Cisternotomy and Lamina Terminalis Fenestration as a New Surgical Treatment for Idiopathic Intracranial Hypertension (Pseudotumor Cerebri): Case Report

Introduction: Idiopathic intracranial hypertension presents with manifestations of increased intracranial pressure, such as headache, vomiting, and papilledema. Surgical management of cases refractory to medical therapy involves placement of a cerebral spinal fluid (CSF) shunt. Subarachnoid basal cistern opening is used during many microsurgical operations to relax the brain by removing or diverting CSF. Recent developments indicate that CSF from the ventricles does not communicate with the parenchyma but rather CSF from the cisterns communicates with the parenchyma through the Virchow-Robin spaces via the glymphatic system. Therefore, release of CSF via cisternotomy is purported to increase removal of interstitial fluid and CSF.

Methods: A review of the literature did not reveal any similar cases. This is considered the first reported case of pseudotumor cerebri treatment with cisternotomy.

Results:

Case Presentation: A 39-year-old woman presented with headache and nausea with grade-1 papilledema in the setting of maximally tolerated medical management. She was diagnosed with idiopathic intracranial hypertension and had significant benefit with lumbar drain trial therefore a ventriculoperitoneal shunt was placed. Five months later her shunt had to be removed due to infection, and Acetazolamide was restarted.
One month later, she presented with three weeks of progressive headaches and nausea. Headache improved after large volume lumbar puncture showing opening pressure of 27cmH2O. CSF labs were normal. She declined replacement of her shunt as the next step and therefore underwent a right eyebrow craniotomy for cisternotomy and lamina terminals fenestration. At surgery, the arachnoid was found to be exceptionally thick and required nearly exclusive sharp dissection. Surgery was uncomplicated. Postoperatively her symptoms improved completely. She was off Acetazolamide at 6-week follow up and no longer had her headaches. Neuro-ophthalmology visit demonstrated stable grade-1 papilledema.

Conclusion : Cisternotomy and lamina terminals fenestration presents a potential novel surgical option for patients with pseudotumor cerebri and will require larger trials.