The role of minimal surgery with renal preservation in abnormal complete duplex systems

Among 36 children, mostly less than 1 year of age, referred for a complete duplex-system anomaly, 18 were diagnosed before birth by obstetrical ultras onography where a diagnosis of dilated upper urinary tract was the most fre quent report; in the remaining cases urinary tract infection was the main p resenting symptom. Five children had bilateral anomalies. Principal diagnos es associated with the complete duplex system were: 18 ureteroceles (UC) (1 1 extravesical [EUC] with bladder-neck or urethral extension, 7 intravesica l [IUC]), 5 ectopic ureters without UC and 28 lower-polar vesicoureteral re flux (VUR) associated in 6 with upper polar VUR. The initial assessment was based on a voiding cystogram and radionuclide scan. Penal-polar function w as severely impaired when major ureteral ectopia or severe primary reflux w as present. Primary surgery was performed in 8 patients, demolitive in 4 (3 upper-polar nephrectomies, 1 nephrectomy) and reconstructive in 4 (duplex en-bloc reimplantation); staged management with minimal endoscopic incision was undertaken in 15 UCs (9 EUCs and 6 IUCs). Expectant management was ele cted in all cases of mild primary, single, or double VUR not associated wit h UC and was followed by spontaneous reduction in one-half of the cases. Se condary VUR complicated endoscopic incision in 2 of 6 IUCs and 5 of 9 EUCs; a certain degree of functional improvement after decompression could be ob served in all IUCs versus only 2 EUCs. Twenty-one patients requested second ary surgery; 17 needed an open intravesical procedure for ureteral reimplan tation, combined in 8 with UC excision and bladder-floor reconstruction and in 5 with upper-pole nephrectomy. A nephrectomy was required in 4 cases. A ll primary or secondary demolitive procedures involved 9 of 11 EUCs extrave sical and 2 of 5 ectopic ureters. EUCs and ureteral ectopia were associated with severe renal-polar damage, and function was rarely affected by primar y decompressive procedures even in prenatally detected, uninfected cases. E ndoscopic incision of EUCs was frequently followed by secondary VUR, which made secondary intravesical operations more complex. For these reasons, pri mary elective resection of a dysplastic upper pole is preferable in most ca ses to temporary decompressive measures. Conservative surgery is always ind icated in IUC, which may benefit from endoscopic decompression. Isolated VU R involving the lower pole of a completely duplicated system may respond to expectant management in a significant number of cases.