January 1997

Sudden Death in Septo-Optic DysplasiaReport of 5 Cases

Author Affiliations

From the Department of Ophthalmology, University of Arkansas for Medical Sciences (Drs Brodsky and Mrak), and the Department of Veterans Affairs (Dr Mrak), Little Rock; the Departments of Pediatrics (Dr Conte) and Ophthalmology (Dr Hoyt), University of California at San Francisco; and the Great Ormond Street Hospital for Children, London, England (Dr Taylor).

Arch Ophthalmol. 1997;115(1):66-70. doi:10.1001/archopht.1997.01100150068011

Objectives:  To report our experience with sudden death in children with septo-optic dysplasia and to identify specific risk factors and suggest preventive measures to minimize mortality.

Methods:  Clinical data from 5 children with septooptic dysplasia who died suddenly and unexpectedly were evaluated retrospectively.

Results:  All children had corticotropin deficiency, all had thermoregulatory disturbances, and 4 children had diabetes insipidus. In at least 4 children, clinical deterioration was caused by fever and dehydration from a presumed viral illness, which appeared to precipitate adrenal crisis.

Conclusions:  Children with septo-optic dysplasia and hypocortisolism are at risk for sudden death during febrile illness. Thermoregulatory disturbances and dehydration from diabetes insipidus may potentiate clinical deterioration. Prevention of sudden death in septo-optic dysplasia requires early recognition and treatment of these major risk factors.