A population based and validated 10 year cohort of 94 cases with split
hand/foot born in Hungary, 1975-1984 was evaluated. This type of cong
enital limb deficiency was relatively frequently (43%) associated with
non-limb defects. Fifty-four cases with isolated split hand/foot are
evaluated in this paper. A single limb was affected in 78% of cases. T
he upper limbs were 21 times more frequently affected in unimelic case
s with a right sided predominance and male excess. Case-control analys
is indicated intrauterine growth retardation and lower socioeconomic s
tatus of parents of cases. Family study showed six familial cases with
autosomal dominant inheritance among 152 first and 452 second degree
relatives. All familial cases were males.