Endocrine Abstracts

Multiple pituitary hormone deficit and hypothalamic obesity are common complications after brain surgery for childhood tumors. Despite adequate replacement hormone therapy, obesity develops equally, but liver failure is not usually described. In this study, four subjects who had undergone surgery for brain tumors involving the peri-hypothalamic area when they were nursing or as youths (9 months, 6 years, 9 years and 20 years). They were referred to our Unit when they were 19, 23, 23 and 31 years old, respectively. One of this patients had a pilocytic astrocytoma, two of these patients had a craniopharyngioma, and the last a third ventricle germinoma. One patient underwent radiotherapy and chemotherapy after surgery and another one received four transphenoidal or transcranial surgical operations. All patients developed panhypopituitarism, but only two had taken in childhood and adolescent age adequate hormone replacement therapies since brain damage. Since the other two patients came to our observation, adequate hormonal replacement therapy was proposed. All patients developed obesity (BMI 39.2–46.6 kg/m²) or overweight (BMI: 27.1). The two patients adequately treated for hypopituitarism after surgery, developed a fatty liver when they were 16 and 21 years old respectively. In both cases, steatosis progressively evolved into cirrhosis after 2 years. One of these patients also developed a hepatic-pulmonary syndrome and underwent liver transplant at the age of 25 years. In the two patients with inadequate or absent hormone replacement therapy, a diagnosis of cirrhosis was concomitant with the first appropriate endocrinological care during hospitalization for liver failure. The analysis of these four cases shows that pediatric peri-hypothalamic surgery may be associated with very severe hepatic clinical features, induced by mechanisms not yet known, regardless of hormone replacement therapy. It is therefore very important to start a careful follow-up of these patients from childhood for early detection of possible liver failure.