Technical NoteEndoscopic Management of Spontaneous Clival Cerebrospinal Fluid Leaks: Case Series and Literature Review
Introduction
Spontaneous cerebrospinal fluid (CSF) leaks consist of idiopathic CSF leakages in which no definable cause, such as trauma, previous surgery, or tumor, is present.1 Spontaneous CSF leaks have been associated with increased intracranial pressure (ICP) and an underlying diagnosis of intracranial hypertension (ICH); moreover, patients with spontaneous CSF leaks are generally middle-aged and obese women—a group of patients who are also at high risk of developing ICH.2 In patients with spontaneous CSF leaks, clinical and radiographic signs of increased ICP, such as empty sella (80%), arachnoid pits (63%), dural sinus stenosis or thrombosis, dilation of the optic nerve sheaths, and a thinned bone of the skull base, can be found.3 Patients with spontaneous CSF leaks typically present with a long history of CSF rhinorrhea and episodes of meningitis or brain abscess, which represent the main complications of persistent leaks and could be life-threatening. Spontaneous CSF leaks can occur anywhere along the skull base at the cribriform plate, ethmoid sinus, frontal sinus, sphenoid sinus, and middle ear. However, CSF leaks are generally more common along the anterior skull base, in particular, in the cribriform plate, where sphenoidal localization is less common. Moreover, as reported by Van Zele et al.,4 transclival meningoceles and spontaneous CSF leaks located at the clivus are extremely rare entities, and only a few have previously been described.
Persistent CSF leaks should be repaired because of the risk of complications. Endoscopic endonasal surgery for the repair of CSF leaks is currently considered as the gold standard management process.5, 6 A review by Psaltis et al.7 presented an overall success rate for primary endoscopic repair of spontaneous CSF leaks of 90%, increasing to 96.6% on a second surgical attempt. Moreover, leakages from the sphenoid sinus represent a unique challenge, owing to the anatomic relationship and the extreme variability in the shape and pneumatization of the sinus itself.8
Multilayer closure is usually considered as the standard of care for the endoscopic repair of sphenoid sinus CSF leaks.8 The multilayer technique involves intracranially and extracranially inserting ≥1 layers of grafting material. In 2006, Hadad et al.9 described a technique consisting of a vascular pedicled flap of the nasal septum mucoperiosteum and mucoperichondrium based on the nasoseptal artery (a branch of the posterior septal artery and the terminal branch of the internal maxillary artery) for the endoscopic repair of skull base defects (Figure 1). Up to now, this vascularized flap has been considered the “workhorse” for the management of CSF leaks along the entire cranial base, in particular, in transsphenoidal corridors (planum, sella, and clivus defects).
In this report, we describe 6 cases of clival spontaneous CSF leaks that were surgically treated with an endoscopic endonasal approach; we also analyzed the surgical outcome of these patients and discussed the role of the nasoseptal flap (NSF) in this anatomic region. We also performed a literature review regarding clival localization of spontaneous CSF leaks.
Section snippets
Materials and Methods
The study was conducted in 2 tertiary, university-affiliated medical centers in Italy. As stated by the local ethics committee, patients gave informed consent for review of their clinical data. A retrospective analysis of patients treated for CSF leaks between 2005 and 2014 was performed. Only spontaneous clival leaks were considered in this study; other localizations were not considered. Postoperative and traumatic leaks were also excluded. Preoperative assessments included checking symptoms,
Results
Between 2005 and 2014, 67 patients underwent endoscopic surgery for spontaneous CSF leaks in the departments of our institutions. Of these, 6 patients (9%) presented with CSF leaks in the clival region (Table 1). Primary spontaneous CSF leaks were diagnosed in all patients as previously described.
The mean age was 60 years (range, 36–91 years old), and all patients were women (100%). Mean body mass index in our group was 28.3 kg/m2 (range, 21–35 kg/m2). All patients presented with a history of
Discussion
Spontaneous CSF leaks account for 40% of fistulas as reported in the systematic review by Psaltis et al.7 Moreover, Psaltis et al. described the cribriform plate as the most commonly affected site, which is involved in 52.7% of cases, whereas the sphenoid sinus accounts for 30.2% of all fistulas. Among the sphenoidal localizations, spontaneous CSF leaks arising from the prepontine cistern through the clivus are extremely rare. As stated in the literature review and reported in Table 2, to date,
Conclusions
Clival spontaneous CSF leaks represent an extremely rare condition. Analysis of previously published reports identified 15 articles with descriptions of 37 cases of clival localization of the defect, with the largest series of 6 consecutive cases by Van Zele et al.4 Etiologic factors of this condition may include cranial malformations, hyperpneumatized sphenoid sinus, and functional factors such as increased ICP. In particular, an accurate assessment of ICP should be performed in all patients
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