[Short rib-polydactyly syndrome of the Saldino-Noonan type in 2 siblings].

Two stillborn female siblings with short rib-polydactyly syndrome type I (Saldino-Noonan) are described. Besides the characteristic narrow thorax, the pronounced micromelia and a severe dysplasia of the skeleton, a series of organic malformations have been found, in particular in the gastro-intestinal tract and in the urogenital system. The clinical, radiological and anatomo-pathological differences between the four presently known types of the SRP syndrome are described. The importance of postmortem X-ray diagnosis and of genetic counseling (autosomal recessive transmission) is emphasized.