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Prenatal diagnosis and epidemiology of multicystic kidney dysplasia in Europe.

Authors :: Winding L
Loane M
Wellesley D
Addor MC
Arriola L
Bakker MK
Bianchi F
Calzolari E
Gatt M
Haeusler M
Lelong N
Mullaney C
Scarano G
Tucker D
Wiesel A
Garne E
Source :: Prenatal diagnosis [Prenat Diagn] 2014 Nov; Vol. 34 (11), pp. 1093-8. Date of Electronic Publication: 2014 Jul 02.
Publication Year :: 2014
Abstract: Objectives: The aim of this study is to describe the prenatal diagnosis and epidemiology of multicystic kidney dysplasia (MCKD).<br />Methods: The study is based on routinely collected data from a European database of major congenital anomalies including 13 registries with cases born in 1997-2006 and covering 1 458 552 births.<br />Results: There were 601 MCKD cases giving an overall prevalence of 4.12 per 10 000 births with regional variation. In live births, 87% of cases had an isolated renal anomaly and 13% had associated major nonrenal anomalies (chromosomal, syndrome or other major anomalies). For the cases with isolated renal anomalies, 51/386 (11%) and 7/386 (2%) choose to terminate the pregnancy or resulted in an intrauterine fetal death, respectively. The prenatal detection rate was 88% in both unilateral and bilateral cases. Birth outcome differed with 92% of unilateral MCKD cases being liveborn compared with 33% of bilateral MCKD cases. For unilateral MCKD cases, 84% had an isolated renal anomaly compared with 51% of bilateral MCKD cases (p < 0.001).<br />Conclusions: Cases with unilateral MCKD are mainly liveborn, and only 16% have associated major malformations or a syndrome. Cases with bilateral MCKD are often associated with nonrenal major congenital anomalies or part of a syndrome, and only one third of bilateral MCKD cases in this study were liveborn. Prenatal detection rate of MCKD was high for both unilateral and bilateral cases. © 2014 John Wiley & Sons, Ltd.<br /> (© 2014 John Wiley & Sons, Ltd.)