Urethral atresia and anhydramnios at 18 weeks of gestation can result in normal development

Abstract

INTRODUCTION: Second-trimester anhydramnios is incompatible with survival unless amniotic fluid volume is restored. We describe the long-term outcome of a child with documented anhydramnios at 17 weeks. CASE REPORT: A 28-year-old gravida II had anhydramnios at 17 weeks. At 19 weeks, the bladder measured 12 cm, and karyotype was XY. At 21 weeks, a fistula between the bladder and the amniotic space developed. At 36 weeks, a 2800 g boy with prune belly phenotype was delivered. The urachus was patent and drained urine. There was a completely obstructing membrane below the veru montanum and a hypoplastic urethra distal to it. The right kidney had no function and the left had grade V reflux. A P.A.D.U.A. (progressive augmentation by dilatating the urethra anterior) procedure was performed and the urachal fistula closed. At 6 months of age abdominoplasty, bilateral orchidopexy, right nephrectomy and left to right transuretero-ureterostomy were performed. At age 30 months he was toilet trained, with creatinine 0.5 mg/dl and normal growth. At 10 years of age he continues to be continent, with serum creatinine 0.9 mg/dl. CONCLUSION: Resolution of anhydramnios at 21 weeks of gestation can result in normal pulmonary function and acceptable renal and bladder function in childhood. Efforts to develop effective methods of fetal bladder decompression should continue.