Sibs diagnosed prenatally with situs inversus totalis, renal and pancreatic dysplasia, and cysts: A new syndrome?

We describe two sib fetuses with situs inversus, cystic dysplastic kidney a nd pancreas, bowing of the lower limbs and clavicles, severe intrauterine g rowth retardation (IUGR), and oligohydramnios. Early prenatal diagnosis of pancreatic and dysplastic renal cysts and situs inversus totalis were made in the 18-week-old fetus. This syndrome differs from that of Ivemark and re lated syndromes because of the presence of situs inversus totalis and absen ce of hepatic fibrosis and cysts. The parents were first cousins, and did n ot have any cysts of kidney, liver, or pancreas detected by ultrasonography . (C) 1999 Wiley-Liss, Inc.