khan SA, Thompson D, Jenkins AJ. A multi-centre analysis of the outcomes of cranioplasty in the Northeast of England. Acta Neurochir (Wien). 2014. 156: 1361-8

7. Gooch MR, Gin GE, Kenning TJ, German JW. Complication of cranioplasty following decompressive craniectomy: Analysis of 62 cases. Neurosurg Focus. 2009. 26: E9-

8. Gürbüz MS, Celik O, Berkman MZ. Infection of cranioplasty seen twenty years later. J Korean Neurosurg Soc. 2012. 52: 498-500

9. Graziano F, Certo F, Basile L, Maugeri R, Grasso G, Meccio F. Autologous fibrin sealant (Vivostat(®)) in the neurosurgical practice: Part I: Intracranial surgical procedure. Surg Neurol Int. 2015. 6: 77-

10. Graziano F, Maugeri R, Basile L, Meccio F, Iacopino DG. Aulogous fibrin sealant (Vivostat(®)) in the neurosurgical practice: Part II: Vertebro-spinal procedures. Surg Neurol Int. 2016. 7: S77-82

11. Honeybul S, Ho KM. Cranioplasty: Morbidity and failure. Br J Neurosurg. 2016. 30: 523-8

12. Honeybul S, Janzen C, Kruger K, Ho KM. The impact of cranioplasty on neurological function. Br J Neurosurg. 2013. 27: 636-41

13. Jelcic N, De Pellegrin S, Cecchin D, Della Puppa A, Cagnin A. Cognitive improvement after cranioplasty: A possible volume transmission-related effect. Acta Neurochir (Wien). 2013. 155: 1597-9

14. Kim JS, Park IS, Kim SK, Park H, Kang DH, Lee CH. Analysis of the Risk Factors Affecting the Surgical Site Infection after Cranioplasty Following Decompressive Craniectomy. Korean J Neurotrauma. 2015. 11: 100-15

15. Kimchi G, Stlylianou P, Wohl A, Hadani M, Cohen ZR, Zauberman J. Predicting and reducing cranioplasty infections by clinical, radiographic and operative parameters-A historical cohort study. J Clin Neurosci. 2016. 34: 182-6

16. Klinger DR, Madden C, Beshay J, White J, Gambrell K, Rickert K. Autologous and acrylic cranioplasty: A review of 10 years and 258 cases. World Neurosurg. 2014. 82: 525-30

17. Martin KD, Franz B, Kirsch M, Polanski W, von der Hagen M, Schackert G. Autologous bone flap cranioplasty following decompressive craniectomy is combined with a high complication rate in pediatric traumatic brain injury patients. Acta Neurochir (Wien). 2014. 156: 813-24

18. Maugeri R, Basile L, Giugno A, Graziano F, Iacopino DG. Impasse in the management of recurrent basal cell carcinoma of the skull with sagittal sinus erosion, Interdiscip. Neurosurg. 2015. 2: 160-3

19. Maugeri R, Giammalva GR, Graziano F, Iacopino DG. May autologue fibrin glue alone enhance ossification? An unexpected spinal fusion. World Neurosurg. 2016. 95: 611-2

20. Matsuno A, Tanaka H, Iwamuro H, Takanashi S, Miyawaki S, Nakashima M. Analyses of the factors influencing bone graft infection after delayed cranioplasty. Acta Neurochir (Wien). 2006. 148: 535-40

21. Mattogno PP, LA Rocca G, Signorelli F, Visocchi M. Intracranial subdural empyema: Diagnosis and treatment update. J Neurosurg Sci. 2017. p.

22. Morioka T, Fujiwara S, Akimoto T, Nishio S, Fukui M. Intracranial epidural abscess: Late complication of allograft cranioplasty. Fukuoka Igaku Zasshi. 1996. 87: 57-9

23. Piedra MP, Nemecek AN, Ragel BT. Timing of cranioplasty after decompressive craniectomy for trauma. Surg Neurol Int. 2014. 5: 25-

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Abstract

Background:Intracranial prepontine cysts are rare and include epidermoid cysts, arachnoid cysts, and neurenteric cysts. Symptomatic prepontine cysts may require surgical intervention. Reports of spontaneous resolution of cysts are rare.

Case Description:We describe the case of a young gentleman who presented with headache and fever. Magnetic resonance imaging of the brain identified a prepontine lesion with features consistent with epidermoid cyst. During admission, the patient received symptomatic management in addition to empirical antibiotic therapy and dexamethasone. The patient improved symptomatically in the next 48 hours and was discharged. Follow-up imaging at 6 months and 1 year showed significant reduction in size of the lesion.

Conclusion:For asymptomatic prepontine cysts, a close radiological and clinical follow-up may prove useful.

Keywords: Adult brain cyst, MRI brain, prepotine cyst, self-resolution

INTRODUCTION

Prepontine cystic lesions are rare. Common types of cysts reported in this area include epidermoid cysts, arachnoid cysts, and neurenteric cysts.[ 4 6 12 14 ] Symptomatic lesions often require surgical intervention. According to our literature search, spontaneous resolution of a cyst in prepontine area has been reported once before.[ 8 ]

In this report, we describe the case of a young gentleman who presented with headache and fever. A large prepontine cyst was found on magnetic resonance imaging (MRI) with features consistent with epidermoid cyst. The possibility of neurenteric could not be excluded. Patient was treated symptomatically. Follow-up MRI at 1 year showed significant reduction in the size of the cyst.

CASE REPORT

A 41-year-old, right-handed gentleman presented to us in the emergency room with history of severe occipital headache for 4 days. It was constant and moderate to severe in intensity. There was no association with daytime or cough. Headache was associated with several episodes of vomiting. There were no mental status changes, seizure, or complain of motor weakness in any of his limbs.

On examination, he was well oriented to time, place, and person. Signs of meningeal irritation were absent. We also did not find any cranial nerve deficit, papilledema, or long tract signs. Systemic examination was also unremarkable. He had taken symptomatic treatment without much relief in his symptoms. Family history was unremarkable for any intracranial pathologies.

Considering the nature and severity of his symptoms, we obtained an MRI brain with and without contrast. MRI brain showed a midline prepontine cyst with signals identical to cerebrospinal fluid (CSF) on T1 and T2-weighted images [Figures 1a , 2a and 3a ]. Pre-pontine cistern was effaced with significant compression on basilar artery. The cyst showed diffusion restriction. There was no hydrocephalus.

Figure 1

(a) A well-defined extra-axial lesion is identified anterior to the brainstem on this sagittal view, appearing hyper intense relative to brain parenchyma on the T2 weighted image (White arrows). (b) Similar section noted one year later with reduction in mass of the cystic lesion identified in the image a

Figure 2

(a) T1 weighted Brain MRI, this axial section identifies the lesion anterior to the brain stem appearing hyper intense relative to brain parenchyma (White arrows). (b) Similar section noted one year later with reduction in mass of the cystic lesion identified in the image a

Figure 3

(a) T1 weighted image with contrast Brain MRI, this axial section identifies the lesion anterior to the brain stem appearing hyper intense relative to brain parenchyma with no enhancement noticed on contrast (White arrows). (b) Similar section noted one year later with reduction in mass of the cystic lesion identified in the image a

We admitted the patient for further management. He received symptomatic management along with empirical antibiotics. Considering the possibility of chemical meningeal irritation, we also started dexamethasone for 1 week. The patient improved within the next 48 hours and was discharged with a plan for close clinical and radiological follow-up.

After discharge, the patient remained symptom free, and returned with a repeat MRI brain after 1 year. Repeat imaging showed a significant size reduction and decrease in mass effect [Figure 1b , 2b and 3b ].

DISCUSSION

We have described the case of a patient with self-resolving prepontine cyst. Considering the location and radiological features, our top differentials were epidermoid and neurenteric cyst. Epidermoid cysts comprise 0.2–1.8% of primary intracranial tumors.[ 2 ] They arise from ectodermal inclusions formed during the neural tube closure in the third to fifth weeks of gestation at the same time as the optic and octic vessels develop. This explains the frequent occurrence of epidermoid cyst in the cerebellopontine (angle 40–50%)[ 7 9 10 15 ] and the parasellar region (10–15%).[ 5 ] Prepontine area is a rare location for an epidermoid cyst.

Epidermoid cyst on a computed tomography (CT) scan is a round/lobulated mass with a density resembling CSF, calcification is seen in 10% of the cases.[ 11 ] On MRI, hypointensity on T1 and hyperintensity on T2 is noted. Internal heterogeneity on FLAIR images this helps distinguish epidermoid cysts from arachnoid cysts.[ 3 ]

Neurenteric cysts are mostly found in the posterior fossa and are typically midline, anterior to the brainstem.[ 1 13 ] These may arise at the time of notochordal development during the transitory existence of the neurenteric canal. The notochord and foregut fail to separate, causing primitive endodermal cells to be incorporated into the notochord leaving behind a cyst. These cause headache, cranial neuropathies, recurrent aseptic meningitis, and motor and sensory deficits. Best diagnostic tool for a neurenteric cyst is a nonenhancing round or lobulated mass in front of the medulla which appears isointense to hyperintensive on T1, hyperintensive on T2 and also hyperintense on FLAIR images.

CONCLUSION

Although spontaneous resolution of prepontine cysts is rare, in patients who are neurologically intact, symptomatic management with close clinical and radiological follow-up may prove useful.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1. Basheer N, Kasliwal MK, Suri A, Sharma MC, Arora A, Sharma BS. Lateral extradural, supratentorial neurenteric cyst. J Clin Neurosci. 2010. 17: 639-41

2. Bejjani GK, Wright DC, Schessel D, Sekhar LN. Endodermal cysts of the posterior fossa: Report of three cases and review of the literature. J Neurosurg. 1998. 89: 326-35

3. Chen CY, Wong JS, Hsieh SC, Chu JS, Chan WP. Intracranial epidermoid cyst with hemorrhage: MR imaging findings. Am J Neuroradiol. 2006. 27: 427-9

4. Chen CY, Wong JS, Hsieh SC, Chu JS, Chan WP. Intracranial epidermoid cyst with hemorrhage: MR imaging findings. AJNR Am J Neuroradiol. 2006. 27: 427-9

5. Chowdhury FH, Haque MR, Sarker MH. Intracranial epidermoid tumor; microneurosurgical management: An experience of 23 cases. Asian J Neurosurg. 2013. 8: 21-

6. Christov C, Chretien F, Brugieres P, Djindjian M. Giant supratentorial enterogenous cyst: Report of a case, literature review, and discussion of pathogenesis. Neurosurgery. 2004. 54: 759-63

7. deSouza CE, deSouza R, da Costa S, Sperling N, Yoon TH, Abdelhamid MM. Cerebellopontine angle epidermoid cysts: A report on 30 cases. J Neurol Neurosurg Psychiatry. 1989. 52: 986-90

8. Dodd RL, Barnes PD, Huhn SL. Spontaneous resolution of a prepontine arachnoid cyst. Case report and review of the literature. Pediatr Neurosurg. 2002. 37: 152-7

9. Kobata H, Kondo A, Iwasaki K. Cerebellopontine angle epidermoids presenting with cranial nerve hyperactive dysfunction. Skull Base. 2002. 12: 178-

10. Miyazaki S, Fukushima T, Takusagawa Y. Epidermoid presenting as trigeminal neuralgia. Neurol Med Chir. 1984. 24: 774-81

11. Osborn AG, Preece MT. Intracranial cysts: Radiologic-pathologic correlation and imaging approach. Radiology. 2006. 239: 650-64

12. Osborn AG, Preece MT. Intracranial cysts: Radiologic-pathologic correlation and imaging approach 1. Radiology. 2006. 239: 650-664

13. Preece M, Osborn A, Chin S, Smirniotopoulos J. Intracranial neurenteric cysts: Imaging and pathology spectrum. Am J Neuroradiol. 2006. 27: 1211-6

14. Sampath S, Yasha TC, Shetty S, Chandramouli BA. Parasellar neurenteric cyst: Unusual site and histology: Case report. Neurosurgery. 1999. 44: 1335-

15. Zhou LF. Intracranial epidermoid tumours: Thirty-seven years of diagnosis and treatment. Br J Neurosurg. 1990. 4: 211-6

Abstract

Background:Unilateral diaphragmatic paralysis (UDP) can be a very disabling, typically causing shortness of breath and reduced exercise tolerance. We present a case of a surgical decompression of the phrenic nerve of a patient who presented with UDP, which occurred following cervical spine surgery.

Methods:The workup for the etiology of UDP demonstrated paradoxical movement on “sniff test” and notably impaired pulmonary function tests. Seven months following the onset of the UDP, he underwent a surgical decompression of the phrenic nerve at the level of the anterior scalene.

Results:He noted rapid symptomatic improvement following surgery and reversal of the above noted objective findings was documented. At his 4-year follow-up, he had complete resolution of his clinical symptoms. Repeated physiologic testing of his respiratory function had shown a complete reversal of his UDP.

Conclusions:Anatomical compression of the phrenic nerve by redundant neck vasculature should be considered in the differential diagnosis of UDP. Here we demonstrated the techniques in workup and surgical management, with both subjective and objective evidence of success.

Keywords: diaphragmatic paralysis, neurolysis, peripheral nerve surgery, phrenic nerve

INTRODUCTION

Unilateral diaphragmatic paralysis (UDP), secondary to phrenic nerve palsy, can be caused by a multitude of etiologies, but the actual cause is often elusive. It has been recently proposed that vascular compression from traversing vessels at the level of the thoracic outlet may contribute as in other compressive neurapraxias; in such a case surgical decompression may result in functional recovery.

Here we describe the preoperative assessment and the operative technique used to address a unilateral phrenic nerve palsy. Tightly adherent traversing vessels were identified at the time of exploration, specifically the transverse cervical and suprascapular arteries. Following ligation and sacrifice of these traversing vessels with neurolysis of the phrenic nerve over this segment, the patient's clinical symptoms resolved and the previously paralyzed diaphragm recovered.

PATIENT HISTORY

The patient was a 60-year-old male with a remote history of a motor vehicle accident who originally presented with symptoms of bilateral arm weakness and hyperesthesia, symptoms consistent with central cord syndrome. He underwent an anterior cervical discectomy and fusion at C5-C6 and C6-C7 levels through a left-sided approach. His weakness improved after surgery and he recalled no shortness of breath related to the accident or surgery at that time. Seven years later, he developed progressive myelopathic symptoms and was subsequently taken for a C4 to T2 laminectomy and posterior fusion, which was successful. Shortly following this operation, he began to experience shortness of breath. These symptoms persisted and gradually worsened over the course of 6 months to the point where he no longer had the respiratory stamina to do activities that he used to enjoy previously. At that time, the patient presented to our center and underwent workup for these symptoms. A fluoroscopic sniff test demonstrated paradoxical elevation of the right hemidiaphragm on rapid, forced inspiration with normal excursion of the left hemidiaphragm [ Figure 1a ]. On deep, forced expiration, the right hemidiaphragm had a paradoxical downward excursion, while the left hemidiaphragm had normal upward excursion [ Figure 1b ]. Both of these findings were consistent with right hemidiaphragm paralysis.

Figure 1

(a) Fluoroscopic sniff test on deep inspiration, showing elevation of the right hemidiaphragm with depression of the left hemidiaphragm. (b) Fluoroscopic sniff test on deep expiration, showing depression of the right hemidiaphragm with elevation of the left hemidiaphragm

He additionally underwent pulmonary function tests (PFTs), which demonstrated findings similar to that of restrictive airway disease, as represented by a decreased 1-second forced expiratory volume (FEV1), decreased forced vital capacity (FVC), but with a preserved FEV1/FVC ratio. Moreover, he had a reduced total lung capacity (TLC) and vital capacity (VC). These values had negligible improvement following the administration of inhaled albuterol, distinguishing his diaphragmatic paralysis from intrinsic restrictive airway disease [ Table 1 ].

Table 1

Pulmonary function test measurements of our patient with suspected unilateral diaphragmatic paralysis secondary to phrenic nerve compression

Analysis by electromyography (EMG) demonstrated an isolated, paralysis of the right phrenic nerve as evidenced by a lack of response at the recording lead within the diaphragm at the T8/9 intercostal space when stimulating the phrenic nerve at the level of the sternocleidomastoid [ Table 2 ]. This was determined to be incomplete because direct EMG evaluation at the same location demonstrated few long duration complex motor unit potentials. The left phrenic nerve was intact as verified by both robust stimulation using the same technique and a full interference pattern on needle EMG.

Table 2

Electromyography and nerve conduction testing of the left and right phrenic nerve of our patient with suspected unilateral diaphragmatic paralysis secondary to phrenic nerve compression

It was concluded that his right hemidiaphragm was paralyzed due to a severe but incomplete right phrenic nerve injury. Due to the chronicity of this incomplete deficit, we offered a phrenic nerve exploration and decompression.

OPERATIVE TECHNIQUE

At 7 months from the onset of symptoms, the patient was taken for exploration of the phrenic nerve. He was brought to the operating room and placed under general anesthesia. The anesthesia team avoided the use of paralytic agents in order to preserve neuromonitoring capabilities during the case. The patient was positioned supine with his head turned to the left. A small bump was placed vertically between his shoulder blades to allow his shoulder to fall posteriorly, thereby maximizing the exposure of the right clavicular area. A 3 cm horizontal linear incision was made of a fingerbreadth superior to the clavicle, centered over the sternocleidomastoid. The platysma was divided, the sternocleidomastoid was retracted medially, and the supraclavicular fat pad was reflected superolaterally, exposing the anterior scalene. The phrenic nerve was identified on the surface of the anterior scalene with associated vessels crossing superficially, horizontally, and in contact with the phrenic nerve. These two arteries were running in parallel fashion immediately adjacent to one another – the suprascapular and transverse cervical arteries.[ 10 ] These arteries were also intimately impressed upon the phrenic nerve with shared fascial investment. Stimulation of the phrenic nerve proximal and distal to these intersections yielded no observable response of the diaphragm, in accordance with the findings on the preoperative compound muscle action potential (CMAP) study. The suprascapular and transverse cervical arteries were suture-ligated and sacrificed where they crossed the phrenic nerve. Finally, neurolysis of the nerve from these fascial investments at the level of the anterior scalene was performed. Additional stimulation of the phrenic nerve proximal and distal to the arterial contacts was performed, with no appreciable change in stimulatory response from the diaphragm.

In the immediate postop period, the patient did not endorse any improvement in his symptoms, nor did he describe any complications of the procedure. He was discharged home later that day after a period of postanesthesia observation.

Follow-up

At his 1-month follow-up, the patient reported that soon after surgery he developed a mild cramping sensation in his right chest wall at the level of the diaphragm, which was exacerbated by deep inspiration. In fact, he described that he would “guard” during deep inspiration due to the cramping pain. No testing was performed at this time.

At his 6-month follow-up, he endorsed that the cramping pains had resolved shortly thereafter and he subjectively felt that his work of breathing improved. He noted that within a few weeks of the prior visit he felt that he was able to take fuller breaths than before. He was subsequently able to return to his normal level of activity, no longer finding himself “winded” when ascending his steep driveway. His incision was well-healed and he tolerated the sacrifice of the compressive arteries well. A sniff test was performed prior to this follow-up, revealing an improved excursion of the right diaphragm, now symmetric with the left diaphragm [Figure 2a and b ].

Figure 2

(a) Upon deep inspiration, both the left and right hemidiaphragms move symmetrically downward. (b) Upon deep expiration, both the left and right hemidiaphragms move symmetrically upward

Four years following surgery, he returned to our clinic after obtaining a new sniff test and a new PFT. At this time, the patient reported that he had returned to his baseline exercise tolerance. He had no additional neurological complaints. His PFTs performed at this visit demonstrated a significant improvement in FEV1, FVC, VC, and TLC [ Table 3 ]. The FEV1/FVC ratio was stable from preop and postop, which is a result of both values increasing symmetrically. The ratio was not abnormal preoperatively, but the individual values of the FEV1 and the FVC were below the reference values.

Table 3

Preoperative and postoperative comparison of pulmonary function measurements following phrenic nerve decompression

His sniff test demonstrated durable improvement, and was stable compared to the previous sniff test performed 6 months postoperatively. The diaphragmatic excursion was symmetric in both the maximum exhalation and maximum inhalation sequences [Figure 3a and b ]. The degree of excursion difference between preoperative and postoperative imaging was not feasible to measure, due to the ambiguity of rib-counting within the focused field of view of the X-rays. However, the paradoxical excursions of the diaphragm were not present on this postoperative sniff test.

Figure 3

(a) Upon deep inspiration, both the left and right hemidiaphragms move symmetrically downward. (b) Upon deep expiration, both the left and right hemidiaphragms move symmetrically upward

Because of his overall improvement in objective measures (PFTs and fluoroscopic sniff test), coupled with his overall clinical subjective improvement, we decided not to pursue a new EMG/NCS study.

DISCUSSION

UDP is an uncommon affliction. The most common symptom of UDP is shortness of breath on exertion. However, many patients are asymptomatic and have incidental diagnoses based on chest radiographs for other reasons.[ 9 ] Iatrogenic injury from cardiothoracic and neck surgery is the most common identifiable cause of phrenic nerve palsies (36%), which is secondary to incidental transection, stretching, or thermal injury to the nerve.[ 2 ] Other causes of phrenic nerve palsies include tumor compression (19%), inflammation (14%), and trauma (14%).[ 1 8 ] In many cases, however, the etiology is unknown (17%).[ 1 ] A new, emerging vascular etiology has been considered, potentially caused by a vascular compression of the phrenic nerve by the transverse cervical artery or suprascapular artery at the level of the thoracic outlet.[ 5 ] The relationship of the phrenic nerve to these arteries and their surrounding structures is shown in Figure 4 .

Figure 4

Illustration of the relationship between the arteries, musculature, and nerves of the clavicular area. (1) Middle scalene muscle, (2) anterior scalene muscle, (3) dorsal scapular nerve, (4) transverse cervical artery, (5) phrenic nerve, (6) brachial plexus, (7) dorsal scapular artery, (8) suprascapular artery, (9) thyrocervical artery, (10) lung, (11) inferior cervical sympathetic ganglion, (12) longus colli muscle, (13) vertebral artery, (14) vagus, (15) inferior thyroid artery, (16) middle cervical sympathetic ganglion, (17) recurrent laryngeal nerve. Original image reprinted from: Int J Shoulder Surg. 2010 Jul-Sep; 4 (3): 63–74. Creative Commons Attribution 4.0 (CC BY-SA 4.0). Full terms at https://creativecommons.org/licenses/by-sa/4.0

It was difficult to determine the exact pathophysiologic mechanism behind our patient's UDP. Certainly, patient positioning and shoulder traction in preparation for the surgery could have led to traction of the nerve, particularly given the anatomical arrangement at the level of the anterior scalene. Following anterior cervical discectomy and fusion, there is typically height added to the spine as the large intervertebral disc replacements are placed to distract the foramen. In doing so, the scalene muscles which originate from the transverse processes and insert on the first rib can experience additional traction. In a patient with a history of whiplash injury, the scalene muscles are frequently more fibrotic with thicker fascia and thus have less “give.” This could theoretically lead to tensioning of the scalene muscles and associated fascia and the invested structures (arteries), pressing them against adjacent nervous structures (the phrenic nerve in this case). Curiously, his symptoms arose immediately following a posterior cervical spine fusion, which does not typically result in the same type of cervical distraction. Ultimately, it could be inflammatory, such as a Parsonage-Turner Syndrome, or a combination of many different etiologies. In our case the exact cause of the phrenic palsy cannot be clearly identified and whether our intervention was the source of the improvement or simply corresponded temporally with the natural recovery of the nerve is impossible to determine.

The pathophysiologic mechanisms of shortness of breath in UDP are two-fold.[ 6 ] Primarily, the paralysis of the hemidiaphragm increases mechanical respiratory effort. Secondarily, there is paradoxical diaphragmatic elevation on the affected side due to a normal downward excursion of the contralateral diaphragm which increases intraabdominal pressures that apply a force to elevate the affected diaphragm. This paradoxical motion antagonizes the work of the accessory muscles of respiration, thereby increasing the work of breathing even further.[ 6 ] This paradoxical motion was seen on our patient's sniff test and returned to normal motion on postoperative imaging.

Due to the rarity of this disease, there is no single well-studied course of management of patients with UDP. The single-most studied treatment has been diaphragmatic plication.[ 3 4 11 ] Plication of the diaphragm surgically increases the tensile strength of the diaphragm by removing the redundancy, which prevents paradoxical excursion. Prevention of paradoxical excursion allows movement of the contralateral diaphragm to indirectly pull the ipsilateral diaphragm as it descends. Plication of the diaphragm certainly has intraoperative and postoperative risks.[ 11 ] The decision for surgical correction must weigh in the manifestation of disease in the patient's day-to-day livelihood. Results of plication have been shown to be quite successful.[ 3 4 11 ] Though diaphragmatic plication does not reverse the etiological cause of UDP, it manages the symptoms. Another option more recently introduced has involved transfer of intercostal nerves to the distal phrenic to restore tone to this muscle.[ 7 ]

Recent advances in surgical technique have aimed at reversing the etiologic causes of UDP. Kaufman et al. were the first to describe the concept presented here that ligation of the transverse cervical artery at the phrenic nerve may have contributed to restored diaphragmatic function immediately postoperatively in three patients who they believed suffered from a compressive vascular etiology.[ 5 ] They also demonstrated intraoperative evidence of a conduction block within the phrenic nerve, in which proximal stimulation elicited no response, whereas distal stimulation did result in robust activation prior to ligation. Here, we presented a case of vascular decompression of the phrenic nerve in a patient with UDP with postoperative resolution of symptoms, which lacked intraoperative confirmation of a conduction block. This case demonstrated postoperative success of decompression of the phrenic nerve by ligation and sacrifice of the transverse cervical and the suprascapular arteries. Symptoms of returning diaphragmatic function appeared very soon after the procedure, making it a feasible explanation for this sudden change after 7 months of paralysis. This case showcases the preoperative workup and the operative technique to decompress the phrenic nerve. Unlike diaphragmatic plication, phrenic nerve decompression targets the etiology rather than the pathophysiology of UDP secondary to phrenic nerve compression, and should be considered in the management of this disabling disease entity.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1. Elefteriades J, Singh M, Tang P, Siegel MD, Kenney B, Pandey A. Unilateral diaphragm paralysis: Etiology, impact, and natural history. J Cardiovasc Surg (Torino). 2008. 49: 289-95

2. Fell SC. Surgical anatomy of the diaphragm and the phrenic nerve. Chest Surg Clin N Am. 1998. 8: 281-94

3. Graham DR, Kaplan D, Evans CC, Hind CR, Donnelly RJ. Diaphragmatic plication for unilateral diaphragmatic paralysis: A 10-year experience. Ann Thorac Surg. 1990. 49: 248-51

4. Higgs SM, Hussain A, Jackson M, Donnelly RJ, Berrisford RG. Long term results of diaphragmatic plication for unilateral diaphragm paralysis. Eur J Cardiothorac Surg. 2002. 21: 294-7

5. Kaufman MR, Willekes LJ, Elkwood AI, Rose MI, Patel TR, Ashinoff RL. Diaphragm paralysis caused by transverse cervical artery compression of the phrenic nerve: The Red Cross syndrome. Clin Neurol Neurosurg. 2012. 114: 502-5

6. Ko MA, Darling GE. Acquired paralysis of the diaphragm. Thorac Surg Clin. 2009. 19: 501-10

7. Krieger LM, Krieger AJ. The intercostal to phrenic nerve transfer: An effective means of reanimating the diaphragm in patients with high cervical spine injury. Plast Reconstr Surg. 2000. 105: 1255-61

8. McCool FD, Tzelepis GE. Dysfunction of the diaphragm. N Engl J Med. 2012. 366: 932-42

9. Piehler JM, Pairolero PC, Gracey DR, Bernatz PE. Unexplained diaphragmatic paralysis: A harbinger of malignant disease?. J Thorac Cardiovasc Surg. 1982. 84: 861-4

10. Verenna AA, Alexandru D, Karimi A, Brown JM, Bove GM, Daly FJ. Dorsal scapular artery variations and relationship to the brachial plexus, and a related thoracic outlet syndrome case. J Brachial Plex Peripher Nerve Inj. 2016. 11: e21-8

11. Versteegh MI, Braun J, Voigt PG, Bosman DB, Stolk J, Rabe KF. Diaphragm plication in adult patients with diaphragm paralysis leads to long-term improvement of pulmonary function and level of dyspnea. Eur J Cardiothorac Surg. 2007. 32: 449-56

Abstract

Background:Chronic subdural hematoma (CSDH) of the posterior fossa is uncommon in adults. Only a few cases have been reported, and most of these were secondary to head injury or anticoagulant therapy. We herein describe a case of successful surgical treatment of CSDH in the posterior fossa after surgical removal of a large supratentorial and infratentorial dermoid cyst.

Case Description:A 71-year-old woman underwent removal of a left supratentorial and infratentorial dermoid cyst via a left transzygomatic approach. Three years, 6 months after surgery, screening computed tomography revealed CSDH in the supratentorial and infratentorial regions. Four months later, the patient was transferred to the emergency department with cerebellar ataxia, vomiting, and deterioration of consciousness. Two hematomas, one in the supratentorial region and one in the infratentorial region, were greatly compressing the brain, and seemed to be separate lesions. It was difficult to judge on computed tomography whether there was communication between these two hematoma cavities. The patient underwent hematoma removal via suboccipital craniotomy for the posterior fossa CSDH to resolve brain stem compression. Burr-hole irrigation was used for the supratentorial CSDH to avoid upper herniation. The patient recovered uneventfully and was discharged with no neurological deficits.

Conclusion:Although the optimal treatment for CSDH of the posterior fossa remains unclear because of the limited number of previous reports, direct decompression of the posterior fossa via suboccipital craniotomy should be considered, especially when CSDH exists primarily at the cerebellopontine angle and strongly compresses the brain stem.

Keywords: Chronic subdural hematoma, craniotomy, dermal cyst, posterior fossa, trepanation

INTRODUCTION

Chronic subdural hematoma (CSDH) of the posterior fossa is uncommon in adults. Only a few cases have been reported,[ 2 3 5 ] and most of these were secondary to head injury or anticoagulant therapy. We herein report a case of successful treatment of CSDH in the posterior fossa that developed after surgical removal of a large supratentorial and infratentorial dermoid cyst. Our report emphasizes the strategy of suboccipital craniotomy with supratentorial burr-hole irrigation.

CASE REPORT

An asymptomatic 71-year-old woman was diagnosed with a left supratentorial and infratentorial dermoid cyst on screening magnetic resonance imaging (MRI) [ Figure 1 ] and admitted to our hospital. She had a past history of atrial fibrillation, tympanoplasty for tympanitis, and total ovariohysterectomy. Removal of the supratentorial and infratentorial cyst was performed via a left transzygomatic approach. The tumor was almost totally resected with no sequelae; the supratentorial and infratentorial compartments remained. Because the patient had a preoperative history of atrial fibrillation, postoperative warfarin therapy was administered to prevent embolism. The patient was followed-up by the outpatient service and developed no recurrence or any abnormal imaging signals in the residual cavity [ Figure 2 ]. Although the patient had no history of head injury, a screening computed tomography (CT) scan performed 3.5 years postoperatively as part of annual follow-up revealed a density change within the supratentorial and infratentorial regions. At that point, we decided to perform periodic follow-ups once monthly because the patient was asymptomatic, and the hematoma did not appear to be compressing the brain stem. Four months after the start of follow-up, the patient was transferred to the emergency department because of cerebellar ataxia, vomiting, and deterioration of consciousness (Glasgow Coma Scale score: E3V4M6) with no paresis. A CT scan revealed enlargement of the hematoma in the posterior fossa and compression of the brain stem [ Figure 3 ]. Two hematomas, one in the supratentorial region and one in the infratentorial region, were greatly compressing the brain, and seemed to be separate lesions. It was difficult to judge on CT whether there was communication between the two hematoma cavities. Because the patient's symptoms were considered to have resulted from compression of the brain stem and cerebellum, we urgently performed hematoma removal under general anesthesia via suboccipital craniotomy with a 6-cm linear incision behind the mastoid process along the hairline for the posterior fossa CSDH. Burr-hole irrigation was performed via a linear incision for the supratentorial CSDH. The tension in the posterior fossa was high, and the outer membrane was seen under the dura mater [ Figure 4a ]. We resected the outer membrane of the CSDH as completely as possible and copiously irrigated the hematoma cavity. The fluid in both the supratentorial and infratentorial portions was the same reddish brown. After adequate irrigation of both fields, we confirmed communication between the supratentorial and infratentorial spaces. Closed-system subdural drainage catheters were inserted in both cavities until the day after the operation. Postoperative CT scans of the head revealed disappearance of the CSDH in both the supratentorial and infratentorial spaces [Figure 4b and c ]. Signal change was also confirmed on postoperative MRI [ Figure 4d ]. The patient's symptoms gradually subsided, and she was discharged to her home with no neurological deficits on postoperative day 12. Apixaban was prescribed as anticoagulation therapy upon discharge. No recurrence was detected on CT scan 1 month postoperatively. At 1 year, CSDH had not recurred.

Figure 1

T1-weighted magnetic resonance imaging with contrast enhancement before tumour removal. The images revealed a supratentorial and infratentorial mass with a cyst. (a) Coronal view (b) Axial view

Figure 2

Postoperative imaging findings. (a) T1-weighted magnetic resonance imaging without enhancement 6 months postoperatively showed low signal intensity within the cavity. (b and c) Computed tomography 6 months postoperatively showed low density in the supratentorial and infratentorial cavities

Figure 3

Imaging findings upon admission. (a) T1-weighted magnetic resonance imaging with contrast enhancement on admission showed a signal change from low to mildly high within the cavity. (b) Computed tomography on admission revealed low and iso-density of the infratentorial lesion with compression of the brain stem. (c) The supratentorial lesion also exhibited mixed density on computed tomography

Figure 4

Intraoperative and postoperative findings. (a) Intraoperative image after dural incision shows the outer membrane of the haematoma on the cerebellar hemisphere. (b and c) Computed tomography after haematoma evacuation shows removal of the haematoma and resolution of the brain stem compression. (d) Postoperative MRI shows signal change in the cavity

DISCUSSION

The reported incidence of postoperative CSDH is 1.6% after brain tumor surgery, and the period between craniotomy and development of CSDH ranges from 3 to 5 months (mean, 4.3 months).[ 4 ] In the present case, CSDH occurred 3.5 years after tumor removal. To the best of our knowledge, this is the latest reported onset of CSDH after tumor removal.[ 4 ]

CSDH of the posterior fossa is rare in adults.[ 2 3 5 ] Infratentorial subdural hematomas can result from mild traumatic events, anticoagulation therapy, blood clotting disorders, and intracranial hypotension. In some cases, no cause is found.[ 1 3 ] Stendl et al. reported that six out of 15 patients with posterior fossa CSDH had taken anticoagulant drugs.[ 3 ] Previous reports have indicated that there is no difference in the mechanism of occurrence of supratentorial and infratentorial CSDH.[ 1 ] The rather low incidence of CSDH in the posterior fossa may be explained by the rare occurrence of venous sinus injuries and the low number of bridging veins present in the posterior fossa.[ 3 ] There are no previous reports of dermoid cyst removal resulting in postoperative CSDH. In our case, the patient did not have a history of head trauma or prolongation of international normalized ratio resulting from anticoagulant drugs. The mechanism of this delayed CSDH is unclear. However, our case indicates that patients with a postoperative cavity who take anticoagulant drugs need periodic imaging follow-up for at least several years.

Previous reports have described surgical drainage (craniotomy or trepanation) and conservative therapy for treatment of CSDH.[ 1 2 ] However, the optimal treatment is unknown because of the limited number of reports. In the present case, because the CSDH developed in the residual cavity, the hematoma was mainly located at the anterior surface of the brain stem and cerebellopontine angle, and strongly compressed these structures. Based on the state of the residual cavity, we initially predicted the presence of communication between the supratentorial and infratentorial hematomas; we therefore considered performing only supratentorial irrigation. However, we removed the hematoma via suboccipital craniotomy for the posterior fossa CSDH and with burr-hole irrigation for the supratentorial CSDH for the following two reasons. First, the patient developed vomiting and cerebellar ataxia without paresis, and we considered these symptoms to be caused by severe compression of the brain stem. CSDH in the posterior fossa often causes nonspecific symptoms.[ 1 ] More than half cases of CSDH in the posterior fossa are associated with symptoms such as vertigo, cerebellar ataxia, and nystagmus without hemiparesis.[ 2 ] Because the hematoma in the present case was mainly located at the left cerebellopontine angle and anterior surface of the brain stem, and not on the surface of the cerebellar hemisphere, we had to directly open the cerebellopontine angle to resect the outer membrane and irrigate the hematoma via suboccipital craniotomy. Second, although we performed preoperative MRI, it was not clear whether a communication existed between the supratentorial and infratentorial hematomas. Additionally, even if such hematomas communicate, adequate irrigation is difficult if approached only from the supratentorial portion.

We finally confirmed the presence of communication between the supratentorial and infratentorial compartments and observed drainage to the infratentorial space upon irrigation of the supratentorial portion. Because we opened the top and bottom of the long hematoma cavity, including both the supratentorial and infratentorial portions, gravity induced the formation of a spontaneous drainage route, and complete removal of the hematoma was achieved.

CONCLUSION

We have herein described suboccipital craniotomy with supratentorial burr-hole irrigation for resolution of brain stem compression resulting from CSDH in the posterior fossa, with a good functional outcome. From a clinical viewpoint, we recommend that clinicians consider direct decompression of the posterior fossa via suboccipital craniotomy, especially when CSDH exists primarily in the cerebellopontine angle and strongly compresses the brain stem.

Disclosure

The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this article.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that name and initial will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1. Costa LB, De Andrade A, Fonseca Valadão G. Chronic subdural hematoma of the posterior fossa associated with cerebellar hemorrhage: Report of a rare disease with MRI findings. Arq Neuropsiquiatr. 2004. 62: 170-2

2. Kurisu K, Kawabori M, Niiya Y, Ohta Y, Mabuchi S, Houkin K. Bilateral chronic subdural hematomas of the posterior fossae. Neurol Med Chir (Tokyo). 2012. 52: 822-5

3. Stendel R, Schulte T, Pietila TA, Suess O, Brock M. Spontaneous bilateral chronic subdural haematoma of the posterior fossa. Case report and review of the literature. Acta Neurochir (Wien). 2002. 144: 497-500

4. Takahashi Y, Ohkura A, Sugita Y, Sugita S, Miyagi J, Shigemori M. Postoperative Chronic Subdural Hematoma Following Craniotomy. Neurol Med Chir. 1995. 35: 78-81

5. Takami H, Oshiro N, Hiraoka F, Murao M, Ide T. Rapid resolution of a spontaneous large chronic subdural haematoma in the posterior fossa under conservative treatment with platelet administration to aplastic anaemia. Clin Neurol Neurosurg. 2013. 115: 2236-9

Abstract

Background:Von Hippel–Lindau (VHL) disease is an autosomal dominant condition characterized by formation of multiple benign and malignant tumors. In this disease supratentorial lesions are rare and no falcine meningioma has been previously reported. Differential diagnosis is very difficult and the histopathological examination is the definitive method for diagnosis.

Case Description:A patient with VHL underwent a suboccipital craniotomy for removal of cerebellar hemangioblastoma and after 2 years magnetic resonance imaging (MRI) showed an iperintense solid mass located at posterior part of the falx. Histological diagnosis revealed meningioma.

Conclusion:The only case in the literature of falcine meningioma in a patient with Von Hippel–Lindau disease, discovered during radiological follow-up, is described and a surgical management is proposed.

Keywords: Falcine meningioma, hemangioblastoma, Von Hippel-Lindau disease

INTRODUCTION

Von Hippel–Lindau (VHL) disease is a rare condition caused by genetic mutations on chromosome 3,[ 1 6 12 13 ] transmitted in an autosomal dominant fashion with near complete penetrance and characterized by formation of multiple benign and malignant tumors, as well as cysts in multiple organs.[ 7 ] Affected patients frequently develop retinal and central nervous system hemangioblastomas (HB), clear cell renal cell carcinomas (RCC), pheochromocytomas, pancreatic neuroendocrine tumors, and endolymphatic sac tumors (ELSTs).[ 13 ] In VHL disease supratentorial lesions, usually HB, are rare and meningiomas are extremely rare.[ 11 ] In literature no case of falcine meningioma in VHL has been previously reported. We describe the first case of meningioma located at the third posterior of the falx in a patient with VHL.

CASE REPORT

A 55-year-old Caucasian female patient underwent a suboccipital craniotomy for removal of left cerebellar hemisphere HB. Abdominal computed tomography revealed a pheochromocytoma that was excised. Genetic analysis showed the presence of a VHL gene mutation and an evaluation of the family history demonstrated VHL disease in two of the patient's siblings. Thus this patient's condition had been diagnosed as VHL disease. Follow-up magnetic resonance imaging (MRI) performed 1 year after the first operation had shown no evidence of recurrence or abnormal findings in the supratentorial region [ Figure 1 ]. However, MRI performed 2 years after the first operation showed a solid mass with strong enhancement in the right cerebellar hemisphere as recurrence and an iperintense solid mass located at posterior part of the falx [ Figure 2 ]. MRI performed at 4 years [ Figure 3 ] revealed slow growth of both lesions that were asymptomatic. A frameless stereotactic biopsy with the Leksell Model G stereotactic system (Elekta, Inc., Norcross, GA) of falcine lesion was performed. The intra and postoperative histological diagnosis was meningothelial meningioma of World Health Organization Grade 1 [ Figure 4 ]. There were no postoperative complications. The patient decided for clinical and radiological follow-up, temporarily delaying a following definitive surgical or radiotherapical treatment.

Figure 1

One year postoperative axial (a), sagittal (b), a coronal (c) T1-weighted MR images showing no evidence of recurrence or abnormal findings in the supratentorial region

Figure 2

Two years postoperative axial (a and b), sagittal (c and d), a coronal (e) T1-weighted MR images with gadolinium detecting a solid mass with strong enhancement in the right cerebellar hemisphere (yellow arrow) and an hyperintense extra-axial solid mass located at posterior part of the falx (green arrow)

Figure 3

Four years postoperative axial (a and b), sagittal (c and d), a coronal (e) T1-weighted MR images with gadolinium demonstrating further slow growth of both lesions described in Figure 2

Figure 4

Histological images of meningothelial meningioma showing syncytial clusters of meningothelial cells

DISCUSSION

Melmon and Rosen[ 14 ] first proposed clinical criteria for the diagnosis of VHL, recognizing cerebellar HB, the hallmark of Lindau's tumor. Later Lamiell[ 10 ] redefined the clinical diagnosis of VHL including an expanded list of associated visceral tumors, as ELST, RCC, pheochromocytoma, paraganglioma, neuroendocrine neoplasm, and/or multiple cysts of the pancreas. The diagnosis of VHL is made if a patient has at least two central nervous system HB or at least one central nervous system HB and one of the visceral lesions described above or at least one of the visceral lesions previously described above, and a pathogenic mutation in VHL gene or a first-degree relative with VHL.[ 1 ] The incidence of VHL ranges from 1 in 40,000 live births and typically it first manifests in the second decade of life.[ 3 ] Because of the phenomenon of genetic anticipation, evidence of progressively earlier age of onset and more severe presentation in successive generations have been reported.[ 6 16 ] Life expectancy in VHL remains the lowest among common inherited tumor syndromes, with a male life expectancy (59.4 years) significantly higher than female (48.4 years) and with HB or RCC as the major cause of mortality.[ 13 ] In 1995, Bleggi-Torres[ 2 ] first reported a case of meningioma in VHL, and in the pertinent literature only other two cases[ 4 8 ] have been previously described [ Table 1 ]. Because of a very difficult preoperative radiological differential diagnosis, the histopathological examination after surgical resection, if indicated, is the definitive method for diagnosis.[ 7 9 ] The main differential diagnoses of these lesions include meningeal HB, hemangiopericytoma, and metastatic RCC. Epithelial membrane antigen (EMA) immunostaining can help distinguish supratentorial meningeal HB from meningioma.[ 15 ] Furthermore, HB tumors are generally negative for glial fibrillary acidic protein and positive for neuron-specific enolase.[ 17 ] Finally, vimentin is positive in HB tumors, supporting the hypothesis that HBs originate from the mesenchyme.[ 3 ] Recently, inhibin-α has been described as a useful marker for distinguishing HB from angiomatous meningioma.[ 9 ] Immunohistochemically, RCC stain positive for EMA whereas HB are EMA negative.[ 5 ] Because of the extreme rarity of falcine meningiomas in VHL, treatment modality has never been analyzed. In our opinion, in asymptomatic lesions stereotactic biopsy is suggested to design the next therapeutic step, while in symptomatic or hemorrhagic lesions or asymptomatic lesions that show significant growth during radiological follow-up, maximal resection is recommended to prevent recurrence and postoperative hemorrhage. Radiotherapy is useful for multiple, subtotally resected, and recurrent tumors.

Table 1

Review of meningiomas in VHL

CONCLUSIONS

We report a rare case of asymptomatic slow-growing meningioma located at the third posterior of the falx in a patient with VHL, detected 2 years after surgical resection of a cerebellar HB, in which no surgical or radiotherapical treatment has been previously performed. In VHL, supratentorial meningeal masses are extremely rare and no other case of falcine involvement has been previously reported. Pathological and immunohistochemical techniques are helpful for differential diagnosis. Regular follow-up is mandatory and surgical resection is the definitive treatment for these lesions.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1. Binderup ML, Bisgaard ML, Harbud V, Moller HU, Gimsing S, Friis-Hansen L.editors. Von Hippel-Lindau disease (vHL). National clinical guideline for diagnosis and surveillance in Denmark. Dan Med J. 2013. 60: B4763-

2. Bleggi-Torres LF, De Noranha L, Fillus Neto J, Telles JE, Madalozzo LE. Von Hippel-Lindau's disease: Report of three cases and review of the literature. Arq Neuropsiquiatr. 1995. 53: 782-8

3. Chittiboina P, Lonser RR. Von Hippel-Lindau disease. Handb Clin Neurol. 2015. 132: 139-56

4. Governale LS, Vortmeyer AO, Zhuang Z, Oldfield EH. Fibrous meningioma in a patient with von Hippel-Lindau disease: A genetic analysis. J Neurosurg. 2001. 95: 1045-9

5. Ishwar S, Taniguchi RM, Vogel FS. Multiple supratentorial hemangioblastomas. Case study and ultrastructural characteristics. J Neurosurg. 1971. 35: 396-405

6. Kaelin WG. Molecular basis of the VHL hereditary cancer syndrome. Nat Rev Cancer. 2002. 2: 673-82

7. Kaloostian P, Taylor C. Supratentorial dural-based haemangioblastoma in a native American patient without Von Hippel Lindau Syndrome. J Surg Case Rep. 2012. 2012: 11-

8. Kanno H, Yamamoto I, Yoshida M, Kitamura H. Meningioma showing VHL gene inactivation in a patient with von Hippel-Lindau disease. Neurology. 2003. 60: 1197-9

9. Kim H, Park IS, Jo KW. Meningeal supratentorial hemangioblastoma in a patient with von Hippel-Lindau disease mimicking angioblastic meningioma. J Korean Neurosurg Soc. 2013. 54: 415-9

10. Lamiell JM. Army clinical investigation of von Hippel-Lindau disease, 1977-2000. Mil Med. 2001. 166: 839-42

11. Lee KR, Kishore PR, Wulfsberg E, Kepes JJ. Supratentorial leptomeningeal hemangioblastoma. Neurology. 1978. 28: 727-30

12. Lonser RR, Glenn GM, Walther M, Chew EY, Libutti SK, Linehan WM. von Hippel-Lindau disease. Lancet. 2003. 361: 2059-67

13. Maddock IR, Moran A, Maher ER, Teare MD, Norman A, Payne SJ. A genetic register for von Hippel-Lindau disease. J Med Genet. 1996. 33: 120-7

14. Melmon KL, Rosen SW. Lindau's disease. Review of the literature and study of a large kindred. Am J Med. 1964. 36: 595-617

15. Murali R, Jones WI, Ma Wyatt J. A 57-year-old man with a dural-based parietal lobe tumor. Brain Pathol. 2007. 17: 460-3

16. Ning XH, Zhang N, Li T, Wu PJ, Wang X, Li XY. Telomere shortening is associated with genetic anticipation in Chinese Von Hippel-Lindau disease families. Cancer Res. 2014. 74: 3802-9

17. Takei H, Bhattacharjee MB, Rivera A, Dancer Y, Powell SZ. New immunohistochemical markers in the evaluation of central nervous system tumors: A review of 7 selected adult and pediatric brain tumors. Arch Pathol Lab Med. 2007. 131: 234-41

Abstract

Background:Although it is well known that most choroid plexus cysts (CPCs) are asymptomatic, previous studies have reported that they can infrequently cause progressive hydrocephalus along with their increasing sizes. Among those cases, some patients needed cyst fenestration or cerebrospinal fluid (CSF) diversion to recover neurological deterioration. Meanwhile, some CPCs revealed spontaneous resolution, and in rare cases, they developed re-accumulation. Some reports have described series of radiological findings about their changes in location.

Case Description:We present a 47-year-old male with CPC manifesting obstructive hydrocephalus. Radiological findings of the lateral and the third ventricles changed along with their different obstructive points, leading to their own symptoms. Because the patient's symptoms were not resolved completely, he underwent endoscopic fenestration for the cyst at the third ventricle. We could perform near-total resection, resulting in recovery of normal CSF flow. Postoperatively, the size of the ventricles decreased, with histological confirmation of a CPC. His symptoms resolved clearly without any complications.

Conclusions:It seems quite unusual that shift of the CPC location in the ventricle systems could induce not only different types of hydrocephalus but also their own symptoms. We need to consider that the location of CPCs might change when patients present with fluctuating symptoms over time.

Keywords: Choroid plexus cyst, endoscopic surgery, obstructive hydrocephalus

INTRODUCTION

Choroid plexus cysts (CPCs) rarely become symptomatic and are reported in 2.8% of fetal subjects upon second-trimester ultrasound examinations.[ 7 ] In adults, most CPCs are asymptomatic and discovered incidentally.[ 6 ] However, some previous reports suggested that CPCs could cause obstructive hydrocephalus.[ 2 11 ] Meanwhile, in other patients, they infrequently appear spontaneously resolved, and in rare cases, they re-accumulate.[ 1 3 8 9 10 11 ] Some authors suggested a mechanism in which CPCs can be flexible and cause repeated symptomatic obstructive hydrocephalus, and cerebral ultrasonography revealed that the floating CPC shifted to the foramen of Monro and obstructed cerebrospinal fluid (CSF) pathways.[ 12 ] We experienced an adult patient with fluctuating symptoms associated with CPCs. The series of radiological findings indicated that the flexible CPCs shifted their locations, resulting in different types of obstructive hydrocephalus, each with their own symptoms.

CASE REPORT

A 47-year-old healthy man consulted a local hospital with complaints of unsteadiness and dizziness. Despite magnetic resonance imaging (MRI) revealing a cystic lesion in the third ventricle, he presented with no neurological abnormalities and his symptoms disappeared shortly. One year later, he was referred to the hospital again with complaints of headache. MRI revealed unilateral obstructive hydrocephalus with enlargement of the cyst, which occluded the foramen of Monro and protruded into the left lateral ventricle [ Figure 1a ]. He was referred to our hospital for the surgery of cyst fenestration, and his headache resolved spontaneously. Endoscopic biopsy of the cyst wall was scheduled for the relief of headache and histological confirmation was obtained one month later. Upon admission for operation, MRI revealed that the cyst drew back in the third ventricle, and the shape of the ventricles turned out to be symmetrical. However, the ventricles still remained enlarged [ Figure 1b ]. The sequential MRI findings demonstrated that the cyst at the third ventricle was flexible in its location and induced noncommunicative hydrocephalus with different obstructive points. He complained of different types of headaches, and finally we decided to perform endoscopic surgery to eliminate the symptom and normalize CSF flow in the ventricles.

Figure 1

T2-weighted coronal magnetic resonance images. (a) The cystic lesion (black arrow) located in the third ventricle caused obstructive hydrocephalus with asymmetrical enlargement of the ventricles. (b) Preoperative image showing the asymmetrical enlargement of ventricles was resolved but the enlargement of ventricles remained because of previous protrusion of the cyst into the left lateral ventricle regressed into the third ventricle (white arrow). (c) Postoperative image revealing the ventricular size decreased

The patient underwent endoscopic cyst fenestration at the third ventricle via the left lateral ventricle with a fiberscope (VEF-V, Olympus, Japan). The cystic lesion occupied the enlarged foramen of Monro [ Figure 2a ]. There was a slight gap around the cyst wall at the foramen of Monro and the cyst wall, and some granular tissue could be found through the cyst wall. The cyst wall was connected to the choroid plexus behind the foramen of Monro [ Figure 2b ]. Most of the cyst wall could be easily removed using endoscopic forceps, but the small part of the cyst wall was tightly adhered to the choroid plexus and was left untouched [ Figure 2c ]. Finally, near-total resection was achieved to recover the physiological CSF flow through the foramen of Monro [ Figure 2d ].

Figure 2

Operative findings. (a) The cyst arising from the third ventricle and protruding into the left foramen of Monro. (b) A slight gap between the cyst wall and the edge of the foramen of Monro (black arrow). (c) The cyst adhered to the choroid plexus at the edge of the foramen of Monro (black arrow). d) The cyst decreased in size after surgery, confirming the bottom of the third ventricle

Upon pathological examination of the surgical specimen, cuboidal epithelia lined with connective tissue were found in papillary patterns with some calcification and no malignant appearances [ Figure 3 ]. Immunoreactivity for epithelial membrane antigen and glial fibrillary acidic protein were positive in cuboidal epithelia, and these findings are consistent with CPCs.

Figure 3

Hematoxylin and eosin staining shows cuboidal epithelium lining with the collagen tissue, leading to the diagnosis of choroid plexus cyst

The postoperative course was excellent, and his symptoms disappeared completely. MRI revealed the ventricles were reduced in size compared to preoperatively [ Figure 1c ]. The patient was discharged on postoperative day 10 without any complications. He presented no complications for 6 months postoperatively.

DISCUSSION

In the present report, we described a case of a previously healthy man who complained of fluctuating symptoms with a CPC at the third ventricle. The cyst changed its location and caused different types of obstructive hydrocephalus. Endoscopic surgery relieved his symptoms without any neurological deficits. This is the first case report that demonstrates a flexible CPC manifesting different types of obstructive hydrocephalus, as revealed on the chronological MRI findings.

CPCs are kinds of neuroepithelial cysts, usually located in the lateral ventricles, and rarely in the third ventricle.[ 11 ] In general, these cysts are asymptomatic, but sometimes cause symptomatic obstructive hydrocephalus.[ 2 11 ] Their symptoms are reported as acute headache, vomiting, somnolence, and declining mental status.[ 8 ] MRI is useful to identify CPCs associated with obstructive hydrocephalus.[ 6 8 ]

Flexible CPCs sometimes recur as symptomatic obstructive hydrocephalus. These cysts change their locations causing obstructive hydrocephalus along with the alternation of CSF pressure balance. In cerebral ultrasonography, some case reports described CPC in childhood showed that the CPC obstructed CSF pathways at the foramen of Monro while crying.[ 12 ] Crying is considered to be one mechanism to induce changes in CSF pressure balance, while the cyst prolapses from the third ventricle into the left lateral ventricle. Unilateral obstructive hydrocephalus occurs with the cystic lesion occluding the foramen of Monro. The CPCs would change locations with posture changes, leading to changes in symptoms, such as acute debilitating, nausea, vomiting, and posture headaches.[ 2 ] These reports indicated that the changing locations of flexible CPCs can cause “intermittent” obstructive hydrocephalus. However, it had never been shown that sequential changes in locations of CPCs resulted in different types of CSF dynamic disturbances.

The effectiveness of endoscopic surgery for intraventricular cysts has been reported by many authors.[ 4 6 ] The endoscopic approach is less invasive than craniotomy, and can avoid both aggressive craniotomy and inserting shunt systems into the body. However, endoscopic treatments require skillful techniques.[ 10 ] The total resection of the cyst wall is not always recommended, and the part of the cyst wall adhering to the surrounding neural tissue should not be removed.[ 5 ] In our case, tightly adhered cyst walls were left to avoid massive bleeding from the choroid plexus.

There are some differences in clinical symptoms between adults and children. Most cases of flexible CPCs in childhood have been reported to present as acute obstructive hydrocephalus.[ 1 3 8 9 10 11 12 ] However, it is difficult to detect these symptoms and signs accurately in children. In contrast, some adult patients with obstructive hydrocephalus caused by CPCs complained of chronic obscure symptoms.[ 2 6 ] Obstructive hydrocephalus caused by CPCs in adulthood progressed more gradually than that in childhood, because there is greater ventricle volume in adult patients to compensate for cyst growth.

We experienced the first case of an adult patient who suffered from a flexible CPC inducing different types of obstructive hydrocephalus. These cysts in adults would sometimes develop as chronic obscure symptoms. We need to consider CPCs that are flexible when patients present with fluctuating symptoms as a possible operative indication.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1. Azab WA, Mijalcic RM, Aboalhasan AA, Khan TA, Abdelnabi EA. Endoscopic management of a choroid plexus cyst of the third ventricle: Case report and documentation of dynamic behavior. Childs Nerv Syst. 2015. 31: 815-9

2. Chamczuk AJ, Grand W. Endoscopic cauterization of a symptomatic choroid plexus cyst at the foramen of Monro: Case report. Neurosurgery. 2010. 66: 376-7

3. Filardi TZ, Finn L, Gabikian P, Giussani C, Ebenezer S, Avellino AM. Treatment of intermittent obstructive hydrocephalus secondary to a choroid plexus cyst. J Neurosurg Pediatr. 2009. 4: 571-4

4. Gangemi M, Maiuri F, Godano U, Mascari C, Longatti PL, Marzucco M. Endoscopic treatment of para- and intraventricular cerebrospinal fluid cysts. Minim Invasive Neurosurg. 2000. 43: 153-8

5. Hanbali F, Fuller GN, Leeds NE, Sawaya R. Choroid plexus cyst and chordoid glioma. Report of two cases. Neurosurg Focus. 2001. 10: E5-

6. Jeon JH, Lee SW, Ko JK, Choi BG, Cha SH, Song GS. Neuroendoscopic removal of large choroid plexus cyst: A case report. J Korean Med Sci. 2005. 20: 335-9

7. Morcos CL, Platt LD, Carlson DE, Gregory KD, Greene NH, Korst LM. The isolated choroid plexus cyst. Obstet Gynecol. 1998. 92: 232-6

8. Nahed BV, Darbar A, Doiron R, Saad A, Robson CD, Smith ER. Acute hydrocephalus secondary to obstruction of the foramen of Monro and cerebral aqueduct caused by a choroid plexus cyst in the lateral ventricle. Case report. J Neurosurg. 2007. 107: 236-9

9. Ormond DR, Omeis I, Mohan A, Murali R, Narayan P. Obstructive hydrocephalus due to a third ventricular neuroepithelial cyst. J Neurosurg Pediatr. 2008. 1: 481-4

10. Parizek J, Jakubec J, Hobza V, Nemeckova J, Cernoch Z, Sercl M. Choroid plexus cyst of the left lateral ventricle with intermittent blockage of the foramen of Monro, and initial invagination into the III ventricle in a child. Childs Nerv Syst. 1998. 14: 700-8

11. Spennato P, Chiaramonte C, Cicala D, Donofrio V, Barbarisi M, Nastro A. Acute triventricular hydrocephalus caused by choroid plexus cysts: A diagnostic and neurosurgical challenge. Neurosurg Focus. 2016. 41: E9-

12. van Baalen A, Stephani U. Flexible and floating choroid plexus cyst of the third ventricle: An ultrasonographic video documentation. Child Nerv Syst. 2007. 23: 259-61

Abstract

Background:Meningiomas give rise to the dural tail sign (DTS) on contrast-enhanced magnetic resonance imaging (CEMRI). The presence of DTS does not always qualify for a meningioma, as it is seen in only 60-72% of cases. This sign has been described in various other lesions like lymphomas, metastasis, hemangiopericytomas, schwannomas and very rarely glioblastoma multiforme (GBM). The characteristics of dural-based GBMs are discussed here, as only eleven such cases are reported in the literature till date. Here we discuss the unique features of this rare presentation.

Case Description:A 17-year-old male presented to the emergency department (ED) with, complaints of headache, recurrent vomiting, vision loss in right eye and altered sensorium. On examination patient was drowsy with right hemiparesis, secondary optic atrophy in the right eye and papilledema in the left eye. MRI brain showed, heterogeneous predominantly solid cystic lesion with central hypo-intense core suggestive of necrosis with heterogeneous enhancement and a positive DTS. Patient underwent emergency left parasagittal parieto-occipital craniotomy and gross total tumor excision including the involved dura and the falx. On opening the dura, tumor was surfacing, invading the superior sagittal sinus and the falx, greyish, soft to firm in consistency with central necrosis and highly vascular suggesting a high-grade lesion. Postoperative computed tomography (CT) of the brain showed evidence of gross total tumor (GTR) excision. The postoperative course of the patient was uneventful. Histopathological analysis revealed GBM with PNET like components. The dura as well as the falx were involved by the tumor.

Conclusion:GBMs can arise in typical locations along with DTS mimicking meningiomas. Excision of the involved dura and the falx becomes important in this scenario, so as to achieve GTR. Hence high index of suspicion preoperatively aided by Magnetic Resonance Imaging (MRS) can help distinguish GBMs from meningioma, thereby impacting upon the prognosis.

Keywords: Dural tail sign, glioblastoma multiforme, meningioma, posterior third parasagittal

INTRODUCTION

Dural tail sign (DTS) is considered the hallmark for the radiological diagnosis of a meningioma. It is seen in 60–72% cases of meningiomas and would represent either direct tumor invasion or reactive changes surrounding the tumor itself.[ 1 2 4 6 10 11 12 ] Dural tail has been reported in the literature in nonmeningiomatous pathologies such as lymphomas/chloromas, dural-based metastasis, hemangiopericytomas, schwannoma, chordomas, pleomorphic xantho-astrocytomas, and very rarely glioblastoma multiforme (GBM).[ 3 ] Literature regarding GBMs presenting with dural tail mimicking meningiomas is sparse. Here, we report a rare case of GBM with dural tail mimicking a posterior one-third parasagittal meningioma and review the relevant literature.

CASE REPORT

A 17-year-old male with no comorbidities presented to the emergency department (ED) with complaints of headache and recurrent vomiting for 2 weeks, vision loss in right eye for 1 week, and altered sensorium for 2 days. On examination, the patient was drowsy but arousable, right hemiparesis grade 4/5, right-sided secondary optic atrophy, and left-sided papilledema (pseudofoster Kennedy syndrome). Magnetic resonance imaging (MRI) of the brain showed, T1-weighted images heterogeneous predominantly solid (iso-intense) cystic with central hypo-intense core suggestive of necrosis [ Figure 1 ]. T2-weighted images showed, solid (iso-intense) cystic (hyper-intense) with hyper-intense central core suggestive of necrosis [ Figure 1 ]. On contrast administration, the lesion demonstrated heterogeneous enhancement with central necrosis with a positive DTS [ Figure 2 ].

Figure 1

MRI brain. (a) T1-weighted images: Posterior one-third iso-to-hypointense lesion abutting the falx and the convexity dura. (b) T2-weighted images: iso-to-hyperintense lesion with perilesional edema. (c) MRS showing choline and lipid lactate peak

Figure 2

Contrast-enhanced MRI in all three planes. (a) Axial images exhibiting heterogeneous enhancement with DTS (open arrow). (b) Coronal images: parasagittal location with DTS (open arrow). (c) Sagittal images demonstrating DTS (open arrow)

Patient was taken up for emergency surgery, and left parasagittal parieto-occipital craniotomy fashioned and gross total tumor excision was done. On opening the dura, tumor was seen surfacing and invading the superior sagittal sinus as well as the falx, with infiltration into the adjacent brain parenchyma. Tumor was greyish soft to firm in consistency with central necrosis and highly vascular suggestive of a high-grade lesion. Per-operatively patient had a transient episode of hypotension, which was managed. Approximate blood loss was 2 liters. Postoperative computed tomography scans showed complete tumor removal [ Figure 3 ]. Postoperative recovery was uneventful and patient was discharged in a stable condition. Final biopsy revealed GBM with primitive neuro-ectodermal (PNET) like components.

Figure 3

CT brain. (a) Preoperative contrast CT brain showing heterogeneously enhancing parasagittal lesion. (b) Postoperative plain CT brain showing gross total tumor excision

DISCUSSION

The presence of dural tail sign on MRI is highly suggestive of a meningioma but not a pathognomonic sign. The presence of a dural tail in GBM is very rare, and a thorough review of English literature revealed 10 cases of GBM exhibiting DTS mimicking meningioma. The demographic and clinical data are listed in Table 1 . All except 2 patients including the index case were elderly, suggesting its common occurrence in that age group. Meningiomas are extra-axial tumors, arising from arachnoid cap cells and parasitize on the dural blood supply with subsequent invasion. Approximately 60–72% of meningiomas show classical DTS.[ 2 ] Controversy exists regarding the nature of the dura showing the tail sign, with majority of the published studies claiming it to be reactive changes, whereas few studies have shown it to be due to actual tumoral involvement.[ 1 2 4 6 10 11 12 ] GBMs are intra-axial lesions exhibiting ring-like contrast enhancement with areas of central necrosis and gross perilesional edema. GBMs presenting as extra-axial mass and DTS is unusual, thereby leading to a diagnostic dilemma in the preoperative period. The criteria for the diagnosis of dural tail was given by Aoki et al.[ 1 ] which included:

Table 1

Demographic and clinical features of all the cases reported in literature

a.

Linear enhancement was present along the duramater originating from and extending outward from the tumor margin

All the criteria were fulfilled in our case, thereby confirming DTS leading to the provisional diagnosis of a meningioma.

Wilms et al.[ 11 ] first reported the significance of the DTS in GBMs through histopathological confirmation of the involved dura. None of the five patients with the final biopsy of GBM reported in their series showed invasion of the dural tail by the tumor. Hence, they concluded that the dural tail to be just a reactive change rather than actual infiltration of the tumor. Ten cases of GBMs with a DTS have been reported in the literature till date, except Wilms et al.[ 11 ] none of the other reports included dural biopsy. In the index case reported here, we have histological confirmation of the involvement of both the falx and the dura by the tumor beyond attachment. There were lytic changes on the inner table of the overlying bone, which were drilled away.

Unlike meningiomas, GBMs are highly vascular and aggressive lesions invading normal brain, deriving their blood supply from pial vasculature. The vessels of duramater rarely feed GBMs, the enhanced dural tail sign is likely to develop from vascular congestion or proliferation.[ 3 ] On the other hand, meningiomas derive their blood supply from dural vessels, mostly external carotid circulation (ECA) with few exceptions. Patel et al.[ 8 ] demonstrated tumor blush and ECA supply in both their cases on angiography, and thereby misleading the preoperative diagnosis as meningioma. They subjected both the patients for angio-embolization followed by surgery. Blood loss was less than 500 ml in both their cases. Similarly, in one of the cases reported by Wilms et al.[ 11 ] angiogram was performed demonstrating feeders from middle meningeal artery (MMA) similar to meningiomas [ Table 2 ]. Angiogram was not performed in our case in view of the emergency setting, but the tumor had parasitized the falx and the convexity dura for its nutrition, as noted during the surgery. The massive blood loss encountered during the surgery would have been reduced, by prior angio-embolization, as was the case with Patel et al.,[ 8 ] but for the emergency situation.

Table 2

Imaging, operative, and pathological findings of all the cases reported in the literature

Magnetic resonance spectroscopy (MRS) is a useful adjunct in differentiating preoperatively meningiomas from GBMs. Majos et al.[ 7 ] studied the role of proton MRS in differentiating various tumors and found large lipid/lactate resonance to be characteristic of GBMs and large alanine peaks to be characteristically seen in meningiomas. Heish et al.[ 5 ] reported a case of GBM mimicking meningioma with the classical dural tail, where MRS revealed characteristic lipid/lactate peak strongly suggesting GBM, which was subsequently confirmed on histopathology.

The origin of extra-axial GBMs has been a matter of debate. Various authors have proposed two mechanisms by which these lesions develop. One hypothesis involving GBM of the cranial nerves (CN) states that, GBM arise primarily from the CNS tissue that lay within the proximal parts of the CN itself. CNS tissue may extend well into the CN, and isolated islands of CNS tissue may even be found within the CN at a considerable distance from its exit point. The second hypothesis is that the tumor originated as primary in the heterotopic neuroglial cell nests in the leptomeninges of the adjacent brain.[ 9 ] In the index case, considering the dural and falcine invasion can be possibly explained by the second hypothesis.

CONCLUSION

Lesions arising in typical locations for meningiomas but with atypical appearances, GBM should be considered in the differential diagnosis. MRS is a very valuable method of differentiating GBMs from meningiomas. Preoperative angiography appears to have a role in reducing the blood loss although not performed in the present case. High index of suspicion prior to surgery and excision of the involved dural elements would lead to a better outcome.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1. Aoki S, Sasaki Y, Machida T, Tanioka H. Contrast-enhanced MR images in patients with meningioma: Importance of enhancement of the dura adjacent to the tumor. AJNR Am J Neuroradiol. 1990. 11: 935-8

2. Goldsher D, Litt AW, Pinto RS, Bannon KR, Kricheff II. Dural “tail”associated with meningiomas on Gd-DTPA-enhanced MR images: Characteristics, differential diagnostic value, and possible implications for treatment. Radiology. 1990. 176: 447-50

3. Guermazi A, Lafitte F, Miaux Y, Adem C, Bonneville JF, Chiras J. The dural tail sign beyond meningioma. Clin Radiol. 2005. 60: 171-88

4. Gupta S, Gupta RK, Banerjee D, Gujral RB. Problems with the “dural tail” sign. Neuroradiology. 1993. 35: 541-2

5. Hsieh CT, Liu MY, Tang CT, Sun JM, Tsai WC, Hsia CC. Problem of dural tail sign in glioblastoma multiforme?. Acta Neurol Belg. 2009. 109: 310-3

6. Kayaci S, Şengöz A, Köksal V, Gunver F, Kiliç K. Glioblastoma multiforme mimicking falx meningioma with achondroplasia. Neurosurgery Quarterly. 2014. 24: 53-5

7. Majos C, Alonso J, Aguilera C, Serrallonga M, Perez- Martin J. Proton magnetic resonance spectroscopy ((1) H MRS) of human brain tumours: Assessment of differences between tumour types and its applicability in brain tumour categorization. Eur Radiol. 2003. 13: 582-91

8. Patel M, Nguyen HS, Doan N, Gelsomino M, Shabani S, Mueller W. Glioblastoma Mimicking Meningioma: Report of 2 Cases. World Neurosurg 2016;95:624. e-. 4. p.

9. Reifenberger G, Boström J, Bettag M, Bock WJ, Wechsler W, Kepes J. Primary glioblastoma multiforme of the oculomotor nerve.Case report. J Neurosurg. 1996. 84: 1062-6

10. Tokumaru A, O'uchi T, Eguchi T, Kawamoti S, Kokubo T, Suzuki M. Prominent meningeal enhancement adjacent to meningioma on Gd-DTPA-enhanced MR images: Histopathologic correlation. Radiology. 1990. 175: 431-3

11. Wilms G, Lammens M, Marchal G, Van Calenbergh F, Plets C, Van Fraeyenhoven L. Thickening of dura surrounding meningiomas: MR features. J Comput Assist Tomogr. 1989. 13: 763-8

12. Wu B, Liu W, Zhu H, Feng H, Liu J. Primary glioblastoma of the cerebellopontine angle in adults. J Neurosurg. 2011. 114: 1288-93

Abstract

Background:Exostoses of the internal auditory canal is a rare finding that may present with disabling symptoms of dizziness, hearing loss, and vestibular dysfunction based on the extent of cranial nerve compression. The purpose of this case report is to discuss the presentation and outcomes in a patient who presented with this disorder.

Case Description:A 19-year-old female presented to the neurotologist with left ear discomfort, pain with left lateral gaze, and dizziness. She underwent extensive evaluation including audiometric testing, videonystagmography, and neuroimaging, which confirmed left auditory and vestibular hypofunction and compression of the contents of the internal auditory canal from the exostosis. After extensive counseling, the patient elected to undergo a suboccipital craniectomy to remove the internal auditory canal exostosis. She experienced complete resolution of symptoms.

Conclusions:Exostoses of the internal auditory canal, although rare, can present with severe symptoms of dizziness, hearing loss, and vestibular hypofunction based on the extent of cranial nerve compression. Imaging, particularly with thin-cut computed tomography, is invaluable in making the correct diagnosis. Severe cases can be treated successfully with surgery with minimal or no complications and excellent outcome.

Keywords: Exostoses, exostosis, hearing loss, internal auditory canal, osteoma, vertigo

BACKGROUND

Exostoses of the internal auditory canal are rare, and sometimes seen only on postmortem examinations. Not to be confused with osteomas of the external auditory canal, which are slow- growing tumors that arise from the tympano-squamous suture, exostoses of the internal auditory canal are broad-based elevations found anywhere in the tympanic bone.[ 1 ] They are less common than exostoses of the external auditory canal,[ 5 ] which are readily encountered in standard otolaryngology clinics around the United States. Despite their rarity, when they occur, exostoses of the internal auditory canal can present with severe symptoms related to compression of the cranial nerve seven and eight complex.[ 3 ] Symptoms include decreased hearing, tinnitus, and vertigo, which can be disabling. Thus, the purpose of this report is to describe the clinical presentation, including signs and symptoms, surgical approach, and postsurgical outcome in a patient who was treated at our institution with unilateral exostosis of the internal auditory canal.

CLINICAL PRESENTATION

A 19-year-old female presented to a Neurotologic colleague with a several-month history of discomfort of the left ear that was exacerbated on left lateral gaze. In addition, she experienced dizziness triggered on sitting up and physical activity. Audiometric testing revealed minimal, bilateral low-frequency hearing loss, and videonystagmography demonstrated a 67% reduced vestibular response in the left ear. These symptoms were significantly impacting her quality of life. Past medical history was notable for thoracic outlet syndrome. She was otherwise intact on neurological examination. Thin-cut temporal bone computed tomography (CT) and magnetic resonance imaging (MRI) [Figures 1 and 2 ] revealed an asymmetric narrowing of the left porus acousticus due to a bony overgrowth at the anterior inferior wall of the left internal auditory canal. Semicircular canals, vestibule, cochlea, and ossicles appeared intact. After discussion at the multidisciplinary skull base tumor board, it was decided to schedule her for a suboccipital craniectomy to remove the exostosis on the lower aspect of the internal auditory canal.

Figure 1

Coronal view showing the exostosis in the internal auditory canal. The red circle shows how the left is severely narrowed as compared to the right

Figure 2

Axial view showing the exostosis in the internal auditory canal. The red circle shows the severe narrowing of the left internal auditory canal

Surgery was performed in lateral position, with the left side up [ Video 1 ]. A spinal drain was placed to aid with brain relaxation. A curvilinear incision was made behind the ear for a retromastoid approach. A standard suboccipital craniectomy was fashioned. The cisterna magna was decompressed by allowing cerebrospinal fluid to drain through the spinal drain. The brain was retracted medial and inferior to the seventh and eighth cranial nerve complex. After splitting the arachnoid, the exostoses were visible on the base of the porus acousticus below the anterior inferior cerebellar artery and appeared to be directly compressing the intermediate nerve. At this point, with the assistance of the neurotologic surgeon, the bone was drilled with a series of diamond drills until it was flat. Thereafter, Teflon was laid between the bone and the seventh and eight cranial nerve complexes. Intraoperative monitoring of cranial nerves V, VII, and VIII remained unchanged at the end of the case.

Postoperatively, she was admitted to the neurosurgical intensive care unit where she was neurologically stable. Due to complaints of a low-pressure headache, she underwent blood patch with resolution of her headache. She was seen in follow-up 6 weeks later with neurosurgery and 7 weeks later in the neurotologic surgery clinic. She had mild discomfort around her left ear, but the episodes of dizziness as well as hearing difficulty had resolved. She remains symptom free and neurologically intact to this date.

DISCUSSION

Exostoses of the internal auditory canal are rare. The underlying cause of the exostoses is not understood.[ 2 ] Diagnosis can be delayed and confused with other, more common pathologies seen by ENT and neurosurgery.[ 4 ] Therefore, imaging, particularly with thin-cut CT is fundamental in arriving at a correct diagnosis. Exostoses can often be confused with osteomas of the internal auditory canal. Osteomas, which are slow-growing osseous tumors, typically present as solitary, pedunculated lesions extending into the internal auditory canal.[ 6 ] On the other hand, exostoses present as a smooth-bordered, broad-based growth extending into the canal. Diagnosing either of the two can be aided with a tissue diagnosis obtained during surgery, but is not necessary. Treatment of exostoses of the internal auditory canal depends on the severity of symptoms and the extent of cranial nerve compression. In some mild cases, patients may be observed and their exostoses followed on serial imaging to monitor for development of nerve compression. Therefore, surgical treatment to decompress the exostoses is reserved for severe cases and in patients with neurological deficit. Our patient, with severe symptoms that were negatively impacting her quality of life, elected to proceed with surgery.

CONCLUSION

Exostoses of the internal auditory canal, although rare, can present with disabling and severe symptoms of dizziness, hearing loss, and vestibular dysfunction based on the extent of cranial nerve compression. Imaging, particularly with thin-cut CT, is invaluable in making the correct diagnosis. Severe cases can be successfully treated with surgery with minimal complications and excellent outcome.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1. Baik FM, Nguyen L, Doherty JK, Harris JP, Mafee MF, Nguyen QT. Comparative case series of exostoses and osteomas of the internal auditory canal. Ann Otol Rhinol Laryngol. 2011. 120: 255-60

2. Doan HT, Powell JS. Exostosis of the internal auditory canal. J Laryngol Otol. 1988. 102: 173-5

3. Nguyen LT, Baik FM, Doherty JK, Harris JP, Nguyen QT. Exostoses and osteomas of the internal auditory canal. Laryngoscope. 2010. p. S215-

4. Polat C, Baykara M, Ergen B. Evaluation of internal auditory canal structures in tinnitus of unknown origin. Clin Exp Otorhinolaryngol. 2014. 7: 160-4

5. Turetsky A, Vines F, Clayman A. Surfer's ear: Exostoses of the external auditory canal. Am J Neuroradiol. 1990. 11: 1217-8

6. Wright A, Corbridge R, Bradford R. Osteoma of the internal auditory canal. Br J Neurosurg. 1996. 10: 503-6

Abstract

Background:Post-surgical empty sella is related to the removal of pituitary tumors either from the transcranial or transphenoidal route, rendering diaphragma sellae incompetent at the end of the procedure. This subsequently leads to herniation of the third ventricle and optic apparatus into the empty sella. Studies have shown that in 50% of the cases, individuals with primary and secondary empty sella syndrome have developed defects in the visual fields. Benign increased intracranial pressure, cerebrospinal rhinorrhea, papilledema, and abnormalities affecting visual acuity may also occur as a result of empty sella.

Case Description:This case report discusses a rare treatment option for the symptomatic secondary empty sella syndrome. Patient underwent lumbar drain placement and that resulted in astonishingly significant improvement in vision. Keeping in view the beneficial effect of lumbar drain, lumbar–peritoneal (LP) shunt was inserted which showed drastic improvement in vision.

Conclusion:The surgical outcome of symptomatic cases of ESS is favorable. Various common surgical options were reported in literature; however, we have discussed an unconventional surgical option with an impressive outcome.

Keywords: Empty sella turcica, optic chiasma, pituitary adenoma, visual fields

INTRODUCTION

Post-surgical empty sella is related to the removal of pituitary tumors either from the transcranial or transphenoidal route, rendering diaphragma sellae incompetent at the end of the procedure.[ 1 ] This subsequently leads to herniation of the third ventricle and optic apparatus into the empty sella.[ 1 2 ] Studies have shown that in 50% of the cases, individuals with primary and secondary empty sella syndrome have developed defects in the visual fields.[ 2 ] Benign increased intracranial pressure, cerebrospinal rhinorrhea, papilledema, and abnormalities affecting visual acuity may also occur as a result of empty sella.[ 1 2 ] In this report we have described the case of a patient who developed empty sella syndrome after transsphenoidal surgery. She presented with profound visual impairment. She was treated successfully using a lumbo-peritoneal shunt.

CASE REPORT

A 51-year-old lady was admitted in Aga Khan University Hospital, Karachi, with complaints of headache and blurring of vision. She was diagnosed as having pituitary macroadenoma [ Figure 1a ] 8 months back for which she underwent endoscopic pituitary adenoma resection. The patient had persistent generalized headache that ameliorated on head elevation up to 40°-45° and got worse in the morning. It was associated with phonophobia but not associated with nausea or vomiting. She had bitemporal hemianopia with decreased visual acuity in both eyes. The symptoms got worsened following surgery. She also developed increased urinary frequency post-operatively. Other than pituitary macroadenoma resection, the patient underwent partial thyroidectomy for a solitary nodule in 2000 (euthyroid), and hysterectomy in 2001 due to dysfunctional uterine bleeding. She had a medical history of hepatitis C treatment with interferon 10 years back. Currently, she is on selective serotonin reuptake inhibitor (SSRI), desmopressin nasal spray, and certain analgesics. An examination showed significant bitemporal hemianopia without papilloedema. Higher mental functions and motor and sensory examination were unremarkable. Cranial nerves were all intact. Magnetic resonance imaging (MRI) of the brain showed evident empty sella turcica with downward herniation of optic chiasm [Figure 1b – d ]. The patient was planned for lumbar drain insertion to lower down intracranial pressure (ICP) which would help in ameliorating downward herniation of optic chiasm. The opening pressure of more than 35 cm of H₂O was measured. The outcome of the intervention was noticed soon after the surgery with transient improvement in vision. For long-term outcome, lumbar–peritoneal (LP) shunt was placed after a period of 1 month which resulted in both subjective and objective improvement in visual acuity [Figures 2 and 3 ]. Inclusively, associated diabetes insipidus got better

Figure 1

(a) Presurgical contrast enhanced T1 Sagittal MR image of the pituitary gland showing macroadenoma. Post surgical images (b) Sagittal T2 (c) Coronal T2 and (d) Post contrast T1 weighted Coronal images of the pituitary gland showing residual gland and evident empty sella turcica with downward herniation of optic chiasm and suprasellar structures

Figure 2

(a and b) Right and left eye visual field before LP shunt insertion (c and d) Right and left eye Visual field after LP shunt insertion. Visual field testing results obtained showed dramatic improvement of the preprocedural visual field defects

Figure 3

(a and b) T1 and T2 weight MR images from the sellar region following Lumbar-Peritoneal shunt procedure shows a reversal of chiasmatic herniation and reduction in the degree of empty sella turcica

DISCUSSION

This case report highlights a rare treatment option for the symptomatic secondary empty sella syndrome resulting from post-surgical pituitary resection. Various studies have mentioned ways to prevent downward herniation of optic chiasma that have been resulting in visual defects in subjects. Around 50% of patients show visual field defects associated with both primary and secondary empty sella syndromes.[ 1 ] Visual alterations may be due to traction on the chiasm or involvement of chiasmal blood vessels. In secondary empty sella syndrome, the incidence is much higher because of the underlying sellar pathology. Clinically, the patients complain about clouding of vision, color vision defects, photophobia, and various visual field defects (bitemporal hemi- or quadrantanopia, generalized field constriction, quadrine constriction, central scotoma, homonymous hemiachromatopsia mimicking the lesion in patients with a suprasellar pituitary tumor).[ 2 ] Secondary empty sella has been noted to follow the below:

Sellar or parasellar surgery

Radiation-induced vascular changes or strangulation of the optic nerves or chiasm are thought to compromise local blood flow. Downward herniation of the optic pathways is present in the majority of cases.[ 5 ] Several materials have been suggested for filling the sellar space and reconstruction of the sellar floor. They include bioabsorbable materials, muscle, fat, dural substitutes, cartilage, bone fragments, ceramic substances, titanium plates, and others.[ 3 4 ] The surgical process of propping up the optic chiasm is called chiasmopexy, which includes inserting muscle, cartilage, or silicone sponge under optic chiasm, packing the sella with fat, muscle, or cartilage to elevate the pituitary gland, pituitary stalk, and chiasm and transsphenoidal placement of a detachable balloon.[ 6 ] Lumbar drain insertion has a mechanism of reducing the cerebrospinal fluid (CSF) pressure that may build up and contribute in downward optic chaisma herniation. The ultimate possible improvement in symptoms of a patient with secondary empty sella syndrome is placing ventriculoperitoneal shunt for long-term beneficial effects.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1. Fouad W. Review of empty sella syndrome and its surgical management. Alexandria J Med. 2011. 47: 139-47

2. Guinto G, del Valle R, Nishimura E, Mercado M, Nettel B, Salazar F. Primary empty sella syndrome: The role of visual system herniation. Surg Neurol. 2002. 58: 42-7

3. Kaptain GJ, Vincent DA, Laws ER. Cranial base reconstruction after transsphenoidal surgery with bioabsorbable implants: Technical note. Neurosurgery. 2001. 48: 232-4

4. Seiler RW, Mariani L. Sellar reconstruction with resorbable Vicryl patches, gelatin foam, and fibrin glue in transsphenoidal surgery: A 10-year experience with 376 patients. J Neurosurg. 2000. 93: 762-5

5. Thomé C, Zevgaridis D. Delayed visual deterioration after pituitary surgery-a review introducing the concept of vascular compression of the optic pathways. Acta Neurochir (Wien). 2004. 146: 1131-

6. Cybulski GR, Stone JL, Geremia G, Anson J. Intrasellar balloon inflation for treatment of symptomatic empty sella syndrome. Neurosurgery. 1989. 24: 105-9

Abstract

Background:Cerebral hemangioblastomas are benign tumors with abundant blood flow that occur mainly in the posterior fossa. Tumor removal en bloc is important in surgical treatment because of the risk of bleeding; however, it is actually rather difficult in practice. Therefore, we propose a surgical strategy for visualizing hypervascular tumors of the posterior fossa utilizing indocyanine green (ICG).

Case Description:Case 1 involved a 48-year-old male with a history of von Hippel–Lindau (VHL) disease. Magnetic resonance imaging (MRI) revealed a solid tumor measuring 3.0 cm in diameter in the right cerebellopontine angle. We performed surgery because the tumor was pressing against the brainstem. Surgery was performed via the posterior subtemporal transtentorial approach in order to visualize the feeding artery and draining vein intraoperatively. The vessels were confirmed by ICG and the tumor was removed en bloc. Case 2 involved a 30-year-old woman. Signs of increased intracranial pressure were noted, and an MRI revealed a solid tumor 3.5 cm in diameter in the left cerebellar hemisphere. Surgery was performed via the midline suboccipital approach. Similarly, we confirmed the vessels using ICG and the tumor was removed en bloc.

Conclusions:For hypervascular tumors of the posterior fossa, preoperative image assessment is important. Furthermore, the use of ICG during surgery is advantageous for surgical strategies where the feeding arteries and draining veins exist superficially in the operative field and are therefore easier to remove en bloc.

Keywords: Hemangioblastoma, hypervascular, indocyanine green, surgical videoangiography

INTRODUCTION

In high vascular tumors of the posterior fossa, surgical resection can be extremely challenging. Hemangioblastomas are common hypervascular tumors that occur in the posterior fossa. The prevalence of hemangioblastoma has been estimated to be approximately 1.5–2.5% of all intracranial tumors and 7–8% of posterior fossa tumors.[ 1 8 9 ] Most lesions arise sporadically, although nearly one-third of patients have been diagnosed with von Hippel–Lindau (VHL) disease.[ 3 ] Hemangioblastomas can be either predominantly cystic or solid tumors. These tumors are known to be highly vascularized with corresponding histological features of stromal cells strongly expressing vascular endothelial growth factor and abundant vascular cells.[ 12 ] Treatment by surgical intervention may be necessary in certain cases, and removal of the tumors en bloc is important during surgery in order to minimize blood loss. However, because hemangioblastomas are frail and hypervascular, surgery carries a substantial risk for bleeding and resection can be difficult.[ 19 ] Accordingly, tumor embolization before surgery has been considered to control bleeding during surgery, but there are a considerable number of reports detailing complications, such as brain swelling and/or intratumoral hemorrhage after embolism.[ 4 6 ] Hence, the use of presurgical embolization has not been established as a standard practice.[ 4 6 ] As another surgical strategy, several studies have reported on the use of indocyanine green (ICG) during surgery for tumor removal.[ 7 8 10 17 19 20 21 ] However, ICG videoangiography is limited in that only tissue in the operative field is visible. When a deep-seated tumor is approached through a long, narrow corridor, it is not easy to clearly visualize tumor-related vessels with ICG videoangiography, and consequently, the technique may not be useful in the instance.[ 10 ] For tumors of the posterior fossa, the surgical field is often deeper. Therefore, we report two cases in which the assessment of preoperative imaging with ICG could be advantageous from a surgical strategic viewpoint, where the feeding artery and draining vein exist superficially.

CASE HISTORY

Case 1

A 48-year-old male patient with a family history of VHL disease consulted an ophthalmologist because he had experienced blurred vision for 2 months prior to consultation. A diagnosis of bilateral retinal angioblastoma was made. Subsequently, magnetic resonance imaging (MRI) brain was performed to assess the patient for VHL. MRI showed a predominantly solid tumor with a maximum diameter of 3.0 cm accompanying an internal cyst close to the right cerebellopontine angle. In addition, edema extending to the brainstem was noted. The solid portion of the lesion was uniformly contrast-enhancing according to an MRI. Subsequent cerebral angiography identified feeding arteries from the superior cerebellar artery (SCA) and draining veins that converged onto the tentorial sinus [ Figure 1 ]. The patient also exhibited deficits in working memory. We performed surgery because the tumor was compressing the posterior lobe of cerebellum and there was a decline in cognitive function.

Figure 1

The presurgical magnetic resonance (MR) image depicting an enhanced solid haemangioblastoma in the cerebellopontine angle (a). Postsurgical MR image depicting the resection of the tumour (b). Right vertebral artery angiogram exhibiting a hypervascular tumour near the superior cerebellar artery (c and d)

Surgery was performed via the posterior subtemporal transtentorial approach with the patient in the left supine lateral position. We intravenously injected mannitol and inserted a ventricular drainage tube into the supramarginal gyrus due to brain relaxation. After opening the dura mater, a surgical corridor was created by dissecting the bridging vein, and the tumor was confirmed by the retraction of the temporal lobe. The tumor was detached from the quadrigeminal bodies, followed by ICG fluorescence vascular angiography. First, the feeding arteries from the SCA were visualized, then the draining veins as they were converging with the tentorial sinus, and finally the transit arteries were carefully identified [ Figure 2 ]. After careful assessment of the vasculature, the feeding arteries from the SCA were coagulated and cut. Finally, the draining veins were taken and the tumor was removed en bloc. The quantity of bleeding was approximately 640 ml. The pathological examination confirmed a hemangioblastoma World Health Organization (WHO) I.

Figure 2

Surgical view (a) and indocyanine green (ICG) videography (b-d) during an operation. The main feeding arteries (arrow) are first filled with ICG (b), followed by transit feeders (open arrowhead), and then by simultaneous filling of drainers (open arrow) approximately 4 seconds after the main feeding arteries are visualized (c). Finally, non-feeding arteries (arrowhead) are filled with ICG approximately 10 seconds after the main feeding arteries (d)

Case 2

A 30-year-old woman patient visited our outpatient clinic after experiencing headache and blurred vision for 1 month. Her neurological examination was within the normal limits, except that bilateral congested papillae were identified. Her family history was unavailable because she was adopted. MRI revealed a solid tumor with a maximum diameter of 3.5 cm accompanying an internal cyst in the left cerebellar hemisphere. In addition, extensive cerebral edema and ventricular dilation were noted. On presurgical brain MRI, the solid region showed uniform contrast enhancement. On cerebral angiography, feeding arteries were noted to arise from the left anterior inferior cerebellar artery (AICA) and left posterior inferior cerebellar artery (PICA), and both the draining veins to the petrosal vein and the inferior vermian vein were identified [ Figure 3 ].

Figure 3

The presurgical magnetic resonance (MR) image demonstrating the enhanced solid haemangioblastoma in the cerebellum (a). The postsurgical MR image demonstrating the resection of the tumour (b). A Three-dimensional computed tomography angiogram during the late phase, exhibiting a draining vein (open arrowhead) flowing into the petrosal vein on the ventral side of the tumour (c). Left vertebral artery angiogram exhibiting a hypervascular tumour near the anterior inferior cerebellar artery and the posterior inferior cerebellar artery (d and e)

We performed 200 ml of 10% glycerol (twice a day) with dexamethasone (8 mg/day) for 6 days before the operation. Because the symptoms improved, we did not perform external ventricular drainage prior to the operation. Surgery was performed via the midline suboccipital approach with the patient in the prone position. The dura mater was dissected and ICG fluorescence angiography was performed. We observed the feeding artery coming off the PICA arose from the cerebellar hemisphere and drained via the horizontal fissure, additionally feeding vessels from the AICA were confirmed on the outside. The lesional veins drained into the inferior vermian vein [ Figure 4 ]. Intraoperatively, the feeding artery from the PICA was coagulated first and then cut. Because there was a clear cleavage plane from the surrounding tissue, which was not swollen, detachment was performed, leaving the thick draining veins intact. The most significant draining veins, which converged onto the petrosal vein, were positioned anteriorly on the outside. Finally, these veins were coagulated and cut, and the tumor was removed en bloc. The estimated blood loss was 500 ml. Pathological examination of the specimen confirmed hemangioblastoma WHO I.

Figure 4

Surgical view (a) and indocyanine green (ICG) videography (b-e) during the operation. The main feeding arteries (arrows) are first filled with ICG (b and c), followed by transit feeders (open arrowhead) and the simultaneous filling of drainers (open arrow) approximately 8 seconds after the main feeding arteries are visualized (d). Finally, the non-feeding arteries (arrowhead) are filled with ICG approximately 15 seconds after the main feeding arteries are visualized (e)

DISCUSSION

Hemangioblastomas are benign tumors (WHO I) comprising neoplastic stromal cells and abundant small vessels.[ 12 ] Surgical resection is the first line treatment, which is indicated in (a) symptomatic lesions, (b) urgency before symptoms develop, (c) lesions that are progressively enlarging on sequential scans, and (d) lesions that can or should not be treated with other modalities such as stereotactic radiosurgery.[ 2 8 13 ] In the first case, we performed surgery because the tumor was compressing the posterior lobe of cerebellum, causing a decline in cognitive function.[ 15 16 ] In the second case, surgery was performed because the patient exhibited signs of intracranial hypertension, such as headache and nausea. Hemangioblastomas have abundant blood flow comparable to arteriovenous malformations. The lesions are frail, and it can be difficult to control bleeding if removed in a piecemeal fashion, which also carries the risk of leaving some tumor behind,[ 5 ] posing a high probability of recurrence. It has been widely advocated that these tumors must be removed en bloc.[ 5 8 9 ]

In recent years, there have been several reports of presurgical embolization being performed in order to control bleeding during hemangioblastoma surgeries.[ 3 4 5 6 9 11 14 18 ] However, serious complications such as stroke, hemorrhage, and swelling have also been reported,[ 4 6 ] and therefore the procedure's utility has not been established.

Liu et al. compared the outcomes of groups in which presurgical embolization was or was not performed for cerebellar hemispheric hemangioblastomas. The authors reported that the duration of surgery was shortened, and the amount of blood loss and subsequent need for transfused blood were decreased in the embolization group.[ 11 ] There are other studies reporting that fewer complications arose in cases where n-butyl-cyanoacrylate (NBCA) was used as the embolization material.[ 9 14 ] However, this can result in recanalization after embolization because a tumor can develop collateral circulation from other vessels that embolized.[ 11 ] Particulate embolizing materials such as polyvinyl alcohol particles and embospheres are also widely used.[ 4 6 ] However, there are a significant number of reports in which bleeding complications or swelling in the brain occurred after embolization with particular embolizing substances.[ 4 6 ] There are other studies contesting the usefulness of presurgical embolization. According to Ampie et al., there are no significant differences in total tumor removal rate or the quantity of bleeding during surgery between embolized and nonembolized patients, but there is a high rate of complication associated with the embolism procedure itself.[ 3 ] In addition, Takeuchi et al. reported swelling in the brain or cerebral hemorrhaging occurring in 70% of cases after partial embolization, rather than decreasing the risk of complications during surgery.[ 18 ] Given that there are usually many feeding vessels in posterior fossa hemangioblastomas, complete embolization can be difficult and therefore, considerable care must be taken in order to avoid strokes.[ 18 ]

Hemangioblastomas are characterized by abundant blood flow and it has been strongly advocated that they should be removed en bloc.[ 5 8 9 ] Although evaluation of the respective vasculature is necessary before surgery in order to identify the anatomy of the relevant blood vessels, surgery can be made even safer if the blood vessels are visualized during surgery.

Hitherto, ICG has been employed routinely during surgery for brain aneurysms, cerebral arterial malformations, and bypass surgeries.[ 7 17 19 20 21 ] Beyond that, the use of ICG for the identification of blood vessels in hemangioblastoma has previously been reported, with favorable results.[ 7 8 10 17 19 20 21 ] According to Hojo et al., ICG can detect specific blood vessels via differences in the filling pattern.[ 8 ] First, ICG fills the main feeding arteries and then transit arteries simultaneously with the draining veins. Finally, ICG fills the adjacent nonfeeding arteries.[ 8 ]

However, the use of ICG also has its drawbacks. Deep blood vessels, covered by the cerebral parenchyma, cannot be visualized.[ 7 ] In fact, there are usually many feeding vessels and they may be deep within posterior fossa hemangioblastomas.[ 18 ] However, it can be considered that ICG could be useful in planning the surgical approach when using comprehensive assessment by presurgical angiography or three-dimensional visualization.[ 22 ] In Case 1, we considered a suboccipital infratentorial approach, an occipital transtentorial approach, or a subtemporal transtentorial approach as alternative methods of approach. Both the suboccipital infratentorial approach and occipital transtentorial approach required excessive traction of the cerebellum and occipital lobe, and it was considered difficult to identify both the SCA and trochlear nerve in front of the tumor. However, the subtemporal transtentorial approach does not require excessive traction of temporal lobe, and it was possible to identify both the SCA and trochlear nerve in front of the tumor. In fact, we used ICG to confirm both the feeding artery and draining vein; therefore, the tumor was able to be removed en bloc. In Case 2, because we used ICG to discover that the feeding arteries and the draining vein to the inferior vermian vein were on the surface, the surgery was able to be performed using the midline suboccipital approach. In this case as well, ICG was useful in making it possible to both control bleeding and remove the tumor en bloc, while preserving the normal blood vessels. However, it was impossible to identify the deep draining vein flowing into the petrosal vein during the initial administration of ICG, but it was assumed from the angiographic evaluation prior to surgery that the draining vein was in the outer basal region of the tumor.

We did not perform an embolization in either of these cases owing to concerns of the possibility of complications. Such complications could have been fatal (e.g., in Case #1) because of the proximity of the tumors to the brainstem. In such cases, we demonstrated that there is a way to use ICG without embolization. It seems that ICG can be successfully utilized by examining the surgical strategy with preoperative images.

CONCLUSION

For hypervascular tumors of the posterior fossa, preoperative image assessment is important. Furthermore, the use of ICG during surgery can be advantageous when the feeding arteries and draining veins exist superficially in the operative field and as a result, making tumor removal en bloc safer.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1. Amano T, Tokunaga S, Shono T, Mizoguchi M, Matsumoto K, Yoshida T. Cerebellar hemangioblastoma manifesting as hearing disturbance. Neurol Med Chir (Tokyo). 2009. 49: 418-20

2. Ammerman JM, Lonser RR, Dambrosia J, Butman JA, Oldfield EH. Long-term natural history of hemangioblastomas in patients with von Hippel-Lindau disease: Implications for treatment. J Neurosurg. 2006. 105: 248-55

3. Ampie L, Choy W, Lamano JB, Kesavabhotla K, Kaur R, Parsa AT. Safety and outcomes of preoperative embolization of intracranial hemangioblastomas: A systematic review. Clin Neurol Neurosurg. 2016. 150: 143-51

4. Cornelius JF, Saint-Maurice JP, Bresson D, George B, Houdart E. Hemorrhage after particle embolization of hemangioblastomas: Comparison of outcomes in spinal and cerebellar lesions.J. Neurosurg. 2007. 106: 994-8

5. Cui H, Zou J, Bao YH, Wang MS, Wang Y. Surgical treatment of solid hemangioblastomas of the posterior fossa: A report of 28 cases. Oncol Lett. 2017. 13: 1125-30

6. Eskridge JM, McAuliffe W, Harris B, Kim DK, Scott J, Winn HR. Preoperative endovascular embolization of craniospinal hemangioblastomas. AJNR Am J Neuroradiol. 1996. 17: 525-31

7. Hao S, Li D, Ma G, Yang J, Wang G. Application of intraoperative indocyanine green videoangiography for resection of spinal cord hemangioblastoma: Advantages and limitations. J Clin Neurosci. 2013. 20: 1269-75

8. Hojo M, Arakawa Y, Funaki T, Yoshida K, Kikuchi T, Takagi Y. Usefulness of tumour blood flow imaging by intraoperative indocyanine green videoangiography in hemangioblastoma surgery. World Neurosurg. 2014. 82: E498-E501

9. Katayama S, Kidoguchi K, Takeda N. Preoperative embolization of solid cerebellar hemangioblastomas using n-butyl-cyanoacrylate. JNET. 2013. 7: 286-93

10. Kim EH, Cho JM, Chang JH, Kim SH, Lee KS. Application of intraoperative indocyanine green videoangiography to brain tumour surgery. Acta Neurochir. 2011. 153: 1487-95

11. Liu AH, Peng TM, Wu Z, Xiao XR, Jiang CH, Wu ZX. Clinical effectiveness of preoperative embolization for cerebella hemangioblastoma. Asian Pac J Cancer Prev. 2013. 14: 5179-83

12. Louis DN, Ohaki H, Wiestler OD, Cavenee WK.editorsWHO classification of tumours of the central nervous system. Lyon: IART; 2016. p.

13. Nakamura H, Kuratsu J, Shuuin T. Craniospinal hemangioblastoma associated with Von Hippel-Lindau disease review. Jpn J Neurosurg (Tokyo). 2013. 22: 52-60

14. Sakamoto N, Ishikawa E, Nakai Y, Akutsu H, Yamamoto T, Nakai K. Preoperative endovascular embolization for hemangioblastoma in the posterior fossa. Neurol Med Chir. 2012. 52: 878-84

15. Schmahmann JD, Sherman JC. The cerebellar cognitive affective syndrome. Brain. 1998. 121: 561-79

16. Shiroma A, Nishimura M, Nagamine H, Miyagi T, Hokama Y, Watanabe T. Cerebellar contribution to pattern separation of human hippocampal memory circuits. Cerebellum. 2016. 15: 645-62

17. Takeshima Y, Tanaka Y, Hironaka Y, Shida Y, Nakase H. Visualization of vascular structure of spinal hemangioblastoma using intraoperative indocyanine green videoangiography and temporary feeder occlusion. Eur Spine J. 2015. 24: S585-9

18. Takeuchi S, Tanaka R, Fujii Y, Abe H, Ito Y. Surgical treatment of hemangioblastomas with presurgical endovascular embolization. Neuro Med Chir. 2001. 41: 246-52

19. Tamura Y, Hirota Y, Miyata S, Yamada Y, Adam T, Kuroiwa T. The use of intraoperative near-infrared indocyanine green videoangiography in the microscopic resection of hemangioblastomas. Acta Neurochir. 2012. 154: 1407-14

20. Ueba T, Abe H, Matsumoto J, Higashi T, Inoue T. Efficacy of indocyanine green videography and real-time evaluation by FLOW 800 in the resection of a spinal cord hemangioblastoma in a child. J Neurosurg Pediatrics. 2012. 9: 428-31

21. Ueba T. Application of intraoperative indocyanine green videography to brain tumour surgery. Jpn J Neurosurg. 2014. 23: 871-5

22. Yoshino M, Nakatomi H, Matsumoto J, Kin T, Saito T, Shono N. Usefulness of high-resolution 3D multifusion medical imaging for preoperative planning in patients with posterior fossa hemangioblastoma: Technical note. J Neurosurg. 2017. 127: 139-47

Abstract

Background:Ependymomas are relatively uncommon tumors that constitute about 7% of all primary intracranial neoplasms. Among these, high-grade ependymomas are locally aggressive and recur most commonly at the primary site following resection. Ependymomas are also known to be the one glial neoplasm that tends to frequently metastasize inside and outside the central nervous system (CNS) that complicates workup and management. Metastasis due to surgical manipulation is common and neurosurgeons should be well-versed in the most effective methods to remove these tumors in order to avoid such metastases.

Case Description:Here, we report a case of a 28-year-old female who initially presented with a parenchymal World Health Organization (WHO) grade III anaplastic ependymoma of the occipital lobe without metastasis. After multiple resections, the patient showed no evidence of disease recurrence for 2 years. During follow-up, new metastasis to the frontal lobe as well as to the lung were discovered 2 years after the initial surgery, without recurrence at the tumor's primary site.

Conclusions:While uncommon, this case demonstrates the possibility for ependymomas to metastasize via cerebrospinal fluid to other locations within the CNS and hematologically to extraneural locations without recurring locally.

Keywords: Cerebrospinal fluid metastasis, ependymoma, hematologic metastasis

INTRODUCTION

Ependymomas are primary glial cell tumors that arise from ependymal cells lining the ventricular system.[ 1 ] These neoplasms constitute 6.8% of all primary intracranial neoplasms; in adults, these tumors make up 3 9 19 ] In adults, ependymomas are found most commonly in the spinal cord (46%), infratentorial (35%), and supratentorial (19%) locations within the posterior fossa.[ 26 ] The World Health Organization (WHO) grade classification system outlines three grades of this disease—with the most malignant subtype being grade III, anaplastic ependymoma.[ 10 15 ] These grade III neoplasms are locally aggressive and recur most commonly at the primary site following resection.[ 29 31 ]

At present, the primary therapeutic intervention for intracranial ependymomas is surgery.[ 9 ] However, due to a high rate of local recurrence, many patients require adjuvant radiotherapy, chemotherapy, or surgical re-resection.[ 14 24 ] Ependymomas, like other gliomas, have been shown to also metastasize to extracranial sites, albeit at a very low rate; however, the incidence is difficult to calculate since this is a rare event.[ 1 ]

The majority of ependymomas are not located in the parenchyma but located within the cerebrospinal fluid (CSF) pathways and have a relatively high rate of metastasis compared to other brain tumors. Workup for these neoplasms, therefore, includes imaging of the entire cranio-spinal axis and between 8 and 20% of high-grade ependymomas demonstrate CSF spread at presentation.[ 32 ] High-grade, infratentorial ependymomas occurring in children are most likely to be associated with CSF seeding.[ 20 ] Due to CSF dynamics, the spinal cord is the most likely location for tumor seeding, though a very few case reports have demonstrated spinal cord and/or infratentorial to supratentorial spread as well.[ 2 ]

Here, we report a case of a 28-year-old female who initially presented with a WHO grade III anaplastic ependymoma of the occipital lobe without any signs of metastasis. After multiple resections, which were required for the index site, the patient showed no evidence of disease recurrence in the CNS for 2 years. At the 2-year follow-up appointment, new metastasis to the frontal lobe and lung were discovered in the absence of any recurrence at the tumor's primary site.

The case presented is of particular interest for multiple reasons. First, it is highly uncommon for an ependymoma to metastasize in retrograde to the primary lesion. Second, concurrent brain and lung metastases suggest sequential CSF and hematologic spread, which, to our knowledge, has yet to be reported within the literature.

CASE REPORT

A 28-year-old female presented to an outside hospital in 2007 with fainting spells that were suspicious for seizures by family's report. A magnetic resonance imaging (MRI) was obtained and revealed a right occipital tumor. The patient underwent a right occipital craniotomy and image-guided resection of the lesion. It was noted that there were three areas which were concerning for tumor invasion of the surrounding parenchyma due to its general discoloration. Frozen biopsies were sent for evaluation. The pathology report came back negative for marginal tumor infiltration. The patient was incorrectly told that the tumor was meningioma due to an incorrect preliminary read and that no further treatment was necessary; however, the final pathological diagnosis revealed a WHO grade III anaplastic ependymoma. Unfortunately, the revised diagnosis was not made known to the patient, nor her future physicians. She did not undergo chemotherapy or radiation at that time.

In 2012, the patient presented to our hospital with severe (8/10) headaches, nausea, and photophobia. Computed tomography (CT) and MRI of the brain did not show any acute pathology or evidence of tumor recurrence. The patient was given a diagnosis of migraine headaches and treated medically.

The patient re-presented in 2014 with relapse of her headaches and fainting spells. An MRI of the brain was obtained, revealing a recurrent 4.1 cm enhancing mass in the right occipital lobe with surrounding edema (without evidence of drop metastasis or other enhancing lesions on spinal MRI [ Figure 1 ]). In light of the rather rapid interval growth of the lesion, despite no new onset focal deficits, and concern for malignant transformation to a WHO grade IV glioma, the neurosurgical team recommended re-resection of the tumor. The patient underwent subsequently re-do craniotomy, and did well clinically postoperatively as she remained neurologically intact and showed improvement in her headaches. The postoperative MRI demonstrated a gross total resection and she subsequently underwent adjuvant radiotherapy.

Figure 1

Preoperative axial MR images showing an enhancing mass in the occipital lobe with isointensity on T1-weighted images (a), hyper intensity on a gadolinium enhanced T1-weighted image (b), and hyper intensity on T2-weighted image (c). Postoperative axial gadolinium enhanced T1-weighted image demonstrating complete resection of the mass with no regions of hyper intensity (d)

Two years after her second resection, the patient presented to her primary care physician with a cough lasting for several weeks. A chest X-ray was performed, which revealed multiple, bilateral pulmonary nodules. The largest lesion was seen in the left lower lobe, measuring 4.5 × 3.4 × 3.9 cm [ Figure 2 ]. The second largest lesion was in the left upper lobe, in close proximity to the major fissure. Additional pleural-based masses were seen. A CT-guided biopsy of the mass was performed, confirming the diagnosis of grade III anaplastic ependymoma.

Figure 2

Imaging of multiple pulmonary nodules in the left lower and upper lobe seen on lateral (a) and anterior posterior (b), X-ray views, as well as on sagittal (c) and axial (d) CT imaging

An MRI of the brain was obtained at the same time, which revealed the interval development of several new right-sided intracranial frontal extraaxial masses side, but without any evidence of tumor recurrence in the right occipital lobe tumor resection bed [Figure 3a – d ]. The largest mass measured at 4.7 × 4.4 × 3.0 cm and was located along the right temporal–frontal convexity. Imaging displayed heterogeneous hyper-intense T2- and hypo-intense T1-signal characteristics and some foci of restricted diffusion. There was diffuse heterogeneous enhancement on postcontrast imaging. The lesion exerted significant mass effect upon the adjacent right temporal lobe gyri, uncus, and cerebral peduncle and a second mass (measuring 2.1 × 2.1 × 1.7 cm) and was located along the right posterior frontal convexity, also causing mass effect. Two additional masses were detected immediately anterior to the second largest mass along the right posterior frontal lobe, measuring 1.3 cm each in the greatest dimension. There was no evidence of recurrent disease at the initial occipital site of presentation, nor evidence of drop metastasis or other enhancing lesions on spinal MRI [Figure 3e , f ].

Figure 3

Two year post operative MR axial images demonstrating T1 weighted a non enhancing fronto-temporal mass (a), with hyperintensity following gadolinium enhancement on T1-weighted axial (b), sagittal (c) and coronal views (d). Images demonstrating the absence of drop metastases or other enhancing lesions T1-weighted gadolinium enhanced images of thoracic (e), and lumbar (f) spinal cord

The patient once again underwent respective surgery and all cranial tumors were removed successfully as seen on postoperative imaging [ Figure 4 ].

Figure 4

Post-operative MR images showing complete resection of the tumor and the absence of hyper intense lesions near the site of tumor excision on axial gadolinium enhanced T1-weighted axial (Left), and coronal images (Right)

The patient is currently under close follow-up, with adjuvant radiotherapy and chemotherapy for her lung metastases without evidence of intracranial recurrence 12 months following her most recent resection seen on imaging.

DISCUSSION

Ependymomas are primary tumors of the CNS that primarily affect children and young adults.[ 11 28 ] Traditionally, ependymomas were thought to originate from the lining of the ventricles, and believed to be of glial cell origin.[ 4 ] Management of ependymomas is largely limited to surgical resection and radiation therapy, with chemotherapy playing a minimal role.[ 13 18 23 25 ] Because of their locally invasive nature and their tendency to recur as well as the fact that the prognosis is correlated to the extent of tumor resection, aggressive gross total resection is the preferred treatment. Following appropriate treatment, adult 5-year overall survival ranges between 60 and 90%.[ 17 30 ] However, patients with aggressive recurrence and/or metastasis have worse prognoses. Unfortunately, the rarity of these conditions makes it difficult to establish an accurate survival rates.[ 2 ]

The scenario of intracranial tumors showing metastasis to an extraneural location has been intensely studied, but the mechanism of this spread of disease is not yet fully understood.[ 2 16 27 ] A clear prerequisite seems to be tumor access to extracranial soft tissue, blood, or CSF. Several hypotheses of the underlying mechanisms have been brought up and include: direct tumor invasion of the dural sinuses, tumor access to local lymphatic vessels, spread to adjacent extracranial tissue, and, most commonly, CSF seeding via the ventricular system leading to drop or spinal metastases.[ 22 ]

Extracranial ependymoma metastases are exceedingly rare, and are most often found at the time of primary tumor recurrence.[ 4 5 12 ] Ependymomas, like all primary brain tumors, have low potential for spread outside the CNS due to the blood–brain barrier, matrix proteins, and microglia.[ 1 ] However, metastases of anaplastic ependymomas have been observed in the lungs, liver, and cervical lymph nodes, suggesting the possibility of both hematologic and lymphatic spread.[ 8 21 ]

Direct surgical manipulation of the tumor and CSF-shunt placement are potent avenues for intra- and extraneural metastasis formation in these cases. The physical disruption of the blood–brain barrier during surgery, combined with the dislodging of malignant cells into local structures, following direct tumor manipulation, is likely to play a role in the formation of about 8.5% of extracranial metastases.[ 1 7 21 ] Studies of venous blood samples during and after surgery have also demonstrated a significant presence of brain tumor cells which gained access to the systemic circulation.[ 7 ] Additionally, direct seeding to the extraneural space may occur in setting to any placement of ventricular shunts and is associated with about 27.3% of extraneural metastases observed in cases of other primary CNS tumors.[ 4 ]

High-grade ependymomas are locally aggressive and recur most commonly at the primary site following resection. They are also known to be the glial tumor most prone to metastasis. Malignant cells have been identified within the CSF, drawn in the typical fashion of three vials of 2 cc. On average, 16% of patients with known ependymomas.[ 17 ] We speculate that it is through CSF dissemination that our patient's occipital lobe tumor reoccurred in the frontal/temporal region without recurrence at the primary site. Furthermore, this leptomeningeal spread occurred most likely after tumor cells were introduced into the subarachnoid space at the time of surgery.

Pulmonary metastases, on the contrary, are likely the result of hematogenous spread via tumor cells that gained access to the circulation during surgery. Such CSF seeding to extracranial sites is possible via mechanical disruption of the blood–brain barrier at the time of surgery allowing tumor cell to reach the venous system, which then permits hematogenous spread to the lung. Similarly, it is possible that the intracranial tumor could have spontaneously invaded either vascular or lymphatic structures, leading to seeding of tumor cells to the lungs.

While uncommon, and not extensively written about in the literature, this case demonstrates the possibility for ependymomas to simultaneously metastasize both through CSF to other CNS locations and hematologically to extraneural locations. Such pathways should be considered when routinely monitoring patients for tumor growth or recurrence.

CONCLUSION

Ependymomas rarely metastasize without local recurrence. To the best of our knowledge, our case is the first report on anaplastic ependymoma with concurrent CSF and hematologic metastasis, occurring without recurrence at the primary site. This unusual pattern of neoplasm recurrence prompts a discussion of the pathogenesis of extraneural metastasis and highlights a possible risk of surgical intervention.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1. Alzahrani A, Alassiri A, Kashgari A, Alrehaili J, Alshaalan H3, Zakzouk R4. Extraneural metastasis of an ependymoma: A rare occurrence. Neuroradiol J. 2014. 27: 175-8

2. Ambekar S, Ranjan M, Prasad C, Santosh V, Somanna S. Fourth ventricular ependymoma with a distant intraventricular metastasis: Report of a rare case. J Neurosci Rural Pract. 2013. 4: S121-4

3. Brandao LA, Young Poussaint T. Posterior fossa tumors. Neuroimaging Clin N Am. 2017. 27: 1-37

4. Chao MM, Packer RJ, Myseros JS, Rood BR. Isolated extracranial recurrence of anaplastic ependymoma. Pediatr Blood Cancer. 2011. 56: 317-8

5. Davis MJ, Hasan F, Weinreb I, Wallace MC, Kiehl TR. Extraventricular anaplastic ependymoma with metastasis to scalp and neck. J Neurooncol. 2011. 104: 599-604

6. Dolecek TA, Propp JM, Stroup NE, Kruchko C. CBTRUS statistical report: Primary brain and central nervous system tumors diagnosed in the United States in 2005-2009. Neuro Oncol. 2012. 14: v1-49

7. Duffner PK, Cohen ME. Extraneural metastases in childhood brain tumors. Ann Neurol. 1981. 10: 261-5

8. Fischer C, Haque SS, Huse JT, Blochin E, Souweidane MM, Lis E. Extraneural ependymoma: Distant bone, lung, liver, and lymph node metastases following bevacizumab. Pediatr Blood Cancer. 2013. 60: 143-5

9. Gilbert MR, Ruda R, Soffietti R. Ependymomas in adults. Curr Neurol Neurosci Rep. 2010. 10: 240-7

10. Godfraind C. Classification and controversies in pathology of ependymomas. Childs Nerv Syst. 2009. 25: 1185-93

11. Gramatzki D, Roth P, Felsberg J, Hofer S, Rushing EJ, Hentschel B. Chemotherapy for intracranial ependymoma in adults. BMC Cancer. 2016. 16: 287-

12. Itoh J, Usui K, Itoh M, Hashizume Y. Extracranial metastases of malignant ependymoma--case report. Neurol Med Chir (Tokyo). 1990. 30: 339-45

13. Jung J, Choi W, Ahn SD, Park JH, Kim SS, Kim YS. Postoperative radiotherapy for ependymoma. Radiat Oncol J. 2012. 30: 158-64

14. Kinoshita M, Izumoto S, Kagawa N, Hashimoto N, Maruno M, Yoshimine T. Long-term control of recurrent anaplastic ependymoma with extracranial metastasis: Importance of multiple surgery and stereotactic radiosurgery procedures—case report. Neurol Med Chir (Tokyo). 2004. 44: 669-73

15. Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, Burger PC, Jouvet A. The 2007 WHO classification of tumours of the central nervous system. Acta Neuropathol. 2007. 114: 97-109

16. Mavroudis C, Townsend JJ, Wilson CB. A metastasizing ependymoma of the cauda equina. Case report. J Neurosurg. 1977. 47: 771-5

17. Merchant TE. Current management of childhood ependymoma. Oncology (Williston Park). 2002. 16: 629-42

18. Merchant TE. Current clinical challenges in childhood ependymoma: A focused review. J Clin Oncol. 2017. 35: 2364-9

19. Mete O, Lopes MB. Overview of the 2017 WHO classification of pituitary tumors. Endocr Pathol. 2017. 28: 228-43

20. Murakami M, Kuratsu J, Takeshima H, Soyama N, Shinojima N, Ushio Y. Spinal seeding of anaplastic ependymoma mimicking fungal meningitis. A case report and review of the literature. J Neurosurg Sci. 2000. 44: 46-51

21. Newton HB, Henson J, Walker RW. Extraneural metastases in ependymoma. J Neurooncol. 1992. 14: 135-42

22. Pollack IF, Gerszten PC, Martinez AJ, Lo KH, Shultz B, Albright AL. Intracranial ependymomas of childhood: Long-term outcome and prognostic factors. Neurosurgery. 1995. 37: 655-

23. Ramaswamy V, Taylor MD. Treatment implications of posterior fossa ependymoma subgroups. Chin J Cancer. 2016. 35: 93-

24. Severino M, Consales A, Doglio M, Tortora D, Morana G, Barra S. Intradural extramedullary ependymoma with leptomeningeal dissemination: The first case report in a child and literature review. World Neurosurg. 2015. 84: 865 e813-869

25. Shim KW, Kim DS, Choi JU. The history of ependymoma management. Childs Nerv Syst. 2009. 25: 1167-83

26. Vera-Bolanos E, Aldape K, Yuan Y, Wu J, Wani K, Necesito-Reyes MJ. Clinical course and progression-free survival of adult intracranial and spinal ependymoma patients. Neuro Oncol. 2015. 17: 440-7

27. Wight DG, Holley KJ, Finbow JA. Metastasizing ependymoma of the cauda equina. J Clin Pathol. 1973. 26: 929-35

28. Wood H. Neuro-oncology: A new approach to ependymoma subtyping. Nat Rev Neurol. 2017. 13: 512-3

29. Wu J, Armstrong TS, Gilbert MR. Biology and management of ependymomas. Neuro Oncol. 2016. 18: 902-13

30. Yao Y, Mack SC, Taylor MD. Molecular genetics of ependymoma. Chin J Cancer. 2011. 30: 669-81

31. Zhang XP, Liu Y, Zhang D, Zheng Q, Wang C, Wang L. Cerebellar ependymoma with overlapping features of clear-cell and tanycytic variants mimicking hemangioblastoma: A case report and literature review. Diagn Pathol. 2017. 12: 28-

32. Zhao C, Wang C, Zhang M, Jiang T, Liu W, Li W. Primary cerebellopontine angle ependymoma with spinal metastasis in an adult patient: A case report. Oncol Lett. 2015. 10: 1755-58

Abstract

Background:Intracranial dermoid cysts are uncommon lesions with characteristic imaging appearances. Symptomatic clinical presentation usually occurs in one of two ways: mass effect or rupture. Radiologically, dermoid cysts typically present as low density masses on computed tomography (CT) scan and are generally hyperintense on T1-weighted magnetic resonance imaging (MRI) sequences with variable signal on T2-weighted sequences.

Case Description:We present the case of a 35-year-old female presented with symptoms of increased intracranial pressure. Radiological investigations showed a cystic posterior fossa tumor that was not only hyperdense on CT scans but also hypointense on MRI T1-weighted images. The patient underwent a total-gross resection of an extra-parenchymal posterior fossa tumor. Pathologic examination of the specimen concluded to dermoid cyst.

Conclusion:Dermoid cyst of the posterior fossa is a benign lesion surgically treatable. Only an appropriate radiological diagnosis of this lesion would permit a well-targeted therapeutic approach.

Keywords: Computed tomography, dermoid cyst, magnetic resonance imaging, posterior fossa

INTRODUCTION

Dermoid cysts are rare congenital lesions as a result of developmental malformation, with the defect in gastrulation affecting the surface ectoderm and causing a secondary disruption of neural tube closure.[ 10 ] Computed tomography (CT) and magnetic resonance imaging (MRI) delineate many characteristic features of dermoid cysts. Their most common site is the posterior cranial fossa nearby the midline, when extradural, may arise at the anterior fontanelle.[ 3 6 ] However, some unusual radiological features may elude radiologists and surgeons to other differential diagnosis.

In this paper, we report a patient having fourth ventricle dermoid cyst with atypical radiololgical features.

CASE REPORT

We report the case of a 35-year-old female presented with headaches, vomiting, and gradually worsening blurred vision. Physical examination was unremarkable except for a grade 2 papillary edema on the fundi. Brain CT scan showed imaging a large median and paramedian spontaneous homogenous hyperdense lesion in the posterior fossa. Mild surrounding edema and mass effect on the fourth ventricle was associated provoking a triventricular hydrocephalus [ Figure 1 ]. On MRI, the lesion appeared hypointense on T1-weighted images and extremely hypointense on T2-weighted images with a hyperintense central area on T1 and T2 without enhancement after injection of gadolinium [Figures 2 – 4 ]. A total resection of an extra-parenchymal tumor was performed through a midline suboccipital craniotomy. The lesion was cystic and nonhemorrhagic. After opening the thin cyst wall, a viscid and light brown substance containing few short dark hairs was removed. The patient had a good postoperative recovery and was discharged on the 5^th-postoperative day. Histopathologic examination [ Figure 5 ] revealed a stratified squamous epithelium with fatty cellular debris, few hair follicles, and scattered sebaceous glands. The diagnosis of dermoid cyst was retained.

Figure 1

Axial CT scan showing a hyper-attenuating lesion centered by an hypodensity of the posterior fossa

Figure 2

MRI on axial T1 weighted image revealing an hypointense and heterogeneous contrast enhancement of the solid component of the dermoid cyst

Figure 3

MRI on axial T2 weighted images showing a hypointense signal of the dermoid cyst

Figure 4

MRI on Sagittal T2 weighted images showing a hypointense signal of the lesion

Figure 5

Histopathological examination of the surgical specimen revealing a stratified squamous epithelium with fatty cellular debris, few hair follicles and scattered sebaceous glands consistent with the diagnosis of dermoid cyst

DISCUSSION

The intracranial dermoid cyst is a rare entity, accounting for 0.1–0.7% of all intracranial tumors.[ 14 ] Dermoid cysts are derived from the ectopic inclusion of epithelial cells during neural tube closure between the 3^rd and 5^th week of fetal development, so that the preferential location in the medial line of the posterior fossa and the spinal cord.[ 11 13 ] They are slow growing lesions and may become quite large before becoming symptomatic. Rarely, they may rupture and present with meningitis.[ 3 13 ] Dermoid cysts are well-circumscribed lesions lined by stratified squamous epithelium. They contain thick, foul-smelling, yellowish material from the secretion of sebaceous glands, lipid metabolites, whorls of hair, calcifications, and desquamated epithelium.[ 5 ]

To our knowledge, nine cases have been reported about posterior fossa's dermoid cyst so far. All those cases are resumed in Table 1 .

Table 1

Cases of posterior fossa's dermoid cyst reported in the literature

The appearance of the dermoid cyst in typical cases is easily recognizable in imaging. They are usually extremely hypodense on CT scan with a Hounsfield unit (HU) of −20 to −140, explained by their lipid content.[ 4 5 ] On MRI, dermoid cysts typically appear hyperintense on both T1-weighted and FLAIR sequences, with variable signal on T2-weighted sequences that ranges from hypointense to inhomogeneously hyperintense. There is usually associated neither vasogenic edema nor contrast enhancement. The presence of spontaneous hemorrhage is extremely rare.[ 3 ] On both CT and MRI images, fat density droplets may be seen throughout the subarachnoid space and inside the ventricular system if rupture of the cyst has occurred.

The unusual radiological finding in our patient was the hyperdensity on CT scan and the hyposignal on MRI T1-weighted images. The hyperdensity could be explained by the high protein content of the necrotic tissue within the lesion.[ 4 ] The hyposignal on T1-weighted images could be explained by the saponification of lipid or keratinized debris with secondary microcalcification in suspension.[ 12 ]

CONCLUSION

The dermoid cyst of the posterior fossa is a benign lesion surgically treatable. The appropriate radiological diagnosis of this lesion permits a well-targeted therapeutic approach. The radiological study is often unequivocal during this lesion. Nevertheless, the hyperdense aspect of the lesion on CT associated with hyposignal on T1-weighted images on MRI may make the diagnosis of a dermoid cyst more difficult.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1. Bizzozero L, Talamonti G, D'Angelo V, Casadei G, Arrigoni G, Collice M. Dermoid cyst mimicking hematoma in the posterior fossa. Clin Neurol Neurourg. 1992. 94: 61-3

2. Brown J, Morokoff A, Mitchell P. Unusual imaging appearance of an intracranial dermoid cyst. Am J Neuroradiol. 2001. 22: 1970-2

3. Douvoyiannis M, Goldman D, Abbott I, Litman N. Posterior fossa dermoid cyst with sinus tract and meningitis in a toddler. Pediatr Neurol. 2008. 39: 63-6

4. Goh G, Page R, Nixon T. An unusual CT and MR appearance of a posterior fossa dermoid cyst. Eur J Radiol. 1995. 20: 46-7

5. Lunardi P, Missori P, Gagliardi F, Fortuna A. Dermoid cysts of the posterior cranial fossa in children. Report of nine cases and review of the literature. Surg Neurol. 1990. 34: 39-42

6. Matthys M, Long S, Huisman T, Pindrik J, Tekes A. Posterior fossa dermoid cyst with a sinus tract and restricted diffusion on MR imaging: Value of structural imaging findings and signal characteristics. J Neuroradiol. 2012. 39: 134-5

7. Morina A, Kelmendil F, Morina Q, Morina D. Cerebellar dermoid cyst with contrast enhancement mural nodule. Acta Clin Croat. 2014. 53: 479-82

8. Neugroschl P, David N, Sadeghi A, Soebert B, Pirotte S, Rorive D. Unusual CT features of dermoid cyst in the posterior fossa. Eur Radiol. 2002. 12: 2726-9

9. Sanchez-Mejia R, Limbo M, Tihan T, Galvez M, Woodward M, Gupta N. Intracranial dermoid cyst mimicking hemorrhage. Case report and review of the literature. J Neurosurg. 2006. 105: 311-4

10. Sharma M, Sharma B, Yadav A, Khosla V. Posterior fossa dermoid in association with Klippel-Feil syndrome: A short report. Neurol India. 2001. 49: 210-2

11. Tan L, Kasliwal M, Harbhajanka A, Kellogg R, Arvanitis L, Munoz L. Hyperdense suprasellar mass: An unusual radiological presentation of intracranial dermoid cyst. J Clin Neurosci. 2015. 22: 1208-10

12. Wallace D, Tress B, Kwan P. Radiologically atypical congenital posterior fossa dermoid cyst presenting late in life. J Clin Neurosci. 2008. 15: 835-8

13. Wang Y, Chang T, Lo C, Tu M. Spontaneous rupture of intracranial dermoid cyst with chemical meningitis. J Emerg Med. 2013. 44: 275-6

14. Yasargil M, Abernathey C, Sarioglu A. Microneurosurgical treatment of intracranialdermoid and epidermoid cyst. Neurosurgery. 1989. 24: 561-7

Abstract

Background:Giant cell tumor of bone originating from the connective tissue within the bone marrow is benign but locally aggressive lesion. In all, 90% of the cases involve the epiphysis of long bones and less than 2% involve the skull. Giant cell tumors of the skull occur most frequently in the sphenoid and temporal bones, and very rarely in the ethmoid, frontal, parietal, and occipital bones. We would like to share a case of giant cell tumor of bone arising from the left orbital roof with involving ethmoid sinus, which was diagnosed to be a meningioma before surgery.

Case Description:A 32-year-old lady presented to us with the chief complain of left proptosis, diplopia, and left eye soreness without decline of visual acuity for about 2 months. Her orbital magnetic resonance imaging (MRI) disclosed a mass lesion located in the left frontal base, orbital roof, and upper medial orbital region with adjacent dural-tail sign favoring meningioma. She underwent a left supraorbital pterional craniotomy with the gross total removal of tumor and dura reconstruction. Histology examination of the tumor showed a picture of giant cell tumor of bone. Considering giant cell tumor of bone is locally aggressive, postoperative adjuvant therapy with Denosumab was introduced after full explanation.

Conclusion:Standard treatments of skull-base giant cell tumors have yet to be established due to small number of cases reported in the literature. The standard treatment of giant cell tumor of bone is complete resection of the tumor.

Keywords: Anti-RANKL monoclonal antibody, giant cell tumor of bone, orbital roof tumor

INTRODUCTION

Giant cell tumor of bone was described by Cooper and Travers in 1818, which is characterized histologically by multinucleated giant cells with a background of mononuclear stromal cells.[ 4 ] Giant cell tumor of bone originating from the connective tissue within the bone marrow is benign but locally aggressive with high recurrent rate after treatment.[ 1 3 8 ] Giant cell tumor of bone accounts for about 3% to 7% of primary bone tumors. In all, 90% of the cases involve the epiphysis of long bones and less than 2% involve the skull.[ 1 6 8 ] Giant cell tumors of the skull occur most frequently in the sphenoid and temporal bones, and very rarely in the ethmoid, frontal, parietal, and occipital bones.[ 1 6 8 ] The first case of giant cell tumor of the orbit reported in the English literature was published in 1993.[ 12 ] We would like to share a case of giant cell tumor of bone arising from the left orbital roof with involving ethmoid sinus, which was diagnosed to be a meningioma before surgery.

CASE REPORT

In March 2017, a 32-year-old woman without any systemic disease or ocular traumatic event presented to us with the chief complain of left proptosis, left eye soreness, and diplopia on upper right gaze without decline of visual acuity since January 2017. Noncontrast computed tomography (CT) of orbit done at an outside hospital showed a left intra-orbital tumor with intracranial invasion. Orbital magnetic resonance imaging (MRI) was arranged and it disclosed a mass lesion about 3.8 cm × 3.7 cm × 3.3 cm in size, located in the left frontal base, orbital roof, and upper medial orbital region. This mass lesion showed relative intermediate intensity on T1-weighted image and T2-weighted image, and the postcontrast study showed good enhancement with adjacent dural tail sign causing mass effect on the left eyeball as well as the left frontal brain parenchyma, which favored meningioma [ Figure 1 ]. On admission, her neurological examination showed impairment of the left eye ball movement to upward gaze and medial gaze, otherwise essentially negative finding. Under general anesthesia, she was put in the supine position and underwent a left supraorbital pterional craniotomy with the gross total removal of tumor and dura reconstruction. Grossly, the tumor was hypervascular, relatively firm in consistency, which destructed the left orbital roof thoroughly and invaded the dura of the left frontal base causing the compression of the left frontal lobe and displacement of the left eye ball. This tumor also extended to ethmoid sinus. Histology examination showed that the tumor consisted of evenly distributed osteoclast-like giant cells in a background of round or spindle-shaped mononuclear cells. By immunohistochemistry, the tumor cells showed CD68(+), GFAP(−), EMA(−), p63(−), S100(+scattered), CD1a(+scattered), and p53(−) [ Figure 2 ]. Proliferation index was about 6% by Ki-67 immunostain. Giant cell tumor of bone was diagnosed based on the morphology of the tumor cells and the result of immunohistochemical stains. The resected left orbital roof remnant and diseased dura showed focal involvement by the tumor. The patient's postoperative course was uneventful. After surgery, her left proptosis and limitation of the left eye ball movement were resolved. The postoperative orbital MRI showed some postoperative change without definite residual tumor [ Figure 3 ]. Considering giant cell tumor of bone is locally aggressive with high recurrent rate, postoperative adjuvant therapy with Denosumab was introduced after full explanation. She is doing well and is undergoing regular follow-up at our outpatient department.

Figure 1

Preoperative orbit MRI. Coronal T1 weighted image (a), coronal T1 weighted image post gadolinium enhancement (b), coronal T2 weighted image (c), and sagittal T1 weighted image post gadolinium enhancement (d) showing a well-circumscribed lesion isointensity on T1-weighted images and hypointensity on T2-weighted image with good enhancement and has small cystic/necrotic change noted in the left frontal base, orbital roof, and upper-medial orbital region. The black arrows point out the dural tail sign

Figure 2

Histology of the specimen. Orbital roof HE stain 200X (a), Dura HE stain 100X (b), Tumor HE stain 200X (c) showing osteoclast-like giant cells in a background of round or spindle-shaped mononuclear cells. The resected orbital roof and dura had been involved by the tumor. Tumor CD68 stain 100X (d) showing positive staining. CD68 is particularly useful as a marker for giant cells, osteoclasts

Figure 3

Post-operative orbit MRI. Coronal T1 weighted image (a), coronal T1 weighted image post gadolinium enhancement (b), coronal T2 weighted image (c) showing gross total removal of the tumor

DISCUSSION

Giant cell tumor of bone is an aggressive, bone lytic, osteoclastogenic stromal tumor.[ 6 ] Lung metastasis and malignant transformation to high grade osteosarcoma have been reported although rare.[ 1 2 7 8 11 ] The majority of cases develop at the epiphyses of long bones, but may occur in the sacrum, vertebral body and occasionally in the small bones of the hands and feet.[ 2 7 ] Less than 2% of the cases involve the skull.[ 8 ] The radiographic findings of giant cell tumor of bone on CT scan are osteolytic, radiolucent, and well-circumscribed.[ 8 ] The MRI features of giant cell tumor of bone are nonspecific, usually demonstrating a well-circumscribed lesion with low to intermediate signal intensity or isointense on T1-weighted images; intermediate to high intensity or hypointense on T2-weighted images; and homogenous enhancement after gadolinium injection.[ 8 9 10 11 ] However, there are no MRI features sufficiently characteristic to allow a preoperative diagnosis of giant cell tumor of bone especially those in the rare locations.[ 8 12 ] Due to the small number of skull-base giant cell tumors reported in the literature, standard treatments have yet to be established.[ 6 ] Total surgical resection is the treatment of choice of giant cell tumor of bone.[ 1 6 7 12 ] However, in some instances, radical resection is not feasible when the tumor involves the skull base. Chemotherapy may be beneficial for incompletely resected tumors.[ 8 ] Adjuvant radiation therapy is controversial because giant cell tumor of bone is not radiosensitive and irradiation may predispose the tumor to subsequent malignant transformation.[ 1 7 8 12 ] However, radiotherapy with the dosage around 40 to 60 Gy is still recommended as a postoperative adjuvant therapy in cases of incompletely resected skull base tumor.[ 6 ] Histologically, giant cell tumor of bone consists of reactive multinuclear osteoclast-like giant cells expressing receptor activator of nuclear factor kappa B (RANK) and neoplastic mononuclear stromal cells expressing receptor activator of nuclear factor kappa B ligand (RANKL).[ 1 2 10 ] Blocking of the receptor activator of NF-kappa B ligand (RANKL) signaling pathway, which plays a role in the pathogenesis of giant cell tumor of bone by the anti-RANKL monoclonal antibody “Denosumab,” is an additional adjuvant therapeutic option.[ 1 2 5 10 ] Recently, Denosumab has been reported to provide promising therapeutic effect on giant cell tumor of bone in cases of inoperable or locally advanced situation.[ 1 2 10 ] Nevertheless, the long-term effect and the duration of treatment of Denosumab on giant cell tumor of bone and the safety of Denosumab need further clinical evaluation and basic research.[ 5 11 ]

CONCLUSION

Orbitofrontal giant cell tumor of bone is very rare. To our best knowledge, only three cases had been reported in the English literature [ Table 1 ]. Total surgical resection is the treatment of choice. The dural tail sign occurs as a result of thickening and enhancement of the dura and is most often seen adjacent to a meningioma, but interestingly, it was present in our patient who was confirmed to be a case of orbital roof giant cell tumor of bone.

Table 1

Cases of orbitofrontal giant cell tumor of bone reported in the English literature

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1. Bardakhchyan S, Kager L, Danielyan S, Avagyan A, Karamyan N, Vardevanyan H. Denosumab treatment for progressive skull base giant cell tumor of bone in a 14 year old female- a case report and literature review. Ital J Pediatr. 2017. 43: 32-

2. Borkowska A, Goryn T, Pienkowski A, Wagrodzki M, Jagiello-Wieczorek E, Rogala P. Denosumab treatment of inoperable or locally advanced giant cell tumor of bone. Oncol Lett. 2016. 12: 4312-8

3. Chakarun CF, Forrester DM, Gottsegen CF, Patel DB, White EA, Matcuk GR. Giant Cell Tumor of Bone: Review, Mimics, and New Developments in Treatment. Radiographics. 2013. 33: 197-211

4. Cooper AS, Travers B.editors. Surgical essays. London, England: Cox Longman and Co; 1818. p. 178-9

5. Gaston CL, Grimer RJ, Parry M, Stacchiotti S, Tos APD, Gelderblom H. Current status and unanswered questions on the use of Denosumab in giant cell tumor of bone. Clin Sarcoma Res. 2016. 6: 15-

6. Goto Y, Furuno Y, Kawabe T, Ohwada K, Tatsuzawa K, Sasajima H. Treatment of a skull-base giant cell tumor with endoscopic endonasal resection and denosumab: Case report. J Neurosurg. 2017. 126: 431-4

7. Harris AE, Beckner ME, Barnes L, Kassam A, Horowitz M. Giant cell tumor of the skull: A case report and review of the literature. Surg Neurol. 2004. 61: 274-7

8. Kamoshima Y, Sawamura Y, Imai T, Furukawa H, Kubota K, Houkin K. Giant Cell Tumor of the Frontal Bone in a Girl- Case Report. Neurol Med Chir (Tokyo). 2011. 51: 798-800

9. Tang PH, Mettu P, Maltry AC, Harrison AR, Mokhtarzadeh A. Giant Cell Tumor of the Frontal Bone Presenting as an Orbital Mass. Ophthalmol Ther. 2017. 6: 215-20

10. Van der Heijden L, Dijkstra S, Blay JY, Gelderblom H. Giant cell tumour of bone in the denosumab era. Eur J Cancer. 2017. 77: 75-83

11. Van der Heijden L, Dijkstra S, Van de Sande MAJ, Kroep JR, Nout RA, Van Rijswijk CSP. The Clinical Approach Toward Giant Cell Tumor of Bone. Oncologist. 2014. 19: 550-61

12. Vernet O, Ducrey N, Deruaz JP, Tribolet ND. Giant Cell Tumor of the Orbit. Neurosurgery. 1993. 32: 848-51