[Sphenoido-nasopharyngeal craniopharyngioma. A case with radical excision by Le Fort I-type maxillotomy]

A case of infra-sellar (sphenoidal nasopharyngeal) craniopharyngioma is reported. A 16 year-old girl presented with progressive nasal obstruction. 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 craniopharyngioma. The tumor was successfully removed via an inferior route using a Le Fort I maxillotomy. Plates and screws were used to achieve osteosynthesis. The post-operative course was uneventful and there was no residual tumor on post-operative CT. Craniopharyngiomas with strictly infra-sellar development are very rare. Up to now, 19 cases have been reported in the literature. This condition likely results from growth of embryonic remnants lying along the craniopharyngeal duct. Several surgical approaches have been proposed, most of those giving poor exposure to the clival and lateral tumorous extensions. Of particular interest in the authors' experience was the use of a Le Fort I maxillotomy which provided optimal exposure to the skull-base and facilitated radical excision. CT and MR studies have proved of upmost importance in delineating the area of bone to be removed at operation.