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Journal of Hand Surgery (British and European Volume)
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Articles

Hypoplastic Synpolydactyly as a New Clinical Subgroup of Synpolydactyly

ILHAMI KURU, HALE SAMLI, AYLIN YUCEL, M. ERAY BOZAN, SEVAL TURKMEN and MUSTAFA SOLAK

From the Orthopedics Department, Medical School of Kocatepe University, Afyon, Turkey, Genetics Department, Medical School of Kocatepe University, Afyon, Turkey, Radiology Department, Medical School of Kocatepe University, Afyon, Turkey, Institute of Medical Genetics, Humboldt University, Berlin, Germany

Correspondence: Dr Ilhami Kuru, Umit Sitesi 31/9 Umitkoy 06530, Ankara, Turkey. Tel.: +90-532-415-1857; fax: +90-272-217-2029;; E-mail: ikuru{at}isnet.net.tr

A large kindred which was first described in 1995 was investigated again. We present the clinical, radiological, genetic and surgical findings of the hand deformities found in homozygote individuals which we called "hypoplastic synpolydactyly".

There were 125 affected (heterozygote or homozygote) people out of 245 subjects in the five last generations. We identified seven marriages of two affected people. Twelve offsprings, of these marriages had a homozygote genetic pattern and "hypoplastic synpolydactyly". From both the clinical and surgical perspectives, their hand deformity was distinctive from that of their parents. We surgically treated both hands of three individuals with this deformity.

The hand deformity of these homozygotes was so complicated and distinctive that it can be evaluated as a new subgroup of synpolydactyly.

Key Words: hypoplastic synpolydactyly • polysyndactyly • synpolydactyly • syndactyly

Journal of Hand Surgery (British and European Volume), Vol. 29, No. 6, 614-620 (2004)
DOI: 10.1016/J.JHSB.2004.06.008
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