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Dorsal Dimelia in Patau Syndrome: A Case Report

From the Department of Plastic Surgery, The Queen Victoria Hospital, East Grinstead, Sussex, UK

Correspondence: St. Andrew's Centre for Plastic Surgery, Broomfield Hospital, Court Road, Chelmsford, Essex CM1, UK. Tel.: +44 7900 67 37 59; fax: +44 1245 516 172. E-mail:adel_fattah{at}hotmail.com

We present a case of a child with Patau syndrome that exhibits features consistent with congenital palmar nail syndrome. The literature is reviewed and evidence presented to demonstrate that this is a defect in the dorso-ventral patterning of the limb and thus a form of dorsal dimelia. In order to differentiate this from other instances of ectopic nail tissue we suggest congenital palmar nail syndrome should be more specifically defined as duplicated nails, absent flexion creases, non-glabrous skin on the palmar surface, reduced movement at the interphalangeal joints and hypoplastic terminal phalanges.

Key Words: limb deformities • congenital • nail • trisomy • polydactyly

Journal of Hand Surgery (European Volume), Vol. 32, No. 5, 534-536 (2007)
DOI: 10.1016/J.JHSE.2007.03.001


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