Giant spheno-ethmoidal encephalocele associated with mucinous sinonasal hamartoma

Abstract

Congenital encephalocele appears as the result of neural tissue herniation through a cranial defect. Basal encephaloceles can be classified as follows: sphenoethmoidal, trans-sphenoidal spheno-orbital, transethmoidal, 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 examination as 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