A case of transsphenoidal meningoencephalocele

A 6-year-old boy was sent to us due to basal meningoencephalocele detected by MRI. He had had a past history of strabismus and morning glory syndrome since 6 months of age, as well as hypopituitary dwarfism since he was 3 years old. On admission, physical examination revealed hypertelorism and left coloboma. Laryngoscopic examination revealed a round mass covered by normal nasal mucose in the midline of the tegmen of the choana. X-ray tomogram and CT scan demonstrated an isodense mass protruding into the nasopharyngeal cavity through a bony defect of the sella turcica. Right carotid angiogram showed stenosis of the right internal carotid artery and abnormal fine vessels in the basal ganglia similar to basal Moyamoya network. There was an irregular filling of the right anterior cerebral artery. Left carotid angiogram showed an irregular filling of the left middle cerebral artery. MRI showed an anterior part of the third ventricle descending into, and the meningocele protruding into the nasopharyngeal cavity. It also showed a stalk extending from the hypothalamus into the meningocele, but the pituitary gland was not recognized. The meningocele was of the same signal intensity as CSF in both T1 and T2 weighted images. Growth hormone deficiency was confirmed by radioimmunoassay of the peripheral blood. An operation was scheduled transcranial to prevent snoring during sleep at age 9. The stalk was observed behind the chiasm during the operation, but a radical operation was not performed because of a possibility of postoperative hypothalamic-pituitary dysfunction. Transsphenoidal encephalocele is rare. So far as we could see, only 30 cases have been reported.(ABSTRACT TRUNCATED AT 250 WORDS)