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

doi:10.1002/lary.28828

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

Istituto Regina Elena

Research output: Contribution to journal › Article › peer-review

Abstract

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 language	English
Pages (from-to)	E401-E407
Journal	Laryngoscope
Volume	131
Issue number	2
DOIs	https://doi.org/10.1002/lary.28828
Publication status	Published - Feb 2021

Keywords

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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10.1002/lary.28828

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title = "Spontaneous CSF Leaks and IIH: A Flawless Connection? An Experience With 167 Patients",

abstract = "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.",

keywords = "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",

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

note = "{\textcopyright} 2020 The American Laryngological, Rhinological and Otological Society, Inc.",

year = "2021",

month = feb,

doi = "10.1002/lary.28828",

language = "English",

volume = "131",

pages = "E401--E407",

journal = "Laryngoscope",

issn = "0023-852X",

publisher = "John Wiley and Sons Inc.",

number = "2",

}

TY - JOUR

T1 - Spontaneous CSF Leaks and IIH

T2 - A Flawless Connection? An Experience With 167 Patients

AU - Zocchi, Jacopo

AU - Pietrobon, Giacomo

AU - Lepera, Davide

AU - Gallo, Stefania

AU - Russo, Federico

AU - Volpi, Luca

AU - Pellini, Raul

AU - Bignami, Maurizio

AU - Castelnuovo, Paolo

PY - 2021/2

Y1 - 2021/2

N2 - 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.

AB - 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.

KW - Adult

KW - Aged

KW - Aged, 80 and over

KW - Cerebrospinal Fluid Rhinorrhea/diagnosis

KW - Female

KW - Follow-Up Studies

KW - Humans

KW - Male

KW - Middle Aged

KW - Natural Orifice Endoscopic Surgery/methods

KW - Pseudotumor Cerebri/complications

KW - Recurrence

KW - Retrospective Studies

KW - Secondary Prevention/methods

KW - Tomography, X-Ray Computed

KW - Transferrin/analysis

KW - Treatment Outcome

KW - Young Adult

U2 - 10.1002/lary.28828

DO - 10.1002/lary.28828

M3 - Article

C2 - 32557740

SN - 0023-852X

VL - 131

SP - E401-E407

JO - Laryngoscope

JF - Laryngoscope

IS - 2

ER -

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

Abstract

Keywords

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