Progressive superficial siderosis from Chronic CSF leak as a long-term complication of cervical anterior corpectomy: A case report and review of the literature
- Department of Radiology, Weill Cornell Medicine, New York City, New York, United States,
- Department of Neurology, Weill Cornell Medicine, New York City, New York, United States,
- Department of Neurosurgery, Weill Cornell Medicine, New York City, New York, United States.
Pierce W. McMahon, Department of Radiology, Weill Cornell Medicine, New York City, New York, United States.
DOI:10.25259/SNI_493_2022Copyright: © 2022 Surgical Neurology International This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, transform, 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: Pierce W. McMahon1, Joshua Loewenstern1, Peter Girgis2, Apostolos John Tsiouris1, Matthew Fink2, Roger Härtl3, Gayle Salama1. Progressive superficial siderosis from Chronic CSF leak as a long-term complication of cervical anterior corpectomy: A case report and review of the literature. 05-Aug-2022;13:341
How to cite this URL: Pierce W. McMahon1, Joshua Loewenstern1, Peter Girgis2, Apostolos John Tsiouris1, Matthew Fink2, Roger Härtl3, Gayle Salama1. Progressive superficial siderosis from Chronic CSF leak as a long-term complication of cervical anterior corpectomy: A case report and review of the literature. 05-Aug-2022;13:341. Available from: https://surgicalneurologyint.com/surgicalint-articles/11765/
Background: Superficial siderosis of the central nervous system (SSCNS) is a rare progressive neurological disorder resulting from chronic subarachnoid hemorrhage and subsequent subpial hemosiderin deposition. A prolonged cerebrospinal fluid (CSF) leak is a known cause of SSCNS. We present a novel case where progressive SSCNS resulted from a chronic CSF leak related to an anterior cervical corpectomy.
Case Description: A 73-year-old man presented with gait ataxia and progressive hearing loss. Thirteen years before, he had undergone a combined anterior-posterior cervical decompression for symptomatic ossification of the posterior longitudinal ligament (OPLL). The presenting MR imaging showed extensive superficial siderosis and focal spinal cord herniation at the site of a ventral dural defect at the corpectomy site. A CT myelogram showed extensive CSF leakage into the corpectomy surgical site and a communicating pseudomeningocele in the anterior neck.
Conclusion: This is the first reported case of progressive SSCNS as a long-term complication of an anterior cervical corpectomy for OPLL. Clinicians should be aware of SSCNS secondary to a chronic CSF leak in patients with a prior corpectomy.
Keywords: Anterior corpectomy, CSF leak, superficial siderosis, Ossified posterior longitudinal ligament, Pseudomeningocele, Spinal cord herniation
Ossification of the posterior longitudinal ligament (OPLL) is a known cause of myelopathy that may necessitate surgical decompression for spinal cord decompression.[
The incidence of dural tears after anterior decompression varies from 0% to 3%; however, in studies specifically reporting on anterior corpectomy with an OPLL, the incidence of a dural tear is as high as 32%.[
Superficial siderosis of the central nervous system (SSCNS) is an uncommon progressive neurological disorder that is the consequence of hemosiderin deposition in the subpial layer due to recurrent chronic subarachnoid hemorrhage. SSCNS typically presents with gait ataxia, hearing loss, and other cranial neuropathies. The cause of bleeding remains unknown in many cases, although dural tears are one of the most common etiologies.[
In addition to SSCNS, spinal cord herniation (SCH) is another problematic long-term postoperative complication of CSF leak following an anterior corpectomy that has been scarcely described in the literature.[
In addition, in rare occasions, iatrogenic cervical pseudomeningoceles may occur in the postoperative period after anterior cervical procedures, especially anterior corpectomy, as a consequence of an inadvertent tear of the dura mater and resultant CSF leakage into the anterior compartment of the neck.[
In this report, we discuss the case of a patient who developed classic neurologic symptoms of SSCNS years after an anterior corpectomy for symptomatic OPLL. Further workup of the supratentorial and infratentorial superficial siderosis, which was identified on brain MRI imaging, led to the discovery of the surgical site CSF leak, anterior neck pseudomeningocele, and mild SCH.
Presenting history and examination
A 73-year-old man who reported chronic gait impairment and progressive hearing loss presented with worsening balance and weakness in his arms and legs over the past 2 years. Neurologic physical examination demonstrated that the range of motion of his neck was severely impaired on extension and lateral rotation. He had hearing loss, motor weakness, abnormal coordination and gait, and poor balance. He had also noted loss of sense of smell and diminished sense of taste. He had difficulty walking on his toes and heels without assistance. Upper extremity weakness was noted especially at the triceps, bilaterally, with distal muscles relatively normal, but poor fine motor movements. Reflexes were diminished at the biceps and brachioradialis muscles and absent at the triceps. He had decreased perception of pin prick and light touch in fingers of both hands. Of note, he denied current neck or back pain.
His initial symptoms of gait ataxia began in 2008, when he was diagnosed with cervical spinal stenosis due to marginal disc osteophytosis and OPLL. He subsequently underwent a combined anterior-posterior surgery at an outside medical center including anterior decompression of the spinal cord with corpectomy, removal of bone spurs, and placement of a vertebral titanium mesh cage. In the same operation, he also had posterior decompression with bilateral laminectomy from C2 to C4. He suffered extensive postoperative complications, including a lower extremity deep vein thrombosis, pneumothorax, and residual weakness in his arms and legs. He was hospitalized for several weeks after his operation, and eventually made a full recovery and went home with improved neurological symptoms compared to before the operation.
MRI demonstrated extensive superficial siderosis over the cerebral hemispheres, cerebellar hemispheres, and the spinal cord extending from the foramen magnum down into the thoracic spinal cord. Prior imaging at an outside medical center did reveal evidence of mild superficial siderosis 5 years postoperatively [
Representative axial MR GRE sequences from 5 years after surgery (a) and 13 years after surgery (b) shows low intensity signal in the superior cerebellum, indicative of hemosiderin deposition compatible with superficial siderosis. The superficial siderosis is expanding in area from 5 years to 13 years after initial anterior corpectomy and fusion.
(a) Representative axial (left) and sagittal (right) T2-weighted fast spin echo images from 13 years after surgery demonstrating a ventral dural defect (dotted-line arrow) at approximately level C2 and CSF extending from the corpectomy site around the titanium mesh cage (solid-line arrow). (b) Additional axial (left) and sagittal (right) slices demonstrating a fluid collection (solid-line arrows) in the right anterolateral soft tissues of the neck extending from corpectomy site at approximately level C4.
During evaluation at our medical center, the patient underwent a total spine CT myelogram due to concern for a CSF leak which may be contributing to the progressive SSCNS findings [
Coronal (upper left) and sagittal (lower left) scout images of the intact anterior corpectomy with titanium mesh cage, anterior plate and screw construct from C2-C4, and posterior laminectomy and fusion with transpedicular screws and interconnecting rods from C2-C4. Axial (upper middle) and sagittal (upper right) post-injection CT myelogram images demonstrating a ventral dural defect (dotted-line arrow) at level C2 with borderline spinal cord herniation and CSF throughout the corpectomy site and within the titanium mesh cage. Axial (lower middle) and sagittal (lower right) postinjection CT myelogram images showing a fluid collection in the right anterolateral soft tissues of the neck extending from the corpectomy site (solid-line arrows) at level C4.
At the C3 level, there was a large ventral dural defect with extrathecal contrast completely opacifying the corpectomy surgical site [
Shortly after CT myelogram was performed, the patient had a mechanical fall as a result of his progressive impaired balance and weakness, sustaining a hip fracture requiring repair. Given his age, clinical condition, and comorbidities, the likelihood of life-threatening complications was deemed prohibitive to justify a revision and repair operation at the corpectomy site.
Anterior corpectomy for OPLL related spinal canal stenosis is associated with an increased risk of creating a ventral dural tear because of adhesion between the OPLL and dura.[
SCH through an iatrogenic dural defect following anterior corpectomy with postoperative CSF leak is another complication that can produce neurological symptoms.[
In rare occasions, iatrogenic cervical pseudomeningoceles may occur after anterior corpectomy procedures, likely as a consequence of an inadvertent tear of the dura mater with breach into the subarachnoid space or a lack of closure of the dural tear with resultant CSF leakage into the anterior compartment of the neck. The dural defects permit CSF egress to the surrounding tissues through a fistula when the CSF pulse pressure exceeds the interstitial pressure of the surrounding tissue, therefore, leading to pseudomeningocele formation, typically in the anterior compartment of the neck.[
Typically, SCH and CSF leak is repaired through anterior approach by removing the titanium cage and associated scarring and adhesions and then repairing the dural tear with replacement of the herniated spinal cord.[
This case highlights the importance of identifying a CSF leak as a long-term postoperative complication of an anterior corpectomy for OPLL. Clinicians should consider a CSF leak in their differential when patients present with neurological symptoms and imaging findings consistent with SSCNS following an anterior corpectomy, even if the surgery was remote. Therefore, patients can be immediately referred to myelography, resulting in earlier CSF leak diagnosis, which can lead to targeted intervention and symptom amelioration.[
Patient’s consent not required as patient’s identity is not disclosed or compromised.
There are no conflicts of interest.
1. An HS, Al-Shihabi L, Kurd M. Surgical treatment for ossification of the posterior longitudinal ligament in the cervical spine. J Am Acad Orthop Surg. 2014. 22: 420-9
2. Bertalanffy H, Eggert HR. Complications of anterior cervical discectomy without fusion in 450 consecutive patients. Acta Neurochir (Wien). 1989. 99: 41-50
3. Brembilla C, Lanterna LA, Bonito V, Gardinetti M, Dorelli G, Rampini AD. Updating superficial siderosis of the central nervous system: Bleeding of a dorsal osteophyte into the subarachnoid space from a perforating artery. J Neurosurg Spine. 2018. 30: 106-10
4. Fountas KN, Kapsalaki EZ, Johnston KW. Cerebrospinal fluid fistula secondary to dural tear in anterior cervical discectomy and fusion: Case report. Spine (Phila Pa 1976). 2005. 30: E277-80
5. Foyt D, Johnson JP, Kirsch AJ, Bruce JN, Wazen JJ. Dural closure with laser tissue welding. Otolaryngol Head Neck Surg. 1996. 115: 513-8
6. Guppy KH, Silverthorn JW. Spinal cord herniation after cervical corpectomy with cerebrospinal fluid leak: Case report and review of the literature. World Neurosurg. 2017. 100: 711.e7-711.e12
7. Hannallah D, Lee J, Khan M, Donaldson WF, Kang JD. Cerebrospinal fluid leaks following cervical spine surgery. J Bone Joint Surg Am. 2008. 90: 1101-5
8. Horowitz SW, Azar-Kia B, Fine M. Postoperative cervical pseudomeningocele. AJNR Am J Neuroradiol. 1990. 11: 784
9. Joseph V, Kumar GS, Rajshekhar V. Cerebrospinal fluid leak during cervical corpectomy for ossified posterior longitudinal ligament: Incidence, management, and outcome. Spine (Phila Pa 1976). 2009. 34: 491-4
10. Kala M. Cerebrospinal fluid pseudocyst after anterior stabilization for cervical spine injury treated by ventricular drainage: Case report. Surg Neurol. 1996. 45: 293-5
11. Kitchel SH, Eismont FJ, Green BA. Closed subarachnoid drainage for management of cerebrospinal fluid leakage after an operation on the spine. J Bone Joint Surg Am. 1989. 71: 984-7
12. Kizilay Z, Yilmaz A, Ismailoglu O, Coskun ME. Anterior spinal cord herniation after multilevel anterior cervical corpectomy: A case report. Neurol Neurochir Pol. 2016. 50: 190-4
13. Kumar N. Neuroimaging in superficial siderosis: An in-depth look. AJNR Am J Neuroradiol. 2010. 31: 5-14
14. Mazur M, Jost GF, Schmidt MH, Bisson EF. Management of cerebrospinal fluid leaks after anterior decompression for ossification of the posterior longitudinal ligament: A review of the literature. Neurosurg Focus. 2011. 30: E13
15. Min JH, Jung BJ, Jang JS, Kim SK, Jung DJ, Lee SH. Spinal cord herniation after multilevel anterior cervical corpectomy and fusion for ossification of the posterior longitudinal ligament of the cervical spine. J Neurosurg Spine. 2009. 10: 240-3
16. Nakase H, Park YS, Kimura H, Sakaki T, Morimoto T. Complications and long-term follow-up results in titanium mesh cage reconstruction after cervical corpectomy. J Spinal Disord Tech. 2006. 19: 353-7
17. Rahimizadeh A, Soufiani H, Rahimizadeh S. Remote cervical pseudomeningocele following anterior cervical corpectomy and fusion: report of a case and review of the literature. Int J Spine Surg. 2016. 10: 36
18. Schievink WI, Maya MM, Nuño M. Chronic cerebellar hemorrhage in spontaneous intracranial hypotension: Association with ventral spinal cerebrospinal fluid leaks: Clinical article. J Neurosurg Spine. 2011. 15: 433-40
19. Smith MD, Bolesta MJ, Leventhal M, Bohlman HH. Postoperative cerebrospinal-fluid fistula associated with erosion of the dura. Findings after anterior resection of ossification of the posterior longitudinal ligament in the cervical spine. J Bone Joint Surg Am. 1992. 74: 270-7
20. Takai K, Taniguchi M. Superficial siderosis of the central nervous system associated with ventral dural defects: Bleeding from the epidural venous plexus. J Neurol. 2021. 268: 1491-4
21. Wipplinger C, Simian A, Hernandez RN, Navarro-Ramirez R, Kim E, Kirnaz S. Superficial siderosis of central nervous system as primary clinical manifestation secondary to intradural thoracic disk herniation. World Neurosurg. 2018. 119: 40-4
22. Yokosuka J, Takai K, Komori T, Taniguchi M. Superficial siderosis: Bleeding from the bone marrow after laminectomy for spinal tumor removal. J Neurosurg Spine. 2014. 21: 905-8