Gorham Stout disease of the temporal bone with cerebrospinal fluid leak

Pascale Aouad ◽  
Nancy M. Young ◽  
Amanda M. Saratsis ◽  
Meredith A. Reynolds ◽  
Maura E. Ryan
2008 ◽  
Vol 122 (11) ◽  
pp. 1168-1174 ◽  
K K Ramalingam ◽  
R Ramalingam ◽  
T M SreenivasaMurthy ◽  
G R Chandrakala

AbstractMeningo-encephalocoele of the temporal bone, also known as fungus cerebri, is a rare occurrence in clinical practice. We present a series of 13 patients with chronic otitis media who suffered brain herniation into the mastoid cavity. We also discuss the presentation and management of brain herniation with or without cerebrospinal fluid leak.Study design:Retrospective.Methods:Among 963 cases undergoing revision mastoid surgery, 13 patients suffered brain herniation. These cases were identified and analysed.Results:All 13 patients' initial diagnosis was chronic suppurative otitis media with cholesteatoma, and all had undergone previous mastoid surgery resulting in a defect in the tegmen and weakening of the dura mater. The revision procedures performed included 10 (76.9 per cent) modified radical mastoidectomies without ossicular chain reconstruction and one (7.6 per cent) modified radical mastoidectomy with ossicular chain reconstruction; two (15.3 per cent) patients required a blind sac closure. Brain herniation and/or cerebrospinal fluid leak were repaired by a transmastoid ± minicraniotomy procedure.Conclusions:Injury to the tegmen and dura should be avoided during surgery for chronic middle-ear disease. Cerebrospinal fluid leaks, if encountered, should be managed in the same surgical session. The transmastoid approach is helpful in repairing defects smaller than 1 cm in diameter, whereas the combined transmastoid-minicraniotomy approach provides good access when closing defects larger than 1 cm in diameter and also enables auto-calvarial grafting.

2019 ◽  
Vol 46 (3) ◽  
pp. 483
Federico Maria Gioacchini ◽  
Ettore Cassandro ◽  
Matteo Alicandri-Ciufelli ◽  
Shaniko Kaleci ◽  
Claudia Cassandro ◽  

2013 ◽  
Vol 74 (S 01) ◽  
Matthew Carlson ◽  
William Copeland ◽  
Stanley Pelosi ◽  
Colin Driscoll ◽  
Michael Link ◽  

1995 ◽  
Vol 112 (5) ◽  
pp. P39-P39
Ronald A. Hoffman

Educational objectives: To diagnose cerebrospinal fluid leak and to be knowledgeable regarding therapy.

T Hendriks ◽  
A Thompson ◽  
R Boeddinghaus ◽  
H E I Tan ◽  
J Kuthubutheen

Abstract Background and objective Spontaneous cerebrospinal fluid leak of the temporal bone is an emerging clinical entity for which prompt and accurate diagnosis is difficult given the subtle signs and symptoms that patients present with. This study sought to describe the key temporal bone abnormalities in patients with spontaneous cerebrospinal fluid leak. Methods A retrospective cohort study was conducted of adult patients with biochemically confirmed spontaneous cerebrospinal fluid leak. Demographics and radiological features identified on computed tomography imaging of the temporal bones and/or magnetic resonance imaging were analysed. Results Sixty-one patients with spontaneous cerebrospinal fluid leak were identified. Fifty-four patients (88.5 per cent) underwent both temporal bone computed tomography and magnetic resonance imaging. Despite imaging revealing bilateral defects in over 75 per cent of the cohort, only two patients presented with bilateral spontaneous cerebrospinal fluid leaks. Anterior tegmen mastoideum defects were most common, with an average size of 2.5 mm (range, 1–10 mm). Conclusion Temporal bone computed tomography is sensitive for the identification of defects when suspicion exists. In the setting of an opacified middle ear and/or mastoid, close examination of the skull base is crucial given that this fluid is potentially cerebrospinal fluid.

2019 ◽  
Vol 161 (3) ◽  
pp. 493-498
Joseph T. Breen ◽  
Colin R. Edwards ◽  
Rebecca S. Cornelius ◽  
J. Michael Hazenfield ◽  
Gavriel D. Kohlberg ◽  

ObjectiveTo demonstrate the clinical utility, sensitivity, and specificity of standard magnetic resonance imaging (MRI) sequences in differentiating temporal bone cerebrospinal fluid leaks from all other middle ear effusions.Study DesignRetrospective imaging review.SettingAcademic medical center.SubjectsPatients with cerebrospinal fluid leaks or other middle ear effusions who also underwent MRI.MethodsPatients were assigned to cerebrospinal fluid leak and other effusion cohorts based on clinical course, findings at surgery/myringotomy, and beta-2 transferrin fluid analysis. Reviewers blinded to the clinical outcome examined T1-weighted, T2-weighted, diffusion-weighted, fluid-attenuated inversion recovery (FLAIR), and 3-dimensional (3D) acquired T2-weighted MRI sequences. For each sequence, fluid imaged in the temporal bone was graded as either similar or dissimilar in signal intensity to cerebrospinal fluid in the adjacent subarachnoid space. Signal similarity was interpreted as being diagnostic of a leak. Test characteristics in predicting the presence of a leak were calculated for each series.ResultsEighty patients met criteria (41 leaks, 39 other effusions). The 3D T2 series was 76% sensitive and 100% specific in diagnosing a leak, and FLAIR was 44% sensitive and 100% specific. The T1-weighted (73% sensitive, 69% specific), T2-weighted (98% sensitive, 5.1% specific), and diffusion-weighted (63% sensitive, 66% specific) series were less useful.ConclusionsMRI, with attention to 3D T2 and FLAIR series, is a noninvasive and highly specific test for diagnosing cerebrospinal fluid leak in the setting of an indeterminate middle ear effusion.

2021 ◽  
pp. 014556132098456
Massimo Re ◽  
Mirko Giannoni ◽  
Alfonso Scarpa ◽  
Claudia Cassandro ◽  
Massimo Ralli ◽  

Stapes gusher is a massive flow of perilymph and cerebrospinal fluid leak that fills the middle ear immediately after surgical opening of the labyrinth, such as during stapedectomy. Stapes gusher usually occurs as the result of a congenital malformation that causes an abnormal communication between the perilymphatic space and the subarachnoid space involving the internal auditory canal or the cochlear duct. To date, the potential risk of stapes gusher cannot be assessed preoperatively, as there are not pathognomonic signs suggestive of this complication. However, high-resolution computed tomography scan (HRCT) of the temporal bone can provide information that may help recognizing patients at risk. Recently, an anatomic evaluation of the inner ear with oblique reformation at HRCT has been described. This reformation offers a new and more detailed topographic vision of temporal bone structures compared to the classic axial and coronal planes and may help identifying anatomical alterations otherwise not visible. In this article, we present a case of stapes gusher and the role of preoperative HRCT with oblique reformation in its prevention.

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