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British Journal of Ophthalmology 2005;89:1275-1277; doi:10.1136/bjo.2005.069609
Copyright © 2005 by the BMJ Publishing Group Ltd.

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SCIENTIFIC REPORT

Infantile infection and diabetes insipidus in children with optic nerve hypoplasia

S P Donahue1,2,3, A Lavina1, J Najjar3

1 Department of Ophthalmology and Visual Sciences, Vanderbilt University School of Medicine, Nashville, TN, USA
2 Department of Neurology, Vanderbilt University School of Medicine, Nashville, TN, USA
3 Department of Pediatrics, Vanderbilt University School of Medicine, Nashville, TN, USA

Correspondence to:
Correspondence to:
Sean P Donahue
MD, PhD, Department of Ophthalmology and Visual Sciences, Vanderbilt University School of Medicine, 8000 Medical Center East, Nashville, TN 37232-8808, USA; sean.donahue{at}vanderbilt.edu


ABSTRACT
Background: Bilateral optic nerve hypoplasia (BONH) is often associated with other central nervous system midline abnormalities (septo-optic dysplasia). Hormonal dysfunction, caused by anterior (cortisol) and posterior (ADH) pituitary involvement, can be sudden, severe, and life threatening.

Methods: Case series. Three cases of septo-optic dysplasia (SOD) presenting as infantile infection with associated diabetes insipidus are reported. The diagnosis of SOD was suspected only after ophthalmological evaluation; further evaluation led to the diagnosis of panhypopituitarism.

Conclusions: A high index of suspicion is required to diagnose SOD in children when the disorder presents with infantile infection and hypernatraemia. Early warning signs of neonatal jaundice and hypoglycaemia should prompt ophthalmological evaluation.


Abbreviations: ACTH, adrenocorticotrophic hormone; BONH, bilateral optic nerve hypoplasia; SOD, septo-optic dysplasia

Keywords: diabetes insipidus; optic nerve hypoplasia; septo-optic dysplasia; hypopituitarism


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Br. J. Ophthalmol. 2005 89: 1231. [Extract] [Full Text] [PDF]






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Copyright © 2005 by the BMJ Publishing Group Ltd.