THE IVEMARK-SYNDROME - PRENATAL-DIAGNOSIS OF AN UNCOMMON CYSTIC RENALLESION WITH HETEROGENEOUS ASSOCIATIONS

Renal dysplasia has been reported in association with a number of anat omical abnormalities, including pancreatic dysgenesis and hepatic anom alies. The combination of renal, hepatic, and pancreatic dysplasia (RH PD), also known as Ivemark syndrome, is rare and uniformly fatal. We r eport here the gross and histological findings in 4 cases of combined RHPD, 2 of which were detected by prenatal ultrasonography. Evaluation of these 4 and the other 20 reported cases shows that combined RHPD h as considerable variability in the histological findings and in organ involvement. In addition, nearly half were associated with anomalies i n other organ systems, and 11 of the 24 were familial. In this study, ultrasonographic and histological abnormalities were seen as early as 18.5 weeks gestation in 1 case.