Sudden death in septo-optic dysplasia. Report of 5 cases

Arch Ophthalmol. 1997 Jan;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 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.

Publication types

  • Case Reports

MeSH terms

  • Adrenocorticotropic Hormone / deficiency
  • Body Temperature Regulation
  • Child
  • Child, Preschool
  • Death, Sudden / etiology*
  • Diabetes Insipidus / complications
  • Female
  • Humans
  • Hypoglycemia / complications
  • Hypopituitarism / complications
  • Hypopituitarism / pathology
  • Infant
  • Magnetic Resonance Imaging
  • Male
  • Optic Nerve / abnormalities*
  • Retrospective Studies
  • Risk Factors
  • Septum Pellucidum / abnormalities*
  • Septum Pellucidum / pathology


  • Adrenocorticotropic Hormone