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Septo-Optic Dysplasia

Background

Septo-optic dysplasia (SOD) is the commonest of the midline cerebral/cranial abnormalities involving the spectrum of holoprosencephaly, absence of the corpus callosum (see entry, Agenesis of the Corpus Callosum) and midline cleft palate and also involving the hypothalamopituitary region.

It comprises two out of the three features of optic nerve hypoplasia (ONH) unilateral or bilateral, absence of the septum pellucidum and pituitary dysfunction. Because fifty per cent of patients with this condition have a septum pellucidum present, the older name of De Morsier's syndrome may be preferable.

What are the symptoms? View What are the symptoms?

Medical text written October 2001 by Dr R Stanhope and Dr M Dattani. Last updated September 2005 by Dr R Stanhope, Consultant Paediatric Endocrinologist, Great Ormond Street Hospital, London, UK and Dr M Dattani, Reader and Hon. Consultant in Paediatric Endocrinology, Institute of Child Health, London, UK.

 

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