title: Ankyloblepharon-ectodermal defects-cleft lip/palate (AEC) syndrome in monozygotic twins: two cases report and reviews.
abstract: Ankyloblepharon-ectodermal defects-cleft lip/palate (AEC) syndrome (or Hay-Wells syndrome) is a rare congenital malformation. Our first cases were a pair of female monozygotic twins with AEC syndrome at Srinagarind Hospital. In this study, we describe monozygotic female twins concordant for ankyloblephaon, ectodermal dysplasia and helical rim deformities, but discordant for cleft, syndactyly of toes, heart and urinary tract abnormalities. Twin A had syndactyly of the right third and fourth toes with incomplete bilateral cleft lip and complete bilateral cleft palate. Twin B had left ventricular enlargement, caliectasia of both kidneys with complete left unilateral cleft lip and cleft palate. The twins were treated by multidisciplinary teams with satisfactory results.
authors.affiliation: Division of Plastic Surgery, Department of Surgery, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand. krijen@kku.ac.th
authors.forename: Kamonwan
authors.initials: K
authors.lastname: Jenwitheesuk
authors.forename: Palakorn
authors.initials: P
authors.lastname: Surakunprapha
authors.forename: Bowornsilp
authors.initials: B
authors.lastname: Chowchuen
authors.forename: Charoon
authors.initials: C
authors.lastname: Jetsrisuparb
mesh_terms: D000015:Abnormalities, Multiple; D002971:Cleft Lip / Q000175:diagnosis / Q000601:surgery*; D002972:Cleft Palate / Q000175:diagnosis / Q000601:surgery*; D004476:Ectodermal Dysplasia / Q000175:diagnosis / Q000601:surgery; D005124:Eye Abnormalities / Q000175:diagnosis / Q000601:surgery; D005143:Eyelids / Q000002:abnormalities / Q000601:surgery; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D019651:Plastic Surgery Procedures / Q000379:methods*; D016896:Treatment Outcome; D014430:Twins, Monozygotic