Abstract

Background: Pseudotumor cerebri (PTC) or idiopathic intracranial hypertension (IIH) is characterized by elevated intracranial pressure without hydrocephalus or mass lesion, with normal cerebrospinal fluid (CSF) studies and neuroimaging. The exact cause remains uncertain, but potential mechanisms include increased CSF production, impaired CSF absorption, cerebral edema, and abnormal cerebral venous pressure gradients. Patients may present with various accompanying symptoms such as unilateral or bilateral visual obscuration, pulsatile tinnitus, back pain, dizziness, neck pain, blurred vision, cognitive difficulties, radicular pain, and typically intermittent horizontal diplopia.

Case Description: We report a case of a 32-year-old female who initially presented with chronic headaches and oligomenorrhea, which resulted in the diagnosis of polycystic ovary syndrome (PCOS) a few years before the initial diagnosis of PTC. Despite receiving maximum medical treatment and undergoing optic nerve sheath fenestration, the patient experienced complete bilateral vision loss. Nearly 5 years later, the patient sought care at our outpatient neurology clinic, presenting with symptoms including tinnitus, left-sided hearing loss, and joint pain with elevated inflammatory markers and headaches. The focus of this research was to discuss the pathophysiology of each of these comorbidities.

Conclusion: This case report aims to explore the pathophysiological relationships between PTC and concurrent comorbidities, including PCOS, sensorineural hearing loss, empty sella (ES) syndrome, and elevated inflammatory markers. Remarkably, no other PTC case with this unique constellation of concurrent comorbidities have been reported in existing medical literature. The case report underscores the critical importance of early diagnosis of IIH and prompt medical intervention, particularly in patients with PCOS experiencing chronic headaches.

Keywords: Bilateral blindness, Empty sella syndrome, Idiopathic intracranial hypertension, Polycystic ovary syndrome, Sensorineural hearing loss, Pseudotumor cerebri

INTRODUCTION

In this case report, we will study the association of idiopathic intracranial hypertension (IIH), polycystic ovary syndrome (PCOS), sensorineural hearing loss, and elevated inflammatory markers, in a patient who, despite receiving optimal medical treatment and undergoing optic nerve sheath fenestration (ONSF), unfortunately, experienced bilateral blindness. The focus of the case report will be to delve into the pathophysiology of each of these comorbidities and explore how they can manifest and interact differently in individual patients. Emphasizing the importance of early diagnosis, particularly in patients with PCOS who experience chronic headaches, this research underscores the significance of prompt treatment and the need to investigate the underlying mechanisms.

Pseudotumor cerebri (PTC) also known as IIH is primarily associated with raised intracranial pressure with no hydrocephalus or mass lesion, in the presence of normal cerebrospinal fluid (CSF) studies and neuroimaging, without any evident underlying cause.[ 13 ]

PTC commonly affects women aged 20–44, with a female-to-male ratio of 8:1, with a yearly incidence of 19.3/100,000 within the United States, especially among obese women of child-bearing age who weigh more than 20% of their ideal body weight.[ 7 ] A recent meta-analysis of 15 different studies, which included population-based studies from 10 different countries, found a pooled annual worldwide incidence of 1.2/100,000 and a prevalence of 5.1–14.3/100,000.[ 12 ]

The exact underlying cause of IIH remains uncertain, but various mechanisms have been suggested to elucidate the elevated CSF pressure. These mechanisms include increased production of CSF, impaired absorption of CSF at arachnoid granulations or lymphatics, cerebral edema, and elevated cerebral venous pressure resulting from abnormal venous pressure gradients. These factors have been proposed as potential contributors to the raised CSF pressure in IIH.[ 20 ]

The majority of individuals with IIH commonly experience a headache that progressively becomes more severe and frequent, as outlined in the International Classification of Headache Disorders, 3^rd edition. This headache phenotype can vary significantly and may resemble other primary headache disorders. In addition, patients may present with various accompanying symptoms such as unilateral or bilateral visual obscuration, pulsatile tinnitus, back pain, dizziness, neck pain, blurred vision, cognitive difficulties, radicular pain, and typically intermittent horizontal diplopia. It is important to note that none of these symptoms are specific to IIH and cannot definitively indicate its presence. The investigation and management of IIH rely on the assessment of individual symptoms and signs, necessitating a collaborative approach involving experts from multiple disciplines.[ 13 ]

The potential of neuroimaging signs of elevated intracranial pressure is often underutilized in the diagnostic evaluation of suspected PTC. Studies have found that flattening of the posterior sclera emerged as the most sensitive indicator of elevated intracranial pressure in patients diagnosed with PTC.[ 3 ] In addition, empty sella (ES), prelaminar optic nerve enhancement, distension of the peri-optic nerve subarachnoid CSF space, and vertical tortuosity of optic nerve complex demonstrated intermediate sensitivities, while intraocular protrusion of the prelaminar optic nerve exhibited a low sensitivity.[ 3 ] The bony enlargement of the sella turcica caused by chronic elevation of intracranial pressure contributes to the partially empty appearance of the sella turcica.[ 11 ] The multiplicity of these neuroimaging signs, each with a high specificity, enables an observant clinician to confirm or rule out elevated intracranial pressure using magnetic resonance imaging (MRI) criteria alone in the majority of cases.[ 3 ]

Diagnosis of PTC is based on the modified Dandy criteria and varies based on the presence or absence of papilledema. Required criteria of PTC in patients with papilledema include normal neurologic examination except for cranial nerve abnormalities, normal brain parenchyma without evidence of hydrocephalus, mass, or structural lesion on MRI neuroimaging with and without gadolinium contrast, normal CSF composition, and an elevated lumbar puncture opening pressure >250 mm in adults or 280 mm in children.[ 8 ] In patients without papilledema, the diagnosis of PTC can be made if all other previously discussed criteria are satisfied, in addition to unilateral or bilateral abducens nerve palsy.[ 8 ] Furthermore, if both papilledema and abducens nerve palsy are absent, the diagnosis of PTC can be suggested if at least 3 of the following are present on neuroimaging: an ES turcica, flattening of the posterior aspect of the globe, distention of the perioptic subarachnoid space with or without a tortuous optic nerve, and transverse venous sinus stenosis.[ 8 ]

Conservative management for PTC begins with identifying and targeting known risk factors. Therefore, regardless of disease severity, initiating a weight loss program has been shown to improve the disease course.[ 6 ] In a study of 25 obese women, it was concluded that losing weight corresponded to fewer headaches, papilledema, and intracranial pressure.[ 17 ] Medical management is implicated in symptomatic PTC. First-line treatment in patients with symptoms such as intracranial pressure and headaches includes carbonic anhydrase inhibitors including acetazolamide and methazolamide.[ 6 ] In cases where symptoms are rapidly progressing, severe optic neuropathy is present or symptoms progress despite medical management, surgical intervention is recommended. These procedures can include but are not limited to CSF diversion such as ventriculoperitoneal (VP), lumboperitoneal, ventriculoatrial shunting, and optic nerve sheath decompression. Highly severe cases can utilize subtemporal decompression surgery.[ 6 ]

The main objective of this case is to delve into the pathophysiological associations between PTC and concurrent comorbidities, including PCOS, sensorineural hearing loss, ES syndrome, and elevated inflammatory markers. In the current medical literature, there is no documentation of another PTC case presenting the constellation of concurrent comorbidities observed in this patient. The manuscript underscores the importance of early diagnosis and prompt medical management, particularly in patients with PCOS who experience chronic headaches. It highlights the significance of recognizing and addressing IIH and its associated comorbidities to prevent potential complications and optimize patient outcomes.

CASE DESCRIPTION

A 32-year-old female with a medical history of PTC presented to the outpatient neurology clinic complaining of worsening headache, joint pain, hearing loss in the left ear, and tinnitus. She had previously been diagnosed with PTC and her symptoms were progressively increasing for 5 years. Family history and psychosocial history were unremarkable. Initially, the patient was on acetazolamide but showed no improvement. The patient has had a lumbar puncture that led to the relief of her symptoms for only a few days. A VP shunt (VPS) placement surgery could not be performed due to the patient having an implantable cardioverter defibrillator (ICD) for cardiomyopathy a few years earlier.

On physical examination, the patient was found to be morbidly obese with a body mass index (BMI) of 64.5. Eye examination revealed mildly dilated pupils bilaterally with significant loss of vision in both eyes, with only minimal perception of hand waving. The patient had light perception but exhibited an afferent pupillary defect and abducens nerve palsy. A fundoscopic examination revealed gross papilledema bilaterally with optic disc pallor that indicated optic nerve atrophy. The otoscopic examination was normal and Weber and Rinne tests suggested left-sided sensorineural hearing loss.

The patient’s remaining neurological examinations such as the remainder of cranial nerves, motor and sensory functions, reflexes, and mental status were unremarkable.

A review of the patient’s medical record revealed that the patient had also received ONSF which entails creating an opening or window in the optic nerve sheath to drain CSF from the subarachnoid space surrounding the optic nerves for decompression.

According to the patient, optic nerve fenestration initially was helpful, but later she became legally blind despite the maximum medical management and even after optic nerve fenestration. Subsequent investigation revealed that the patient initially presented with chronic headaches and oligomenorrhea, which led to the diagnosis of polycystic ovarian syndrome (PCOS) years before her initial diagnosis of PTC [ Figures 1 and 2 ]. However, it took some time for her primary care physician to refer her to a neurologist until her visual disturbance symptoms were progressively worsening. This referral to a neurologist ultimately facilitated the further diagnosis of PTC.

Figure 1:

(a) Transvaginal ultrasound of the right ovary in grayscale view demonstrates the morphological features of the polycystic ovarian disease: Hyperechoic central stroma, peripheral location of follicles (a string of pearls sign), follicles of similar size measuring 2–9 mm, and increased right ovary size and volume; the right ovary measures approximately 40 mm × 22 mm × 24 mm (length × width × thickness) (corresponding to a volume of 11.65 cm³). An ovary typically weighs 2–8 g. However, they change during life and double in size during pregnancy. (b) Transvaginal ultrasound of the right ovary in color Doppler study demonstrates the mean spectral of the ovarian stromal arteries. However, using color Doppler transvaginal ultrasonography in the clinical diagnosis of patients with polycystic ovary syndrome is not beneficial.

Figure 2:

(a) Transvaginal ultrasound of the left ovary in grayscale view demonstrates the morphological features of polycystic ovarian disease: Hyperechoic central stroma, peripheral location of follicles (a string of pearls sign), follicles of similar size measuring 2–9 mm, and increased left ovary size and volume; the left ovary measures approximately 34 mm × 19 mm × 33 mm (length × width × thickness) (corresponding to a volume of 11.73 cm³). An ovary typically weighs 2–8 g. However, they change during life and double in size during pregnancy, (b) transvaginal ultrasound of the left ovary in color Doppler study demonstrates the mean spectral of the ovarian stromal arteries. However, using color Doppler transvaginal ultrasonography in the clinical diagnosis of patients with polycystic ovary syndrome is not beneficial.

During the follow-up visit at our outpatient neurology clinic, visual-evoked potential showed normal latencies of bilateral optic nerves. Brain auditory-evoked response test revealed left peripheral acoustic nerve neuropathy. The patient’s laboratory result showed rheumatoid factor: 119 IU/mL (normal reference 0.0–13.9) and a high level of C-reactive protein: 13.2 mg/L (normal reference 0.0–4.9); negative Sjogren antibodies (SS-A, SS-B); and erythrocyte sedimentation rate was within normal range. The patient reported experiencing joint pain and stiffness, leading to a referral to a rheumatologist for further evaluation. She was also ordered a non-contrast brain computed tomography (CT) scan to exclude any space-occupying lesions. CT scan findings showed a 3 × 5 mm fat density structure in the third ventricular roof region beneath the body of the corpus callosum and an empty appearance of the sella turcica [ Figure 3 ]. No acute intracranial hemorrhage, significant mass effect, or midline shift, the gray-white matter differentiation was grossly preserved.

Figure 3:

(a) Brain computed tomography scan in two axial planes without contrast demonstrates an empty sella (white arrows) filled with cerebrospinal fluid (CSF) without visualization of the pituitary gland. (b) The sella is empty and enlarged. The sella contains fluid of CSF density.

The patient was started on Topiramate 25 mg oral tablets. During the 3-month follow-up visit, the patient’s condition did not show any improvement. To address this, the dosage of topiramate was increased to 100 mg, while closely monitoring for any potential side effects and she was also started on Furosemide 80 mg. At the subsequent visit, there was still no improvement observed. The patient still complained of headaches and tinnitus. As a result, the dosage of topiramate was further increased to 150 mg, which provided some relief from headaches for a short period of time. To address the ongoing symptoms, the medication regimen was adjusted once again. Promethazine HCL 25 mg oral tablets and Diamox Sequel 500 mg oral extended-release were incorporated into the treatment plan. After a period of 3 months, the dosage of Diamox was raised to 1000 mg, and careful monitoring for potential side effects was implemented. In addition, Phenergan 25 mg was continued alongside the adjusted medication plan. The patient stayed on the regimen for over 6 months and is still being followed up.

DISCUSSION

This case report describes a 32-year-old female who initially presented with chronic headaches and oligomenorrhea, which resulted in the diagnosis of PCOS a few years before the initial diagnosis of PTC. Despite receiving maximum medical treatment and undergoing ONSF, the patient experienced complete bilateral vision loss. Nearly 5 years later, the patient sought care at our outpatient neurology clinic, presenting with symptoms including tinnitus, left-sided hearing loss, and joint pain with elevated inflammatory markers and headaches.

Hence, in this case report, we will explore the pathophysiological connections between PTC and its concurrent comorbidities, which encompass PCOS, sensorineural hearing loss, ES syndrome, and elevated inflammatory markers. In the existing medical literature, there is no documented instance of another PTC case manifesting this particular combination of concurrent comorbidities. This case report underscores the critical importance of early diagnosis and expeditious medical intervention, especially in patients with PCOS who are afflicted by chronic headaches.

Despite receiving optimal medical treatment and undergoing ONSF, this patient unfortunately experienced legal blindness. ONSF is generally advised for individuals presenting with visual symptoms, whereas shunting procedures are typically reserved for those with headaches.[ 19 ] However, in this particular case, the patient was unable to undergo VPS placement surgery due to a previous ICD placement for cardiomyopathy a few years prior.

In most patients, prompt management of PTC results in a good prognosis with a rapid improvement of symptoms after conservative medical management including diuretics and acetazolamide.[ 18 ] In a prospective study of 24 patients conducted by Sørensen et al., only 25% suffered a more chronic disease course that interfered with their daily lives.[ 18 ] Patients with refractory PTC who do not respond to the mainstays of conservative medical treatment can benefit from CSF diversion through VP shunting which results in rapid intracranial pressure reduction and symptom alleviation.[ 14 ] Permanent vision loss is the only major complication of PTC if left untreated.[ 1 ] With poor management of PTC, including those who do not receive surgical treatment with VP shunting, fulminant IIH, defined as severe vision loss within 4 weeks of symptom onset, may ensue.[ 2 ] A meta-analysis of 17 CSF diversion studies, including VP shunting, consisting of 435 patients diagnosed with PTC resulted in a postprocedural normalization of papilledema, headache, and visual acuity of 70%, 80%, and 54% of patients, respectively.[ 16 ]

Our patient also exhibited symptoms related to ES syndrome. ES is characterized by the filling of the sella turcica with CSF, resulting in the compression of pituitary tissue until it lines the floor and walls of the sella. Primary ES refers to the entry of CSF into the sella through a defect in the sellar diaphragm, which could be linked to elevated intracranial pressure.

When comparing PTC patients to a control population, it was found that the sagittal cross-sectional area of the sella turcica was increased by an average of 38% in patients with PTC compared to the control group.[ 10 ] The bone of the floor of the sella turcica is extremely malleable due to its thin nature. As in the case of an expanding pituitary tumor, this floor can be stretched and lengthened due to an increase in intracranial pressure due to excess CSF. The high rates of sella remodeling in response to the increased CSF gradient are thought to contribute to the appearance of an ES turcica on neuroimaging.[ 11 ]

As previously mentioned, this patient was diagnosed with PCOS a few years before her diagnosis of IIH. The study conducted by Cosar et al. observed an incidence of three cases of IIH per 30 women with PCOS and headache. The previous studies reported a prevalence of well-documented PCOS in women referred for IIH ranging from 39% to 57%. These rates were significantly higher, 5–8 times greater, than the 4–10% prevalence of PCOS in the general unselected population.[ 5 ]

Hence given the association between PCOS, obesity, and the development of IIH, it is crucial to carefully evaluate and monitor patients with PCOS who present with chronic headaches, especially when accompanied by ES. Prompt diagnosis and management of IIH are important to prevent potential complications and optimize patient outcomes.

This patient also exhibited left-sided sensorineural hearing loss. It is widely recognized that the visual impairments observed in IIH are primarily attributed to the compression of the optic nerve. The increased pressure within the intracranial space transmits to the optic nerve sheath through the optic canal, obstructing the axoplasmic transmission in the optic nerve axons. Consequently, this leads to swelling of the optic nerve fibers. Similarly, it is postulated that the audio-vestibular symptoms experienced by individuals with IIH result from the compression and subsequent edema of the vestibulocochlear nerve due to elevated intracranial pressure.[ 4 ] In a study by Giuseffi et al., it was reported that 58% of IIH cases presented with pulsatile tinnitus.[ 9 ] While Rudnick and Sismanis suggested a potential association between hearing loss and the masking effect of tinnitus, the underlying pathophysiology of both tinnitus and hearing loss in IIH remains unclear.[ 15 ] Another proposed mechanism for hearing loss is the transmission of heightened intracranial pressure to the perilymph through the cochlear aqueduct. Consequently, this increased tension of the basilar membrane and hindered mobility of the stapes footplate can contribute to hearing loss.[ 17 ]

Furthermore, this patient presented with elevated inflammatory markers and experienced joint pains, necessitating a referral to a rheumatologist for further evaluation and management.

Considering the patient’s elevated BMI value, it is important to acknowledge that obesity plays a significant role in triggering a chronic proinflammatory state within the body. This state of inflammation has been strongly associated with the development of IIH. The association between obesity and the development of IIH may be partly explained by proposed mechanisms involving inflammatory processes. One such mechanism involves the dysregulation of the enzyme 11b-hydroxysteroid dehydrogenase type 1 (11b-HSD1), which plays a role in modulating glucocorticoids and regulating CSF secretion. Increased activity of 11b-HSD1 has been observed in both obesity and IIH, potentially leading to elevated local cortisol levels. Prolonged exposure to high cortisol levels can stimulate the production of proinflammatory mediators and potentially impact CSF production. Studies have shown that weight loss and reduced intracranial pressure in IIH patients are associated with decreased levels of 11b-HSD1.[ 20 ]

The management of our patient’s symptoms predominantly focused on individualized adjustments to medication types and dosages. Given the complex nature of her condition, a multidisciplinary approach to management was deemed necessary.

It is important to emphasize that the overall risk of mortality in PTC is low, and most individuals respond well to appropriate management and treatment. However, studies on patients enrolled in the intracranial hypertension registry show patients with PTC possess a significantly elevated risk of death attributed to suicide and accidental overdose when compared to the general population.[ 10 ]

CONCLUSION

This case underscores the importance of early diagnosis and management of IIH, particularly in patients with PCOS who present with chronic headaches. In fact, in the course of reviewing this manuscript and learning its objective for timely diagnosis of IIH among PCOS patients, one of our health-care providers encountered a 22-year-old patient with a history of PCOS seeking treatment at our neurology clinic due to recent complaints of debilitating headaches and blurry vision. After a comprehensive evaluation, we expeditiously arrived at the diagnosis of IIH.

The patient exhibited clinical signs of papilledema along with an elevated opening pressure of 36 mm H₂O during the lumbar puncture procedure. In accordance with our diagnosis, an appropriate therapeutic regimen was promptly initiated, comprising Diamox 250 mg twice daily and Topamax 25 mg. Recognizing the complexity of this case, the patient was referred to both an ophthalmologist and a neurosurgeon for further evaluation and consideration of treatment options. Following thorough discussions and shared decision-making, the patient opted to postpone the consideration of a VP shunting procedure, electing instead to pursue a treatment plan involving medication management and a focused approach to weight loss. Subsequent follow-up visits revealed significant clinical improvement, with resolution of the papilledema and notable improvement of the patient’s previously reported visual disturbances and blurry vision. We find significant reassurance in the patient’s response to treatment, emphasizing the paramount importance of timely diagnosis and intervention in cases of IIH, particularly among patients with PCOS and a history of headaches. Prompt recognition and treatment are crucial to prevent potential complications and optimize patient outcomes.

Furthermore, this case report aims to investigate the pathophysiological mechanisms underlying PCOS, sensorineural hearing loss, ES, and elevated inflammatory markers in a patient diagnosed with IIH. Obesity-induced chronic inflammation and dysregulation of cortisol metabolism may contribute to the pathogenesis of IIH in these individuals.

A multidisciplinary approach involving neurologists, ophthalmologists, endocrinologists, and rheumatologists is essential for the comprehensive management of IIH and its associated comorbidities. Further research and studies are needed to enhance our understanding of the underlying mechanisms and to identify more effective treatment strategies for this challenging condition.

It is crucial to recognize the potential psychological impact of IIH on patients, as studies have shown an increased risk of mortality attributed to suicide and accidental overdose. Addressing the mental health needs of patients with IIH is an integral part of their overall care. Overall, this case report contributes to the existing literature on the clinical presentation, diagnosis, and management of IIH, emphasizing the importance of a multidisciplinary approach and individualized treatment strategies for optimal patient outcomes.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

Use of artificial intelligence (AI)-assisted technology for manuscript preparation

The authors confirm that there was no use of artificial intelligence (AI)-assisted technology for assisting in the writing or editing of the manuscript and no images were manipulated using AI.

Disclaimer

The views and opinions expressed in this article are those of the authors and do not necessarily reflect the official policy or position of the Journal or its management. The information contained in this article should not be considered to be medical advice; patients should consult their own physicians for advice as to their specific medical needs.

References

1. Best J, Silvestri G, Burton B, Foot B, Acheson J. The incidence of blindness due to idiopathic intracranial hypertension in the UK. Open Ophthalmol J. 2013. 7: 26-9

2. Bouffard MA. Fulminant idiopathic intracranial hypertension. Curr Neurol Neurosci Rep. 2020. 20: 8

3. Brodsky MC, Vaphiades M. Magnetic resonance imaging in pseudotumor cerebri. Ophthalmology. 1998. 105: 1686-93

4. Çoban K, Aydın E, Özlüoğlu LN. Audio-vestibular findings in increased intracranial hypertension syndrome. J Int Adv Otol. 2017. 13: 100-4

5. Cosar E, Cosar M, Köken G, Sahin FK, Caliskan G, Haktanir A. Polycystic ovary syndrome is related to idiopathic intracranial hypertension according to magnetic resonance imaging and magnetic resonance venography. Fertil Steril. 2008. 89: 1245-6

6. Dave SB, Subramanian PS. Pseudotumor cerebri: An update on treatment options. Indian J Ophthalmol. 2014. 62: 996-8

7. Durcan FJ, Corbett JJ, Wall M. The incidence of pseudotumor cerebri. Population studies in Iowa and Louisiana. Arch Neurol. 1988. 45: 875-7

8. Friedman DI, Liu GT, Digre KB. Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology. 2013. 81: 1159-65

9. Giuseffi V, Wall M, Siegel PZ, Rojas PB. Symptoms and disease associations in idiopathic intracranial hypertension (Pseudotumor cerebri): A case-control study. Neurology. 1991. 41: 239-44

10. Hermes SM, Miller NR, Waslo CS, Benes SC, Tanne E. Mortality among patients with idiopathic intracranial hypertension enrolled in the IH registry. Neurology. 2020. 95: e921-9

11. Kyung SE, Botelho JV, Horton JC. Enlargement of the sella Turcica in pseudotumor cerebri. J Neurosurg. 2014. 120: 538-42

12. McCluskey G, Doherty-Allan R, McCarron P, Loftus AM, McCarron LV, Mulholland D. Meta-analysis and systematic review of population-based epidemiological studies in idiopathic intracranial hypertension. Eur J Neurol. 2018. 25: 1218-27

13. Mollan SP, Davies B, Silver NC, Shaw S, Mallucci CL, Wakerley BR. Idiopathic intracranial hypertension: Consensus guidelines on management. J Neurol Neurosurg Psychiatry. 2018. 89: 1088-100

14. Mondragon J, Klovenski V, editors. Pseudotumor cerebri. StatPearls. Treasure Island, FL: StatPearls Publishing; 2022. p.

15. Rudnick E, Sismanis A. Pulsatile tinnitus and spontaneous cerebrospinal fluid rhinorrhea: Indicators of benign intracranial hypertension syndrome. Otol Neurotol. 2005. 26: 166-8

16. Satti SR, Leishangthem L, Chaudry MI. Meta-analysis of CSF diversion procedures and dural venous sinus stenting in the setting of medically refractory idiopathic intracranial hypertension. AJNR Am J Neuroradiol. 2015. 36: 1899-904

17. Sinclair AJ, Burdon MA, Nightingale PG, Ball AK, Good P, Matthews TD. Low energy diet and intracranial pressure in women with idiopathic intracranial hypertension: Prospective cohort study. BMJ. 2010. 341: c2701

18. Sørensen PS, Krogsaa B, Gjerris F. Clinical course and prognosis of pseudotumor cerebri. A prospective study of 24 patients. Acta Neurol Scand. 1988. 77: 164-72

19. Spitze A, Lam P, Al-Zubidi N, Yalamanchili S, Lee AG. Controversies: Optic nerve sheath fenestration versus shunt placement for the treatment of idiopathic intracranial hypertension. Indian J Ophthalmol. 2014. 62: 1015-21

20. Sudhakar P. Commentary: The role of inflammation in idiopathic intracranial hypertension. Indian J Ophthalmol. 2021. 69: 1506-7

Abstract

Background: FD is relatively rare in the craniofacial region, accounting for only 20% of all cases. Currently, two general subtypes of FD are recognized: monostotic and polyostotic. The monostotic form is more frequent, accounting for 75% to 80% of fibrous dysplasia cases.

Case Description: An 18-year-old male presented with the complaint of bony-hard swelling over the forehead for 8 years. Radiology showed an expansile osseous lesion involving frontal bones. The patient underwent bi-frontal craniectomy with gross total resection of tumour mass with titanium mesh cranioplasty. His postoperative period was uneventful and was discharged on the seventh postoperative day.

Conclusion: The cases of monostotic skull fibrous dysplasia should be treated by resection of the affected bone and cranioplasty. However, a more conservative re-contouring may be carried out in cases with multifocal involvement or when the excision is considered risky due to proximity to the major venous sinuses.

Keywords: Fibrous dysplasia, Frontal, Hump on the head, Parietal

INTRODUCTION

Fibrous dysplasia (FD) is a benign, congenital, and noncommunicable disease affecting both genders equally. A benign proliferation of fibrous tissue in the bone marrow characterizes it. FD is relatively rare in the craniofacial region, and incidence has been estimated at 1 in 5,000–10,000.[ 3 ]

The authors wish to report a rare case of monostotic FD, as it involves the bifrontal bone, overlying the superior sagittal sinus. We used the “cut, remove, and replace” method of dealing with FD and would like to discuss the management options of this rare entity.

CASE DESCRIPTION

An 18-year-old male presented with the complaint of a bony, hard swelling over the forehead for eight years. As per the patient’s description, the swelling was “initially the size of a pea,” but it gradually increased to “the size of a tennis ball.” There was no history of rupture or discharge from it. This was associated with mild headaches without any aggravating or relieving factors. There was no history of seizure, vomiting, trauma, or blurring of vision. On examination, a bony, hard, nonpulsatile, immobile, non-tender swelling, 8 × 10 × 15 cm in size, was present over the midline, bifrontal region [ Figure 1 ]. The rest of his neurological examination was unremarkable.

Figure 1:

(a) Clinical photograph of patient lateral view and (b) front view.

Computed tomography (CT) of the head showed an expansile osseous lesion involving the bifrontal bones with linear trabecular striations [ Figure 2 ]. Magnetic resonance imaging (MRI) of the brain revealed a swelling in the frontal region that was seen involving both the inner and outer table of the frontal bone, causing the widening of the diploic spaces [ Figure 3 ]. No significant enhancement was seen on postcontrast images.

Figure 2:

(a and b) Three-dimensional computed tomography (CT) scan reconstruction showing frontal bone hump. (c and d) Bone window of coronal and sagittal CT scan.

Figure 3:

(a) Magnetic resonance imaging T2 sagittal and (b) T1 contrast sagittal.

The tumor was resected by bifrontal craniotomy [ Figure 4a ]. Burr holes were made circumferentially around the lesion, avoiding the midline and leaving some margin. Gradually, the dura was separated circumferentially, avoiding any sinus injury. The tumor mass was enveloped in the bone (both inner and outer tables). Following this, cranioplasty was done using titanium mesh (Johnson and Johnson, India), fixed with miniplate and screw [ Figure 4b ].

Figure 4:

(a) Intraoperative image after skin flap elevation, (b) after resection and placement of mesh over the craniectomy defect.

The postoperative period was uneventful, and the patient was discharged on the 7^th postoperative day. Histopathology revealed a fibro-osseous lesion with irregular curvilinear woven bone without conspicuous osteoblastic rimming, intervening fibrous tissue composed of bland fibroblastic spindle cells [ Figure 5 ]. It was suggestive of benign fibro-osseous lesion favoring FD. He is doing well at a 12-month follow-up that shows an excellent cosmetic result [ Figure 6 ] without any recurrence of symptoms.

Figure 5:

(a and b) Fibro-osseous lesion with irregular curvilinear woven bone without conspicuous osteoblastic rimming, intervening fibrous tissue is composed of bland fibroblastic spindle cells (H&E).

Figure 6:

Clinical photograph of the patient at 12-month follow-up.

DISCUSSION

FD is characterized by fibro-osseous lesions with blending of fibrous and osseous tissue, with resultant secondary bony metaplasia, without osteoblast maturation producing immature, newly formed, and weakly calcified bone.[ 7 ] It usually presents as a painless swelling, which progressively increases in size.

CT best characterizes FD. Its appearance pattern on CT may vary because of variable combinations of osseous as well as fibrous elements. It may be ground glass or mixed (56%), homogeneous radiopaque (sclerotic) (23%), and radiolucent with a lytic pattern of trabeculae (cystic) (21%).[ 2 ] On MRI, FD shows homogenous, moderately low-signal intensity on T1 and T2-weighted images. Most of the lesions display moderate-to-high central contrast enhancement with some rim enhancement. The degree of contrast enhancement on T1-weighted images depends on the amount and degree of bone trabeculae and collagen present. Likewise, in our case, there was no contrast enhancement.[ 1 ]

The aim of the treatment is correction of the facial deformity, prevention of pathological fracture, control of pain, and prevention of recurrence.[ 5 ] Radiation therapy and chemotherapy have no role in the treatment of this disease, and radiation therapy may increase the risk of malignant transformation.[ 4 ] Surgery is the mainstay of treatment in FD. We carried out a radical resection with mesh cranioplasty to correct the deformity to prevent recurrence.[ 6 ]

CONCLUSION

To conclude, cases of monostotic skull FD should be treated by resection of the affected bone and cranioplasty. As we have shown, even a case involving the bifrontal bones and lying over the superior sagittal sinus can be resected completely, followed by reconstruction. However, a more conservative recontouring may be carried out in cases with multifocal involvement or when the excision is considered risky due to proximity to the major venous sinuses.

Ethical approval

Not applicable.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

Use of artificial intelligence (AI)-assisted technology for manuscript preparation

The authors confirm that there was no use of artificial intelligence (AI)-assisted technology for assisting in the writing or editing of the manuscript and no images were manipulated using AI.

Disclaimer

The views and opinions expressed in this article are those of the authors and do not necessarily reflect the official policy or position of the Journal or its management. The information contained in this article should not be considered to be medical advice; patients should consult their own physicians for advice as to their specific medical needs.

References

1. Chandavarkar V, Patil PM, Bhargava D, Mishra MN. A rare case report of craniofacial fibrous dysplasia. J Oral Maxillofac Pathol. 2018. 22: 406-9

2. Gupta D, Garg P, Mittal A. Computed tomography in craniofacial fibrous dysplasia: A case series with review of literature and classification update. Open Dent J. 2017. 11: 384-403

3. Javaid MK, Boyce A, Appelman-Dijkstra N, Ong J, Defabianis P, Offiah A. Best practice management guidelines for fibrous dysplasia/McCune-Albright syndrome: A consensus statement from the FD/MAS international consortium. Orphanet J Rare Dis. 2019. 14: 139

4. Maher CO, Friedman JA, Meyer FB, Lynch JJ, Unni K, Raffel C. Surgical treatment of fibrous dysplasia of the skull in children. Pediatr Neurosurg. 2002. 37: 87-92

5. Menon S, Venkatswamy S, Ramu V, Banu K, Ehtaih S, Kashyap VM. Craniofacial fibrous dysplasia: Surgery and literature review. Ann Maxillofac Surg. 2013. 3: 66-71

6. Ozek C, Gundogan H, Bilkay U, Tokat C, Gurler T, Songur E. Craniomaxillofacial fibrous dysplasia. J Craniofac Surg. 2002. 13: 382-9

7. Xu J, Li X, Lv CS, Chen Y, Wang M, Liu JF. Treatment protocols for growth hormonesecreting pituitary adenomas combined with craniofacial fibrous dysplasia: A case report of atypical McCuneAlbright syndrome. Exp Ther Med. 2014. 8: 877-80

Abstract

Background: The effectiveness of autologous stem cell transplantation (ASCT) in preventing the development of central nervous system (CNS) plasmacytomas in multiple myeloma (MM) patients is not well understood. An ASCT patient who developed CNS extramedullary (EM) lesions is presented. The literature was reviewed for similar cases in which the transplant did not prevent the development of CNS lesions.

Case Description: A 42-year-old female was evaluated after complaining of a sudden severe headache and complete vision loss. Two years before, she was diagnosed with MM and treated with systemic chemotherapy and an ASCT. The patient was in remission; however, a new brain magnetic resonance imaging showed a sellar and suprasellar mass. Additional smaller lesions were identified at the parietal convexity and the splenium. Due to the history of MM and evidence of multiple intracranial lesions, it was suspected that the lesions were secondary to EM disseminated disease. Due to the sudden loss of vision, the patient underwent a right frontotemporal craniotomy with subtotal sellar/suprasellar tumor resection to decompress the optic nerves. Histopathological examination of the lesion confirmed an immunoglobulin A (IgA) EM sellar and suprasellar plasmacytoma.

Conclusion: In the majority of MM patients with CNS involvement, ASCT did not prevent the development of EM sellar plasmacytomas. The IgA subtype is associated with more aggressive disease biology for CNS relapses.

Keywords: Extramedullary, Multiple myeloma, Plasmacytoma, Relapse, Sella, Stem cell, Suprasellar, Transplantation

INTRODUCTION

Multiple myeloma (MM) is a plasma cell proliferative disorder displaying an abnormal increase of monoclonal immunoglobulins. The overall 5-year survival rate ranges from 40% to 82%.[ 23 ] High-dose chemotherapy with autologous stem cell transplantation (ASCT) is the mainstay treatment.[ 4 , 6 , 7 , 29 , 34 ] ASCT significantly improves the remission rate and minimal residual disease of MM patients.[ 23 ] In patients with MM, deposits of plasma cells can cluster together to form tumors (plasmacytomas) in several body areas. The most frequent mechanism causing soft-tissue plasmacytomas is direct growth from skeletal tumors by disrupting the cortical bone.[ 27 ] Other mechanisms for their formation include hematogenous spread or clonal heterogeneity in which a new clone population resistant to previous treatments develops.[ 5 , 27 ] Plasmacytomas may require neurosurgical management if they involve the skull, brain, or spine. Extramedullary (EM) plasmacytomas outside the bone marrow are highly radiosensitive, with 80–100% local control rates.[ 10 , 18 ] About 13% of MM patients develop EM plasmacytomas, occurring in 7% of newly diagnosed cases or 8% later in the disease.[ 31 ]

Central nervous system (CNS) involvement of MM is rare, occurring in 0.7–1% of the patients.[ 1 , 22 , 24 , 28 ] It can emerge in newly diagnosed, relapsed, or refractory MM patients; however, it is more frequent in relapsed cases and younger patients.[ 1 , 16 , 21 , 28 ] The median time from MM diagnosis to CNS involvement is approximately two years.[ 16 ] It has a poor prognosis, with 1 , 5 , 16 , 28 ] Abdallah et al. showed that patients who received only systemic chemotherapy when the primary MM diagnosis was established had a significantly shorter 8-month median time to CNS myeloma diagnosis compared to those patients who received systemic chemotherapy and ASCT, with a median time of 32 months.[ 1 ] The study by Liu et al. suggested that for MM patients with CNS involvement, ASCT can improve the prognosis.[ 21 ] However, in most studies, the benefit of ASCT in patients with CNS disease appears to be limited.[ 1 , 28 ] The effectiveness of ASCT in preventing the development of CNS plasmacytomas is not well understood. This report presented an MM patient with an ASCT who later developed CNS EM lesions. The literature was reviewed for similar cases in which the transplant did not prevent the development of CNS lesions.

CASE REPORT

A 42-year-old female was evaluated at the emergency department (ED) after experiencing a severe headache and sudden complete vision loss. Two years before, she was diagnosed with MM and treated with systemic chemotherapy and an ASCT. She was in remission; however, eight months before her current evaluation, a right intracranial occipital dural base lesion was identified, for which she received fractionated radiotherapy. Three months later, brain magnetic resonance imaging (MRI) showed a significant reduction in the lesion size. During the two months before her evaluation at the ED, she noticed a slow but progressive visual acuity loss; however, she did not seek medical attention until she developed complete vision loss. A new brain MRI at the ED demonstrated a sellar and suprasellar mass measuring 1.9 cm anteroposterior × 2.1 cm transverse × 3.2 cm craniocaudal, showing avid contrast enhancement causing significant compression on the optic chiasm superiorly [ Figure 1 ]. The lesion had an invasion of the right cavernous sinus and extended inferiorly into the right sphenoid sinus and anteriorly into the ethmoidal air cells. A 2.4 cm parietal dural-based lesion and a 1.5 cm lesion at the splenium of the corpus callosum were also identified.

Figure 1:

Brain magnetic resonance imaging with gadolinium T1-weighted (a) coronal and (b) sagittal images showing a large sellar/ suprasellar mass with avid contrast enhancement with invasion of the right cavernous sinus and encasement of the right carotid artery.

On physical examination, the patient had bilateral optic nerve swelling with no light perception. Bilateral six-nerve paresis was present. Motor and sensory examinations were normal. Due to the history of MM and evidence of multiple intracranial lesions, it was suspected that the lesions were secondary to her disseminated disease. Due to the sudden loss of vision, the patient underwent a right frontotemporal craniotomy with subtotal sellar/suprasellar tumor resection to decompress the optic nerves. The histopathological examination of the lesion was compatible with an immunoglobulin A (IgA) plasma cell neoplasia, CD19 positive, and negative for kappa or lambda light chain. She died three months later from her disseminated disease without receiving additional chemotherapy or radiotherapy.

DISCUSSION

Plasmacytomas are tumors that should be considered in the differential diagnosis for lesions involving the sella even in the absence of known MM, especially when cranial nerve paresis is present.[ 10 , 18 ] However, these patients should be rigorously monitored for progression to MM.[ 18 ] DiDomenico et al. showed that in 45% of the patients with a sellar plasmacytoma, the initial plasmacytoma diagnosis led to the diagnosis of MM.[ 10 ] For patients with known MM and parasellar masses, the treatment plan should include a combination of induction therapy, immunomodulatory drugs, radiotherapy, and systemic therapy.[ 10 , 18 , 28 ] In the review of sellar and clival plasmacytomas by Lee et al., 67% of the patients were alive at a median follow-up of 12 months.[ 18 ] However, 18% of them develop parasellar recurrences.

Relapse of MM after ASCT usually presents with a recurrence of plasma cells in the marrow.[ 22 ] Some authors have reported that the presentation of localized EM plasmacytoma after ASCT in MM patients is very unusual.[ 11 , 26 ] However, in the study by Zeiser et al., the relapse rate after ASCT was 63%.[ 34 ]

Alegre et al. reported that 52% of MM patients relapsed or progressed after ASCT.[ 2 ] In their study, among the patients with relapse, 14% presented EM presentations with multiple plasmacytomas as the predominant symptom, carrying a significantly shorter median overall survival.[ 2 ] Yue et al. showed that among newly diagnosed MM patients where ASCT was used, 57% had disease relapse during follow-up, including 24% of patients with EM relapse.[ 33 ] EM relapse of MM is often resistant to existing treatments and has an extremely poor prognosis.[ 8 , 33 ] Survival analysis showed that EM relapse patients had significantly worse median overall survival than patients with relapse but without EM involvement.[ 33 ] Plasmacytoma occurrence at relapse in patients with upfront ASCT is significantly associated with a poor prognosis.[ 8 ]

CNS involvement following ASCT has been described in very few MM patients. Isolated CNS relapse after ASCT is extremely rare, with only 14 cases reported.[ 20 , 22 ] Among the isolated CNS relapse cases, 43% had parenchymal lesions.[ 3 , 5 , 22 , 25 , 29 , 32 ] Despite ASCT, the prognosis after CNS relapse is extremely poor, with a very short median survival.[ 14 , 20 , 22 , 25 , 29 ] For MM patients who are ASCT candidates, IgA MM has been recognized as a risk factor that adversely affects survival.[ 9 , 12 ] Li et al. noticed that more patients with EM plasmacytoma at the time of MM diagnosis carried the IgA subtype than those without plasmacytoma.[ 19 ] Seftel et al. showed that IgA MM patients have a significant risk of CNS relapse after ASCT.[ 29 ] In the study of MM patients with CNS involvement by Jurczyszyn et al., 27% of them had the IgA subtype.[ 16 ] Among the 14 patients with isolated CNS relapse after ASCT, 43% had the IgA subtype. The IgA subtype was present in 50% of the patients with parenchymal CNS lesions. These findings suggest that the IgA subtype is associated with more aggressive disease biology for CNS relapses.

This report highlighted an MM patient with an EM lesion at the sellar/suprasellar area that developed two years after receiving an ASCT. After reviewing the literature, four case reports of sellar/suprasellar plasma cell tumors were identified in which the patient received a stem cell transplant during the disease process.[ 13 , 15 , 17 , 30 ] The case presented by Khan et al. is similar to that reported herein, where the lesion developed after the patient received the ASCT.[ 17 ] However, the patients reported by Fukai et al. and Sinnott et al. underwent stem cell transplants after they were operated on for the plasmacytic lesion in the Sella.[ 13 , 30 ] For both cases, the MM diagnosis was established after the seller plasmacytoma was operated on.[ 13 , 30 ] The patient presented by Jiang et al. did not undergo surgery for the sellar lesion as a diagnosis of MM was made a few days before the scheduled surgery, being treated with radiotherapy, chemotherapy, and ASCT with complete remission.[ 15 ] The patients presented by Fukai et al., Khan et al., and Jiang et al. received a peripheral ASCT.[ 13 , 15 , 17 ] However, the patient presented by Sinnott et al. received a bone marrow ASCT.[ 30 ] Interestingly, the IgA subtype was not identified in any of the four sellar cases, with 75% of them having light chain MM. Similar to all 14 patients with isolated CNS relapse after ASCT, in the present case, the transplant did not prevent the development of an EM sellar plasmacytoma.

CONCLUSION

In the majority of patients with CNS MM involvement, ASCT did not prevent the development of EM sellar plasmacytomas. The IgA subtype is associated with more aggressive disease biology for CNS relapses.

Ethical approval

The Institutional Review Board approval is not required.

Declaration of patient consent

Patient’s consent is not required as patients identity is not disclosed or compromised.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

Use of artificial intelligence (AI)-assisted technology for manuscript preparation

The author confirms that there was no use of artificial intelligence (AI)-assisted technology for assisting in the writing or editing of the manuscript and no images were manipulated using AI.

Disclaimer

The views and opinions expressed in this article are those of the authors and do not necessarily reflect the official policy or position of the Journal or its management. The information contained in this article should not be considered to be medical advice; patients should consult their own physicians for advice as to their specific medical needs.

References

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2. Alegre A, Granda A, Martínez-Chamorro C, Díaz-Mediavilla J, Martínez R, García-Laraña J. Different patterns of relapse after autologous peripheral blood stem cell transplantation in multiple myeloma: Clinical results of 280 cases from the Spanish Registry. Haematologica. 2002. 87: 609-14

3. Annibali O, Nobile C, Greco R, Cellini F, Quattrocchi CC, Tirindelli MC. The combination of topotecan, temozolomide, and dexamethasone is associated with radiotherapy as a treatment of central nervous system myeloma relapse. Int J Hematol. 2009. 89: 513-6

4. Attal M, Harousseau JL, Stoppa AM, Sotto JJ, Fuzibet JG, Rossi JF. A prospective, randomized trial of autologous bone marrow transplantation and chemotherapy in multiple myeloma. Intergroupe Français du Myélome. N Engl J Med. 1996. 335: 91-7

5. Bergantim R, Bastos J, Soares MJ, Carvalho B, Soares P, Marques C. Aggressive central nervous system relapse after autologous stem cell transplant in multiple myeloma: Case reports and literature review. Case Rep Hematol. 2020. 2020: 8563098

6. Bove V, Garrido D, Riva E. Young age and autologous stem cell transplantation are associated with improved survival in newly diagnosed multiple myeloma. Hematol Transfus Cell Ther. 2021. 43: 295-302

7. Child JA, Morgan GJ, Davies FE, Owen RG, Bell SE, Hawkins K. High-dose chemotherapy with hematopoietic stem-cell rescue for multiple myeloma. N Engl J Med. 2003. 348: 1875-83

8. Chu TH, Jung SH, Kim K, Lee JH, Mun JC, Bang SM. Relapse with plasmacytoma after upfront autologous stem cell transplantation in multiple myeloma. Ann Hematol. 2022. 101: 1217-26

9. Desikan R, Barlogie B, Sawyer J, Ayers D, Tricot G, Badros A. Results of high-dose therapy for 1000 patients with multiple myeloma: Durable complete remissions and superior survival in the absence of chromosome 13 abnormalities. Blood. 2000. 95: 4008-10

10. DiDomenico J, Ampie L, Choy W, Lamano JB, Oyon DE, Kesavabhotla K. Sellar plasmacytomas masquerading as pituitary adenomas: A systematic review. J Clin Neurosci. 2018. 50: 20-3

11. Erkus M, Bolaman Z, Meteoglu I, Kadikoylu G. Localized extramedullary relapse after autologous hematopoietic stem cell transplantation in multiple myeloma. Saudi Med J. 2005. 26: 989-91

12. Facon T, Avet-Loiseau H, Guillerm G, Moreau P, Geneviève F, Zandecki M. Chromosome 13 abnormalities identified by FISH analysis and serum beta2-microglobulin produce a powerful myeloma staging system for patients receiving high-dose therapy. Blood. 2001. 97: 1566-71

13. Fukai J, Nohgawa M, Uematsu Y, Itakura T, Kamei I. Immunoglobulin D multiple myeloma involving the sella manifesting as oculomotor palsy: Case report. Neurosurgery. 2010. 67: E505-6

14. Hotta M, Ito T, Konishi A, Yoshimura H, Nakanishi T, Fujita S. Multiple myeloma with central nervous system relapse early after autologous stem cell transplantation: A case report and literature review. Intern Med. 2021. 60: 463-8

15. Jiang CZ, Lin QS, Wu XY, Wang CY, Kang DZ. Sellar solitary plasmacytoma progressing to multiple myeloma: A case report and literature review. Medicine (Baltimore). 2014. 93: e58

16. Jurczyszyn A, Grzasko N, Gozzetti A, Czepiel J, Cerase A, Hungria V. Central nervous system involvement by multiple myeloma: A multi-institutional retrospective study of 172 patients in daily clinical practice. Am J Hematol. 2016. 91: 575-80

17. Khan IS, Javalkar V, Thakur JD, Nanda A. Intrasellar plasmacytoma: An illustrative case and literature review. J Clin Neurosci. 2012. 19: 210-3

18. Lee J, Kulubya E, Pressman BD, Mamelak A, Bannykh S, Zada G. Sellar and clival plasmacytomas: Case series of 5 patients with systematic review of 65 published cases. Pituitary. 2017. 20: 381-92

19. Li J, Shen KN, Huang WR, Li LH, Chen H, Chen WM. Autologous stem cell transplant can overcome poor prognosis in patients with multiple myeloma with extramedullary plasmacytoma. Leuk Lymphoma. 2014. 55: 1687-90

20. Li X, Wang W, Zhang X, Liang Y. Multiple myeloma with isolated central nervous system relapse after autologous stem cell transplantation: A case report and review of the literature. Front Oncol. 2022. 12: 1027585

21. Liu J, Shen J, Liu D. Case reports: Central nervous system involvement in patients with newly diagnosed multiple myeloma. Front Neurol. 2023. 14: 1072490

22. Mittal A, Pushpam D, Kumar L. Isolated central nervous system relapse of multiple myeloma post autologous stem cell transplant-A rare presentation. Leuk Res Rep. 2020. 14: 100207

23. Nishimura KK, Barlogie B, van Rhee F, Zangari M, Walker BA, Rosenthal A. Long-term outcomes after autologous stem cell transplantation for multiple myeloma. Blood Adv. 2020. 4: 422-31

24. Paludo J, Painuly U, Kumar S, Gonsalves WI, Rajkumar V, Buadi F. Myelomatous involvement of the central nervous system. Clin Lymphoma Myeloma Leuk. 2016. 16: 644-54

25. Petersen SL, Wagner A, Gimsing P. Cerebral and meningeal multiple myeloma after autologous stem cell transplantation. A case report and review of the literature. Am J Hematol. 1999. 62: 228-33

26. Rezvani A, Shahriarirad R, Fallahi MJ, Zeighami A. Extramedullary relapse of immunoglobulin A-kappa myeloma manifesting as plasmacytoma of the pleura without bone marrow involvement and following autologous bone marrow transplant: A case report. J Med Case Rep. 2023. 17: 42

27. Rosiñol L, Beksac M, Zamagni E, Van de Donk NW, Anderson KC, Badros A. Expert review on soft-tissue plasmacytomas in multiple myeloma: Definition, disease assessment and treatment considerations. Br J Haematol. 2021. 194: 496-507

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29. Seftel MD, Maguire J, Voss N, Woodhurst WB, Dalal BI, Shepherd JD. Intra-cerebral relapse following prolonged remission after autologous stem cell transplantation for multiple myeloma. Leuk Lymphoma. 2002. 43: 2399-403

30. Sinnott BP, Hatipoglu B, Sarne DH. Intrasellar plasmacytoma presenting as a non-functional invasive pituitary macro-adenoma: Case report & literature review. Pituitary. 2006. 9: 65-72

31. Varettoni M, Corso A, Pica G, Mangiacavalli S, Pascutto C, Lazzarino M. Incidence, presenting features and outcome of extramedullary disease in multiple myeloma: A longitudinal study on 1003 consecutive patients. Ann Oncol. 2010. 21: 325-30

32. Varga G, Mikala G, Gopcsa L, Csukly Z, Kollai S, Balázs G. Multiple myeloma of the central nervous system: 13 Cases and review of the literature. J Oncol. 2018. 2018: 3970169

33. Yue X, He D, Zheng G, Yang Y, Han X, Li Y. Analysis of high-risk extramedullary relapse factors in newly diagnosed MM patients. Cancers (Basel). 2022. 14: 6106

34. Zeiser R, Deschler B, Bertz H, Finke J, Engelhardt M. Extramedullary vs medullary relapse after autologous or allogeneic hematopoietic stem cell transplantation (HSCT) in multiple myeloma (MM) and its correlation to clinical outcome. Bone Marrow Transplant. 2004. 34: 1057-65

Abstract

Background: Brachial artery pseudoaneurysms (PSAs) are a rare complication of trauma and medical intervention, estimated to constitute 0.3–0.7% of all PSAs. Although neurologic symptoms are common in patients undergoing hemodialysis, direct nerve compression by large PSAs is rare.

Case Description: We report a case of median nerve compression by a brachial artery PSA treated by PSA resection and distal nerve transfer of the extensor carpi radialis brevis nerve to the anterior interosseous nerve.

Conclusion: This case illustrates the successful use of distal nerve transfers for the treatment of median neuropathy secondary to brachial PSA. In addition, this case highlights the importance of imaging before any exploratory nerve surgery in the setting of a mass and/or prior vascular procedure. Embarking on a nerve release/ repair surgery in the absence of a vascular surgeon would be disastrous.

Keywords: Brachial artery pseudoaneurysm, Nerve compression, Nerve transfer

INTRODUCTION

Brachial artery pseudoaneurysms (PSAs) are a rare complication of trauma and medical intervention, estimated to constitute 0.3–0.7% of all PSAs.[ 1 ] Literature on noninfectious PSAs associated with hemodialysis access is limited to case studies and small case series. Chemla et al. report an overall incidence of 4.5% in a population of patients with arteriovenous fistulas for hemodialysis access.[ 2 ] Although neurologic symptoms are common in patients undergoing hemodialysis, direct nerve compression by large PSA is rare.[ 3 - 10 ] We report a case of median nerve compression by a brachial artery PSA treated by PSA resection and distal nerve transfer.

CASE PRESENTATION

A 69-year-old male presented to our multidisciplinary brachial plexus clinic with severe proximal median neuropathy causing paresthesia in the median nerve distribution, benediction hand [ Figure 1a ], and weakness of his extrinsic finger flexors, flexor digitorum profundus (FDP) to his index finger, flexor pollicis longus (FPL), and median innervated hand intrinsics. His past medical history was significant for end-stage renal failure requiring dialysis through a right brachiocephalic fistula. Nine months prior, during an attempt to cannulate his fistula for dialysis access, he experienced significant pain and paresthesia in the median nerve distribution. Initially, he did not have symptoms of weakness. Over three months, he experienced progressive numbness and weakness in the hand.

Figure 1:

(a) Preoperative presentation of the patient showing physical examination consistent with benediction hand. (b) Preoperative photograph showing compressible swelling in the upper arm. A positive Tinel’s sign over the expected course of the median nerve in the upper arm was also noted to be present.

On initial physical examination, he demonstrated Medical Research Council (MRC) Grade 4/5 pronator teres (PT) strength, 0/5 FPL, 0/5 abductor pollicis brevis (APB), 0/5 flexor digitorum superficialis (FDS), 0/5 FDP to index finger, and 4/5 FDP to the remaining fingers. Sensory examination was significant for diminished sensation to light touch in the palmar cutaneous branch distribution and 10–14 mm static two-point discrimination in the median nerve distribution of the fingers. In addition, he had a positive Tinel sign and sensory collapse test over the carpal tunnel and PT. He was noted to have compressible swelling over the medial upper arm [ Figure 1b ] with a positive Tinel sign over the expected course of the median nerve proximal to the elbow.

Electrodiagnostic studies were obtained and confirmed severe median neuropathy proximal to the takeoff of the PT. These electrodiagnostic findings suggested a severe median neuropathy localizing proximal to the elbow. In addition, low compound muscle action potentials (CMAPs) and absent sensory nerve conduction suggested some degree of axonal injury.

Magnetic resonance imaging and magnetic resonance angiography were also obtained and showed a 4.6 cm × 4.2 cm × 6.7 cm mass, likely a PSA within the brachial artery, compressing the median nerve [ Figure 2 ].

Figure 2:

A 4.2 × 5.2 × 6.7 cm mass located at the level of the mid humeral shaft abutting the medial aspect of the brachial artery (dashed arrow). The median nerve (solid arrow) demonstrates thickening, edema, and abnormal enhancement at the level of the mass through the distal humerus. The median nerve is compressed as it contours the anterior margin of the mass.

In conjunction with vascular surgery who planned to resect and repair the brachial artery PSA, we decided to proceed with decompression of the median nerve in the upper arm, forearm, and carpal tunnel, along with distal extensor carpi radialis brevis (ECRB) to anterior interosseous nerve (AIN) transfer to restore pinch. Intraoperatively, we were able to successfully resect and reconstruct the brachial artery PSA [ Figures 3a and b ]. The median nerve in the upper arm was noted to have significant circumferential adhesions extending distally to the elbow. Circumferential release of the median nerve was performed in the upper arm, at the PT, and distally at the carpal tunnel. After the release of adhesions, the fascicles appeared scarred but in continuity. Nerve action potentials were negative. However, due to the long segment of unhealthy-looking nerve, it was decided to do the nerve transfer and not cut and graft since the grafts would have been longer than 12 cm. In addition, distal ECRB to AIN transfer was performed with coaptation using an 8-0 nylon suture and nerve wrap [ Figure 4 ].

Figure 3:

(a and b) Pseudoaneurysm (PSA) before and after opening.

Figure 4:

Intraoperative photograph showing the proximal extensor carpi radialis brevis (ECRB) branch with a tension-free coaptation to the anterior interosseous nerve (AIN) with nerve wrap.

At 1-year follow-up, the patient has regained some function of the median nerve. On physical examination, he was found to have MRC grade 2/5 FPL strength, FDS 2/5, FDP to the index 2/5, and APB 0/5. On transcutaneous electrical nerve stimulation examination, he notes sensation in the palmar cutaneous branch distribution to be 60% of the contralateral side and sensation in the fingers in the median nerve distribution around 40% of the contralateral side. He is currently planning to undergo opponensplasty to restore thumb opposition.

DISCUSSION

Previous studies reporting on the treatment of arterial PSA causing nerve compression have focused on decompression by surgical management of the PSA. Papadopoulos et al. reported two cases of median neuropathy associated with a brachial artery aneurysm in which neurological symptoms completely resolved after resection of the compressive PSA.[ 7 ] Other case studies also reported complete resolution of symptoms with resection of the PSA alone.[ 4 , 8 , 9 ] However, these studies report on cases that were treated acutely (

We report a case of delayed presentation of severe proximal median neuropathy with evidence of axonal loss on electrodiagnostic studies. In this case, it is unclear whether axonal loss was induced by prolonged unrecognized compression or by direct injury during dialysis access. However, we elected to perform distal nerve transfers due to the low likelihood of spontaneous motor recovery after this prolonged period of denervation and the distance from the level of injury to the target. This is further supported by the lack of recovery of the median innervated hand intrinsics one year after PSA resection and median nerve release.

CONCLUSION

To our knowledge, this is the first reported case of the successful use of distal nerve transfers for the treatment of median neuropathy secondary to brachial PSA. In addition, this case illustrates the importance of imaging before any exploratory nerve surgery in the setting of a mass and/or prior vascular procedure. Embarking on a nerve release/ repair surgery in the absence of a vascular surgeon would have been a disaster in this case.

Ethical approval

Institutional Review Board approval is not required.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

Use of artificial intelligence (AI)-assisted technology for manuscript preparation

The authors confirm that there was no use of artificial intelligence (AI)-assisted technology for assisting in the writing or editing of the manuscript and no images were manipulated using AI.

Disclaimer

The views and opinions expressed in this article are those of the authors and do not necessarily reflect the official policy or position of the Journal or its management. The information contained in this article should not be considered to be medical advice; patients should consult their own physicians for advice as to their specific medical needs.

References

1. Borghese O, Pisani A, DiCenta Isabelle. Treatment of iatrogenic pseudoaneurysm of the brachial artery: Case report and literature review. Vascu Dis Manag. 2019. 16: E87-91

2. Chemla E, Nortley M, Morsy M. Brachial artery aneurysms associated with arteriovenous access for hemodialysis. Semin Dial. 2010. 23: 440-4

3. Ergungor MF, Kars HZ, Yalin R. Median neuralgia caused by brachial pseudoaneurysm. Neurosurgery. 1989. 24: 924-5

4. Hatano M, Kitajima I, Yamamoto S, Nakamura M, Isawa K, Suwabe T. Dialysis-related carpal tunnel syndrome in the past 40 years. Clin Exp Nephrol. 2022. 26: 68-74

5. Lijftogt N, Cancrinus E, Hoogervorst EL, Mortel RH, Vries JP. Median nerve neuropraxia by a large false brachial artery aneurysm. Vascular. 2014. 22: 378-80

6. Lobo J, Ferreira MC, Ramos PN. Pseudoaneurysm of brachial artery: A rare cause of median nerve compression. Trauma Case Rep. 2018. 14: 8-10

7. Papadopoulos SM, McGillicuddy JE, Messina LM. Pseudoaneurysm of the inferior gluteal artery presenting as sciatic nerve compression. Neurosurgery. 1989. 24: 926-8

8. Rahimizadeh A, Davaee M, Shariati M, Rahimizadeh S. Posterior tibial neuropathy secondary to pseudoaneurysm of the proximal segment of the anterior tibial artery with delayed onset. J Brachial Plexus Peripher Nerve Inj. 2018. 13: e15-9

9. Santiago FR, Villares PJ, Fernández JM, Parra FM. Median nerve compression by an iatrogenic pseudoaneurysm of brachial artery. Eur J Radiol Extra. 2004. 50: 75-6

10. Villanueva AG, de Oña IR, de Oya AS. Median nerve compression caused by brachial pseudoaneurysm: Report of two cases and review of the literature. J Hand Microsurg. 2016. 8: 109-10

Abstract

Background: Antiphospholipid syndrome is a complex autoimmune condition associated with the formation of recurrent thrombosis in any vascular bed throughout the body. Jugular vein thrombosis is very rare with only a 0.9% occurrence and is not typically associated with cerebrospinal rhinorrhea as a result of raised intracranial pressure.

Case Description: A 54-year-old patient presented with a 9-month history of cerebrospinal fluid (CSF) rhinorrhea and headache on a background of antiphospholipid syndrome. Investigations showed a superior vena cava (SVC) and right internal jugular vein (IJV) obstruction with moderately elevated intracranial venous pressures. Her magnetic resonance imaging (MRI) brain was consistent with a CSF leak. The patient underwent successful endovascular stenting of her obstructed SVC and right IJV followed by surgical repair of a herniating meningocele in the posterior left ethmoid air cells.

Conclusion: CSF rhinorrhea is uncommon and never previously reported associated with SVC thrombosis induced by antiphospholipid syndrome. A combination of endovascular techniques and surgical repair is recommended for this challenging presentation.

Keywords: Antiphospholipid syndrome, Cerebrospinal fluid rhinorrhea, Endovascular stenting, Superior vena cava obstruction

INTRODUCTION

Cerebrospinal fluid (CSF) rhinorrhea is an uncommon condition classified as either traumatic or nontraumatic and is the result of a pathway formed between the subarachnoid space and the nasopharynx or nasal cavity. Traumatic causes include blunt force injuries and surgical complications.[ 4 , 6 ] Our patient gave no history of trauma. Being an overweight female, she was a classic presentation for raised intracranial pressure (ICP) secondary to benign intracranial hypertension, but she had no intracranial venous sinus abnormalities.

Antiphospholipid syndrome is a complex autoimmune condition with complications, including the formation of recurrent thrombosis in any vascular bed throughout the body.[ 1 , 5 ] It can have severe and life-threatening outcomes, due to the recurring nature of the associated thrombotic events, which necessitates life-long anti-coagulation.[ 1 ] Deep vein thrombosis is the most common peripheral thrombosis with the Euro-Phospholipid project reporting a 38.9% occurrence within their cohort with jugular vein thrombosis being much rarer with only a 0.9% occurrence.[ 5 ] Our patient’s background included antiphospholipid syndrome with chronic thrombosis of the superior vena cava (SVC) and right internal jugular vein (IJV).

We present an unusual case of a patient who presented with CSF rhinorrhea secondary to raised ICP as a result of an SVC and right IJV thrombosis due to antiphospholipid syndrome.

CASE PRESENTATION

A 54-year-old female presented with a 9-month history of spontaneous CSF rhinorrhea associated with blurred vision and headache. This is on a background of antiphospholipid syndrome for which she requires life-long anti-coagulation with warfarin. She also has a history of several deep vein thromboses and pulmonary emboli as well as a near total, chronic thrombosis of her SVC.

Investigations and treatment

She was admitted to the hospital and investigated with an MRI brain demonstrating fluid in the left posterior ethmoid air cells as well as optic nerve sheath enlargement bilaterally, consistent with intracranial hypertension and CSF leak [ Figure 1 ]. A venogram and manometry were also performed, which confirmed the presence of SVC obstruction with extensive venous collaterals [ Figure 2 ] as well as a separate near-complete obstruction of the right IJV at the level of C4 downward. Moderately elevated ICPs were also reported (superior sagittal sinus 22 mmHg), with no intracranial venous gradient and no venous sinus abnormality.

Figure 1:

Magnetic resonance imaging of brain and sinuses showing cerebrospinal fluid leak into the left posterior ethmoidal air cells (arrow).

Figure 2:

Obstructed superior vena cava and extensive collaterals (arrow).

Based on the clinical and radiological findings, a multidisciplinary decision was made to proceed with a combined interventional neuroradiology procedure to stent the SVC and right IJV before endoscopic endonasal repair of the skull base defect.

Under general anesthetic, a 6F Cook Shuttle sheath was introduced through the right common femoral vein. A second venotomy was performed with a 5F short sheath placed in the right IJV and a venography showed severe stenosis of the right IJV at the level of C4 with extensive collaterals flowing into a distended azygos vein. A second severe stenosis was noted in the SVC above the junction with the azygos vein. A communicating channel between the upper and lower SVC was selected from above with a 035 guide wire and an Amplatz super stiff exchange wire was passed to the right common iliac vein. A 10F short sheath was exchanged into the right common femoral vein and a Gooseneck 15 mm device was used to ensnare the exchange wire. Graduated balloon angioplasty (6 mm × 40 mm and 10 mm × 40 mm Advance balloons) of the stenosed SVC was performed and a 16 mm × 40 mm Venovo stent was subsequently deployed over the narrowed segment. A post-stent angioplasty was also completed with a 14 mm × 40 mm XXL balloon. The stenosed section of the right IJV also underwent balloon dilatation (12 mm × 40 mm Advance balloon).

Successful stenting of the patient’s SVC and balloon dilatation of her right IJV [ Figure 3 ] resulted in an improvement in her headaches and blurred vision. The CSF rhinorrhea persisted, and she proceeded to endoscopic endonasal repair of the CSF leak, after 2–3 days.

Figure 3:

Patent superior vena cava post stenting and balloon dilatation.

A preoperative intrathecal injection of fluorescein was used to localize the origin of the leak. An endoscopic approach was employed with a left antrostomy and bilateral ethmoidectomy performed to access the posterior ethmoid space. The source of the leak was identified as a herniating meningocele in the left posterior ethmoid [ Figure 4 ]. The meningocele was reduced and resected with a subsequent vascularized septal mucosal flap raised and rotated to conceal the defect.

Figure 4:

Endoscopic endonasal view of herniating meningocele in the left posterior ethmoid space.

Outcome and follow-up

Following the operation, the patient recovered well and her symptoms continue to improve. There was no further rhinorrhea. She was commenced on a heparin infusion postoperatively and then converted to aspirin and warfarin. She was followed up 6 weeks post-intervention with complete resolution of her CSF leak as well as headache and vision. Her postoperative MRI demonstrated an adequate septal flap placement and replacement of the previous posterior ethmoid fluid with diffuse postoperative changes of chronic sinusitis. Contrast computed tomography of the chest confirmed the patency of the SVC stent. She was returned to her hematologist for the long-term management of her antiphospholipid syndrome.

DISCUSSION

CSF rhinorrhea is an uncommon presentation that can result in serious complications such as bacterial meningitis if left untreated.[ 4 ] There are many underlying etiologies and a thorough understanding of the cause is required to guide treatment decisions.[ 9 ]

There has not been a published case describing a patient whose antiphospholipid syndrome was associated with the incidence of CSF rhinorrhea. This may be due to the rarity of chronic nonmalignant SVC syndrome induced by antiphospholipid syndrome resulting in CSF rhinorrhea.[ 5 ] This downstream occlusion impairs venous outflow, thus disrupting CSF absorption.[ 3 ] This can lead to an abnormally increased ICP, shown to be associated with nontraumatic spontaneous CSF rhinorrhea.[ 4 ]

Surgical endoscopic endonasal repair is the preferred treatment method with good outcomes for most causes of CSF rhinorrhea.[ 2 , 6 , 9 ] However, we considered that surgical treatment would not alleviate our patient’s increased ICP due to the presence of chronic SVC obstruction. Endovascular stenting is an effective and appropriate first-line intervention for nonmalignant SVC syndrome and many patients with benign intracranial hypertension. Studies have shown short-term efficacy for endovascular treatment but there is the absence of substantial data for long-term outcomes with a high likelihood for repeat intervention.[ 7 , 8 ] Our patient tolerated the procedure well with immediate improvement in symptoms.

CONCLUSION

CSF rhinorrhea secondary to SVC obstruction resulting from antiphospholipid syndrome has never been reported in the literature. Surgery is the main treatment modality for CSF leak; however, in this case, stent treatment for the SVC obstruction was also required to alleviate the patient’s raised ICP. Therefore, a combined endovascular and surgical approach is recommended for this challenging presentation.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

Use of artificial intelligence (AI)-assisted technology for manuscript preparation

The author(s) confirms that there was no use of Artificial Intelligence (AI)-Assisted Technology for assisting in the writing or editing of the manuscript and no images were manipulated using the AI.

Disclaimer

The views and opinions expressed in this article are those of the authors and do not necessarily reflect the official policy or position of the Journal or its management. The information contained in this article should not be considered to be medical advice; patients should consult their own physicians for advice as to their specific medical needs.

References

1. Amiral J, Peyrafitte M, Dunois C, Vissac AM, Seghatchian J. Anti-phospholipid syndrome: Current opinion on mechanisms involved, laboratory characterization and diagnostic aspects. Transfus Apher Sci. 2017. 56: 612-25

2. Banu MA, Kim JH, Shin BJ, Woodworth GF, Anand VK, Schwartz TH. Low-dose intrathecal fluorescein and etiology-based graft choice in endoscopic endonasal closure of CSF leaks. Clin Neurol Neurosurg. 2014. 116: 28-34

3. Ducros A, Biousse V. Headache arising from idiopathic changes in CSF pressure. Lancet Neurol. 2015. 14: 655-68

4. Flint PW, Haughey BH, Lund VJ, Robbins KT, Thomas JR, Francis HW, editors. Cummings otolaryngology: Head and neck surgery. Netherlands: Elsevier; 2020. p.

5. Gómez-Puerta JA, Cervera R. Diagnosis and classification of the antiphospholipid syndrome. J Autoimmun. 2014. 48-49: 20-5

6. Gonen L, Monteiro E, Klironomos G, Alghonaim Y, Vescan A, Zadeh G. Endoscopic endonasal repair of spontaneous and traumatic cerebrospinal fluid rhinorrhea: A review and local experience. Neurosurg Clin N Am. 2015. 26: 333-48

7. Rizvi AZ, Kalra M, Bjarnason H, Bower TC, Schleck C, Gloviczki P. Benign superior vena cava syndrome: Stenting is now the first line of treatment. J Vasc Surg. 2008. 47: 372-80

8. Sfyroeras GS, Antonopoulos CN, Mantas G, Moulakakis KG, Kakisis JD, Brountzos E. A review of open and endovascular treatment of superior vena cava syndrome of benign aetiology. Eur J Vasc Endovasc Surg. 2017. 53: 238-54

9. Sharma SD, Kumar G, Bal J, Eweiss A. Endoscopic repair of cerebrospinal fluid rhinorrhoea. Eur Ann Otorhinolaryngol Head Neck Dis. 2016. 133: 187-90

Abstract

Background: Temporal bone squamous cell carcinoma (TBSCC) is a very rare condition. The prognosis is dismal for advanced tumors. Due to its rarity, information in the literature is scarce. Here, we report a unique case of TBSCC with cerebellar invasion and hydrocephalus.

Case Description: A 46-year-old reported right-sided hearing loss and a painful right retroauricular mass for 4 months. Magnetic resonance imaging revealed a 8.7 × 7.6 × 6.4 cm mass invading the right temporal and occipital bones. After a biopsy and 3 surgical procedures over 6 months, the diagnosis of TBSCC was obtained. Due to invasion of the cerebellar tissue and obstructive hydrocephalus, a ventriculoperitoneal shunt was performed. The patient was referred for adjuvant radiotherapy. However, palliative care was initiated due to tumor progression.

Conclusion: We report a case of advanced TBSCC with poor prognosis despite surgical treatment and radiotherapy. More data are necessary to provide new and better treatment to these patients.

Keywords: Cerebellum, Hydrocephalus, Squamous cell carcinoma, Temporal bone

INTRODUCTION

Temporal bone squamous cell carcinoma (TBSCC) is a rare head and neck tumor with aggressive behavior.[ 8 ] Squamous cell carcinoma (SCC) represents around 40% of all temporal bone tumors and accounts for 0.2% of the total head and neck malignancy.[ 5 , 18 ] The worldwide annual incidence is estimated at approximately 1–6 cases per million.[ 14 ] Late stage at presentation is common, resulting in delayed diagnosis and poor prognosis.[ 8 , 11 ] The periauricular soft tissues, parotid gland, temporomandibular joint, and mastoid are often affected by tumor progression. In advanced stages, the carotid canal, jugular foramen, dura mater, middle, and posterior cranial fossae can be invaded.[ 2 , 7 ] The incidence of cerebellar invasion is not known, and after careful literature review, there were no cases reported. Our aim is to report a challenging and unique case of aggressive TBSCC presenting with cerebellar tissue invasion and hydrocephalus.

CASE DESCRIPTION

A 46-year-old longshoreman went to a University Hospital reporting right-sided hearing loss and a painful right retroauricular mass, which was noticed 4 months earlier after being unable to wear his safety helmet. He also reported rapid weight loss of 4 kg in 1 month. His only previous comorbidity was bilateral occupational hypoacusis. Clinical examination revealed a right retroauricular mass, in addition to right anacusis and left hypoacusis. On careful inspection, there was no evidence of skin lesions other than diffuse milia [ Figure 1 ]. He was admitted to the hospital and a brain magnetic resonance imaging (MRI) showed an expansive mass measuring 8.7 × 7.6 × 6.4 cm with heterogeneous enhancement, areas of hemorrhage, and calcification. The lesion invaded the mastoid, petrous part of the right temporal bone, and squamous part of the occipital bone with no evidence of involvement of the cerebellar tissue [ Figure 2 ]. Chest, abdomen, and pelvis computed tomography (CT) did not reveal any other mass, tumor, or nodal involvement.

Figure 1:

The patient exhibited multiple subepidermal keratin cysts that appeared as small, firm white papules in varying numbers, consistent with milia.

Figure 2:

First magnetic resonance imaging of the brain showing a huge lesion in the temporal bone. (a) Axial T1-weighted with contrast exhibiting temporal bone mass with peripheral contrast enhancement – right side. (b) Axial T2-weighted image shows hyperintense signal abnormality in the mastoid part of the temporal bone. The occipital bone was invaded. (c) Contrast-enhanced axial T1-weighted showing a large temporal bone tumor on the right side. (d) The mass was predominantly hyperintense on the T2-weighted image.

A tumor biopsy was performed and the histopathological findings were negative for malignancy and infection screening (GeneXpert – tuberculosis, Gram staining, and mycological culture). After that, due to the rapid tumor growth, he underwent another 2 surgical partial resections with positive margins over a period of 5 months. The tumor was not completely resected due to invasion of petrous bone and the risk of injury to the facial nerve. In the first surgical approaches, samples from the temporal bone tumor were predominantly represented by keratin sheets with parakeratosis, sometimes forming horny pearls, with extensive degenerated areas. Foci of preserved squamous epithelium were observed, showing areas with enlarged nuclei, but without signs of stromal invasion and criteria for malignancy. The sample was also subjected to an immunohistochemical study with p16, which was negative.

Two months after the last surgery, a new brain MRI revealed local recurrence and invasion of the cerebellar tissue [ Figure 3 ]. Another partial resection was performed [ Figure 4 ]. The specimen exhibited fragments of squamous epithelium displaying cells with moderate to marked pleomorphism, with an enlarged and irregular nucleus, and frequent atypical mitotic figures, superficially infiltrating the cerebellar cortex and fibroconnective tissue. The rest of the material was similar to that observed in the first surgeries. In view of all these findings, it was considered to be squamous carcinoma. Pathology samples are collected in Figure 5 . The Modified Pittsburgh Moody Scale was compatible with T4N0M0 (grade IV) TBSCC.

One month after the last procedure, the patient exhibited a recurrent pattern of bulging deformity with a new holocranial headache. He did not present focal deficit symptoms. The new MRI revealed infiltration of the mass into the cerebellar region, accompanied by compression of the fourth ventricle and obstructive hydrocephalus [ Figure 6 ]. Therefore, he underwent a left parietal ventriculoperitoneal shunt (VPS). Radiotherapy was started 6 weeks after VPS. At present, his surgical wound is open [ Figure 7 ] and he is being monitored by palliative care.

Figure 3:

Contrast-enhanced axial T1-weighted brain magnetic resonance imaging shows temporal bone tumor invading the cerebellar tissue with dislocation of the IV ventricle.

Figure 4:

Microsurgical resection of the tumor invading the cerebellar tissue. (a) The tumor was identified after the dural opening. (b) Circumferential dissection between the tumor and normal cerebellum parenchyma. (c) Tumor removal using a microdissector.

Figure 5:

Temporal bone tumor samples: (a) Hematoxylin and Eosin (H&E) stain (×200). Squamous epithelium with some maturation, keratin sheets with parakeratosis, sometimes forming horny pearls. (b) H&E stain (×400). Foci of preserved squamous epithelium showing areas with enlarged nuclei, but no signs of stromal invasion. (c) H&E stain (×100). Sample from the cerebellar region. Squamous epithelium presenting poorly cohesive cells with moderate to marked pleomorphism, superficially infiltrating the cerebellar cortex. (d) H&E stain (×400). Sample from the dural region. Squamous epithelium with an enlarged and irregular nucleus, and frequent atypical mitotic figures infiltrating fibroconnective tissue.

Figure 6:

Comparison between the first brain magnetic resonance imaging (MRI) (fluid-attenuated inversion recovery [FLAIR] sequence) and head computed tomography (CT) after symptoms of hydrocephalus. (a) Preoperative MRI-FLAIR shows a normal lateral ventricle without radiological signs of hydrocephalus. Normal anatomical variation is observed: Cavum septum pellucidum. (b) Head CT showing enlargement of the lateral ventricles and transependymal edema: Low-density change on CT around the margins of the ventricles.

Figure 7:

Final appearance of the wound after four attempts at tumor resection and adjuvant radiotherapy. The surgical wound is open and the patient was referred for palliative care.

DISCUSSION

Temporal bone malignancies are rare, constituting only 0.2% of all head and neck malignancies. The global annual incidence is estimated to be 1.3 cases per million, and it is detected in approximately one in every 5000–20000 patients presenting with otological complaints.[ 4 , 14 ] SCC is responsible for even fewer cases, despite representing the most prevalent histological type among temporal bone tumors.[ 9 ] TBSCC constitutes 60–80% of primary tumors in the temporal bone; yet, it comprises only about 40% of all temporal bone tumors. Across studies on TBSCC, around 60% of patients are men, with the most frequent age of diagnosis falling between 60 and 69 years.[ 5 , 17 ]

In contrast to other head and neck cancers, the risk of primary TBSCC does not appear to be significantly elevated by tobacco and alcohol consumption. Instead, prior radiation emerges as a notable risk factor. In addition, chronic otitis media, otitis externa, and cholesteatoma have been identified as potential contributors to primary TBSCC.[ 1 , 12 ] Recent studies have indicated the existence of human papillomavirus (HPV) genetic material in TBSCC tumors. Case reports have even detailed the malignant transformation of benign HPV papillomas. However, definitive conclusions are still pending. To date, in our patient samples, the expression of biomarker p16 (highly correlated with HPV infection in head and neck SCC) was negative. Sun exposure emerges as a significant risk factor for TBSCC, especially since many of these tumors originate from the skin around the ear. Nonetheless, the precise cause for most cases remains elusive.[ 10 ]

The principal clinical indicators of temporal bone malignancies include otalgia, otorrhea, and hearing loss. Gradually, signs indicating more serious conditions, such as facial weakness or the presence of a parotid or neck mass, may manifest.[ 5 ] Establishing a diagnosis of TBSCC requires a mandatory biopsy. The presence of inflammation or edema can pose a challenge to obtain an accurate biopsy. Consequently, a deep tissue biopsy is often essential to ensure a conclusive and positive outcome.[ 13 ] In our case, it was difficult to obtain a definitive diagnosis due to pathological samples with large amounts of keratin and differentiated tissue. The diagnosis was only confirmed after the last resection including tumoral samples invading the cerebellar tissue.

Given that a substantial portion of the temporal bone is typically not directly visible, imaging studies play a crucial role in both staging and management. CT scan and MRI provide complementary information about the extent of the tumor.[ 8 ] Accurately assessing tumor spread is essential, as the degree of local tumor extension stands out as a key prognostic factor for TBSCC.[ 3 ]

The main staging system used for temporal bone malignancies is the Pittsburgh Staging System (PSS). The PSS adopts the familiar tumor-node-metastasis (TNM) format, relying on CT findings such as bone external auditory canal (EAC) destruction, surrounding soft-tissue infiltration, and involvement of medial bony temporal structures. This approach allows the categorization of patients into treatment and prognostic groups. TNM can be transformed into the standard four-stage system used for other head and neck cancers, with the exception that any temporal bone malignancy with lymph node involvement is automatically classified as stage IV.[ 3 ] This conversion reflects the more favorable prognosis for tumors limited to the EAC (T1 or T2 disease) and the worse prognosis for tumors affecting the middle ear or mastoid (specific T3 or T4 disease).[ 9 ]

Due to the rare occurrence of TBSCC, there is a paucity of adequate randomized trials, leading to wide variation in the extent of resection among different authors and institutions. In general, indicators of low disease-specific survival rates in patients with TBSCC include nodal involvement, poorly differentiated histology, carotid involvement, positive margins, stage T4, dural invasion, and temporomandibular joint invasion.[ 10 ] The standard approach for the oncological management of TBSCC is surgical intervention. Three resection options are available: Lateral temporal bone resection, subtotal temporal bone resection, and total temporal bone resection. Each of these procedures takes advantage of the anatomy of the temporal bone to establish tumor-free margins and can be executed either en bloc or in a piecemeal manner.[ 8 ]

Regarding surgical treatment, we considered the significant morbidity associated with total resection of the temporal bone and the absence of proven survival benefit.[ 16 ] Most radical procedures result in the loss of sensorineural hearing and often compromise facial nerve function.[ 11 ] Furthermore, removing the ear canal and even the auricular pavilion results in significant cosmetic impact.[ 15 , 16 ] In our case, the decision of a piecemeal partial resection was previously shared with the patient that agreed with the non-radical procedure, especially preserving his facial nerve function.

Radiotherapy for TBSCC is typically administered in the adjuvant postoperative period. Some indications include lymph node metastasis, perineural invasion, positive margins, recurrent tumors, and bone invasion.[ 14 ] Numerous studies have also demonstrated better survival rates with adjuvant radiotherapy compared to surgery alone, especially for T2 and higher tumors. On the other hand, there are limited benefits seen with postoperative radiotherapy for patients with completely resected T1 tumors.[ 6 ]

CONCLUSION

TBSCC is a very aggressive tumor associated with poor prognosis. We reported a unique case coursing with cerebellar invasion and hydrocephalus despite multiple surgical resections. There was also tumor progression after radiotherapy. More studies on this topic are necessary to provide new and better treatment to these patients.

Ethical approval

The Institutional Review Board approval is not required.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent.

Financial support and sponsorship

Fundação de Amparo à Pesquisa do Estado do Rio de Janeiro (FAPERJ) and Center of High Complexity Neurosurgery Intern Patients (NIPNAC).

Conflicts of interest

There are no conflicts of interest.

Use of artificial intelligence (AI)-assisted technology for manuscript preparation

The authors confirm that there was no use of artificial intelligence (AI)-assisted technology for assisting in the writing or editing of the manuscript and no images were manipulated using AI.

Disclaimer

The views and opinions expressed in this article are those of the authors and do not necessarily reflect the official policy or position of the Journal or its management. The information contained in this article should not be considered to be medical advice; patients should consult their own physicians for advice as to their specific medical needs.

References

1. Allanson BM, Low TH, Clark JR, Gupta R. Squamous cell carcinoma of the external auditory canal and temporal bone: An update. Head Neck Pathol. 2018. 12: 407-18

2. Arena S, Keen M. Carcinoma of the middle ear and temporal bone. Am J Otol. 1998. 9: 351-6

3. Arriaga M, Curtin H, Takahashi H, Hirsch BE, Kamerer DB. Staging proposal for external auditory meatus carcinoma based on preoperative clinical examination and computed tomography findings. Ann Otol Rhinol Laryngol. 1990. 99: 714-21

4. Conley JJ. Cancer of the middle ear and temporal bone. N Y State J Med. 1974. 74: 1575-9

5. Gidley PW, DeMonte F. Temporal bone malignancies. Neurosurg Clin N Am. 2013. 24: 97-110

6. Kunst H, Lavieille JP, Marres H. Squamous cell carcinoma of the temporal bone: Results and management. Otol Neurotol. 2008. 29: 549-52

7. Leonetti JP, Smith PG, Kletzker GR, Izquierdo R. Invasion patterns of advanced temporal bone malignancies. Am J Otol. 2000. 122: 882-6

8. Lovin BD, Gidley PW. Squamous cell carcinoma of the temporal bone: A current review. Laryngoscope Investig Otolaryngol. 2019. 4: 684-92

9. Madsen AR, Gundgaard MG, Hoff CM, Maare C, Holmboe P, Knap M. Cancer of the external auditory canal and middle ear in Denmark from 1992 to 2001. Head Neck. 2008. 30: 1332-8

10. Masterson L, Winder D, Marker A, Sterling JC, Sudhoff HH, Moffat DA. Investigating the role of human papillomavirus in squamous cell carcinoma of the temporal bone. Head Neck Oncol. 2013. 5: 22-9

11. Mazzoni A, Danesi G, Zanoletti E. Primary squamous cell carcinoma of the external auditory canal: Surgical treatment and long-term outcomes. Acta Otorhinolaryngol Ital. 2014. 34: 129-37

12. McRackan TR, Fang TY, Pelosi S, Rivas A, Dietrich MS, Wanna GB. Factors associated with recurrence of squamous cell carcinoma involving the temporal bone. Ann Otol Rhinol Laryngol. 2014. 123: 235-9

13. Moffat DA, Wagstaff SA. Squamous cell carcinoma of the temporal bone. Curr Opin Otolaryngol Head Neck Surg. 2003. 11: 107-11

14. Moody SA, Hirsch BE, Myers EN. Squamous cell carcinoma of the external auditory canal: An evaluation of a staging system. Am J Otol. 2000. 21: 582-8

15. Ngu CY, Mohd Saad MS, Tang IP. Temporal bone squamous cell carcinoma: A change in treatment. Med J Malaysia. 2021. 76: 725-30

16. Prasad S, Janecka IP. Efficacy of surgical treatment for squamous cell carcinoma of the temporal bone: A literature review. Otolaryngol Head Neck Surg. 1994. 110: 270-80

17. Yin M, Ishikawa K, Honda K, Arakawa T, Harabuchi Y, Nagabashi T. Analysis of 95 cases of squamous cell carcinoma of the external and middle ear. Auris Nasus Larynx. 2006. 33: 251-7

18. Zanoletti E, Marioni G, Stritoni P, Lionello M, Giacomelli L, Martini A. Temporal bone squamous cell carcinoma: Analyzing prognosis with univariate and multivariate models. Laryngoscope. 2014. 124: 1192-8

Abstract

Background: Spinal epidural abscess (SEA) is a rare condition that may result in catastrophic outcomes. On the other hand, calcium pyrophosphate (CPP) deposition disease (CPPD) causes inflammatory arthritis. Spinal involvement of a crystal-induced inflammation caused by CPPD is also common. Surgery is a common risk factor for both SEA and CPPD; however, the postoperative acute onset of SEA complicated with CPPD is extremely rare.

Case Description: A man in his 70s presented to our hospital, complaining of right upper limb weakness, loss of dexterity, and gait disturbance. The diagnosis of cervical spondylotic myelopathy was made, and he performed laminectomy at C3, C4, and C5 levels. Four days after the laminectomy, he suffered from acute neck pain, weakness, and hypoesthesia in his arms and legs. Magnetic resonance imaging revealed a mass occupying the dorsal epidural space of C6 and C7, compressing the cervical spinal cord. Considering the acute symptomatology, an acute spinal epidural hematoma after surgery was suspected; therefore, emergency C6 and C7 laminectomy was performed. Surgical findings indicated that the pressure inside the spinal canal was elevated, and the mass was purulent exudate. Pathological examination showed suppurative inflammation with concomitant deposition of CPP. SEA complicated with CPPD was considered; therefore, antibiotics and non-steroidal anti-inflammatory drugs were administered. The motor weakness and hypoesthesia were improved despite a slight residual deficit in his dexterity.

Conclusion: An acute onset of SEA complicated with CPPD after cervical surgery has rarely been reported. The suppurative inflammation fostered by the crystal-induced inflammation may account for the acute symptomatology.

Keywords: Calcium pyrophosphate deposition disease, Chondrocalcinosis, Epidural abscess, Pseudogout, Surgical site infection

INTRODUCTION

Spinal epidural abscess (SEA) is a rare disease, but the early diagnosis of SEA is mandatory due to its potentially fatal outcome. Since the recent advent of imaging techniques enabled us to detect SEA with high sensitivity, the prevalence of SEA is increasing. However, mortality and the rate of the neurologically poor sequelae of SEA remain high.[ 13 ]

On the other hand, calcium pyrophosphate deposition disease (CPPD) causes crystal-induced arthritis mimicking gout. The patients typically presented with the acute onset of mono- or oligoarticular arthritis and constitutional symptoms in the inflammatory response to calcium pyrophosphate (CPP) crystals.[ 12 ] Although the knee or wrist joints are more susceptible to CPPD, spinal involvement of CPPD has also been reported.[ 6 , 10 , 11 ] Spinal CPPD affects the facet joint, intervertebral discs, and the ligamentum flava, in some cases leading to neurological symptoms due to the compression of the spinal cord.[ 2 , 7 ]

Although a surgical invasion is known to be followed by SEA and CPPD, the acute onset of which after the surgery has been rarely reported.

CASE DESCRIPTION

A man in his 70s presented with a weakness in the right arm, loss of dexterity, and gait disturbance for one month. As the symptoms gradually worsened, the patient came to our department. His medical history included hypertension and hypercholesterolemia. He was an immunocompetent host and did not take any oral antithrombotic medication. Magnetic resonance imaging revealed cervical spinal canal stenosis at the level of C3/4 and C4/5 without ossification of the posterior longitudinal ligament and yellow ligament, and there is no evidence of cervical spinal instability [ Figure 1a ]; hence, he was performed laminectomy at C3, C4, and C5 level. His symptoms subsided after the surgery; however, four days after the procedure, he complained of acute neck pain, motor deficit, and hypoesthesia in four limbs.

Figure 1:

Magnetic resonance image before and after the surgery. (a) Cervical spinal stenosis was found in the T2-weighted image, sagittal section, and the diagnosis of cervical spondylosis was made; therefore, the patient performed the C3 to C5 laminectomy. (b) Four days after the surgery, the mass occupying the dorsal epidural space (arrowhead) was well demarcated and low-intensity in T1-weighted and (c) homogeneously hyper-intensity in T2-weighted image (arrow).

Magnetic resonance imaging showed a mass located in the dorsal epidural space at C6 and C7 levels, which we did not manipulate during the surgery. The mass was well-demarcated, low-intensity in T1-weighted, and homogeneously hyperintensity in T2-weighted image [ Figures 1b and c ]. Based on imaging characteristics and the acute onset, an acute epidural hematoma after the surgery was suspected. The emergency C6 and C7 laminectomy was performed. The surgical findings implied elevated pressure inside the spinal canal because a purulent, whitish exudate erupted from the thinned cortical bone [ Figure 2a ]. Coagulase-negative staphylococci (CNS) were detected in the culture test of the exudate. The pathological examination of the exudate showed the infiltration of neutrophils and the small clusters of weak birefringent, basophilic crystals in hematoxylin and eosin stain, suggesting the SEA of CNS with concurrent crystal-induced inflammation of CPP [ Figures 2b and c ]. During the postoperative course, he complained of pain in his left knee. Arthrocentesis of the joint capsule of the knee also revealed CPP deposition. He was diagnosed with SEA of CNS complicated with CPPD and treated with antibiotics and non-steroidal anti-inflammatory drugs. His motor weakness and hypoesthesia improved despite a mild residual deficit of dexterity ten weeks after the surgery.

Figure 2:

(a) Intraoperative finding. After the laminectomy of C7, a purulent, whitish exudate (arrow) erupted from the thinned cortical bone; Hematoxylin and eosin stain showed the infiltration of neutrophils and the small clusters of weak birefringent, basophilic crystals compatible with calcium pyrophosphate deposition. (b) Low- (× 40), and (c) high-magnification (× 100).

DISCUSSION

SEA is characterized by pus accumulation in the epidural space, causing spinal cord compression and neurological deficit. The diagnosis of SEA is difficult, and the treatment is prone to be delayed.[ 14 ] However, since SEA sometimes leads to catastrophic neurological sequelae, the appropriate and immediate treatment is crucial. The causes of infection can be direct transmission from a local infection, indwelling catheter, surgical invasion, and hematogenous infection; however, in some cases, no obvious origin of infection is proven. Surgical drainage with systemic antibiotics is the widely accepted treatment option.[ 1 , 3 , 13 , 14 ]

On the other hand, CPPD is characterized as the crystal deposition in several joints, causing joint pain and constitutional symptoms. CPPD commonly involves the large joints such as knees, wrists, shoulders, and hips, but it also involves the spine.[ 2 ] CPPD is associated with several metabolic diseases, such as calcium metabolic disorders, iron overload, and hypothyroidism. Mechanical stress, including trauma and surgical invasion, is also relevant to the onset of CPPD. According to the reported cases of spinal involvement of CPPD, a surgical procedure is indicative to patients who suffer from neurological symptoms, including a motor deficit, sensory abnormality or bladder, and bowel incontinence. Histopathological findings obtained from the surgical specimen showed the birefringent rhomboid-shaped crystals, indicating CPP deposition.[ 4 , 8 - 10 ]

The present patient complained of neck pain and neurological symptoms immediately after the surgery for cervical spondylotic myelopathy, suggesting cervical spinal cord compression. Although a surgical procedure is the common predisposing factor of SEA and CPPD, the acute onset of 4 days of SEA and CPPD after the preceding surgery has been rarely reported. The interval time between the onset of SEA and the spinal surgery has not been well investigated. Literally reviewed, five cases of CPPD after the spinal surgery were reported, and the interval time after the surgical procedure ranged from 4 weeks to 8 years; however, acute onset within a week has not been reported [ Table 1 ].[ 2 , 5 , 7 , 9 , 11 ] In addition, the mass occupied the epidural space at the C6 and C7 levels where the surgical manipulation was not performed, which either infection or crystal-induced inflammation could not explain. Whether the diagnosis was SEA or CPPD, the acute postoperative interval of 4 days before the onset of neurological symptoms and the level of the mass formation was atypical. Considering the clinical course, the suppurative inflammation fostered by the crystal-induced inflammation may account for the acute symptomatology of this patient.

Table 1:

Reported cases of calcium pyrophosphate deposition disease after the surgical procedure.

CONCLUSION

A case of acute epidural abscess suspected of epidural hematoma after cervical laminectomy was reported. The acute onset of SEA complicated with CPPD after cervical spinal surgery has rarely been reported. The crystal-induced inflammation of the CPP could amplify the pyogenic inflammation.

Ethical approval

The Institutional Review Board approval is not required.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

Use of artificial intelligence (AI)-assisted technology for manuscript preparation

The authors confirm that there was no use of artificial intelligence (AI)-assisted technology for assisting in the writing or editing of the manuscript and no images were manipulated using AI.

Disclaimer

The views and opinions expressed in this article are those of the authors and do not necessarily reflect the official policy or position of the Journal or its management. The information contained in this article should not be considered to be medical advice; patients should consult their own physicians for advice as to their specific medical needs.

References

1. Al-Hourani K, Al-Aref R, Mesfin A. Upper cervical epidural abscess in clinical practice: Diagnosis and management. Global Spine J. 2016. 6: 383-93

2. Bridges KJ, Bullis CL, Wanchu A, Than KD. Pseudogout of the cervical and thoracic spine mimicking infection after lumbar fusion: Case report. J Neurosurg Spine. 2017. 27: 145-9

3. Darouiche RO. Spinal epidural abscess. N Engl J Med. 2006. 355: 2012-20

4. Ehioghae M, Lawlor MC, Mesfin A. Calcium pyrophosphate dihydrate of the ligamentum flavum in the cervical spine-a review of the literature. Surg Neurol Int. 2022. 13: 470

5. Jaffee S, Jeong SW, Dabecco R, Elhamdani S, Yu A. Pseudogout, an unconventional imitator of the lumbosacral spine with associated chronic epidural hematoma: Illustrative cases. J Neurosurg Case Lessons. 2022. 3: CASE21571

6. Kinoshita T, Maruoka S, Yamazaki T, Sakamoto K. Tophaceous pseudogout of the cervical spine: MR imaging and bone scintigraphy findings. Eur J Radiol. 1998. 27: 271-3

7. Lam H, Cheung K, Law S, Fung K. Crystal arthropathy of the lumbar spine: A report of 4 cases. J Orthop Surg. 2007. 15: 94-101

8. Lu YH, Lin HH, Chen HY, Chou PH, Wang ST, Liu CL. Multilevel calcium pyrophosphate dihydrate deposition in cervical ligamentum flavum: Clinical characteristics and imaging features. BMC Musculoskelet Disord. 2021. 22: 929

9. Moon AS, Mabry S, Pittman JL. Calcium pyrophosphate deposition disease of the cervical and thoracolumbar spine: A report of two cases. N Am Spine Soc J. 2020. 3: 100026

10. Muthukumar N, Karuppaswanny U, Sonntag VK, Traynelis VC, Benzel EC, Haid RW. Tumoral calcium pyrophosphate dihydrate deposition disease of the ligamentum flavum. Neurosurgery. 2003. 53: 103-9

11. Ogawa Y, Nagatsuma M, Kubota G, Inoue G, Eguchi Y, Orita S. Acute lumbar spinal pseudogout attack after instrumented surgery. Spine (Phila Pa 1976). 2012. 37: 1529-33

12. Rosenthal AK, Ryan LM. Calcium pyrophosphate deposition disease. N Engl J Med. 2016. 374: 2575-84

13. Sharfman ZT, Gelfand Y, Shah P, Holtzman AJ, Mendelis JR, Kinon MD. Spinal epidural abscess: A review of presentation, management, and medicolegal implications. Asian Spine J. 2020. 14: 742-59

14. Tetsuka S, Suzuki T, Ogawa T, Hashimoto R, Kato H. Spinal epidural abscess: A review highlighting early diagnosis and management. JMA J. 2019. 3: 29-40

Abstract

Background: Intraparenchymal hemorrhage at the operative site is one of the major complications of brain surgery. It is unusual to occur at a site remote from the operative site, but when it happens, it may cause significant morbidity and mortality.

Case Description: We report the case of a 27-year-old male who presented with complaints of paresthesias over the left side of his face and decreased hearing from the left ear for two years. His radiology was suggestive of a large left cerebellopontine angle epidermoid cyst. The patient underwent left retro mastoid suboccipital craniotomy and near-total excision of the epidermoid cyst. The immediate postoperative non-contrast computed tomography scan of the brain was suggestive of no hematoma at the operated site but a remote left parafalcine frontoparietal intraparenchymal bleed, which was managed conservatively. At two months follow-up, he had no neuro deficits, and magnetic resonance imaging of the brain was suggestive of near-total excision of the epidermoid cyst with resolving left parafalcine frontoparietal bleed.

Conclusion: We report this case due to the unique case observation of an intracranial bleed at a remote site rather than at the operated site.

Keywords: Cerebellopontine (CP) angle epidermoid, Intraparenchymal bleed, Posterior fossa surgery, Remote, Supratentorial

INTRODUCTION

Intraparenchymal hemorrhage at the operative site is one of the major complications of brain surgery. It is unusual to occur at a site remote from the operative site, but when it happens, it may cause significant morbidity and mortality.

CASE DESCRIPTION

We report the case of a 27-year-old male who presented with complaints of paresthesias over the left side of his face and decreased hearing from the left ear for two years. Neurological examination revealed horizontal nystagmus, gait ataxia, and mild left sensorineural hearing loss. His radiology was suggestive of a large extra-axial lesion in the left cerebellopontine (CP) angle with diffusion restriction. The lesion was encasing the cisternal segment of the left 7^th–8^th cranial nerves and was indenting the left middle cerebellar peduncle. Superiorly, the lesion was extending into the left half of the ambient and quadrigeminal cisterns and anteriorly, the lesion was bulging into the suprasellar cistern. His imaging was suggestive of a left CP angle epidermoid cyst [ Figures 1 and 2 ]. The patient underwent left retro mastoid suboccipital craniotomy in the park bench position and near-total excision of the epidermoid cyst. In the immediate postoperative period, a non-contrast computed tomography scan of the brain was suggestive of no hematoma at the operated site but a remote left parafalcine frontoparietal intraparenchymal bleed resulting in weakness of the right upper and lower limbs (power 3/5) [ Figure 3 ]. The patient’s coagulation profile was normal. As the bleed was small with no significant mass effect, it was managed conservatively, and gradually, the patient’s right hemiparesis improved. At two months follow-up, he had no neuro deficits and magnetic resonance imaging of the brain was suggestive of near-total excision of the epidermoid cyst with resolving left parafalcine frontoparietal bleed [ Figure 4 ]. Magnetic resonance venography was suggestive of a patent superior sagittal sinus [ Figure 4 ].

Figure 1:

Preoperative magnetic resonance imaging of the brain revealed (a) T1 hypointense, (b) T2 hyperintense lesion in the left cerebellopontine angle cistern with (c and d) diffusion restriction and extensions into the ambient, quadrigeminal, and suprasellar cisterns with no postcontrast enhancement suggestive of epidermoid cyst.

Figure 2:

(a and b) Preoperative computed tomography scan of the brain suggestive of a hypodense lesion in the left cerebellopontine angle with peripheral calcifications.

Figure 3:

(a-d) Immediate postoperative computed tomography scan of the brain suggestive of no hematoma at the operated site but a remote left parafalcine frontoparietal intraparenchymal bleed.

Figure 4:

(a-f) At two months follow-up, magnetic resonance imaging of the brain suggestive of near total excision of the epidermoid cyst with resolving left parafalcine frontoparietal bleed. Magnetic resonance venography was suggestive of a patent superior sagittal sinus.

DISCUSSION

Haines et al., in their paper, reported the incidence of supratentorial hemorrhage after posterior fossa surgeries was 0.6%.[ 5 ] There are various theories regarding the etiology of remote supratentorial bleed after posterior fossa surgery.

The sitting position during surgery can reduce arterial blood flow, resulting in ischemia.[ 4 ] Hyperperfusion of blood occurs once the patient is brought back to the supine position, leading to intraparenchymal hemorrhage.[ 4 ] Even subcortical veins can burst and cause intraparenchymal hemorrhage through the same mechanism.[ 4 ]

CONCLUSION

We reported this case due to the unique case observation of an intracranial bleed at a remote site rather than at the operated site which in our case, was probably due to excessive drainage of cerebrospinal fluid during surgery.

Ethical approval

The Institutional Review Board approval is not required.

Declaration of patient consent

Patient’s consent not required as patient’s identity is not disclosed or compromised.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

Use of artificial intelligence (AI)-assisted technology for manuscript preparation

The authors confirm that there was no use of artificial intelligence (AI)-assisted technology for assisting in the writing or editing of the manuscript and no images were manipulated using AI.

Disclaimer

The views and opinions expressed in this article are those of the authors and do not necessarily reflect the official policy or position of the Journal or its management. The information contained in this article should not be considered to be medical advice; patients should consult their own physicians for advice as to their specific medical needs.

References

1. Agrawal A, Kakani A, Ray K. Extensive supratentorial hemorrhages following posterior fossa meningioma surgery. J Surg Tech Case Rep. 2010. 2: 87-9

2. Cartier-Giroux J, Mohr G, Sautreaux JL. Supratentorial hemorrhage of hypertensive origin during operation: An unusual complication of surgery on the posterior fossa in the sitting position. Neurochirurgie. 1980. 26: 291-4

3. Cloft HJ, Matsumoto JA, Lanzino G, Cail WS. Posterior fossa hemorrhage after supratentorial surgery. AJNR Am J Neuroradiol. 1997. 18: 1573-80

4. De Albuquerque LA, Dourado JC, Almeida JP, Costa BS. Multiple supratentorial intraparenchymal hemorrhage after posterior fossa surgery. Surg Neurol Int. 2015. 6: S104-9

5. Haines SJ, Maroon JC, Jannetta PJ. Supratentorial intracerebral hemorrhage following posterior fossa surgery. J Neurosurg. 1978. 49: 881-6

6. Salunke P, Malik V, Kovai P, Aggarwal A, Khandelwal NK. Delayed supratentorial intracerebral hemorrhage following posterior fossa surgery. Asian J Neurosurg. 2016. 11: 315

Abstract

Background: Although secondary normal pressure hydrocephalus (sNPH) can occur in various central nervous system diseases, there are no reports of sNPH caused by pituitary lesions. Herein, we present a unique case of sNPH caused by pituitary apoplexy.

Case Description: A 70-year-old man was transferred to our hospital because of a sudden onset of headache and loss of consciousness. The cerebrospinal fluid (CSF) test showed slightly elevated cell counts and protein levels but a negative CSF culture test. Magnetic resonance imaging showed a dumbbell-like cystic lesion with hemorrhagic change at the sella turcica. From the above, the patient was diagnosed with aseptic meningitis caused by pituitary apoplexy. Pituitary hormone replacement therapy was undertaken, and his symptoms fully improved. However, two months later, he complained of a gait disturbance and incontinence that had gradually appeared. Brain imaging with computed tomography showed no ventricular enlargement compared with initial images, although the lateral ventricles were slightly enlarged. As a CSF drainage test improved his symptoms temporarily, sNPH with possible longstanding overt ventriculomegaly in adults (LOVA) background was suspected. We performed a lumboperitoneal shunt (LPS) placement, which improved his symptoms.

Conclusion: This case suggests that sNPH can develop even after a small subarachnoid hemorrhage caused by a pituitary apoplexy in LOVA patients. If the aqueduct of Sylvius is open, sNPH with a LOVA background can be successfully treated with LPS placement.

Keywords: Chemical meningitis, Cerebrospinal fluid drainage test, Longstanding overt ventriculomegaly in adults, Pituitary apoplexy, Secondary normal pressure hydrocephalus

INTRODUCTION

Secondary normal pressure hydrocephalus (sNPH) can occur in a number of central nervous system diseases.[ 10 ] The causes of sNPH include aneurysmal subarachnoid hemorrhage, meningitis, and central nervous system tumors. However, to the best of our knowledge, there are no reports of sNPH caused by pituitary lesions. Herein, we present a unique case of sNPH caused by pituitary apoplexy.

CASE REPORT

The authors obtained written informed consent from the patient. No approval from our institutional review board was sought because this article is a case report. A 70-year-old man complained of a sudden sense of physical weakness and headache, for which he was transferred to the emergency room of our hospital. On arrival, he had a high fever and showed nuchal rigidity. His medical history included diabetes mellitus, hepatocellular carcinoma not induced by viral hepatitis, intraductal papillary mucinous neoplasm, deep venous thrombosis, and angina pectoris, all of which had been appropriately treated. He also had regular visits to our hospital, to which he walked. His medication included antiplatelets and anticoagulants.

Laboratory tests showed elevated white blood cell counts and C-reactive protein. Whole-body computed tomography (CT) did not detect any apparent abnormality, except for a slightly high-density 10-mm mass in the sella turcica and enlarged lateral ventricles (Evans index, 0.33; callosal angle, 61°) [ Figures 1a and b ]. By contrast, magnetic resonance imaging (MRI) showed a fluid-attenuated inversion recovery-hyperintensity lesion in the peduncular cistern [ Figure 1c ], although no evidence of vascular lesions was detected on magnetic resonance angiography [ Figure 1d ]. Cerebrospinal fluid (CSF) testing showed slightly elevated cell counts, including red blood cells and white blood cells, and elevated protein levels [ Table 1 ]. These findings suggested meningitis caused by bacterial or viral infection rather than hemorrhagic cerebral vascular lesions. The sellar lesion detected by CT was initially diagnosed as a pituitary tumor because of its density and small size.

Figure 1:

(a and b) Computed tomography imaging. (a) A hyperdense lesion was observed in the sella turcica (9.5 mm in diameter). (b) Lateral ventricles were slightly enlarged. (c-f) Magnetic resonance imaging. (c) Fluid-attenuated inversion recovery image showed a hyperintensity lesion in the cerebral peduncle that implied a hematoma. (d) No cerebrovascular lesion was detected on magnetic resonance angiography. (e and f) The coronal images showed that the sellar snowman-like lesion was hypointense on T1-weighted imaging and hypointense with niveau on T2-weighted imaging.

After admission, intravenous acyclovir, vancomycin, and ampicillin were administered for a possible diagnosis of infectious meningitis. Although his laboratory data (including white blood cell counts and C-reactive protein) improved, his consciousness level remained poor, with a Glasgow Coma Scale of 13 (E3V4M6). Repeated brain CT showed no remarkable changes compared with initial images, including for the sellar lesion. However, pituitary MRI showed a dumbbell-like hypointense core with a hyperintense rim on T1-weighted imaging [ Figure 1e ] and a hypointense mass with niveau on T2-weighted imaging [ Figure 1f ]. Furthermore, laboratory tests indicated marked panhypopituitarism (thyroid-stimulating hormone, 0.078 mIU/L; free T3,

However, two months after discharge, he complained of gait disturbance and incontinence that gradually appeared. Brain CT showed possible hydrocephalus (Evans index, 0.35; callosal angle, 50°), which was similar to the initial CT [ Figures 1a and 2 ]. He could only walk by sliding his feet, and it took >1 min with 132 steps for a 10 m round trip. His Mini–Mental State Examination score was 21, which indicated mild cognitive impairment. Follow-up MRI showed resolution of the pituitary hemorrhagic lesion. Therefore, we performed a tap test on day 120 from initial hospitalization to assess for a hydrocephalus etiology. We punctured the L4/L5 interspinal space with a 21-gauge Quincke needle, and 20 mL of transparent colorless CSF was aspirated. Laboratory tests for CSF detected no viable cells or red blood cells, although the protein level was mildly elevated [ Table 1 ]. After the CSF drainage test, his subjective symptoms improved and were maintained for approximately one month. For example, his gait was smoother, and it took only 38 steps for a 10 m round trip. His Mini–Mental State Examination score improved to 24 at one day after CSF drainage, and he was able to return to work. Due to these improvements, we inserted a conducted lumboperitoneal shunt (LPS) with a CERTAS plus programmable valve (Codman and Shurtleff, Inc., Raynham, MA, USA) under general anesthesia. His postoperative course was uneventful, and he was able to walk without aid at discharge, which suggested the diagnosis of definite hydrocephalus.

Figure 2:

Imaging at three months after symptom onset. (a) Computed tomography imaging shows the enlarged lateral ventricles, which were similar to the prior images taken at admission. His Evans index was 0.36, and the callosal angle was 54°. (b) Magnetic resonance images showed the resolution of the pituitary lesion on T2-weighted imaging.

Table 1:

Sequential laboratory data of the CSF.

DISCUSSION

In the present case, our initial diagnosis was meningitis rather than pituitary apoplexy because of his symptoms and brain imaging findings. However, we eventually diagnosed a pituitary apoplexy following repeated imaging examinations and a negative CSF culture test. At two months after diagnosis, he complained of gait disturbance and mild cognitive impairment, although CT showed almost no further change in his enlarged lateral ventricle. We suspected sNPH based on his CSF tap test, and LPS surgery successfully improved his symptoms.

At admission, the symptoms and CSF results of our case were suggestive of meningitis, although a later CSF culture test did not detect any pathogenic microorganism. The patient also developed sNPH following meningitis. A potential explanation for these findings is that sNPH occurred as a result of pituitary apoplexy-related noninfectious meningitis. Pituitary apoplexy is a rare condition that can occur in a macroadenoma as well as in a normal pituitary gland or microadenoma.[ 1 , 9 ] Several studies have reported that chemical meningitis can be caused by leakage of blood and necrotic debris into the subarachnoid space in some pituitary apoplexy patients.[ 4 , 8 , 11 ] It is well-established that meningitis can cause sNPH. However, to the best of our knowledge, there are no reports of pituitary apoplexy causing sNPH. Clinicians should be aware of the potential for sNPH when treating pituitary apoplexy patients, especially in those complicated with meningitis.

The present case did not show initial gait disturbance or cognitive impairment, although CT at first admission showed enlarged lateral ventricles. This initial diagnosis may have been related to longstanding overt ventriculomegaly in adults (LOVA) without symptoms. LOVA describes a heterogeneous adult hydrocephalus, which usually has a slow progression.[ 3 ] To the best of our knowledge, there are no reports of pituitary apoplexy causing subacute symptomatic hydrocephalus in LOVA patients or reports of pituitary apoplexy causing “nonobstructive” sNPH. Nevertheless, a recent study described a rare case of cerebral vasospasm, which is a well-known complication of aneurysmal SAH, caused by pituitary apoplexy.[ 2 ] Thus, SAH accompanied by pituitary apoplexy may accelerate symptomatic hydrocephalus in LOVA patients.

In the present case, sNPH was successfully treated with LPS placement, although LOVA may have been present before the onset of pituitary apoplexy. Endoscopic third ventriculostomy (ETV) is generally recommended in symptomatic LOVA patients, whereas a ventriculoperitoneal shunt (VPS) can be considered if the aqueduct of Sylvius is open.[ 5 ] VPS and LPS placement are also both effective treatments for sNPH[ 6 ] in patients with an open aqueduct of Sylvius. Given that our patient was taking anti-platelet and anti-coagulant medications for his medical conditions, we considered LPS the safer option compared with ETV or VPS. Moreover, in idiopathic sNPH (a sNPH without preceding etiologies), a lumbar puncture is used to determine whether the CSF findings are normal and whether the symptoms improve with CSF drainage.[ 7 ] Our findings support the efficacy of LPS surgery as an alternative treatment to ETV or VPS if symptoms improve following the tap test and the aqueduct of Sylvius is open.

CONCLUSION

We present a unique case of sNPH following pituitary apoplexy with a possible LOVA background. Clinicians should be aware that sNPH can develop even after a small subarachnoid hemorrhage caused by pituitary apoplexy in LOVA patients. If the aqueduct of Sylvius is open, sNPH with a LOVA background can be successfully treated with LPS placement.

Ethical approval

Institutional Review Board approval is not required.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

Use of artificial intelligence (AI)-assisted technology for manuscript preparation

The authors confirm that there was no use of artificial intelligence (AI)-assisted technology for assisting in the writing or editing of the manuscript, and no images were manipulated using AI.

Disclaimer

The views and opinions expressed in this article are those of the authors and do not necessarily reflect the official policy or position of the Journal or its management. The information contained in this article should not be considered to be medical advice; patients should consult their own physicians for advice as to their specific medical needs.

Acknowledgments

We thank Juntaro Fujita and Shohei Nishigaki for providing us with critical comments.

References

1. Abbara A, Clarke S, Eng PC, Milburn J, Joshi D, Comninos AN. Clinical and biochemical characteristics of patients presenting with pituitary apoplexy. Endocr Connect. 2018. 7: 1058-66

2. Alsayadi S, Ochoa-Sanchez R, Moldovan ID, Alkherayf F. Cerebral vasospasm as a consequence of pituitary apoplexy: Illustrative case. J Neurosurg Case Lessons. 2023. 5: CASE22349

3. Gillespie CS, Fang WY, Lee KS, Clynch AL, Alam AM, McMahon CJ. Long-standing overt ventriculomegaly in adults: A systematic review and meta-analysis of endoscopic third ventriculostomy versus ventriculoperitoneal shunt as first-line treatment. World Neurosurg. 2023. 174: 213-20.e2

4. Huang WY, Chien YY, Wu CL, Weng WC, Peng TI, Chen HC. Pituitary adenoma apoplexy with initial presentation mimicking bacterial meningoencephalitis: A case report. Am J Emerg Med. 2009. 27: 517.e1-4

5. Ibáñez-Botella G, González-García L, Carrasco-Brenes A, RosLópez B, Arráez-Sánchez M. LOVA: The role of endoscopic third ventriculostomy and a new proposal for diagnostic criteria. Neurosurg Rev. 2017. 40: 605-11

6. Liao CL, Tseng PH, Huang HY, Chiu TL, Lin SZ, Tsai ST. Lumbar-peritoneal shunt for idiopathic normal pressure hydrocephalus and secondary normal pressure hydrocephalus. Tzu Chi Med J. 2022. 34: 323-8

7. Nakajima M, Yamada S, Miyajima M, Ishii K, Kuriyama N, Kazui H. Guidelines for management of idiopathic normal pressure hydrocephalus (third edition): Endorsed by the Japanese Society of normal pressure hydrocephalus. Neurol Med Chir (Tokyo). 2021. 61: 63-97

8. Nawar RN, AbdelMannan D, Selman WR, Arafah BM. Pituitary tumor apoplexy: A review. J Intensive Care Med. 2008. 23: 75-90

9. Randeva HS, Schoebel J, Byrne J, Esiri M, Adams CB, Wass JA. Classical pituitary apoplexy: Clinical features, management and outcome. Clin Endocrinol (Oxf). 1999. 51: 181-8

10. Skalický P, Mládek A, Vlasák A, De Lacy P, Beneš V, Bradáč O. Normal pressure hydrocephalus-an overview of pathophysiological mechanisms and diagnostic procedures. Neurosurg Rev. 2020. 43: 1451-64

11. Tumyan G, Mantha Y, Gill R, Feldman M. Acute sterile meningitis as a primary manifestation of pituitary apoplexy. AACE Clin Case Rep. 2021. 7: 117-20

Abstract

Background: Cauda equina syndrome (CES) is a consequence of a variety of etiologies. CES is most commonly due to compression of the thecal sac and nerve roots by a massive disc herniation. However, it rarely presents secondary to aortic occlusion. Aortoiliac occlusive disorder is usually associated with chronic claudication, erectile dysfunction, and diminished lower limb pulses. Acute aortic occlusion, however, is associated with serious complications such as spinal cord infarction and ischemia. It is also associated with a high risk of morbidity and mortality. Moreover, it poses a diagnostic challenge and may be overlooked. This report emphasizes the importance of considering vascular etiology as a differential diagnosis for CES.

Case Description: This case report describes a unique case of aortic occlusion mimicking CES in a 56-year-old female patient.

Conclusion: For patients presenting with cauda equina symptomatology, it is critical to consider vascular etiology, especially for those with cardiovascular risk factors. Spine surgeons and emergency physicians should maintain a high index of suspicion for vascular etiologies and consider appropriate imaging studies to promote early diagnosis and intervention to prevent subsequent neurological and life-threatening consequences.

Keywords: Abdominal aorta, Acute aortoiliac occlusion, Cauda equina syndrome, Emergency physician, Spine surgeon

INTRODUCTION

Cauda equina syndrome (CES) is an uncommon condition that affects 13 ] CES is relatively rare and challenging to diagnose, with no agreed-upon clinical diagnostic criteria.[ 4 , 9 ] Common signs and symptoms of CES may include pain in the lower back, sphincter (bowel and urinary) dysfunction, saddle anesthesia, and lower limb weakness and radicular pain.[ 5 ] CES is a spinal surgical emergency that, if not recognized and emergently treated, may progress to paraplegia and/or permanent sphincter dysfunction.[ 1 ] The clinical presentation may mimic other differential diagnoses, including acute aortoiliac occlusions.

This report emphasizes the importance of considering vascular etiology as a differential diagnosis for CES. Here, we present a challenging clinical scenario of a unique case of aortic occlusion mimicking CES.

CASE PRESENTATION

A 56-year-old female patient with hypertension, hyperlipidemia, and ischemic heart disease underwent coronary artery bypass grafting in 2016. She had a recent history of non-ST-segment elevation myocardial infarction and dual antiplatelet therapy. She was brought to the emergency department with the chief complaint of acute onset of severe lower back pain, bilateral lower limb weakness, non-dermatomal bilateral lower limb pain, and urinary incontinence. Upon examination, the patient experienced distress and pain despite receiving appropriate analgesic medications, including narcotics. The patient was conscious, febrile, and hypertensive (220/104). The power in the left lower limb was 0/5, and 3/5 in the right lower limb in all muscle groups. She had absent deep tendon reflexes and diminished sensation in both lower limbs. The anal tone was weak, but no saddle anesthesia was present. The lower limbs were not cold, and pedal pulses were palpable but diminished. An emergent magnetic resonance imaging (MRI) of the lumbar–sacral spine was performed and showed an L4– L5 diffuse disk bulge indenting the ventral thecal sac and causing bilateral mild-to-moderate neural foraminal stenosis, mild spinal canal stenosis, and bilateral facet arthrosis [ Figure 1 ]. Cervical and thoracic MRIs were also included, given that she presented with a fever and had a recent central catheter insertion to rule out a spinal abscess. However, the results were unremarkable. MRI of the whole spine at this point was not suggestive of any spinal-related etiology. A repeated physical examination a few hours after presentation revealed 0/5 power in both lower limbs, complete loss of sensation, cold extremities, and absent pulses in both lower limbs. Bedside lower limb Doppler ultrasound showed no pulses. Repeated lactic acid increased from 1.3 mmol/L to 6.8 mmol/L. Computed tomography (CT) abdomen with contrast was conducted, and it showed extensive diffuse atherosclerotic changes affecting the abdominal aorta with branches extending to the lower limbs arterial system, which were more severe on the left. Nonopacification of the aorta from the level below the renal arteries extended to the common iliac arteries, and the contrast continued in the external iliac arteries (likely through collaterals) opacifying the right lower limb arteries with the presence of segments of severe atherosclerotic contrast attenuation and faintly opacifying the left lower limb arteries. Hypodensity in the spleen likely represented partial splenic infarction [ Figure 2 ].

The patient then underwent emergent bilateral aortoiliac embolectomy and bilateral four-compartment lower limb fasciotomy. Postoperatively, there was no improvement in neurological function. She was started on heparin infusion, and dual antiplatelet therapy was initiated. The patient’s condition continued to deteriorate a few days later, and she died due to a cardiac event.

Figure 1:

Magnetic resonance imaging of the lumbar–sacral spine without contrast shows multilevel lower lumbar spine degenerative disc disease that is more pronounced at L4–5.

Figure 2:

Computed tomography angiography abdomen aortogram multiple cuts showing a filling defect in the abdominal aorta starting below the level of the renal arteries extending the bilateral proximal part of the common iliac arteries. (The hallmark - Filling defect).

DISCUSSION

Acute aortic occlusion (AAO) is an uncommon vascular disorder and emergency that is associated with a mortality rate ranging from 20% to 75%.[ 10 , 14 , 16 , 18 ] AAO etiologies include embolization into the aortoiliac bifurcation, acute aortoiliac thrombosis, aortoiliac dissection or aneurysm-related acute thrombus formation, and aortoiliac trauma.[ 19 ] Atypical causes include hypercoagulable state, vasculitis, and fungal infections.[ 6 , 12 , 15 , 20 ] Cardiovascular disorders are the most commonly associated comorbidities with AAO.[ 10 ]

Chronic aortoiliac occlusive disorder is likely to manifest as chronic erectile disorder, claudication, diminished lower limb pulses, cool extremities, cyanotic extremities, compartment syndrome, and myonecrosis.[ 3 ] AAO, however, is associated with serious complications ranging from ischemic neuropathy and spinal cord dysfunction to permanent spinal cord infarction. In addition, it imitates CES, poses a diagnostic challenge, and may be overlooked.[ 17 ] AAO may present with profound systemic response, including tachycardia and diaphoresis. Furthermore, it may present with cold extremities, ischemic cutaneous manifestations, and faint or pulselessness in addition to out-of-proportion abdominal pain and oliguria/anuria if there is concomitant renal or visceral involvement.[ 2 , 8 ] CT is the modality of choice due to its wide availability and it is quick to perform. It shows the extent and mechanism of the disease, vessel patency, and signs of poor perfusion.[ 11 ]

Certain aspects of management, including distinguishing between embolism and thrombosis etiologies before surgery, the use of aortography, the best initial treatment modality, the preferred course of management, and the role of long-term or permanent anticoagulation, are subjects of debate.[ 2 , 8 ] Optimizing cardiac function, administration of anticoagulants, and optimization of hydration status is recommended.[ 3 ] AAO is a surgical condition that requires an emergent revascularization/reperfusion procedure along with anticoagulation unless contraindicated.[ 19 ] Nonoperative management has a mortality rate of up to 75%, whereas emergent operative management has significantly lower rates.[ 3 , 19 ]

Revascularization/reperfusion interventions may include thrombectomy, embolectomy, thrombolysis, extra-anatomic bypass, and direct repair.[ 3 , 19 ] The choice between endovascular and open vascular approaches is largely dependent on the surgeon’s expertise, disease extension, and patient comorbidities.[ 7 ] The open surgical approach is the gold standard for AAO management. It has a higher primary patency rate and a relatively higher perioperative mortality rate ranging from 0% to 7%.[ 7 ] However, the endovascular approach has an increasing role in the management of AAO, with a similar secondary patency rate, higher costs, lower hospitalization stay period, and lower perioperative morbidity and mortality risks.[ 7 ] Thrombolysis is usually an adjunct to other treatment modalities.[ 7 ] The neurovascular status upon presentation (severity of the ischemia) and the duration of ischemia, along with early detection and expeditious intervention, are all factors associated with a lower risk of morbidity, mortality, and improved recovery.[ 2 , 3 , 8 , 10 , 19 ]

Patients with AAO may present with clinical pictures mimicking CES, which may lead to a diagnostic puzzle. To preclude misdiagnosis, emergency medicine physicians and spine surgeons must have a high index of suspicion regarding vascular etiology. Along with a detailed neurological examination, peripheral vascular examination should not be overlooked. The consideration of vascular etiologies should be even higher, especially if a spinal MRI does not suggest a compressive neurological etiology and/or a spinal cord lesion.

CONCLUSION

For patients presenting with cauda equina symptomatology, it is critical to consider vascular etiology, especially for those with cardiovascular risk factors. Spine surgeons and emergency physicians should maintain a high index of suspicion for vascular etiologies and consider appropriate imaging studies to promote early diagnosis and intervention to prevent subsequent neurological and life-threatening consequences.

Ethical approval

Institutional Review Board approval is not required.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

Use of artificial intelligence (AI)-assisted technology for manuscript preparation

The authors confirm that there was no use of artificial intelligence (AI)-assisted technology for assisting in the writing or editing of the manuscript and no images were manipulated using AI.

Disclaimer

The views and opinions expressed in this article are those of the authors and do not necessarily reflect the official policy or position of the Journal or its management. The information contained in this article should not be considered to be medical advice; patients should consult their own physicians for advice as to their specific medical needs.

Acknowledgments

American manuscript editors.

References

1. Ahn UM, Ahn NU, Buchowski JM, Garrett ES, Sieber AN, Kostuik JP. Cauda equina syndrome secondary to lumbar disc herniation: A meta-analysis of surgical outcomes. Spine. 2000. 25: 1515-22

2. Azzarone M, De Troia A, Iazzolino L, Nabulsi B, Tecchio T. Hybrid treatment of acute abdominal aortic thrombosis presenting with paraplegia. Ann Vasc Surg. 2016. 33: 228.e5-8

3. Babu SC, Shah PM, Nitahara J. Acute aortic occlusion--factors that influence outcome. J Vasc Surg. 1995. 21: 567-75 discussion 573-5

4. Bakas JM, Bijdevaate DC, Lauw MN, van Veelen-Vincent ML, van Rijn MJ. A case of complete resolution of cauda equina syndrome caused by extensive iliocaval thrombosis: The role of thrombolysis and venous stents. J Endovasc Ther. 2023. p. 15266028231179596

5. Barraclough K. Cauda equina syndrome. BMJ. 2021. 372: n32

6. Bolduc M, Clayson S, Madras P. Acute aortic thrombosis presenting as painless paraplegia. J Cardiovasc Surg (Torino). 1989. 30: 506-8

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