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Midline cleft lip and nasal dermoids over five generations: a distinct entity or autosomal dominant Pai syndrome?

Lees, Melissa M.a; Connelly, Fionab; Kangesu, Loshanc; Sommerlad, Brianc; Barnicoat, Angelaa

doi: 10.1097/01.mcd.0000204987.61990.f0
ORIGINAL ARTICLES
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A family with midline nasal dermoids in four members over five generations is described. Two of these individuals also had a midline cleft lip. The pattern of inheritance appears to be autosomal dominant with variable expression. To our knowledge, no familial cases of midline clefting with nasal dermoids have previously been reported in the literature. Cranial imaging to look for intracerebral extension of the dermoid is recommended in such cases.

aNorth Thames Regional Genetics Service, Great Ormond Street Hospital

bSouth West Thames Regional Genetics Centre, St Georges Hospital, London

cNorth Thames Cleft Centre, Great Ormond Street Hospital and St Andrews Centre, Broomfield, UK

Correspondence and requests for reprints to Dr M.M. Lees, North East Thames Regional Genetics Service, Great Ormond Street NHS Truct, London WC1N 3JH, UK

E-mail: mlees@ich.ucl.ac.uk

Received 16 September 2005; Accepted 20 January 2006

© 2006 Lippincott Williams & Wilkins, Inc.