Combined surgical repair and venous sinus stenting for patients with skull base encephaloceles secondary to dural venous sinus stenosis

Abstract

Background
Chronically elevated intracranial pressure (ICP) seen in idiopathic intracranial hypertension (IIH) can cause the development of skull base encephaloceles and cerebrospinal fluid (CSF) leaks. Surgical repair and ventriculoperitoneal shunt (VPS) placement are mainstays of treatment. Venous sinus stenting (VSS) is a newly accepted treatment modality. The goal of this study was thus to determine if VSS can be used to treat symptoms and prevent recurrence after surgical encephalocele repair.

Methods
Retrospective chart review of patients that had surgical repair of encephaloceles followed by VSS for symptomatic stenosis with elevated pressure gradient.

Results
A total of 13 patients underwent a combined encephalocele repair and VSS. Seventy-two percent were female; 46% had headaches, 69% pulsatile tinnitus, and 92% CSF rhinorrhea or otorrhea. One had seizures. Mean lumbar opening pressure was 23.3 ± 2.6 cm H2O; the average sagittal-to-jugular pressure gradient was 12.7 ± 1.8 cmH2O and was elevated in all patients. Four patients had middle fossa craniotomy for repair of tegmen defect (one bilateral); one had a retrosigmoid craniotomy for repair of a sigmoid plate defect. Eight had an endoscopic endonasal repair for sphenoid or cribriform plate encephalocele. There were no VSS procedural complications or complications associated with dual antiplatelet therapy. One patient had meningitis after endoscopic repair that was treated with antibiotics. One patient had recurrence of both CSF leak and venous stenosis adjacent to the stent requiring repeat repair and VSS. There was no further recurrence.

Conclusion
In patients with dural sinus stenosis and encephaloceles requiring repair, VSS can be performed safely within weeks of surgery for relief of symptoms, resolution of underlying pathology, and prevention of CSF leak recurrence.

Full text

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Acta Neurochirurgica (2023) 165:2283–2292
https://doi.org/10.1007/s00701-023-05680-w
ORIGINAL ARTICLE
Combined surgical repair andvenous sinus stenting forpatients
withskull base encephaloceles secondary todural venous sinus
stenosis
UmbertoTosi1 · AlexanderRamos1· MargheritaRampichini1· GeorgeAlexiades2· SrikanthBoddu1·
BabacarCisse1· AshutoshKacker2· AthosPatsalides1· AbtinTabaee2· JustinSchwarz1· TheodoreH.Schwartz1,2·
RohanRamakrishna1
Received: 4 March 2023 / Accepted: 9 June 2023 / Published online: 21 June 2023
© The Author(s), under exclusive licence to Springer-Verlag GmbH Austria, part of Springer Nature 2023
Abstract
Background Chronically elevated intracranial pressure (ICP) seen in idiopathic intracranial hypertension (IIH) can cause
the development of skull base encephaloceles and cerebrospinal fluid (CSF) leaks. Surgical repair and ventriculoperitoneal
shunt (VPS) placement are mainstays of treatment. Venous sinus stenting (VSS) is a newly accepted treatment modality.
The goal of this study was thus to determine if VSS can be used to treat symptoms and prevent recurrence after surgical
encephalocele repair.
Methods Retrospective chart review of patients that had surgical repair of encephaloceles followed by VSS for symptomatic
stenosis with elevated pressure gradient.
Results A total of 13 patients underwent a combined encephalocele repair and VSS. Seventy-two percent were female;
46% had headaches, 69% pulsatile tinnitus, and 92% CSF rhinorrhea or otorrhea. One had seizures. Mean lumbar opening
pressure was 23.3 ± 2.6cm H2O; the average sagittal-to-jugular pressure gradient was 12.7 ± 1.8 cmH2O and was elevated
in all patients. Four patients had middle fossa craniotomy for repair of tegmen defect (one bilateral); one had a retrosigmoid
craniotomy for repair of a sigmoid plate defect. Eight had an endoscopic endonasal repair for sphenoid or cribriform plate
encephalocele. There were no VSS procedural complications or complications associated with dual antiplatelet therapy. One
patient had meningitis after endoscopic repair that was treated with antibiotics. One patient had recurrence of both CSF leak
and venous stenosis adjacent to the stent requiring repeat repair and VSS. There was no further recurrence.
Conclusion In patients with dural sinus stenosis and encephaloceles requiring repair, VSS can be performed safely within
weeks of surgery for relief of symptoms, resolution of underlying pathology, and prevention of CSF leak recurrence.
Keywords CSF leak· Encephalocele· Sinus stenting· Skull base defect
Introduction
Spontaneous cerebrospinal fluid (CSF) leaks and enceph-
aloceles are rare entities that present with CSF rhino- or
otorrhea and, more rarely, neurological defects or com-
promise (e.g., seizures) [21, 35]. Currently, there is no
uniform understanding about their etiology, with some
believed to be due to prior trauma or developmental
anomalies and others as a consequence of chronically
raised intracranial pressure (ICP), either idiopathic or
secondary to space occupying lesions. For instance,
encephaloceles are believed to be often associated with
idiopathic intracranial hypertension (IIH), a condition
characterized by an absence of an intracranial mass or
* Rohan Ramakrishna
ror9068@med.cornell.edu
Umberto Tosi
umt9001@nyp.org
1 Department ofNeurological Surgery, NewYork-Presbyterian,
Weill Cornell Medicine, 525 E 68th St, Box99, NewYork,
NY10065, USA
2 Department ofOtolaryngology, NewYork-Presbyterian, Weill
Cornell Medicine, NewYork, NY, USA
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