Transsphenoidal meningocele with hypothalamic insufficiency

A 16-year-old girl with transsphenoidal meningocele and signs of hypothalamic insufficiency is presented. Hormonal disturbances have been reported in only three similar cases in the literature. The radiological criteria for the diagnosis are discussed with emphasis on the differential diagnosis of a persistent cranipharyngeal canal. Based on the present case and studies of the pertinent literature we propose that all patients exhibiting signs of dysraphism of the base of the skull should be thoroughly investigated for hormonal disturbances. Children with cleft palate should be examined for possible concomitant dysraphism of the skull.