Scholarly article on topic 'Foramen magnum extramedullary cavernous hemangioma in a pediatric patient'

Foramen magnum extramedullary cavernous hemangioma in a pediatric patient Academic research paper on "Clinical medicine"

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Academic research paper on topic "Foramen magnum extramedullary cavernous hemangioma in a pediatric patient"

explain the proptosis, cranial nerve palsy and chiasmatic meningeal enhancement.12-41 Positive c-ANCA, CT thorax and microscopic hematuria were suggestive of Wegener's granulomatosis. Partial brain autopsy prevented us from confirming Wegener's in lungs and kidneys. Thus Wegener's remains only a speculation in our patient. Angioleiomyoma may have induced hypergammaglobulinemia. This would explain CSF findings, raised c-ANCA, ESR and dural infiltrates. An autoimmune disorder like Wegener's granulomatosis can be associated with reactive myofibroblasts proliferation as in orbital pseudotumor and retroperitoneal fibrosis.181

Gross total resection is possible in solitary angioleiomyoma. None of the cases in the literature reported recurrences for up to a maximum of four years' follow-up.[1-4] Our patient documents for the first time, angioleiomyoma involving the putamen, hippocampus, optic nerves and leptomeninges. Multicentricity would pose a therapeutic challenge to neurosurgeons. We postulate a probable underlying immune-mediated disease stimulating perivascular myoid proliferation, as well as angioleiomyoma-induced paraneoplastic hypergammaglobulinemia.


The authors would like to acknowledge Dr. Shankar S. K., NIMHANS, Bangalore for his valuable assistance in immunohistochemistry and opinion on histopathological diagnosis.

Sweety V. Shinde, Arun B. Shah1, Rahul B. Baviskar1, Jaya R. Deshpande

Departments of Pathology, and Neurology, B.Y.L Nair Hospital, Mumbai, India E-mail:


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3. Gasco J, Franklin B, Rangel-Castila LR, Campbell GA, Eltorky M, Salinas P. Infratentorial angioleiomyoma: A new location for a rare neoplastic entity. J Neurosurg 2009;110:670-74.

4. Figueiredo EG, Gomes M, Vellutini E, Rosemberg S, Marino R. Angioleiomyoma of the cavernous sinus. Neurosurgery 2005;56:411.

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Received: 18-10-2011 Review completed: 14-11-2011 Accepted: 25-12-2011

Foramen magnum extramedullary cavernous hemangioma in a pediatric patient

The reported incidence of central nervous system (CNS) cavernomas ranges from 0.4 to 0.9% and they account for 10-15% of all vascular malformations of the CNS.[1,2] Extra-axial cavernomas are rare and to the best of our knowledge, only one case of intradural extramedullary cavernoma at the foramen magnum has been reported.[3] We are reporting the second such unusual case.

An 11-year-old boy presented with occipital headache, low-grade fever, neck pain and rigidity of two months duration with exaggeration of symptoms a week before presentation. Neurological examination revealed signs of meningism, subtle corticospinal tract signs (motor power 4/5) with bilateral up-going plantars. He was initially treated as sub-acute meningitis by a pediatrician. Cerebrospinal fluid (CSF) was clear with 15 leukocytes/mm3 (95% lymphocytes), few crenated red blood cells, mildly elevated protein and normal glucose. Computed tomography [Figure 1] showed a 3 x 2.5 cm, predominantly hyperdense mass lesion with areas of isodensity in the right postero-lateral foramen magnum region extending up to the C2 spine level. Magnetic resonance image (MRI) [Figures 2 and 3] showed a dural-based extramedullary isointense mass lesion with surrounding hyperintensity and mild contrast enhancement. Cerebellar convexity dura on both the sides and anterior spinal dura at the C1/2 level showed hyperdensities suggestive of sub-acute subarachnoid hemorrhage (SAH). Patient was operated by a sub-occipital craniectomy and C1 posterior arch excision. The dura was opened by a Y-shaped incision.

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Figure 1: CT brain (plain) showing a well-defined hyperdense mass lesion with areas of isodensity located in the right posterolateral aspect of the foramen magnum

Figure 3: MRI brain contrast axial (a), sagittal (b), and coronal (c) images showing an extramedullary mildly enhancing mass lesion with intra-lesional and subarachnoid bleed

A large extra-axial mass lesion with surrounding hemosiderin was noted in the right postero-lateral foramen magnum region crossing the middle onto the left side. The lesion was extending superiorly into the cisterna magna and inferiorly up to the C2 spine level. The mass lesion was completely excised microscopically without any damage to the normal parenchymal

Figure 2: MRI brain (T1-weighted) axial images showing a well-defined extramedullar iso-intense mass with surrounding hyperintensity

Figure 4: Histopathology of the surgical specimen showing numerous thin-walled vascular channels with broad congested lumina and areas of dense fibroblastic reaction with hemosiderin laden macrophages and free hemosiderin pigment (a and b). Hematoxylin and eosin; original modification, x40

and vascular structures. The patient recovered very well postoperatively without any fresh neurological deficits and was discharged on the sixth postoperative day. Histopathology of the lesion [Figure 4] showed numerous thin-walled vascular channels with congested lumina. There was dense fibroblastic reaction with hemosiderin laden macrophages and free hemosiderin pigment. The findings confirmed the diagnosis of cavernous hemangioma with bleed.

Clinical presentation of this patient along with the

preoperative imaging suggested a high possibility of cavernoma with intra-lesional and SAH. Brainstem cavernomas account for 9-35% of the CNS cavernomas.[4,5] The overwhelming majority of intracranial and intraspinal cavernomas are intraparenchymal, with just a small fraction found in the epidural space. Extra-axial cavernomas are rare and are generally located in the cisternal space, arising from the dural surface or cranial nerves.[6] The majority of these are found in the dural sinuses or the cerebellopontine angle and only 29 cases of spinal intradural extramedullary cavernomas have been reported in the literature/-81 Of these, only five cases have been reported at the cervical level. Only one case of extramedullary foramen magnum cavernoma has been reported in the literature till date.

The unique features of our patient are: The location (foramen magnum), clinical presentation (SAH), and age (11 years). In the review of spinal intradural extramedullary cavernomas by Jin et al.,[8] the mean age at diagnosis was 47 (range 20-75), and only 10 (34%) cases presented with SAH. An extramedullary cavernoma presenting as SAH in pediatric age has not been documented. Classic symptoms of brainstem and spinal cavernous malformations include headaches or neck pain and mass effect. Our patient presented with symptoms of meningism and CSF findings were indicative of SAH. Er et al.,[7] suggested that SAH may present as the initial symptom of intradural extramedullary cavernoma. Both the cases of foramen magnum cavernomas reported, including our case, presented with SAH. This may be due to the direct communication of these lesions with the subarachnoid space. It has been postulated that the restriction of movement because of adherence to the spinal roots may make these lesions more likely to bleed. [7] In the author's view, due to the dynamism and wide range of movements at the foramen magnum, detachment of the adherent mass leads to frequent bleeds.


I am extremely thankful to Dr. Anupama Koduru (Consultant Pathologist, Manipal Hospital, Vijayawada) for sharing the histopathological microphotographs of this patient.

Arun Palani

Department of Neurosurgery, Manipal Super-specialty Hospital, Vijayawada, Andhra Pradesh, India E-mail:


1. Batra S, Lin D, Recinos PF, Zhang J, Rigamonti D. Cavernous malformations: natural history, diagnosis and treatment. Nat Rev Neurol 2009;5:659-70.

2. Bertalanfffy H, Benes L, Miyazawa T, Alberti O, Siegel AM, Sure U. Cerebral

cavernomas in the adult. Review of the literature and analysis of 72 surgically treated patients. Neurosurg Rev 2002;25:1-53; discussion 54-5. Mocco J, Laufer I, Mack WJ, Winfree CJ, Libien J, Connolly ES Jr. An extramedullary foramen magnum cavernous malformation presenting with acute subarachnoid hemorrhage: Case report and literature review. Neurosurgery 2005;56:E410; discussion E410.

Fritschi JA, Reulen HJ, Spetzler RF, Zabramski JM. Cavernous malformations of the brain stem: A review of 139 cases. Acta Neurochir (Wien) 1994;130:35-46.

Kondziolka D, Lunsford LD, Kestle JR. The natural history of cerebral cavernous malformations. J Neurosurg 1995;83:820-4. Dörner L, Buhl R, Hugo HH, Jansen O, Barth H, Mehdorn HM. Unusual locations for cavernous hemangiomas: Report of two cases and review of the literature. Acta Neurochir (Wien) 2005;147:1091-6. Er U, Yigitkanli K, Simsek S, Adabag A, Bavbek M. Spinal intradural extramedullary cavernous angioma: Case report and review of the literature. Spinal Cord 2007;45:632-6.

Jin YJ, Chung SB, Kim KJ, Kim HJ. Spinal intradural extramedullary cavernoma presenting with intracranial superficial hemosiderosis. J Korean Neurosurg Soc 2011;49:377-80.

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Received: 20-10-2011 Review completed: 27-11-2011 Accepted: 29-11-2011

Endovascular management of carotid-cavernous fistula in Ehlers-Danlos syndrome Type IV

A 27-year-old lady presented with progressive proptosis of right eye of three months duration. On examination, she was short-statured, had curly wispy hair, pinched nose, hyper-pigmented extremities, swollen joints without hypermobility, wrinkled, dry, thin skin without hyper-elasticity with visible underlying veins. She had proptosis of right eye with chemosis and normal visual acuity and visual fields. Both pupils were equal in size with no relative afferent pupillary defect. Fundoscopy revealed blurring of right disc margin. Movements of the right eye were minimally restricted in all directions. Magnetic resonance imaging of orbits and cerebral computed tomography-angiogram (CTA) revealed a dilated right superior ophthalmic vein (SOV) and dilated cavernous varix [Figure 1]. With a diagnosis of a caroticocavernous fistula (CCF) in a patient with Ehlers-Danlos syndrome (EDS), we planned a combined diagnostic angiogram and intervention to minimize

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