Ramírez-Aguilar R, Escobar GVH, Ayala-Arcipreste A, Padilla L, Mendizabal-Guerra R

Language: Spanish
References: 14
Page: 173-176
PDF size: 197.76 Kb.

ABSTRACT

Congenital encephalocele appears as the result of neural tissue herniation through a cranial defect. Basal encephaloceles can be classified as follows: spheno-ethmoidal, trans-sphenoidal spheno-orbital, trans-ethmoidal, spheno-maxillary. The seromucinous hamartoma was first described in 1974 by Baillie and Batsakis. 22 cases have been previously described, with a predominance of cases in the posterior nasal septum and nasopharinx in 80% of cases and ocassional extension to the skull base. Case report: a 35 year old female with left amaurosis, primary amenorrhea, chronic sinusitis, headache, limitation for nasal breathing and positive Fustemberg Test was admitted in our institution. Magnetic resonance imaging revealed a sellar floor defect with giant hypointense image herniating through the sphenoid and ethmoid bones to the posterior nasal cavity, and a hyperintense rounded image with homogenic enhancement with contrast. A combined craneofacial approach was done, repairing middle fosae floor and excision of polipoid lesion adjacent to the encephalocele, which was classified by histopathologic examinationas a mucinous sinonasal hamartoma. The patient had a good outcome wth abscence of hedaches and nasal obstruction, however there was no visual and hormonal improvement due to the chronicity of the disease. Discussion and conclusions: encephalocele is a rare entity described as an structural anomaly during intrauterine life, and hamartoma is a disturbance in neuronal cell migration, therefore the etiology of both entities may be related. The study of these patients should address both entities initially.

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