Pediatric Nephrology

, Volume 9, Issue 5, pp 594–598 | Cite as

The ivemark syndrome: prenatal diagnosis of an uncommon cystic renal lesion with heterogeneous associations

  • Richard S. Larson
  • Mary A. Rudloff
  • Helen Liapis
  • Jose L. Manes
  • Rosa Davila
  • John Kissane
Original Article

Abstract

Renal dysplasia has been reported in association with a number of anatomical abnormalities, including pancreatic dysgenesis and hepatic anomalies. The combination of renal, hepatic, and pancreatic dysplasia (RHPD), also known as Ivemark syndrome, is rare and uniformly fatal. We report 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 has considerable variability in the histological findings and in organ involvement. In addition, nearly half were associated with anomalies in 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.

Key words

Renal dysplasia Ivemark syndrome Pediatric anomalies 

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Copyright information

© IPNA 1995

Authors and Affiliations

  • Richard S. Larson
    • 1
  • Mary A. Rudloff
    • 2
  • Helen Liapis
    • 2
  • Jose L. Manes
    • 3
  • Rosa Davila
    • 2
  • John Kissane
    • 2
  1. 1.Department of PathologyVanderbilt University Medical CenterNashvilleUSA
  2. 2.Department of PathologyBarnes Hospital/Washington University Medical CenterSt. LouisUSA
  3. 3.Department of PathologySt. Luke's HopitalsSt. LouisUSA

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