Intradural extramedullary cavernous malformation with extensive superficial siderosis of the neuraxis: Case report and review of literature
- Department of Neurological Surgery and Radiology, Northwestern Memorial Hospital, Chicago, Illinois, USA
- Department of Neuropathology, Northwestern Memorial Hospital, Chicago, Illinois, USA
- Department of Neurological Surgery, Advocate Good Samaritan Hospital, Downers Grove, Illinois, USA
Babak S. Jahromi
Department of Neurological Surgery and Radiology, Northwestern Memorial Hospital, Chicago, Illinois, USA
DOI:10.4103/sni.sni_103_17Copyright: © 2017 Surgical Neurology International This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.
How to cite this article: Pedram Golnari, Sameer A. Ansari, Ali Shaibani, Michael C. Hurley, Matthew B. Potts, Missia E. Kohler, Patrick A. Sugrue, Babak S. Jahromi. Intradural extramedullary cavernous malformation with extensive superficial siderosis of the neuraxis: Case report and review of literature. 13-Jun-2017;8:109
How to cite this URL: Pedram Golnari, Sameer A. Ansari, Ali Shaibani, Michael C. Hurley, Matthew B. Potts, Missia E. Kohler, Patrick A. Sugrue, Babak S. Jahromi. Intradural extramedullary cavernous malformation with extensive superficial siderosis of the neuraxis: Case report and review of literature. 13-Jun-2017;8:109. Available from: http://surgicalneurologyint.com/surgicalint-articles/intradural-extramedullary-cavernous-malformation-with-extensive-superficial-siderosis-of-the-neuraxis-case-report-and-review-of-literature/
Background:Spinal cavernous malformations usually affect the vertebral bodies and are seldom intradural. Here, we report a rare spinal intradural-extramedullary cavernous malformation associated with extensive superficial siderosis along the neuraxis in a patient with radicular complaints.
Case Description:A 60-year-old male presented with subacute headaches, intermittent fever, and acute back and radicular leg pain for 1–2 weeks. Magnetic resonance imaging revealed an intradural-extramedullary lesion just below the conus medullaris (at the L2 level). There was associated subarachnoid hemorrhage in the lumbar cistern and superficial siderosis along the entire spinal neuraxis. Following surgical resection, the patient's symptoms resolved. Histopathology of the lesion was of a cavernous malformation.
Conclusions:There are only 56 cases of spinal intradural-extramedullary cavernous malformations published in the literature; however, only 3 described superficial neuraxis siderosis as noted in this case. In the present case, slowly recurring hemorrhages of the lesion located at the conus likely contributed to the complete neuraxis superficial siderosis. Timely evaluation and treatment of these lesions is warranted to avoid further compressive and/or hemorrhagic complications.
Keywords: Cavernous malformation, extramedullary, intradural, superficial siderosis
Spinal cavernous malformations (cavernomas or cavernous hemangiomas) are infrequent vascular malformations which occur primarily in the vertebral body, with or without extradural extension, that constitute 5–12% of all spinal vascular abnormalities.[
History and examination
A 60-year-old male with diffuse headaches and intermittent fever over several months presented with a 1–2-week history of left-sided low back pain radiating to the buttocks and the posterior aspect of the left leg. Other than radicular complaints, he had no focal neurological deficit. Laboratory findings were unremarkable. Magnetic resonance imaging (MRI) of the spine revealed a 1.8 × 1.3 × 1.1 cm intradural-extramedullary hemorrhagic lesion below the conus medullaris (at the L2 level) along with moderate subarachnoid hemorrhage (SAH) in the lumbar cistern and superficial siderosis along the conus [
Lumbar spine MRI demonstrating intradural-extramedullary spinal cavernous malformation. Sagittal T2-weighted (a), fat-suppressed gadolinium-enhanced T1-weighted (b), and T2 inversion recovery turbo-spin echo images show an intradural extramedullary lesion at L2. Hemosiderin deposition is seen along the conus (arrows), and fluid-subarachnoid blood level is layered in the lumbar cistern (arrowheads). Axial T2-weighted images (d-g) corresponding to scout levels on image (c) show the nerve root proximal to the lesion (d), followed by sections in the midst (e) and immediately below (f) the lesion. Layering of subarachnoid blood is well-appreciated on axial images (arrowheads in g)
Brain and cervicothoracic MRI demonstrating extensive superficial siderosis. Cervical (a) and thoracic (b) T2-weighted sagittal MRI images show superficial siderosis (arrows) across the cervical and thoracic spinal cord. Intracranial involvement (arrows) is seen in the cerebellum and along the brain stem on axial gradient-echo sequences (c-f)
An L1-L2 laminectomy and durotomy were performed, and a large vascular lesion with numerous small blood-filled sacs consistent with cavernoma was identified. The lesion was readily separable from the surrounding nerve roots and was dissected out of the thecal sac [
Intraoperative images during resection of spinal cavernous malformation. Stimulation of the proximal (a) and distal (b) ends of the involved nerve root (arrows) did not reveal any detectable motor function, and therefore the lesion was resected en bloc, along with the adjacent nerve root segments entering/exiting the lesion (c)
Histopathological examination revealed that the mass was an encapsulated cavernous malformation with acute and chronic hemorrhages and focal inflammation [
Sections showing a vascular lesion composed of tightly packed vascular channels with varying wall diameters and hyalinization. Vessel walls lacked any significant amount of smooth muscle or elastic tissue. Some vessel walls contained hemosiderin laden macrophages suggesting remote microhemorrhages. Scattered vessels showed thrombi at different stages of organization. These histologic features were consistent with a diagnosis of cavernous angioma. Intermediate power image (a) showing tightly packed vascular channels with vessels walls of varying diameters and some with hyalinization. Vessels walls lack elastic tissue and a significant amount of smooth muscle. High-power H and E image (b) of the cavernous angioma with entrapped nerve fibers. High-power image (c) of neurofilament staining the entrapped nerve fibers
There are 56 reports (including the present) of intradural-extramedullary spinal cavernous malformations [
Superficial siderosis of the central nervous system (CNS) is caused by recurrent hemorrhage into the subarachnoid space, with resultant hemosiderin deposition in the subpial layers of the brain and spinal cord. Extensive hemosiderin deposition can be seen along the surface of the neuraxis, especially along the cerebellum, brainstem, and lower cranial nerves; the triad of sensorineural hearing loss, cerebellar ataxia, and pyramidal tract symptoms reflects the common sites of involvement in this disease.[
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Conflicts of interest
There are no conflicts of interest.
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