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doi: 10.1597/06-065
The Cleft Palate-Craniofacial Journal: Vol. 44, No. 3, pp. 335–339.

Cleft Lip and Palate Repair in Hay-Wells/Ankyloblepharon-Ectodermal Dysplasia-Clefting Syndrome
David S. Cabiling, B.A.
Albert C. Yan, M.D.
Donna M. McDonald-McGinn, M.S., C.G.C.
Elaine H. Zackai, M.D.
Richard E. Kirschner, M.D.

Mr. Cabiling is Research Fellow in the Department of Surgery and Division of Plastic Surgery at The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania. Dr. Yan is Section Chief of Dermatology at The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania. Ms. McDonald-McGinn is Senior Clinical Genetics Counselor and Dr. Zackai is Director of Clinical Genetics in the Division of Clinical Genetics at The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania. Dr. Kirschner is Director of the Cleft Palate Program and Attending Surgeon in the Division of Plastic and Reconstructive Surgery at The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania

Submitted April 2006; Accepted August 2006.

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.

KEY WORDS:AEC, cleft lip, cleft palate, ectodermal dysplasia, Hay-Wells, p63, wound healing

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