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Br J Ophthalmol 2004;88:969 doi:10.1136/bjo.2003.034405
  • Letter

Congenital third nerve palsy in septo-optic dysplasia

  1. A Langmann,
  2. S Lindner
  1. Department of Ophthalmology, University Graz, Austria
  1. Correspondence to: A Langmann MD Department of Ophthalmology, Medical University, Auenbruggerplatz 4, University Graz, Austria; andrea.langmannuni-graz.at
  • Accepted 28 October 2003

Paediatric oculomotor nerve palsies are rare lesions. The most frequently cited mechanism is perinatal injury to the peripheral third nerve,1 although they may be due to congenital absence of the nerve and/or nucleus and be accompanied by neurological deficits.2–4 Septo-optic dysplasia consists of optic hypoplasia, mid-brain malformations, and hypothalamohypophyseal dysfunction. We present three children with congenital third nerve palsy and septo-optic dysplasia.

Case reports

We report on three children with bilateral optic nerve hypoplasia, with visual function from light projection to 0.05 and nystagmus. Magnetic resonance imaging revealed absent septum pellucidum, thinning of corpus callosum, and posterior pituitary ectopia in two cases and infundibular hypoplasia in the third case. They had anterior pituitary hormone deficiency—growth hormone, adrenocorticotrophic hormone, and …

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