Multiple spontaneous skull base cerebrospinal fluid leaks: some insights from an international retrospective collaborative study

• DOI: 10.1007/s00405-020-06227-w
• Publication Date: 2020-07-28T17:26:17Z
• Authors: Iacopo Dallan; Christina Cambi; Enzo Emanuelli; Diego Cazzador; Frank Rikki Canevari; Daniele Borsetto; James R. Tysome; Neil P. Donnelly; Mario Rigante; Christos Georgalas; Isam Alobid; Gabriele Molteni; Daniele Marchioni; Ahmed Khuram Shah...; Mariella Scarano; Veronica Seccia; Ernesto Pasquini
• Keywords: Skull Base; Cerebrospinal Fluid Leak; Greece; United Kingdom; 3. Good health; Idiopathic intracranial hypertension; Skull base; 03 medical and health sciences; Treatment Outcome; 0302 clinical medicine; Italy; Spain; Recurrent cerebrospinal fluid leaks; Spontaneous cerebrospinal fluid leak; Humans; Multiple cerebrospinal fluid leaks; Prospective Studies; Idiopathic intracranial hypertension; Multiple cerebrospinal fluid leaks; Recurrent cerebrospinal fluid leaks; Skull base; Spontaneous cerebrospinal fluid leak; Retrospective Studies

Spontaneous skull base cerebrospinal fluid leaks occurring without any apparent cause are rare. But those patients that present such leaks simultaneously, or successively, in multiple locations are even rarer. Given the rarity of this condition, we collected data from other groups in a multicentre study to reach an adequate number of patients and draw some preliminary considerations.We carried out a multicentre retrospective study on a cohort of patients treated at third level hospitals in Italy, Spain, United Kingdom and Greece for multiple spontaneous-CSF leaks and we compared them with a control group of patients treated for recurrent spontaneous-CSF leaks. Data regarding clinical aspects, radiological findings, surgical techniques and outcome were collected and preliminary considerations on the results were discussed.A total of 25 patients presented multiple simultaneous spontaneous CSF leaks while 18 patients fit with the criteria of recurrent spontaneous CSF leaks. Data analysis was conducted separately.Our understanding of the pathogenesis of this condition is currently very limited. A causative role of IIH may be present but the differences that emerged from the comparison with patients with recurrent fistulas seem to promote the possible role of other cofactors. A longer follow-up period is needed, and, in our opinion, prospective and multicentre studies are the only solution to seriously deal with such a complex topic.