Posterior urethral valves are the most common cause of bladder obstruction in boys. Clinical presentation may vary, depending upon the degree of obstruction and its sequelae. Pathological changes range from relatively normal upper tracts or a small amount of dilatation to a massive dilatation associated with severe infection or sepsis, azotemia and/or renal dysplasia. In our population they constitute about 17% of chronic renal failure in children.
1The development of renal insufficiency in patients with posterior urethral valves may be attributed to the high pressure generated by urethral obstruction transmitted to the upper tracts in utero. In this review, four cases of posterior urethral valves are described, in which we identified certain protective factors which provided a "pop-off" mechanism that resulted in preservation of intact renal and bladder function.
Patients and Methods
Case 1This infant was born with bilateral abdominal masses and mild respiratory distress. Antenatal ultrasound showed bilateral hydronephrosis and oligohydramnios. The initial serum creatinine was 200 μmol/L. Ultrasonogram showed bilateral hydronephrosis. A large collection of fluid was seen around the superior pole of the right kidney, measuring 4.4 x 3.1 cm. A similarly large collection was seen around the left kidney. A micturating cystourethrogram (MCUG) showed multiple small bladder diverticulae, a long dilated posterior urethra and a right Grade V vesicoureteric reflux (VUR). A DMSA scan showed 6% function in the right kidney and 94% in the left kidney. The baby underwent cystourethroscopy, vesicostomy and drainage of both perinephric urinomas. The serum creatinine reached 75 μmol/L. At the age of two years the vesicostomy was closed and the valve fulgurated. Cystometry showed a normal bladder capacity. Subsequently he underwent a right nephroureterectomy. At his last follow-up at the age of three years, his serum creatinine was 40 μmol/L.
Case 2A 45-day-old infant was referred to us with failure to gain weight from birth and repeated vomiting of one week's duration. The abdomen was distended, with well-defined masses palpable in both loin regions. The initial serum creatinine was 190 μmol/L. A sonogram showed hydronephrotic kidneys with hyperechoic parenchyma. A well-defined urinoma was seen on the anteriomedial aspect of the right kidney (2.6 x 1.1 cm). A cystogram showed multiple diverticulae of varying size, a right Grade IV VUR and a dilated posterior urethra. A DMSA scan showed 7% function in the right kidney and 93% in the left kidney. Cystoscopy showed a Type III posterior urethral valve (PUV). A vesicostomy and drainage of the urinoma by a perinephric drain were performed. On the 10th day after admission, the infant's serum creatinine dropped to 54 μmol/L. A repeat ultrasound showed that the urinoma had regressed in size. The vesicostomy was closed and the valve fulgurated. Cystometry showed normal findings for the age. Ultimately, a right nephroureterectomy was performed. At his last follow up at the age of two year...