Hydrocephalus Following Bilateral Dumbbell-Shaped C2 Spinal Neurofibromas Resection and Postoperative Cervical Pseudomeningocele in a Patient with Neurofibromatosis Type 1: A Case Report

Abstract

Study Design Case report.

Objective To present a rare case of hydrocephalus following bilateral dumbbell-shaped C2 spinal neurofibromas resection and postoperative cervical pseudomeningocele in a patient with neurofibromatosis type 1 (NF1).

Methods The patient's clinical course is retrospectively reviewed. A 37-year-old man affected by NF1 referred to our department for progressive weakness of both lower extremities and gait disturbance. Radiological imaging showed bilateral dumbbell-shaped C2 spinal neurofibromas. After its resection, at the 1-month follow-up evaluation, the patient reported headache and nausea. A CT brain scan showed a postoperative cervical pseudomeningocele and an increase in the ventricular sizes, resulting in hydrocephalus.

Results A ventriculoperitoneal shunting was performed using a programmable valve opening pressure set to 120 mmH₂0. After surgery, the patient's neurological status markedly improved.

Conclusion Hydrocephalus must be considered a possible complication of cervical spine tumor resection.

Keywords

spinal cord tumor - cervical spine - dumbbell-shaped neurofibroma - hydrocephalus - postoperative cervical pseudomeningocele - neurofibromatosis type 1

• References

• 1 Conference NIHCD ; National Institutes of Health Consensus Development Conference. Neurofibromatosis. Conference statement. Arch Neurol 1988; 45 (5) 575-578
• 2 Bartolomei JC, Crockard HA. Bilateral posterolateral approach to mirror-image C-2 neurofibromas. Report of four cases. J Neurosurg 2001; 94 (2, Suppl) 292-298
• 3 Taleb FS, Guha A, Arnold PM, Fehlings MG, Massicotte EM. Surgical management of cervical spine manifestations of neurofibromatosis Type 1: long-term clinical and radiological follow-up in 22 cases. J Neurosurg Spine 2011; 14 (3) 356-366
• 4 Junming M, Cheng Y, Dong C , et al. Giant cell tumor of the cervical spine: a series of 22 cases and outcomes. Spine (Phila Pa 1976) 2008; 33 (3) 280-288
• 5 Dinçer A, Yener U, Özek MM. Hydrocephalus in patients with neurofibromatosis type 1: MR imaging findings and the outcome of endoscopic third ventriculostomy. AJNR Am J Neuroradiol 2011; 32 (4) 643-646
• 6 Hosoda K, Kanazawa Y, Tanaka J, Tamaki N, Matsumoto S. Neurofibromatosis presenting with aqueductal stenosis due to a tumor of the aqueduct: case report. Neurosurgery 1986; 19 (6) 1035-1037
• 7 Pollack IF, Shultz B, Mulvihill JJ. The management of brainstem gliomas in patients with neurofibromatosis 1. Neurology 1996; 46 (6) 1652-1660
• 8 Créange A, Zeller J, Rostaing-Rigattieri S , et al. Neurological complications of neurofibromatosis type 1 in adulthood. Brain 1999; 122 (Pt 3) 473-481
• 9 Mirone G, Cinalli G, Spennato P, Ruggiero C, Aliberti F. Hydrocephalus and spinal cord tumors: a review. Childs Nerv Syst 2011; 27 (10) 1741-1749
• 10 Rifkinson-Mann S, Wisoff JH, Epstein F. The association of hydrocephalus with intramedullary spinal cord tumors: a series of 25 patients. Neurosurgery 1990; 27 (5) 749-754 , discussion 754
• 11 Cohen AR, Wisoff JH, Allen JC, Epstein F. Malignant astrocytomas of the spinal cord. J Neurosurg 1989; 70 (1) 50-54