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Purpose: Persistent cloaca is a complex malformation with variable presentation that remains a difficult reconstructive challenge. Previous reviews have concentrated on surgical technique, and data on long-term outcome are scarce. We evaluate long-term outcome and when possible correlate outcome with anatomy at presentation.Materials and Methods: The records and radiographs of 64 patients 0.5 to 25 years old with persistent cloaca treated at 1 institution from 1975 to 2000 were retrospectively reviewed to determine the outcome for urinary and fecal continence. Outcome data were available in 61 girls as 3 died. Of these patients 11 were younger than 3 years, making evaluation of continence difficult, and so the remaining 50 patients constitute the study population. Mean patient age at review was 11.3 years (range 4 to 25).Results: Of the 50 patients 10 (20%) are incontinent of urine (4 with urinary diversion) and 40 (80%) are socially continent, including 11 (22%) who void spontaneously, 6 (12%) wh...

The aim of this study was to determine whether redo bladder neck reconstruction is effective in achieving continence after a failed bladder neck reconstruction procedure.We retrospectively reviewed the hospital records of patients with bladder exstrophy who had undergone redo bladder neck reconstruction. There were 30 patients in the study, including 20 boys and 10 girls. Mean patient age at redo bladder neck reconstruction was 9.3 years (range 3.2 to 15.5). The patients were divided into 3 groups on the basis of the preoperative pattern of incontinence--incomplete wetters, complete wetters and those on continuous suprapubic drainage. Of the patients 15 already had undergone bladder augmentation, 12 had undergone a Mitrofanoff procedure and 12 had been treated with bulking agents injected in the bladder neck in an attempt to achieve continence. Four patients had undergone more than 1 bladder neck procedure. The patients were investigated with a combination of noninvasive urodynamics, cystoscopy, cystogram and ultrasound. All patients underwent Mitchell's modification of Young-Dees-Leadbetter bladder neck reconstruction. Additional procedures performed included augmentation cystoplasty and Mitrofanoff formation.Mean followup was 6.9 years (range 1.2 to 15.5). Postoperatively 28 patients were using clean intermittent catheterization to empty the bladder (5 per urethra, 23 via Mitrofanoff). Two patients remained on continuous suprapubic catheter drainage. A total of 18 patients (60%) were dry postoperatively (80% of girls and 50% of boys). Among dry patients only 3 were performing clean intermittent catheterization per urethra and 15 via a Mitrofanoff channel. No patient was able to void per urethra without the need for clean intermittent catheterization. The 2 patients on continuous suprapubic catheter drainage continued to remain so. At night only 50% of the patients were dry (5 on free drainage, 4 on clean intermittent catheterization, 6 not on any drainage). Those patients who did not respond satisfactorily to redo bladder neck reconstruction underwent subsequent additional procedures, which included injection of bulking agents (3 patients), insertion of an artificial urinary sphincter (1), Mitrofanoff formation (2) and bladder augmentation plus Mitrofanoff channel (1). Postoperative complications included difficulty with clean intermittent catheterization (8 patients), perivesical leak (1), recurrent epididymo-orchitis (1), upper urinary tract dilatation (2) and incisional hernia (1). Bladder neck closure was being considered in 5 patients.In our experience redo bladder neck reconstruction cannot achieve continence with volitional voiding per urethra. Although redo bladder neck reconstruction can render a significant number of patients dry, it is only effective if performed in conjunction with augmentation. Failure of the initial bladder neck reconstruction may be a reflection of a bladder that is of inadequate capacity and/or compliance. Therefore, bladder augmentation should be considered in all patients requiring redo bladder neck reconstruction. Bladder neck closure may be a better alternative to redo bladder neck reconstruction.

Purpose: Since 1980 numerous variations of the Mitrofanoff principle have been described. We report on 22 children in whom a ureteral Mitrofanoff channel was created.Materials and Methods: Between 1986 and 1995 a ureteral Mitrofanoff channel was constructed as a catheterizable conduit in 22 children 2 to 15 years old (average age 6.5) with various abnormalities of the lower urinary tract, mainly exstrophy and neurogenic bladder. Indications included unavailability or unsuitability of the appendix, preference for appendix as a catheterizable colonic stoma for antegrade washouts or concomitant removal of a nonfunctioning kidney, leaving the ureter available for use. Surgical technique was based on the principles of appendicovesicostomy and in 9 cases the ureteral Mitrofanoff channel was reimplanted.Results: Followup ranged from 1 to 72 months (average 30.5). Complications included stenosis of the conduit that caused difficult catheterization in 3 patients, necessitating dilation or minor revision in...

Purpose: We evaluated functional outcome following heminephrectomy in duplex kidneys. Materials and Methods: A total of 101 patients were identified who underwent upper or lower pole heminephrectomy between January 1995 and December 2000. We retrospectively reviewed 60 case notes with complete data. A total of 37 cases (62%) were diagnosed prenatally, while 20 (33%) presented as single or recurrent urinary tract infection and 3 (5%) as urinary incontinence. Of the patients 52 (87%) had unilateral duplex and 8 (13%) had bilateral duplex kidneys. In addition, 20 of the 60 patients (33%) had ureterocele. Mean age at surgery was 46 months (range 3 to 10 years). Results: Mean function as assessed by nuclear renography of the duplex kidney before heminephrectomy was 39.9% (range 22% to 61%) and following heminephrectomy was 33.1% (13% to 60%). Mean decrease in function was 6.8%, with a decrease in function of less than 5% in 18 patients, 5% to 10% in 13 and more than 10% in 5. The functional values were increased by 1% to 5% in 9 patients and unchanged in 15 patients after heminephrectomy. Conclusions: Following heminephrectomy there was a significant decrease in 8% of patients and a small decrease in function in the remaining moiety in 51%.

Purpose: We assessed the role and long-term outcome of lower pole heminephrectomy in the treatment of nonfunctioning lower renal moieties in children with duplex kidneys.Materials and Methods: Between 1979 and 1994, 54 lower pole heminephrectomies were performed in 53 patients 1 to 192 months old (mean age 54) with duplex systems. A total of 15 patients was prenatally diagnosed, while the others presented with a urinary tract infection (36), orchiepididymitis (1) and failure to thrive (1). The surgical technique was essentially similar to that of upper pole heminephrectomy.Results: Operative course was uneventful except for intraoperative bleeding in 5 cases, which necessitated blood transfusion, and a postoperative urinary tract infection in 1. Followup ranged from 3 to 168 months (mean 56.9). Postoperative renograms available in 34 cases showed unchanged differential function in 12 and an ipsilateral 2 to 14 percent decrease (mean 5.65 percent) in 22. No late complications were detected except i...

The outcome and quality of life in children with cloacal exstrophy are greatly influenced by small intestine residual length. We report 2 cases of almost complete midgut deletion in newborns with duodenal atresia in addition to cloacal exstrophy. To our knowledge this combination of anomalies has not been reported previously CASE HISTORIES Case 1. A male neonate born at 36 weeks of gestation with a birth weight of 2,500 gm. had cloacal exstrophy. Prenatal ultrasound had demonstrated an anterior abdominal wall defect. Magnetic resonance imaging of the spine showed a low lying tethered cord with lipoma and sacral hypoplasia. Bilious nasogastric aspirates were observed on day 3 of life and the classic double bubble appearance was evident on plain abdominal x-ray (see figure). Duodenal obstruction was confirmed by an upper gastrointestinal contrast study. Laparotomy revealed atresia of the second part of the duodenum with only 21 cm. of residual intestine. Atresia was corrected but after discussion with the parents other active treatment was withdrawn. The neonate died at age 17 days. Case 2. A male infant born at 32 weeks of gestation with a birth weight of 2,060 gm. had classic cloacal exstrophy. Prenatal scan had shown an anterior abdominal wall defect. Ultrasound of the spine demonstrated sacral agenesis, a tethered cord and myelomeningocele. Abdominal x-ray revealed a double bubble appearance. Laparotomy confirmed atresia of the second part of the duodenum with only 12 cm. of residual small intestine. No further surgery was performed. The neonate died at age 6 days. DISCUSSION

Transverse retubularization of small ileal segments has been described as a new time and labor saving variation of the Mitrofanoff principle in a dog model with good functional results. We report our initial clinical experience with this technique.From May 1996 through January 1997 a new technique of channel formation for intermittent catheterization was applied in 9 children (1 to 16 years old) and 7 adults (18 to 56) with various abnormalities of the lower urinary tract. The new method was used in primary reconstruction of the lower urinary tract and in revision procedures. An ileal segment 2 cm. long was excised. The bowel wall was opened longitudinally about 1 cm. from the mesentery. The resulting rectangle was retubularized over a 14F catheter in transverse direction. The longer portion of the tube was implanted submucosally into the native bladder, the augmented bladder or an intestinal reservoir. The shorter portion was used to form the stoma. In 4 patients we created a double tube.Of the patients 13 (81%) are completely continent day and night with easy catheterization postoperatively. In 2 cases of tunnel failure continuous leakage required reimplantation of the intact ileal tube to achieve continence. Minor leakage with bladder fullness in an 11-year-old boy could be obviated by adjusting the interval of catheterization.With the advantage of constant availability, minimal loss of bowel, relative simplicity (no mesentery interfering with implantation, high tube mobility), minimized risk of stone formation (no staples), reliable continence (no leak point) and easy catheterization (longitudinal folds), this straightforward technique is an excellent second choice use of the Mitrofanoff principle.

The syndrome of posterior urethral valves, persistent unilateral reflux and renal dysplasia (VURD) is said to be protective of the contralateral nonrefluxing kidney and the outcome for renal function is reported to be excellent. We tested this hypothesis in our patients by replicating previous studies but with longer followup and glomerular filtration rate data.We retrospectively reviewed the records of 183 boys presenting with posterior urethral valves between 1980 and 1989, including 12 who underwent nephrectomy for ipsilateral nonfunction and fulfilled all criteria for the VURD syndrome. Mean age at the most recent followup was 8.5 years. Serial serum creatinine levels and glomerular filtration rates were analyzed and compared to age matched normal values.Histological evaluation revealed dysplasia in all kidneys, confirming the VURD syndrome. Followup plasma creatinine was normal 67% of the patients during year 2 of life, 50% between ages 4 and 5 years, and only 30% between ages 8 and 10 years. Glomerular filtration rate was within the normal range in 25% of boys tested in year 2 of life, and between ages 5 and 8 years.Our data do not support the protective effect of the VURD syndrome on long-term renal function. All patients with posterior urethral valves require diligent long-term followup.

The management of 58 patients born with an imperforate anus who presented to the urology service and who are currently 18 years old or older is discussed. Of the 44 patients with a high imperforate anus 43 were treated by a rectal pull-through and 1 by an anal cut back procedure, while an anal cut back procedure was used in all 14 children with a low imperforate anus. A total of 32 children (55%) had a neuropathic bladder (hyperreflexic in 29 and atonic in 3). A spinal deformity was present in 72% of the hyperreflexic group but not in the atonic group. Vesicoureteral reflux occurred in 41% of the patients, two-thirds of whom had a neuropathic bladder. A total of 30 children had an associated genital anomaly and 27 had an upper tract anomaly. Nineteen children underwent an operation for the neuropathic bladder, with modern reconstructive methods involving substitution or augmentation cystoplasty, supplemented with self-catheterization, being superior to older methods with regard to continence. The incidence of a neuropathic bladder in these children is high in both operative groups but it is usually associated with a spinal deformity and unlikely to be iatrogenic in nature unless proved to be of lower motor neuron origin. Early operative management is advised to achieve continence and minimize renal impairment.

We report on a 7-year-old boy with a multilocular cyst of the left kidney, which was managed by conservative surgery. Multilocular renal cysts in early childhood have been reported previously and have invariably resulted in nephrectomy. The unusual features of this case are described and the importance of diagnostic ultrasound and the potential for conservative surgery are emphasized.

We reviewed the patterns of pyelonephritic damage that occurred during surgical management of bladder exstrophy. Renal injury patterns indicate that papillary configuration and obstruction coupled with urinary infection were the primary factors in producing damage. Diversion by the ileal conduit resulted in frequent injury, whereas the colon conduit was associated rarely with renal damage. The incidence of renal injury from bladder closure and continence attempts suggested closure as a reasonable course, especially in view of present continence results.

We report 2 cases of dilatation of the lower moiety ureter in a duplex kidney with ectopic ureterocele owing to intrinsic pathology at the ureterovesical junction. The relevant problems of this rare situation with double pathology are discussed.