[Pyeloureteral junction syndrome in children. Apropos of 178 cases]

The authors review 178 cases of anomalies of the pyelo-ureteric junction, with a total of 189 anomalies. Almost one half of the cases were less than 5 years old (47.2%) and about 1/4 were less than 1 year old (24.7%). In girls, the incident which reveals the diagnosis is most often an infection and in boys, it is pain or haematuria. 26% of the cases had an associated urological lesion (23 cases of vesico-ureteric reflux, 8 cases of renal stones, 5 cases of mega-ureter and 4 cases of horseshoe kidney). 133 children were operated, essentially by resection of the pyelo-ureteric junction with an upstream by-pass. From the 126 cases with sufficient follow-up, 48 (38.1%) obtained great improvement, 63 (50%) a moderate improvement and 15 (11.9%), a stabilisation of the dilatation. In all cases except 3, the pyelo-ureteric junction was perfectly catheterizable. The histological lesions of the junction were variable and difficult to systematise, but usually extended beyond the junction to involve a large part of the pelvis. The variability of the caliceal dilatation makes any attempt at classification difficult. The authors recommend cystography in every case, as associated reflux was discovered in 16% of cases. Retrograde uretero-pyelography is practically no longer indicated. The isotope kidney scan with DTPA technetium, with a hyper-diuresis test, is often used, as it gives an idea about the clearance of filtration and about the degree of obstruction. Quantitative DMSA kidney scan can reveal the effects on the renal parenchyma. Conservation of the isthmus of a horseshoe kidney never interferes with the uretero-pyelic re-implantation. After correction of the anomaly of the uretero-pyelic junction, 5 cases of unilateral reflux out of 8 and 3 cases of bilateral reflux out of 10 resolved spontaneously.