ERKNet

The European Rare Kidney Disease Reference Network

  

Thematic Area: 
Perinatal Management of Cystic Kidney Diseases

 
The following guidance document has been adopted based on standardized reviews:
Perinatal Diagnosis, Mamagement, and Follow-up of Cystic Renal Diseases:
A Clinical Practice Recommendation with Systematic Literature Reviews 
 

JAMA Pediatr 2018; 172: 74-86

 

Core Recommendations:
  1. Because many cystic kidney diseases manifest initially as hyperechogenic kidneys without visible cysts,their differential diagnosis should be considered together.
  2. For fetuses with bilateral cystic kidney disease and/or bilateral hyperechoic or enlarged kidneys, consider nondirective counseling on prenatal genetic testing. For patients with 2 or more renal cysts and/or bilaterally increased echogenicity of the renal cortex of any age, genetic testing should be offered, especially if the result could influence further family planning and/or when an individual benefit from knowledge is expected.
  3. There is insufficient evidence to make recommendations on prenatal lung assessment or on prenatal renal function tests as a means to predict postnatal mortality and morbidity or postnatal renal outcome in children with renal oligohydramnios or cystic kidney disease.
  4. In case of oligohydramnios, parents should be offered counseling by a fetal medicine specialist and a neonatologist; irrespective of the presence of oligohydramnios, parents of fetuses with bilateral cystic renal disease should be offered counseling by a pediatric nephrologist.
  5. Do not offer early delivery for kidney reasons.
  6. In all cases with anticipated pulmonary problems (ie, oligohydramnios from early pregnancy), delivery should be planned in a hospital with high-level neonatal care. For fetuses with bilateral cystic renal disease without oligohydramnios, consider delivery in a hospital with easy access to a pediatric nephrologist.
  7. There is no need to perform blood tests for renal function in children with unilateral cystic disease and a normal contralateral kidney with adequate compensatory hypertrophy.
  8. In patients at risk for neonatal renal failure (oligohydramnios or bilateral renal involvement), urine output should be monitored from birth. Renal replacement therapy should be offered to neonates with life-threatening kidney failure due to cystic renal disease. 
 
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