Antenatal oligohydramnios of renal origin: postnatal therapeutic and prognostic challenges.
Urinary tract anomalies (UTA) including polycystic kidney disease nowadays can be detected antenatally by ultrasound. The concomitant presence of oligohydramnios has been regarded as a severe risk factor for renal dysfunction and pulmonary hypoplasia, although clinical data after birth are scarce. We report the postnatal course and long-term follow-up of 10 infants with oligohydramnios due to congenital UTA from two pediatric nephrology centers. The underlying final diagnoses were autosomal-recessive polycystic kidney disease (ARPKD, n = 2), familial tubular dysgenesis (n = 2) and bilateral renal hypoplasia (n = 6) including 3 children with posterior urethral valves. Two children died in the neonatal period while 8 children are currently alive at a median age of 2.5 (range 1.1-10) years. In the postnatal period, respiratory failure necessitating mechanical ventilation occurred in 7 infants (including the 2 non-survivors). All surviving children had chronic renal failure, which could be managed conservatively in 6 children (median GFR 45 (range 15-53) ml/min/1.73 m2) while 2 reached end-stage renal disease; one undergoing preemptive kidney transplantation and one peritoneal dialysis. Seven of 8 children reached normal developmental milestones. In conclusion, the presence of antenatal oligohydramnios in infants with UTA does not always carry a poor prognosis. The high incidence of perinatal complications, the complexity of underlying causes and the prevalence of postnatal chronic renal dysfunction calls for a multidisciplinary approach in the management of these children.
Kemper
M J
MJ
Pediatric Nephrology, University Hospital Eppendorf, Hamburg, Germany. Markus.Kemper@kispi.unizh.ch
Neuhaus
T J
TJ
Timmermann
K
K
Hueneke
B
B
Laube
G
G
Harps
E
E
Mueller-Wiefel
D E
DE
D005260:Female; D005315:Fetal Diseases / Q000000981:diagnostic imaging*; D005865:Gestational Age; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D016104:Oligohydramnios / Q000000981:diagnostic imaging* / Q000209:etiology; D007690:Polycystic Kidney Diseases / Q000150:complications / Q000000981:diagnostic imaging*; D011247:Pregnancy; D011248:Pregnancy Complications; D011379:Prognosis; D012127:Respiratory Distress Syndrome, Newborn / Q000209:etiology / Q000628:therapy; D012189:Retrospective Studies; D016216:Ultrasonography, Prenatal; D014551:Urinary Tract / Q000002:abnormalities* / Q000000981:diagnostic imaging; D012122:Ventilators, Mechanical