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Purpose: The 2 types of urethral injury that can occur during circumcision are urethrocutaneous fistula and urethral distortion secondary to partial glans amputation. We report the surgical repair of these rare injuries., Materials and Methods: In 8 patients urethrocutaneous fistulas located on the distal penile shaft or at the coronal margin were managed by splitting the glans and using a Mathieu style skin flap in 4 or vascularized penile skin flap in 4 to bridge the urethral defect. Three patients underwent repair of a hypospadiac deviated urethra secondary to partial glans amputation by 1 cm. of urethral mobilization and repositioning the meatus into a terminal position within the remaining glans tissue., Results: The 8 patients with urethrocutaneous fistulas voided via a terminal meatus without fistula recurrence at a mean followup of 3.2 years (range 1 to 6). The 3 patients with partial glans amputation and urethral deviation repaired by short urethral advancement had functionally acceptable results, defined as a normal urinary stream, although 1 required meatal dilation postoperatively., Conclusions: The 2 types of urethral injuries that can occur during circumcision are a subcoronal urethrocutaneous fistula and scarred abnormal urethra from partial glans amputation. The urethrocutaneous fistula can be successfully repaired by splitting the glans and forming a neourethra from a vascularized pedicle flap of penile skin. The abnormal urethra after partial glans amputation is more difficult to repair but repositioning the urethra in a more cosmetic location has restored function.

Purpose: Surgical repair of ureteropelvic junction obstruction is successful in 98% of cases. We evaluated children undergoing repeat pyeloplasty and discuss the etiology of recurrent ureteropelvic junction obstruction, surgical approach and outcome., Materials and Methods: Between 1982 and 1996, 366 children with ureteropelvic junction obstruction were surgically treated at our institution, including 16 who presented with recurrent ureteropelvic junction obstruction and required surgery., Results: Repeat repair was successful in all 16 patients, including ureterocalicostomy in 3 and dismembered pyeloplasty in the remainder. No nephrectomy was necessary. Anteriorly elongated flank incisions were made in all cases. Dense scar tissue around and obstructing the ureteropelvic junction was noted in the majority of cases. In 7 patients a redundant pelvis resulted in a kink at the ureteropelvic junction. A nephrostomy tube was placed in all cases and an additional transanastomotic stent was used in all but 2. Obstruction was relieved with 1 operation., Conclusions: A redundant pelvis resulting in a kink at the ureteropelvic junction may contribute to a higher change of urinary leakage and subsequent obstructive scar formation in cases of failed pyeloplasty. Before repeat surgery anatomy should be precisely identified by antegrade and retrograde studies. The surgical approach usually involves identifying the ureter below the area of the previous surgery and then ensuring a tension-free anastomosis. If inadequate ureteral length or an intrarenal pelvis precludes direct anastomosis, ureterocalicostomy is an alternative. A nephrostomy tube and transanastomotic stent are advisable. Nephrectomy is rarely necessary and a good functional result can be anticipated.

Purpose: We developed and tested an animal model of bladder dysfunction due to posterior urethral valves using partial outlet obstruction of the fetal rabbit bladder., Materials and Methods: Partial bladder outlet obstruction of fetal rabbit bladders was created on day 23 of gestation. Of the litter of 8 to 10 fetuses half was obstructed and the remainder served as controls. The doe and fetuses were sacrificed on day 30 of gestation (full term 31 to 32 days) and the fetal bladders were removed. Bladders that had doubled in weight from the average bladder weight of the control littermates were deemed sufficiently obstructed. Hematoxylin and eosin staining was performed and bladder strip response to 32 Hz. field stimulation, 200 microM. bethanechol and 200 mM. potassium chloride was measured., Results: Average body weight did not differ between the control and obstructed fetuses, indicating that surgery did not hinder fetal development. Hematoxylin and eosin staining confirmed smooth muscle cell hypertrophy and increased connective tissue in the obstructed bladders. Obstructed bladder strips responded significantly less to field stimulation, and significantly more to bethanechol and potassium chloride (mean plus or minus standard deviation 5.18 +/- 1.52, 6.29 +/- 1.3 and 10.15 +/- 2.18 x force per/100 mg. tissue, respectively)than control bladder strips (9.0 +/- 1.19, 3.5 +/- 0.46 and 6.16 +/- 1.33 x force per/100 mg. tissue, respectively) suggesting that denervation supersensitivity may have resulted from obstruction., Conclusions: Partial outlet obstruction of the fetal rabbit bladder results in bladder hypertrophy and dysfunction but these changes are markedly different from those in the adult rabbit. Since rabbit fetal development is delayed compared to human fetal development, this model can be used to assess the consequences of posterior urethral valves.

We evaluated compliance in the developing bladder using a newly developed animal model of posterior urethral valves: partial infravesical obstruction in the fetal rabbit bladder. Partial bladder outlet obstruction was created in fetal rabbits at day 23 of a 31 to 32-day gestation period. An in vitro whole bladder preparation provided data on compliance and an isolated bladder strip preparation provided data on the mechanical properties of the bladder wall. In addition, the influence of calcium on both preparations was evaluated. Partial bladder outlet obstruction in the fetal rabbit resulted in a markedly larger bladder weight (246.4 +/- 22.3 mg, n = 14) than control bladders (90.2 +/- 5.7 mg, n = 13). Isolated smooth muscle strips from obstructed and normal bladders revealed identical stretch-stress patterns. In contrast, obstructed bladders had significantly increased compliance in the whole bladder preparation. Since the increase in compliance was not correlated to mechanical properties of the isolated bladder strips, it must therefore result from the pattern of mass increase of the whole bladder wall. During filling, both the control and obstructed bladders had the same slow, large amplitude spontaneous contractions. In addition, both had rapid contractions: those in the obstructed bladders had significantly lower frequency and higher amplitude than the ones in the control bladders. Removing the calcium from the organ bath eliminated the spontaneous contractions but did not change the baseline pressure or force values, indicating that the compliance of these fetal rabbit bladders is a function of the passive properties of the bladder wall. Three main patterns occur in cystometrograms of patients with posterior urethral valves: myogenic failure, hyperreflexic bladders, and low compliance bladders. Using our model of partial outlet obstruction in the fetal rabbit bladder, we could not imitate the group with low compliance. We therefore hypothesize that the different patterns of bladder dysfunction associated with posterior urethral valves are due to infravesical obstruction occurring with different severities or at different ages of gestation.

Purpose: We evaluated long-term results of patients with bladder exstrophy who underwent ureterosigmoidostomy., Materials and Methods: Of 4 women and 23 men monitored at our institution 16 (59%) underwent primary diversion by ureterosigmoidostomy, while 11 (41%) underwent primary bladder closure or an ileal conduit procedure before conversion to ureterosigmoidostomy. Average followup after ureterosigmoidostomy was 17 years., Results: Significant upper urinary tract changes developed in 18% of the patients. Metabolic acidosis was well compensated in most patients but 2 had problems with urinary retention leading to hyperammonemia and acidosis. Of the 19 patients monitored with biennial colonoscopy benign polyps were removed in 4. Daytime continence was achieved in 92% of cases and nighttime continence in 58%., Conclusions: Our experience with ureterosigmoidostomy in children with bladder exstrophy has been favorable through long-term followup. With proper imaging, metabolic surveillance, biennial colonoscopy and nonsteroidal anti-inflammatory drugs we offer ureterosigmoidostomy as a viable alternative for patients with small bladders.

Purpose: We evaluated the long-term outcome of social integration and sexual function in 29 men with bladder exstrophy and epispadias., Materials and Methods: Four men with epispadias and 25 with bladder exstrophy were available for long-term followup. Patients were interviewed by telephone regarding sexual history and social integration. Semen analyses were obtained in 8 cases., Results: Patient assessment of genital appearance was good or fair in 71%. Potency was present in all patients, and erections were straight in 66% and curved in 34% with no curvature so severe as to prevent sexual intercourse. Semen analysis showed a normal sperm count in 63% of the men and no azoospermia. Social integration was satisfactory: 100% of the men attended high school, 55% have a college education and all who are not attending school have full-time jobs., Conclusions: Our long-term review demonstrates that despite what appears to be a significant sexual handicap, patients with exstrophy/epispadias can have adequate sexual function and overall successful social integration.

The sexual differentiation of a gonad is determined in normal embryology by the presence or absence of the TDF gene, a short segment of DNA localized near the tip of the short arm of the Y chromosome. Under the stimulus of this gene, the somatic cells of the genital ridge differentiate into Sertoli cells and secrete anti-müllerian hormone. This hormone inhibits the germ cells from entering meiosis and may well also trigger the formation of the primary sex cords. Once the Sertoli cells are formed, further differentiation of the gonad is independent of the TDF presence, although this gene may further contribute to the normal functioning of the male gonad. In true hermaphroditism the normal regulation of the gonadal differentiation is missing. Gonadal differentiation may revert to a more primitive evolutionary level at which the rate of growth of the gonad determines its sexual direction. When this exceeds a certain threshold a testis may develop despite the absence of the TDF gene.

Megalourethra is a rare congenital disorder involving the formation of the anterior urethra. Both clinical varieties, the scaphoid and fusiform types, involve extensive defects in the formation of the spongiosum urethra. Our experience with 5 patients is discussed with particular reference to associated anomalies, diagnostic procedures, timing of surgical intervention, and definitive reconstructive surgery.

The best management of fetal hydronephrosis is controversial. Despite the lack of experimental evidence that prenatal drainage of the obstructed urinary tract substantially improves ultimate renal function, various forms of percutaneous intervention on the fetal bladder and kidney have been used. To evaluate the efficacy of intervention for suspected fetal obstructive uropathy, all published reports of drainage of the fetal urinary tract up to December, 1985, were reviewed. In the 57 reported cases, the most common type of intervention was placement of a vesicoamniotic shunt (37%). Complications occurred in 25 cases (44%), including inadequate shunt drainage or migration (19%), onset of premature labour within 48 h (12%), urinary ascites (7%), and chorioamnionitis (5%). Of 28 fetuses with associated oligohydramnios, only 6 (21%) survived. 2 of these survivors had vesicoamniotic shunts, 2 single or multiple bladder aspirations, 1 an external renal drainage catheter, and 1 in-utero vesicostomy. Because of the high complication rate and lack of evidence of improved survival from in-utero drainage procedures, a prospective, randomised trial is needed to compare survival with and without vesicoamniotic shunt placement.

Simple renal cysts and reported in 9 children. Two children presented with hematuria following trauma and the cyst was demonstrated on the initial excretory urogram. The other 7 had cysts found on excretory urograms in be course of evaluation of various urologic complaints. The diagnostic approach used was identical to that advocated for adults, namely, percutaneous needle puncture once a solid renal mass has been excluded by an appropriate screening procedure such as ultrasonography. These cysts are benign lesions similar to cysts in adults and are probably more common than the few reported cases would suggest.

Infants with posterior urethral valves and persistent unilateral reflux after valve resection often have an associated nonfunctioning, dysplastic kidney. Spurious function of the unit with reflux may be apparent on the delayed films of the excretory urogram. Misinterpretation of the initial radiographic studies performed without coincident bladder drainage results in the missed diagnosis of this syndrome and misguided surgical management. Of 82 patients with urethral valves 17 (21 per cent) had persistent unilateral reflux. A renal scan was useful in determining nonfunction in 11 (65 per cent) of those patients with persistent unilateral reflux and 1 with bilateral reflux. The functionless kidney was on the left side in 11 of 12 cases (92 per cent). Early nephroureterectomy is advocated to improve voiding dynamics. The prognosis is generally excellent. The embryogenesis of the syndrome is discussed as it relates to the "bud theory" of renal dysplasia and posterior urethral valves.

We surveyed 321 patients 1 to 18 years old who were followed at the sickle cell clinic at the Children's Hospital of Philadelphia between 1970 and 1984 for urological complications of the disease. Mean followup was 5 years and all patients exhibited a typical spectrum of hemoglobin types. The urological problems encountered were those cited in the literature, namely hematuria, urinary tract infection and priapism. Surprisingly few of our patients experienced significant renal bleeding. Although the number of patients with infection evaluated radiographically was small, the frequency of renal parenchymal scarring was disturbingly high despite the reported rarity of reflux in black subjects. Our survey and a review of the literature indicate that most sicklemic children with urinary infection are not subjected to urological evaluation. We question the wisdom of that policy. Finally, we found that priapism responds most often to nonsurgical therapy and that it rarely results in impotence in young sickle cell patients.

The seventh case of a female subject with a single, unilateral ectopic ureter draining to the vagina is reported. The renal unit was located in the renal fossa, wheras in 3 of the 7 cases reported it was within the pelvis. Although ectopia with ureteral duplication is not an uncommon anomaly the single ectopic ureter in the female subject is the rarest form of ureteral ectopia. Use of phenazopyridine hydrochloric acid as an excertory marker was most helpful in locating the vaginal orifice.

The association of nodular renal blastema, renal dysplasia and duplicated collecting systems may represent more than a histopathological observation. Based on the "double hit" theory of oncogenesis, the appearance of this histologic pattern in kidneys with other developmental alterations may account for an increased risk of neoplasia.

The glans channel technique combined with the use of a transverse preputial island flap to create a neourethra is outlined for one-stage repair of severe hypospadias.

Of the orchiopexies performed for abdominal undescended testicles 85 per cent have resulted in satisfactory testicular growth and position. Three-fourths of these cases were in boys with the prune belly syndrome. We are satisfied with our recent experience using the Fowler-Stephens procedure to salvage intra-abdominal undescended testicles. An analysis of the failures in 5 testes early in this series, resulting in partial atrophy, has helped us to avoid technical errors through attention to the following details: 1) preservation of a broad pedicle of peritoneum overlying the mobilized vas, 2) avoidance of any dissection of the spermatic cord and 3) high ligation of the spermatic vessels well above the point of confluence of the vas and spermatic vessels. Although the Fowler-Stephens procedure has some risk of testicular ischemia and atrophy we believe it to be the preferred way to gain mobility on a short tethering gonadal vascular pedicle.

Complications of hypospadias repair result from errors in the initial evaluation of a patient, the design of the operation, the surgical technique, or the postoperative care. The most commonly encountered complications are discussed, with emphasis both on methods of prevention and of treatment.

A hormone (renotropin) is said to play an important role in compensatory renal growth. The role of renotropin in obligatory growth (normal developmental growth) is unknown. It was observed that contralateral renal size in children with unilateral multicystic kidney was found to be normal at birth but became hypertrophied later. The kidneys of twenty-one-day-old fetal rats were significantly smaller than sham controls in animals whose mothers had uninephrectomies at day 8 (26.5 +/- 1.1 mg. versus 18.5 +/- 1.1 mg.). The mothers' kidneys hypertrophied compared with sham controls (1,065 +/- 23 mg. versus 1,347 +/- 78 mg.). It was concluded that renotropin passes the placenta and modifies so-called obligatory renal growth.

For years the management of blunt and penetrating renal trauma has been the source of controversy in the literature. The indications for a specific therapeutic program vary with each individual case and with the complicating factors involved. An unusual form of penetrating renal trauma in the neonate is presented and the rationale for therapy is discussed.

We treated seven girls with a cloacal anomaly through a posterior sagittal exposure. Six patients, aged 5 months to 2 1/2 years, underwent a primary reconstructive operation with urethroplasty, vaginoplasty, and posterior sagittal anorectoplasty as a single procedure. All had undergone diverting sigmoid colostomies as newborns, four had previous vesicostomies, and one had a vaginostomy. The seventh girl, a 9-year-old, had undergone a number of attempts at reconstruction. All underwent endoscopy and contrast studies of the cloaca before definitive reconstruction. This preoperative evaluation gave necessary information regarding the length and caliber of the cloacal channel and the level of confluence of the urinary, genital, and intestinal tracts. However, major decisions regarding reconstruction were often possible only in the operating room. To construct a neoanus with optimal sphincteric control, all seven underwent posterior sagittal anorectoplasty. Two required laparotomies to mobilize additional colonic length. Three developed urethrovaginal fistulae after repair. Critical decisions regarding urethral and vaginal reconstruction depend on the findings at cystoscopy and operation. The posterior sagittal approach gives excellent exposure for these decisions and the subsequent reconstruction of three functioning perineal orifices.

Two infants with ectopia of the vas deferens are described. To date 11 cases have been reported, comprising a total of 13 ectopic vas insertions, 2 of which are bilateral. A classification of 2 types of ectopic vas is presented with a discussion of their relationship to ureteral ectopias. An embryological hypothesis of a proximal vas precursor segment of the wolffian duct is related closely to Stephens' theory of ureteral development.

We report our experience with 22 Scott genitourinary sphincters implanted in 19 patients during a period of 4 years. Patient selection was rigid. Incontinence must be unremitting and fail to respond to exhaustive efforts at control by pharmacologic manipulation with or without intermittent catheterization. The patient must be able to empty the bladder by Credé's or Valsalva's maneuver, and consider the incontinence intolerable. The artificial sphincter is considered as an alternative to urinary diversion. A second attempt at placement has been done in 3 patients. Removal was required in 32 per cent of the devices. However, 79 per cent of the patients did achieve our goal of continence. Therefore, we have found the artificial sphincter to be a valuable addition to the treatment of pediatric incontinence as an alternative to urinary diversion.

We describe the successful use of an anterolateral, muscle-splitting technique. In the pediatric patient impressive exposure allows for renal pelvic and distal ureteral operations, obviating the necessity for a secondary lower incision. Additional benefits of this incision have been minimal nerve root irritation, incisional pain and muscular weakness.

A functional approach has been used in the management of the pediatric neuropathic bladder. Herein we report on our experience with incontinent children using pharmacologic manipulation with anticholinergic and sympathomimetic agents. Neuropharmacology and neuroanatomy as they apply to this approach are reviewed.