Central hyperthermia control after propranolol therapy in an infant with septo-optic dysplasia

Afonso Sousa, Área de Pediatria, Hospital Dona Estefânia, Centro Hospitalar Lisboa Central, Lisbon, Portugal
Maria João Lage, Unidade funcional de Neonatologia, Hospital Dona Estefânia, Centro Hospitalar Lisboa Central, Lisbon, Portugal
Lurdes Lopes, Unidade de Endocrinologia Pediátrica, Hospital Dona Estefânia, Centro Hospitalar Lisboa Central, Lisbon, Portugal

Introduction: Septo-optic dysplasia (SOD) is a congenital midline brain malformation syndrome involving the hypothalamus-pituitary axis with hypopituitarism and thermal instability. The treatment of central hyperthermia in children with propranolol has already been described. Case report: We report the case of a nine-month-old boy with SOD with prolonged fever and increased urinary output. The physical examination was unremarkable, and a thorough etiological investigation was inconclusive. After adjusting the desmopressin dosage to control the central diabetes insipidus and ruling out infectious, inflammatory, and neoplastic etiologies, the diagnosis of central hyperthermia was established and therapy with propranolol was initiated, with sustained normothermia. Discussion: Although a definite causal relationship could not be proven, this paper is the first to report the successful management of central hyperthermia in an infant with SOD using propranolol. Further trials are needed to evaluate its efficacy and safety in the management of central hyperthermia in children with SOD.

Keywords: Central hyperthermia. Propranolol. Septo-optic dysplasia plus.