Transsphenoidal Meningoencephalocele: Challenge of Early Transsphenoidal Repair

Background: Transsphenoidal meningoencephalocele is a rare congenital anomaly, reported in fewer than 50 patients. It may be subdivided into intrasphenoidal, extending into the sphenoid sinus, and true transsphenoidal, traversing the floor of the sinus and protruding into the nasal cavity or nasopharynx. Surgical treatment of true transsphenoidal meningoencephalocele is debated.

Methods: We present the eight cases of true transsphenoidal meningoencephalocele, with three males and five females, ages from 6 months to 63 years. All patients presented with herniated pituitary gland and third ventricle. Six patients presented with associated cleft palate and other anomalies of skull and brain. Six patients underwent transsphenoidal repair procedures. Different kinds of materials were used to repair these cases. Follow-up was up to 12 years.

Result: All patients underwent surgical procedures and are well recovered except the first intracranial procedure.

Conclusion: The early transsphenoidal repair is important, although the procedure is somewhat difficult and a challenge. Also, early diagnosis of transsphenoidal meningoencephalocele is important, especially in cleft palate children.