Familial Hypopituitarism with Large Sella Turcica

Abstract

We studied endocrine function in three siblings with short stature and enlargement of the sella turcica. Sellar volumes were 5.9, 3.7 and 4.0 standard deviations above age-specific means. Computed tomography or pneumoencephalography showed full sellae without suprasellar extension. Basal thyrotropin levels were low despite hypothyroidism and increments were <3 μU per milliliter after thyrotropin-releasing hormone injection. Stimulated growth hormone levels were <5 ng per milliliter, declining to <2 ng per milliliter after thyroxine treatment. Both thyroxine and growth hormone treatments were required for rapid growth.