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Report of 5 Cases

Michael C. Brodsky, MD; Felix A. Conte, MD; David Taylor, MD; Creig S. Hoyt, MD; Robert E. Mrak, MD, PhD

Arch Ophthalmol. 1997;115(1):66-70.

Abstract
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.

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).

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