Early second trimester intervention in a surviving infant with postnatally diagnosed urethral atresia.

Abstract

A 37-year-old woman (gravida IV, para II) was referred for screening fetal ultrasound at 14 weeks of gestation. The fetus was identified with a massively dilated bladder and normal amniotic fluid. At 15 weeks of gestation level III ultrasound demonstrated oligohydramnios, bilateral echogenic kidneys with hydronephrosis and the “keyhole” sign indicating profound bladder outlet obstruction (fig. 1). The remaining organ systems were unremarkable. Serial bladder electrolytes were obtained at 15, 16 and 17 weeks of gestation (see table). The parents decided to intervene despite a poor prognosis, and at 18 weeks of gestation a vesico-amniotic shunt was placed with immediate decompression of the bladder and restoration of amniotic fluid. At 30 weeks of gestation oligohydramnios was detected despite a decompressed urinary system, and elective cesarean section was performed at 34 weeks of betamethasone treatment. Peritoneal dialysis was instituted in the first week of life to manage renal insufficiency and oliguria. Since neonatal evaluation suggested bladder outlet obstruction, vesicostomy was performed. At age 14 months a 2 cm. proximal congenital urethral atretic segment was identified and serially dilated allowing closure of the vesicostomy (fig. 2). At age 30 months bilateral reimplantation with ureterocystoplasty was performed in an