Acrocephalopolysyndactyly Type Iv

Goodman et al. (1979) applied the designation ACPS IV to a syndrome they observed in 3 offspring of a first-cousin marriage. They felt that the presence of clinodactyly, camptodactyly, and ulnar deviation distinguished the disorder from Carpenter syndrome. In 3 of 8 children of a first-cousin Iranian Jewish couple, Goodman et al. (1979) observed a seemingly new form of acrocephalopolysyndactyly. Two of the 3 sibs were available for study. One had died previously of acyanotic congenital heart malformation at age 2 and one of the living sibs, a female aged 17 years, had Eisenmenger syndrome. Hall et al. (1980) questioned that one can be certain of the distinctness of the autosomal recessive acrocephalopolysyndactylies because of the marked intrafamilial variability. At the least, differentiation on clinical grounds may be difficult. Cohen et al. (1987) concluded that Goodman syndrome is a variant of the Carpenter syndrome (201000).