Hypothalamic Hypopituitarism Presenting with Pituitary Malformation and Morning Glory Syndrome: A Case Report

Abstract

We report a 3-yr-old male with short stature, speech delay, episodes of convulsion, and right morning glory syndrome. He had four episodes of febrile convulsion from the age of 1 yr 6 mo, and two episodes of afebrile convulsion with hypoglycemia. His height was 90.2 cm (-2.3 SD) and his growth rate was decreased from about 2 yr of age. Magnetic resonance imaging of the brain showed an atrophic anterior pituitary lobe, invisible stalk, and ectopic posterior lobe. Septo-optic dysplasia was deniable. We tested pituitary function. Cortisol response to CRH was normal (peak: 21.0 μg/dl). TSH response to TRH was prolonged and exaggerated (peak: 33.0 μU/ml at 120 min), and FT4 response was absent (peak: 0.74 ng/dl). After supplement of l-T4, GH response to arginine was decreased (peak: 5.1 ng/ml). We diagnosed hypothalamic hypopituitarism. We started treatment with l-T4 and growth hormone. Patients with congenital ocular anomaly including morning glory syndrome should be evaluated for brain and pituitary structure with MRI, and further endocrinological examinations should also be considered.