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
- Because many cystic kidney diseases manifest initially as hyperechogenic kidneys without visible cysts,their differential diagnosis should be considered together.
- 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.
- 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.
- 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.
- Do not offer early delivery for kidney reasons.
- 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.
- 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.
- 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.
Comments by Evaluators:
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