NDT Advance Access originally published online on October 25, 2006
Nephrology Dialysis Transplantation 2007 22(2):432-439; doi:10.1093/ndt/gfl591
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Antenatal oligohydramnios of renal origin: long-term outcome
1Pediatric Nephrology, University Medical Center Hamburg-Eppendorf, Martinistr. 52, D-20246 Hamburg, Germany and 2Pediatric Nephrology, University Children's Hospital, Steinwiesstr. 75, CH-8032 Zurich, Swizerland
Correspondence and offprint requests to: Markus J. Kemper, MD, Pediatric Nephrology, University Medical Center, Hamburg-Eppendorf, Martinistr. 52, D-20246 Hamburg, Germany. Email: kemper{at}uke.uni-hamburg.de
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Abstract |
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Background. Prognosis of fetuses with renal oligohydramnios (ROH) is often still regarded as poor. Neonatal complications and the long-term follow-up of fetuses with ROH in two pediatric centres are described.
Method. 23 fetuses (16 males, 7 females) were included as patients. Primary diseases included congenital anomalies of the kidney and urinary tract (n = 16), autosomal recessive polycystic kidney disease (n = 4) and renal tubular dysgenesis (n = 3). The analysis includes retrospective chart review.
Results. Seven children died (30%), the majority (n = 4, 17%) within the neonatal period due to pulmonary hypoplasia and renal insufficiency. Fourteen patients (61%) required postnatal mechanical ventilation for a median of 4 (range 1–60) days; 11 infants had an associated pneumothorax. All 16 surviving children have chronic kidney disease (CKD) at a current median age of 5.7 years (range 0.5–14.5), managed conservatively in eight patients [median glomerular filtration rate 51 (range 20–78) ml/min/1.73 m2]. Eight patients reached end-stage renal disease at a median age of 0.3 years (range 2 days to 8.3 years), including one patient with pre-emptive kidney transplantation. Five of the patients requiring dialysis underwent successful renal transplantation at a median age of 3.5 years (range 2.5–4). Growth was impaired in seven children requiring growth hormone treatment. Cognitive and motor development was normal in 12 (75%) of the 16 patients and showed a delay in four children, including two with associated syndromal features.
Conclusion. ROH is not always associated with a poor prognosis and long-term outcome in survivors is encouraging. The high incidence of neonatal complications and long-term morbidity due to CKD requires a multidisciplinary management of these children.
Keywords: chronic kidney disease; congenital; dialysis; pulmonary hypoplasia; renal oligohydramnios; renal transplantation