Sudden death in septo-optic dysplasia. Report of 5 cases.
OBJECTIVE
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 septo-optic 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.
Brodsky
M C
MC
Department of Ophthalmology, University of Arkansas for Medical Sciences, USA.
Conte
F A
FA
Taylor
D
D
Hoyt
C S
CS
Mrak
R E
RE
D000324:Adrenocorticotropic Hormone / Q000172:deficiency; D001833:Body Temperature Regulation; D002648:Child; D002675:Child, Preschool; D003645:Death, Sudden / Q000209:etiology*; D003919:Diabetes Insipidus / Q000150:complications; D005260:Female; D006801:Humans; D007003:Hypoglycemia / Q000150:complications; D007018:Hypopituitarism / Q000150:complications / Q000473:pathology; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D009900:Optic Nerve / Q000002:abnormalities*; D012189:Retrospective Studies; D012307:Risk Factors; D012688:Septum Pellucidum / Q000002:abnormalities* / Q000473:pathology
D000324:Adrenocorticotropic Hormone