417 EMPTY SELLA SYNDROME IN CHILDHOOD

Abstract

Extension of the subarachnoid space through a defective diaphragma sella may cause progressive enlargement of the pituitary fossa, and may result in secondary endocrine disturbances, CSF rhinorrhea or visual field defects. Symptoms usually present in the fourth or fifth decade, and reports in children are very rare. A 13.4 year old male presented following minor trauma to the face, when symmetrical pituitary fossa enlargement was noted on skull x-ray. The sella volume was 3.2 times normal mean volume for age and there was some minimal calcification within the diaphragm. He was totally asymptomatic, and on physical examination his height, weight and sexual development were appropriate for age, his visual fields were intact and there were no focal CNS signs or thyroid enlargement. Endocrine evaluation showed normal basal plasma levels of T4 (7.7 μgm/dl), TSH (2.2 μU/ml), LH and FSH (2 and 5 mIU/ml) and prolactin (6.9 ng/ml). In response to intravenous insulin and thyrotropin releasing hormone, there were satisfactory increases in GH (Δ 12 ng/ml), cortisol (Δ 13.5 μgm/dl), TSH (Δ 10.6 μU/ml) and prolactin (Δ 11.1 ng/ml), and his responses to gonadotropin releasing hormone were appropriate for his degree of sexual development (Δ LH 26 mIU/ml; Δ FSH 2 mIU/ml). A CT scan performed after injection of matrizamide contrast into the lumbar subarachnoid space, confirmed that the sella was “empty”, with a narrow rim of pituitary tissue visualized against the anterior wall. No pituitary hormones were detected in the CSF. Empty sella syndrome, in some patients, may therefore possibly have its origins in childhood.