Spontaneous Encephalomeningocele: A Case Report

Heloisa dos Santos Sobreira Nunes*, Eduardo Amaro Bogaz, José Antônio Pinto, Kelly Abdo and Andréia Vasconcelos

Received: May 30, 2018;   Published: June 13, 2018

DOI: 10.26717/BJSTR.2018.05.001220

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Abstract

Herniation of meningeal and brain tissue into the middle ear and mastoid through a bony defect that allows meningeal and/or brain tissue to protude into temporal bone is a rare pahtological entity with potentially life-threatening complications, requiring surgery [1,2]. While herniation of brain tissue in middle ear usually occurs from the middle fossa through a defect either in tegmen tympani or in the mastoid tegmen [1,2], the etiology of this defect varies and includes chronic otitis media with or without cholesteatoma, and previous otologic surgery [1-4]. Less commonly, the osseous defect is congenital or follows skull fracture, tumour or irradiation [2]. Nonetheless, since the introduction of more sensitive diagnostic tools in the last few years, reports of spontaneous or idiopathic brain herniations have increased [2].

The most common clinical presentation of encephalomeningocele is a conductive or mixed hearing loss or otitis media with effusion [2,4], other highly suspicious clinical presentations of spontaneous brain Herniation and more specific symptoms [1,4] includes intracranial infection like meningitis and brain abscess, cerebrospinal fuid leak, epilepsy and aphasia [4], which are occasionally sentinel events [2]. The primary modality of treatment is surgery [2], and the management strategy of meningoencephalic Herniation may differ depending upon the etiology, position and size of the bony defect, active cerebrospinal fuid leak, preoperative audiometry and the presence of active infection [1,4]. Whereas the Transmatoid Approach (TM) is generally usefull for single, small (<1cm²) or medium-sized (1 to 2cm²) dehiscences [2,4] located at the level of the tegmen mastoideum or antri when the ossicular chain is not involved, a Middle Cranial Fossa Approach (MCF) is prefered for large or anteriorly located defects or when active infection is present [1,2,4]. The advantage of MCF is the possibility to reach bony defects located anteriolry without any manipulation of the ossicular chain [1].

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