SPHENOIDAL NASO-PHARYNGEAL CRANIOPHARYNGI OMA - REPORT OF A CASE TREATED BY TOTAL RESECTION VIA A LE-FORT-I MAXILLOTOMY

A case of infra-sellar (sphenoidal nasopharyngeal) craniopharyngioma i s reported. A 16 year-old girl presented with progressive nasal obstru ction. CT and MR images showed a heterogeneous tumor originating from the sphenoid bone with no intra-cranial or supra-sellar extension. On a biopsy specimen, the histologic examination was consistent with a cr aniopharyngioma. The tumor was successfully removed via an inferior ro ute using a Le Fort I maxillotomy. Plates and screws were used to achi eve osteosynthesis. The post-operative course was uneventful and there was no residual tumor on post-operative CT. Craniopharyngiomas with s trictly infra-sellar development are very rare. Up to now, 19 cases ha ve been reported in the literature. This condition likely results from growth of embryonic remnants lying along the craniopharyngeal duct. S everal surgical approaches have been proposed, most of those giving po or exposure to the clival and lateral tumorous extensions. Of particul ar interest in the authors' experience was the use of a Le Fort I maxi llotomy which provided optimal exposure to the skull-base and facilita ted radical excision. CT and MR studies have proved of upmost importan ce in delineating the area of bone to be removed at operation.