Sphenoethmoidal cephalocele with cleft palate: transpalatal versus transcranial repair. Report of two cases.

Two cases of sphenoethmoidal encephalocele with cleft palate are reported in detail. The encephaloceles had prolapsed into the nose and nasopharynx, and protruded into the mouth. Previous examples of this entity are reviewed. Such patients have a typical facial appearance: hypertelorism, median cleft lip, and bifid nose of varied severity. Ocular findings include coloboma of the optic nerve or anophthalmia. The prolapsed cerebral tissue varies from nonfunctional glia elements to vital structures of the hypothalamus-pituitary area. Agenesis of the corpus callosum has been reported consistently. Transcranial access to the bone defect, particularly its posterior portion, is difficult because of distorted cerebral anatomy and abnormal vasculature. The fragility of the prolapsed cerebral tissue makes it difficult to preserve the cerebral tissue intact and to reposition it into the cranium. When there is a cleft palate, it is possible to repair the encephalocele extracranially through a transoral, transpalatal approach, preserving and repositioning the content of the sac. Dural closure and obliteration of the bone defects are other essential steps of the operation. Both patients reported here were successfully operated on by the transoral, transpalatal route, at the age of 3 months and 4 years, respectively. They were followed for several years and had comprehensive endocrinological work-ups. One patient exhibited some pituitary deficiency requiring substitution therapy.