title: "ADAM complex" (amniotic deformity, adhesions, mutilations)--a pattern of craniofacial and limb defects.
abstract: We report eight patients with the craniofacial defects and limb anomalies of the (amniotic deformity, adhesions, mutilations) ADAM complex. Facial abnormalities comprise clefts and distortion and dislocation of craniofacial structures; limbs show various combinations of amputation, secondary syndactyly, and constriction. From previous reports and our cases it is obvious that the clinical picture of the ADAM complex varies enormously; a less severe type combines cleft lip and palate with amputations or amniotic bands. Clinical and experimental data suggest that these malformations are of symptomatic (exogenous) origin. Nosologic differentiation from other conditions phenotypically similar but of genetic etiology is important for genetic counseling. Observation of the ADAM complex in two members of a family suggests that genetic factors might operate in some cases.
mesh_terms: D000015:Abnormalities, Multiple / Q000473:pathology*; D000652:Amniotic Band Syndrome / Q000196:embryology / Q000235:genetics / Q000473:pathology*; D002648:Child; D004677:Encephalocele / Q000473:pathology; D005145:Face / Q000002:abnormalities; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D017880:Limb Deformities, Congenital; D008297:Male; D012886:Skull / Q000002:abnormalities