SUDDEN-DEATH IN SEPTO-OPTIC DYSPLASIA - REPORT OF 5 CASES

Objectives: To report our experience with sudden death in children wit h septo-optic dysplasia and to identify specific risk factors and sugg est preventive measures to minimize mortality. Methods: Clinical data from 5 children with septo-optic dysplasia who died suddenly and unexp ectedly were evaluated retrospectively. Results: All children had cort icotropin deficiency, all had thermoregulatory disturbances, and 4 chi ldren had diabetes insipidus. In at least 4 children, clinical deterio ration was caused by fever and dehydration from a presumed viral illne ss, which appeared to precipitate adrenal crisis. Conclusions: Childre n with septo-optic dysplasia and hypocortisolism are at risk for sudde n death during febrile illness. Thermoregulatory disturbances and dehy dration from diabetes insipidus may potentiate clinical deterioration. Prevention of sudden death in septo-optic dysplasia requires early re cognition and treatment of these major risk factors.