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A tubed, free skin graft was used to construct a urethra in 49 young male subjects. Indications included 1) multiple previous hypospadias operations, 2) new severe hypospadias cases, 3) chordee without hypospadias but with a short urethra, 4) epispadias, 5) distal urethral stricture and 6) a small number of patients undergoing an initial operation for hypospadias by the Horton-Devine technique. No fistulas or leaks occurred. The over-all complication rate was low. Based on our own experience and that reported by others we believe that a free tubed graft is an excellent method for making a substitute urethra in a variety of urethral problems in children and young adults. It is important that the graft is of full or nearly full thickness because thin grafts tend to contract.

Since 1969 transureteroureterostomy has been done on 75 patients, mostly children. In the majority of cases the procedure was used simultaneously with reimplantation of the opposite ureter. The patients had been referred with previous failed ureteral reimplantation or for undiversion. The complication rate was low. Many of the cases could not have been reconstructed except by use of transureteroureterostomy or transureteropyelostomy. Transureteroureterostomy is a useful procedure that should be considered in a wide variety of urologic cases. By following certain general principles of avoiding tension, angulation and devascularization it can be done with a high success rate and few complications.

Five incontinent girls between 4 and 18 years old were made continent by constructing in each case a long extension of the urethra up to the clitoris. Previous operations at the proximal end of the urethra, to narrow the bladder neck, had not achieved continence. Lengthening the urethra was accomplished by tubularizing the anterior vaginal wall and covering that with a generous pedicle flap from the perineum. The base of the pedicle flap was divided later, restoring the introitus to a normal appearance. Of the 5 patients 3 had the underlying problem of ureteral ectopia with congenital deficiency of the urethra and the bladder neck, 1 had a marked degree of female hypospadias with absence of the distal urethra and in 1 the cause for incontinence was extensive internal urethrotomy during early childhood. This procedure should be applicable to certain difficult cases of incontinence in pediatric and adult patients.

The management of paratesticular rhabdomyosarcoma has evolved more slowly than that for testicular germ cell tumors, mainly due to its lower incidence and the lack of standardized treatment protocols. With gradual refinements in staging and therapy the prognosis has improved. Between 1960 and 1988, 18 patients 2 to 18 years old were treated at the Children’s Hospital. Management consisted of inguinal orchiectomy, staging evaluation and retroperitoneal lymph node dissection, followed by chemotherapy and radiotherapy according to the stage of the disease.

In 12 of 264 children treated with enterocystoplasty 15 spontaneous perforations occurred. Of the 12 children 9 had myelodysplasia. All segments of the gastrointestinal tract were used for the augmentation and most were detubularized. Surgery to increase bladder outlet resistance was done in 8 cases. At the time of each perforation 9 children had sterile cultures, however, 3 died of overwhelming sepsis. Presenting signs included abdominal pain in 8 cases, septic shock in 4 cases and shoulder pain in 4 older myelodysplastic children with diaphragmatic irritation from escaping urine. Cystography demonstrated a leak in 10 of 11 cases. Urodynamic studies revealed good compliance with low maximum filling pressure in 8 of 10 children. Hyperreflexia was noted in only 5 cases and outlet resistance greater than 85cm. water was demonstrated in 5. Histological analysis showed changes in the bowel wall consistent with ischemia but suture granulomas were present in areas adjacent to the perforation site or thinned areas in biopsy or autopsy specimens. In addition to the theory that overdistention may cause enterocystoplasty perforation, current detubularization techniques may produce areas of relative ischemia, which become accentuated when the augmented bladder is distended beyond a reasonable volume.

Since 1985, 7 children 2 to 17 years old with urinary incontinence from cloacal exstrophy underwent bladder augmentation and creation of a small bowel nipple at the bladder outlet. There were 3 genetic female and 4 genetic male patients being reared in the female role. In 3 early cases augmentation was done with small bowel, while gastric augmentation was used in 3 later cases and 1 had both types. Six patients became completely dry and they empty by intermittent selfcatheterization. In the youngest patient the operation failed because the nipple became infarcted and disappeared. Reoperation will be performed after at least 1 year has passed. In cloacal exstrophy if there is insufficient local tissue to create a bladder neck and urethra, a reversed, catheterizable bowel nipple may be an option to consider.

Unsatisfactory appearance and function of the penis are common after repair of complete epispadias. Reoperation can benefit the majority of these patients. The principles of repair in secondary cases as well as primary cases include wide mobilization of the corpora to gain length, resection of dorsal chordee to straighten the shaft, augmentation of urethral length with a graft, secure closure of the corpora over the urethra to avoid a fistula, wide mobilization of the mons to place hair-bearing skin in proper location and Z-plasty closure.

A personal experience in the management of 73 ureterocele cases during the last 17 years and points of technical importance in the correction of this sometimes complex problem are described. Small adult-type ureteroceles are best resected, reimplanting the ureter, since simple unroofing can cause reflux. The infant-type ureterocele with a duplex collecting system requires individualization according to the status of the patient. In ectopic ureterocele cases the bladder outlet often requires repair. The spectrum of management for this group of problems is shown by representative cases.

A 7½-year-old boy sustained severe trauma to the upper abdomen, including transection of the common bile duct, transection of the second part of the duodenum, contusion of the head of the pancreas, traumatic thrombosis of the left renal artery, thrombosis of the right lower pole renal vasculature and avulsion of the right renal pelvis. A staged elective repair of the upper urinary tract, and later the upper gastrointestinal tract, was accomplished when he was referred 5½ months after the initial injury.

Fetal screening during maternal ultrasonography has changed the mode and age of presentation of congenital uropathies, particularly congenital obstructed megaureter. We studied 47 infants less than 8 months old with primary obstructed megaureter. Surgical correction in 42 patients with moderate to severe obstruction was performed at a mean age of 1.8 months in those detected prenatally and 3.8 months in those presenting after birth. All infants showed functional and structural urographic improvement with a mean followup of 2.3 years. Reflux was seen postoperatively in 8 patients, which subsided spontaneously in 3, continues to be followed in 2 and resulted in repeat reimplantation in 3. Five infants had mild obstruction, which was managed nonoperatively and 2 showed progressive obstruction, which required repair at ages 20 and 28 months. Repair of obstructed megaureters in early infancy improves renal drainage and offers the potential for preventing renal damage before the development of symptoms or infection. With proper attention to detail, excellent results may be achieved.

In the last 13 years splitting the pubic symphysis has been used in 46 patients 1 to 32 years old for wide access to the bladder neck and entire urethra in female, or the bladder neck and urethra down into the bulb in male patients. Each patient had a complex problem to reconstruct and most had undergone a prior operation. Underlying pathological conditions included exstrophy in 16 patients, epispadias in 11, cloacal exstrophy in 6, posterior urethral valves in 3, severe trauma in 2, bilateral single ectopic ureters in 2, radical surgery for sarcoma in 2, myelodysplasia in 2, cloacal anomaly in 1 and iatrogenic injury of the urethra in 1.

Gastrocystoplasty was performed in 29 female and 14 male patients 1 to 23 years old (average age 11 years). Urodynamic studies were completed in 20 patients preoperatively and within 6 months postoperatively. When postoperative studies were compared with the preoperative evaluation, it was noted that overall capacity increased an average of 220% (range 20 to 750%). Compliance also improved dramatically postoperatively. Bladder pressures increased from a mean volume of 34% of capacity preoperatively to 73% of the post-augment capacity. The average maximum filling pressure decreased by 32%, from 57 to 39cm. water. Uninhibited contractions resolved completely in 6 of 10 patients who had them preoperatively. No one had uninhibited contractions postoperatively. Cyclical contractions of the stomach segment not exceeding 50cm. water developed in 6 patients.

The 45 ileal conduits performed on children at the Massachusetts General Hospital from 1955 to 1963 are reviewed and compared to the 45 ileal conduits performed from 1964 to 1970. Late complications involving the conduits occurred in 60 per cent of the early group and in 51 per cent of the late group. Of the renal units judged normal pyelographically preoperatively in the early group 77 per cent went on to at least some deterioration, while 62 per cent of the late group judged normal later deteriorated. Combining all renal units, 34 per cent remained unchanged, 26 per cent improved and 41 per cent showed some degree of deterioration after ileal conduit urinary diversion. The late complication and renal deterioration rates seem to increase progressively with time.

Sixteen bladder exstrophy patients were treated by diverting the urine Into a non-refluxlng colon conduit. There were 4 new, previously unoperated cases and 12 had been diverted earlier by Ileal conduit. Subsequently, 11 conduits were detached from the abdominal wall and joined end-to-side to the rectosigmoid colon. This method has proved satisfactory to date but long-term assessment will be needed. This staged method of ureterosigmoid urinary diversion appears to be a satisfactory way to manage new patients with bladder exstrophy and patients who have been diverted previously by an ileal conduit.

Continent urinary diversion has become increasingly important for treating childhood urinary tract pathology that cannot be managed by direct reconstructive techniques. We review our 9-year experience with continent diversion.

Six cases are described of congenital female epispadias and incontinence. Of these patients 3, who were seen in the last 4 years, were made continent by a 1-stage operation using a combined perineal and transvesical approach. The procedure includes excising the abnormally wide roof of the distal urethra from below, narrowing the urethral caliber and mons plasty. From above the upper urethra and bladder neck are narrowed. Ureters are reimplanted if they have reflux or to facilitate bladder neck narrowing. The 3 other cases are described in which this combined procedure could have been used, in retrospect, to obtain a satisfactory result with fewer operations. In female epispadias complete repair of the abnormally wide urethra is better than other alternatives that have been proposed for treating this rare malformation.

During the last 11 years 37 patients were treated with ureterocolic diversion of the urine by means other than classic ureterosigmoidostomy. By altering the technique of joining the ureters to the colon this method of internal urinary diversion is possible even in cases when it would not be feasible by more traditional methods.

Late stricture is a well known complication of ileal loops. We herein report on late stricture in 5 patients in whom bowel was used as a substitute ureter in closed, nondiverted urinary tracts. This late complication mandates the need to provide continuing followup indefinitely in patients in whom bowel segments are incorporated in the urinary tracts.

Records were reviewed of 100 patients who underwent an operation for ureteropelvic junction obstruction from 1978 to 1989:14 cases were bilateral and 17 were antenatally diagnosed. The ureter was opacified preoperatively in all but 1 patient: in 65 by retrograde pyelography, in 18 by antegrade pyelography, either through a nephrostomy tube with which the patient was referred or during a Whitaker test, in 9 by vesicoureteral reflux seen on voiding cystourethrography performed before an operation for ureteropelvic junction obstruction and in 7 by an excretory urogram. Of the 65 patients who underwent retrograde pyelography 29 had a discrete area of narrowing. However, 36 patients had something more, including a longer segment of narrowing (13), tortuosity of the upper ureter (8), more than 1 area of narrowing (11), high insertion of the ureter on the renal pelvis (3) and compression of the ureter by the lower pole of the kidney (1). In 10 patients referred for reoperation after failed pyeloplasty there was narrowing of the ureter below the level of the prior pyeloplasty.

From 1977 to 1989 bladder augmentation was performed in 56 male and 73 female patients from 1 to 35 years old (average age 12.7 years). In 59 cases augmentation was performed as part of an undiversion operation. Indications for augmentation included neurogenic bladder, severely scarred bladder from prior operations, noncompliant bladder after previous treatment of severe urethral valves, small bladder in former exstrophy patients, epispadias with a small bladder, cloacal exstrophy, and small, noncompliant bladder after therapy for cancer, trauma, cloaca and miscellaneous conditions. We used 145 bowel segments, since 16 patients had 2 bowel segments. Segments included cecum in 65 cases, sigmoid in 46, small bowel in 24, stomach in 4 and left colon in 1. The most common complication was stones. Detubularized bowel for augmentation of small noncompliant bladders allows functional reconstruction in a wide range of urological disorders that were formerly treated by diversion. Reconstruction is possible in many previously diverted patients if bladder augmentation is used.

The intestinal augmented bladder has an increased propensity to form urinary calculi. Predisposing risk factors include chronic bacteriuria, urinary stasis and mucus production. Gastric reservoirs have negligible mucus production and the ability to acidify the urine. We determined whether they also have a decreased incidence of stones.

From 1969 through 1976, 70 non-refluxing colon conduit urinary diversions were performed on 30 adults and 40 children at the Massachusetts General Hospital. Although this is a longer and more complex operation to perform the rate of complications appears significantly less than that seen previously in patients with ileal loop diversions.

There is a wide range of congenital vaginal abnormalities that can be reconstructed using bowel. From 1964 to 1993 we used bowel for vaginal reconstruction in 65 patients and all but 5 were treated since 1981. Patient age ranged from 9 months to 26 years. In 29 patients the bowel was used to extend a short vagina to the perineum, and in 36 the entire vagina was absent and required a substitute vagina. Indications for surgery included cloacal malformations in 34 cases, congenital absence of vagina in 12, cloacal exstrophy in 9 genetic male subjects, postoperative exenteration in 7, intersex in 2 and bladder exstrophy in 1. Rectum was used in 12 cases, sigmoid colon in 27 and small bowel in 26. Ten patients who are now adults have reported satisfactory coitus.

Many children with severe urologic problems have been treated by urinary diversions, often after unsuccessful operations to correct the original pathology. Urinary diversion should be avoided when possible, since often it is accompanied by chronic infection and quality of life is less good than it could be. Most diversions can be prevented by use of other alternatives including 1) reimplanting 1 ureter and transureteroureterostomy of the other, 2) psoas hitch, 3) wide mobilization and downward displacement of the kidney and ureter, 4) use of bowel as ureter, 5) cecal cystoplasty and 6) early repair of complex pathology. Autotransplantation is probably seldom indicated in childhood.

We evaluated urodynamically 41 patients with posterior urethral valves because of signs or symptoms of incontinence (35), frequency (3), hydronephrosis (2) and infection (1). Findings included normal urodynamic evaluations in 3 patients, 2 had high voiding pressures secondary to outlet resistance and 1 had incontinence on the basis of external urethral sphincter damage. In the remainder 3 patterns of bladder dysfunction were identified. Myogenic failure with overflow incontinence occurred in 14 patients. In this group clean intermittent catheterization or Valsalva’s voiding was used for emptying. Hyperreflexic bladders were seen in 10 patients. Pharmacological suppression of instability was effective in 5 of 7 patients treated; 1 required bladder augmentation. Eleven children had a small capacity bladder and poor compliance. Post-void residuals were low and these bladders were generally but not always stable. Pharmacological bladder relaxation was successful in 3 patients, 3 underwent augmentation, 1 did well with a-agonists and followup is unavailable on the other 4.

From 1979 to 1987 we treated 28 patients with severe penile malformations who required a dermal graft to straighten the shaft and a free graft to construct a urethra. In 10 patients the 2 grafts were placed simultaneously with good results. In 15 patients the dermal graft was placed initially and a urethral graft was placed over it later. In 3 patients the urethroplasty has not been done yet. No complications resulted from the combined use of dermal and urethral free grafts. The principle of 舠graft on graft舡 can be applied successfully to correct certain severe penile problems. Indications, technique and results are presented.

From August 1978 to September 1987 a total of 103, 1-stage hypospadias-chordee operations was performed using a free graft of prepuce by the method of Devine and Horton. There were 94 primary cases and 9 secondary cases in which prior operations had been done. Chordee was mild in 11 patients, moderate in 67 and severe in 25. The meatus was at the distal shaft in 56 patients, midshaft in 34 and penoscrotal in 13.

Cloacal malformations occur in a spectrum of severity. In general the urethra (sometimes short or absent), vagina (often 2) and rectum converge in the urogenital sinus. Immediate colostomy is necessary. However, after colostomy the urinary tract assumes prime importance because of life-threatening obstruction and/or reflux. I treated 72 patients with complex cloacal malformations, 33 as primary and 39 as secondary cases. In 40 patients there was severe reflux with severe upper tract damage in more than half of the cases: 19 had prior urinary diversions (4 ileal loop, 5 loop ureterostomy and 10 vesicostomy). In nearly all secondary cases a definitive rectal operation had been done but the more life-threatening urinary tract had not been repaired. Some patients had other urological and nonurological malformations.