Endocrine Abstracts

JOINT1197

Introduction: Hypothalamic syndrome (HS) in childhood is rare and typically is strongly associated with severe obesity. Its incidence ranges from 0.05 to 10 per 100,000 persons per year and may result from genetic or acquired causes that damage the hypothalamic nucleus, such as suprasellar brain tumors or their treatments. Beyond severe obesity, HS may present additional manifestations, including pituitary dysfunction, sleep disturbances, temperature dysregulation, loss of thirst, and behavioral problems. These symptoms are often not promptly recognized, leading to delayed management and a deterioration in quality of life.

Objectives: Present two cases of HS to highlight the complexity of diagnosis and management. Case 1: A 5-year-old male was diagnosed with craniopharyngioma (CP) at age 4 and underwent gross total resection. He developed multiple pituitary hormone deficiencies (MPHD), requiring treatment with levothyroxine, hydrocortisone, desmopressin (DDAVP), and growth hormone (GH). He experienced hyperphagia, resulting in obesity with a BMI of +4 SD in a short time. Hypodipsia necessitated supervision to meet daily water intake, and hyperthermia, with a basal temperature of 38°C that spiked to 40°C on warm days, was managed with cold baths. Case 2: A 12-year-old male was diagnosed with CP at age 2. He underwent multiple surgeries and proton beam therapy, subsequently developing MPHD and requiring treatment with cortisol, levothyroxine, DDAVP, and GH. He also developed adipsia, resulting in recurrent hospitalizations for dehydration and hypernatremia. During his most recent admission, he was clinically dehydrated, tachycardic, and had low-normal arterial pressures. He scored 0 on the thirst scale and had a serum sodium level of 162 mEq/L. Treatment included desmopressin adjustment to 0.375 mg/day and supervised scheduled water intake. Serum sodium normalized by the third day, and he was discharged with a sodium level of 147.3 mEq/land a daily water intake of 1500–2000 mL.

Conclusion: HS is a rare and challenging condition to diagnose, requiring a high index of suspicion in cases involving hypothalamic tumors. Adipsia complicates management, making close clinical and laboratory follow-up essential to optimize outcomes. Hyperthermia is common and should be distinguished from infection-related fever to avoid unnecessary high-dose glucocorticoid treatments.

Key Words: Hypothalamus, Pituitary tumors, Neuroendocrine.