Ankyloblepharon-ectodermal defects-cleft lip/palate (AEC) syndrome in monozygotic twins: two cases report and reviews.
نویسندگان
چکیده
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.
منابع مشابه
Rare Variant of Ankyloblepharon-ectodermal Defect-cleft Lip/Cleft Palate Syndrome: Curly Hair-ankyloblepharon-nail Disease Syndrome
Ankyloblepharon-ectodermal defect-cleft lip/cleft palate (AEC) syndrome is one of the variants of ectodermal dysplasia. It is an autosomal dominant disorder comprising of ankyloblepharon, ectodermal dysplasia, and cleft palate or cleft lip. In 1976, it wasfirst described by Hay and Wells, therefore also known as Hay-Wells syndrome. The characteristic feature of this syndrome is "ankyloblepharon...
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ورودعنوان ژورنال:
- Journal of the Medical Association of Thailand = Chotmaihet thangphaet
دوره 93 Suppl 4 شماره
صفحات -
تاریخ انتشار 2010