Congenital scalp and skull defects with terminal transverse limb anomalies (Adams-Oliver syndrome): report of three additional cases.
Aplasia cutis congenita of the scalp combined with terminal transverse limb defects (Adams-Oliver syndrome) is a rare congenital disorder with autosomal dominant inheritance. Thirty-one patients with the complete syndrome have been published. We report three additional patients (one mother and her affected child, one sporadic case) and discuss the probable vascular disruptive pathogenesis, the clinical variability and the treatment of this condition in the light of a comprehensive review of the literature.
Jaeggi
E
E
Department of Paediatric Surgery, Ostschweizerisches Kinderspital, St. Gallen, Switzerland.
Kind
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C
Morger
R
R
D005192:Family Health; D005260:Female; D005532:Foot Deformities, Congenital / Q000235:genetics*; D005799:Genes, Dominant; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D010294:Parietal Bone / Q000002:abnormalities; D012535:Scalp / Q000002:abnormalities*; D012886:Skull / Q000002:abnormalities*; D013576:Syndactyly / Q000235:genetics*; D013577:Syndrome; D014034:Toes