Cleft lip and palate repair in Hay-Wells/ankyloblepharon-ectodermal dysplasia-clefting syndrome.
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.
Cabiling
David S
DS
Department of Surgery and Division of Plastic Surgery at The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104-4318, USA.
Yan
Albert C
AC
McDonald-McGinn
Donna M
DM
Zackai
Elaine H
EH
Kirschner
Richard E
RE
D000015:Abnormalities, Multiple; D002675:Child, Preschool; D002971:Cleft Lip / Q000150:complications / Q000601:surgery*; D002972:Cleft Palate / Q000150:complications / Q000601:surgery*; D003103:Coloboma / Q000150:complications; D004476:Ectodermal Dysplasia / Q000150:complications*; D005143:Eyelids / Q000002:abnormalities; D005260:Female; D005402:Fistula / Q000209:etiology / Q000601:surgery*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D019651:Plastic Surgery Procedures / Q000379:methods*; D013577:Syndrome; D016896:Treatment Outcome; D014945:Wound Healing