Spontaneous CSF Leaks and IIH: A Flawless Connection? An Experience With 167 Patients

Jacopo Zocchi, Giacomo Pietrobon, Davide Lepera, Stefania Gallo, Federico Russo, Luca Volpi, Raul Pellini, Maurizio Bignami, Paolo Castelnuovo

Research output: Contribution to journalArticlepeer-review


OBJECTIVES: Spontaneous cerebrospinal fluid (CSF) leaks are a rare but insidious clinical entity. In the recent literature, their pathogenesis has been linked with idiopathic intracranial hypertension (IIH). Considering a worse surgical outcome compared to traumatic leaks, a medical treatment of the underlying IIH has been suggested in order to prevent relapses. Nevertheless, some aspects of this rare pathology remain unclear. We report our long experience in the management of spontaneous CSF leaks.

METHODS: A retrospective evaluation of all patients with spontaneous CSF leaks treated from 1998 to 2018 in a tertiary referral center was carried out. All patients received surgery without any adjuvant routine medical treatment. Epidemiological and clinical data were reviewed. A telephone survey was conducted to investigate IIH-related symptoms in this population.

RESULTS: One hundred sixty-seven patients and 195 procedures were included in the study. The total rate of failure after surgical treatment was 9%. Eight patients suffered a relapse in the same site (4%, 8 of 195), whereas seven patients experienced a relapse in a different site (4%, 7 of 167). Twenty-nine percent of the population interviewed referred at least one IIH-related symptom.

CONCLUSION: Spontaneous CSF leaks represent a complex pathology that need a multidisciplinary evaluation. Surgical treatment seems to guarantee favorable results in most of the patients. Despite this, a not negligible percentage of patients experienced a relapse during a long-term follow-up. Further studies are needed to reach a deeper understanding of the presentation and course of this particular condition.

LEVEL OF EVIDENCE: 4 Laryngoscope, 131:E401-E407, 2021.

Original languageEnglish
Pages (from-to)E401-E407
Issue number2
Publication statusPublished - Feb 2021


  • Adult
  • Aged
  • Aged, 80 and over
  • Cerebrospinal Fluid Rhinorrhea/diagnosis
  • Female
  • Follow-Up Studies
  • Humans
  • Male
  • Middle Aged
  • Natural Orifice Endoscopic Surgery/methods
  • Pseudotumor Cerebri/complications
  • Recurrence
  • Retrospective Studies
  • Secondary Prevention/methods
  • Tomography, X-Ray Computed
  • Transferrin/analysis
  • Treatment Outcome
  • Young Adult


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