title: Empty sella syndrome, panhypopituitarism, and diabetes insipidus.
abstract: We present an 18-month-old girl with short stature, obesity, panhypopituitarism, diabetes insipidus, and visual defects. Postmortem examination revealed brain atrophy due to a diffuse encephalopathy, numerous calcified neurons in cerebral cortex, deep telencephalic and diencephalic nuclei, diffuse neuronal necrosis in hypothalamic nuclei, moderate atrophy of optic nerves, very thin hypophyseal stalk, and empty sella with the hypophysis compressed to the dorsal aspect of the concavity. Our hypothesis is that the presence of an empty sella in a child with hypophyseal-hypothalamic abnormalities should alert physicians to the existence of hypothalamic lesions secondary to a perinatal insult. We discuss the possible pathogenesis of these findings as well as lines of evidence available in the literature.
authors.affiliation: Department of Pathology, Children's Hospital Superiora Sor María Ludovica, Buenos Aires, Argentina. jonesmarta3@aol.com
authors.forename: Marta
authors.initials: M
authors.lastname: Jones
authors.forename: Ricardo
authors.initials: R
authors.lastname: Drut
authors.forename: Marina
authors.initials: M
authors.lastname: Valencia
authors.forename: Adriana
authors.initials: A
authors.lastname: Mijalovsky
mesh_terms: D001720:Birth Injuries / Q000473:pathology* / Q000503:physiopathology; D001921:Brain / Q000473:pathology*; D003919:Diabetes Insipidus / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D004652:Empty Sella Syndrome / Q000150:complications* / Q000473:pathology / Q000503:physiopathology; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007018:Hypopituitarism / Q000150:complications* / Q000473:pathology / Q000503:physiopathology; D007223:Infant