GROWTH AND ENDOCRINE SEQUELAE OF CRANIOPHARYNGIOMA

The growth and endocrine sequelae of 75 children (33 girls and 42 boys ) with craniopharyngioma, treated from 1973 to 1994, were studied by r etrospective review and by follow up assessment in 66 survivors, with a mean time from initial surgery of 6.7 years (range 1.5 to 19.8 years ). Although infrequently complained of, 71% of patients had symptoms t o suggest an endocrinopathy at diagnosis. After surgery, multiple endo crinopathies were almost universal, such that 75% of children had panh ypopituitarism at follow up. Hypoadrenal crises in association with in tercurrent illness contributed significantly to morbidity and mortalit y, as did the metabolic consequences of concomitant antidiuretic hormo ne (ADH) insufficiency and absent thirst. Final height in 25 patients was significantly below genetic target height, particularly in the gir ls, with loss of height potential occurring during the pubertal years. The endocrine morbidity associated with craniopharyngioma and its tre atment remains high but manageable with appropriate hormone replacemen t. However, the combination of ADH insufficiency and an impaired sense of thirst following aggressive surgery and severe hypothalamic injury remains one of the most complex management problems.