Literature DB >> 17477760

Cleft lip and palate repair in Hay-Wells/ankyloblepharon-ectodermal dysplasia-clefting syndrome.

David S Cabiling1, Albert C Yan, Donna M McDonald-McGinn, Elaine H Zackai, Richard E Kirschner.   

Abstract

Hay-Wells/ankyloblepharon-ectodermal dysplasia-clefting syndrome is a rare autosomal dominant disorder characterized by ankyloblepharon, ectodermal dysplasia, and cleft lip and/or cleft palate. Mutations in the p63 gene recently have been shown to be etiologic in the majority of cases of ankyloblepharon-ectodermal dysplasia-clefting syndrome. To date, there have been no reports to document wound healing after cleft lip and/or palate repair in ankyloblepharon-ectodermal dysplasia-clefting patients. We describe two patients with ankyloblepharon-ectodermal dysplasia-clefting syndrome and provide a review of the literature. There have been no reported instances of wound healing complications in affected patients. Seventeen percent (3/18) of reported patients required revisions or repair of oronasal fistulae. Cleft lip and palate repair can be performed safely in patients with Hay-Wells syndrome.

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Year:  2007        PMID: 17477760     DOI: 10.1597/06-065

Source DB:  PubMed          Journal:  Cleft Palate Craniofac J        ISSN: 1055-6656


  3 in total

Review 1.  Palatogenesis and cutaneous repair: A two-headed coin.

Authors:  Leah C Biggs; Steven L Goudy; Martine Dunnwald
Journal:  Dev Dyn       Date:  2014-11-25       Impact factor: 3.780

2.  Gene p63: In ectrodactyly-ectodermal dysplasia clefting, ankyloblepharon-ectodermal dysplasia, Rapp-Hodgkin syndrome.

Authors:  Cornelia van Straten; Kurt-W Butow
Journal:  Ann Maxillofac Surg       Date:  2013-01

Review 3.  Master regulatory role of p63 in epidermal development and disease.

Authors:  Eduardo Soares; Huiqing Zhou
Journal:  Cell Mol Life Sci       Date:  2017-11-04       Impact factor: 9.261

  3 in total

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