"Ethmoid Meningoencephalocele in A Patient with Cerebrofacial Arteriovenous metameric syndrome: A Case Report".

"Ethmoid Meningoencephalocele in A Patient with Cerebrofacial Arteriovenous metameric syndrome: A Case Report".

World Neurosurg. 2018 Mar 03;:

Authors: Fernandez-Gajardo R, Almeida JP, Suppiah S, Witterick I, Zadeh G

Abstract
BACKGROUND: Skull base meningoencephaloceles are a rare condition, frequently secondary to traumatic or iatrogenic causes. Cerebrofacial arteriovenous metameric syndrome (CAMS) is characterized by the presence of retinal, facial and cerebral arteriovenous malformations (AVMs) with metameric distribution. To the best of our knowledge, this is the first reported case associating these two conditions.
CASE DESCRIPTION: A 45 years old female previously diagnosed with CAMS type 2 presented with a long history of cerebrospinal fluid (CSF) rhinorrhea. Magnetic resonance imaging and digital subtraction angiography demonstrated a right-sided facial and orbital AVM extending posteriorly along the optic tract into the suprasellar cistern, and a right-sided meningoencephalocele protruding into the olfactory recess and ethmoid sinus. An extended endoscopic endonasal approach was performed to resect the meningoencephalocele and to repair the CSF leak without complications.
CONCLUSIONS: We report the unusual association between the development of a meningoencephalocele and a metameric syndrome, and comment on clinical implications in the management of this patient.

PMID: 29510289 [PubMed - as supplied by publisher]

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