両側単一尿管異所開口および単腎の単一尿管異所開口症例の検討 : とくに膀胱形成不全に対するstaged operation について

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  • Clinical Analysis of Children with Bilateral Single Ectopic Ureter and Single Ectopic Ureter in a Solitary Kidney

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Ureteral ectopy is the result of faulty development of the terminal wolffian duct, and the association with a double ureter and pyelocalyceal system is not uncommon. Although single ectopia accounts for about 70 to 80 per cent of total in Japanese literature, bilateral single ectopic ureter or single ectopic ureter in a solitary kidney is a rare occurence. During the last 17 years we treated 6 children (3 males and 3 females) with bilateral single ectopia and 3 (1 male and 2 females) with single ectopia in a solitary kidney. Age at presentation ranged from 4 days to 3 years. Main clinical presentations included fever from UTI and hematuria. In 5 of 6 children with bilateral ectopia, either one or both of the ureteral openings were found at the bladder neck. In the last case both ureters opened into the vestibulum and the bladder was severely hypoplastic with its capacity less than 5 ml. Among 3 children with solitary kidney, the ureter opened at the bladder neck in one and in the urethra in two. One of the children whose ureter opened in the urethra presented severely hypoplastic bladder of about 3-4ml in capacity. Dilatation of the upper urinary tract was seen in all cases. Ureteral reflux was evident in about half of the ureters. Antireflux ureterovesical anastomosis was done in 9 ureters of 7 children. In two children with severe bladder hypoplasia, we performed staged procedure, first to increase the bladder capacity and second to prevent vesicoureteral reflux. In the first procedure, side-to-side anastomosis of the dilated ureter and lateral wall of the hypoplastic bladder was done using operative loupe. One neonate required temporary diversion of the upper urinary tract prior to the first procedure, since she presented acute renal failure from the obstruction of bilateral ureteral openings. Six to seven months after the first operation the bladder capacity reached about 40 to 70 ml. In the girl with solitary kidney, the second operation of antireflux ureterocy-stoneostomy was performed 9 months after the first procedure with satisfactory result. The second child is now waiting for the second operation.

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