SHORT RIB POLYDACTYLY SYNDROME, VERMA-NAU MOFF TYPE - A CASE-REPORT

The Authors present the case of a fetus spontaneously aborted at 35 we eks gestation because of <<short rib-polydactyly syndrome>>, type Verm a-Naumoff (type III). Principal features of the infant are short limbs , hand and foot polydactyly, hypoplastic chest, protuberant abdomen, m acrocephaly, hypoplastic penis, normal (46XY) chromosomes. Autopsy sho ws chest and pulmonary hypoplasia, bilateral renal dysplasia. Radiogra phic features are narrow chest with short ribs, short limbs with polyd actyly. On the basis of the clinical, histologic and radiological feat ures, exspecially of the bones, the differential diagnosis between the four types of short rib-polydactyly syndromes is discussed.