Use of the mobilized sinus with total urogenital mobilization.

PURPOSE: The surgical treatment of urogenital sinus anomalies has undergone significant advances in recent years. Total urogenital mobilization, which mobilizes the urogenital sinus, vagina and urethra en bloc toward the perineum, represents one of these advances. MATERIALS AND METHODS: We have improved our results with total urogenital mobilization by incorporating the mobilized urogenital sinus tissue into the repair rather than discarding it, as described originally. We have found this a readily available, easily manipulated and well vascularized flap that is a significant aid to reconstruction. RESULTS: We present our 3 favored means of using the mobilized sinus tissue to create a mucosa lined vestibule, a posterior vaginal wall flap and an anterior vaginal wall flap. CONCLUSIONS: We believe that our techniques result in a further advancement in the cosmetic and surgical outcomes in these patients, and are beneficial in the reconstructive surgery armamentarium.

Total continence reconstruction: a comparison to staged reconstruction of neuropathic bowel and bladder.

PURPOSE: Surgical treatment for neuropathic bowel and bladder has become an essential tool in maximizing the quality of life in patients with myelomeningocele. We present our results comparing results in patients who underwent total continence reconstruction of the urinary and gastrointestinal tracts to patients who underwent a separate or single operation. MATERIALS AND METHODS: We performed a retrospective chart review of all patients with myelomeningocele at our institution who underwent reconstruction with a cutaneous catheterizable urinary channel or Malone antegrade continence enema. We compared outcomes with regard to surgical revisions of the channel between patients who underwent the construction of each simultaneously, that is total continence reconstruction, to outcomes in those with a single channel or who underwent reconstruction at 2 or more operations. RESULTS: Most of our patients underwent genitourinary and gastrointestinal reconstruction, and few desired surgical intervention for only a single system. We were unable to find any differences in the continence rate or stomal complications. However, patients who underwent staged reconstruction usually had significant secondary reasons for repeat surgery. CONCLUSIONS: Surgical success for urinary and fecal continence can be safely and effectively achieved through single or multiple procedures. However, because of shared pathophysiology, we believe that most patients benefit from intervention in the gastrointestinal and the genitourinary tract. Therefore, a major advantage of total continence reconstruction is avoidance of the morbidity of a second major surgical procedure.

What is the need for additional bladder surgery after bladder augmentation in childhood?

PURPOSE: Bladder augmentation has revolutionized the care of children with a neuropathic bladder but it remains a major surgical procedure. However, the need for subsequent bladder surgery has not been well defined in a large series with long-term followup. MATERIALS AND METHODS: We retrospectively reviewed the records of the first 500 bladder augmentations performed from 1978 to 2003 at our institution. Charts were reviewed for complications requiring additional surgery, including malignancy, bladder perforation, repeat augmentation, bowel obstruction and bladder calculi. Mean and median followup was 13.3 years. RESULTS: Complications occurred in 169 patients (34%) resulting in a total of 254 surgeries. The cumulative risk of further surgery at the bladder level was 0.04 operations per patient per year of augmentation. Three patients (0.6%) had transitional cell carcinoma, of whom all presented with metastatic disease and died. Bladder perforation occurred in 43 patients (8.6%) with a total of 53 events. Of the patients 16 (3.2%) required laparotomy for bowel obstruction and 47 (9.4%) required repeat augmentation. Bladder stones were treated in 75 patients (15%), who required a total of 125 surgeries. CONCLUSIONS: Bladder augmentation provides immeasurable improvements in quality of life but it requires lifelong dedication from the patient, family and health care providers. While the requirements for additional surgery are not trivial, 66% of our patients have not required any further surgery in the augmented bladder.

Treatment of the occult tethered spinal cord for neuropathic bladder: results of sectioning the filum terminale.

PURPOSE: Occult tethered cord syndrome applies to patients with signs and symptoms consistent with a caudal spinal cord malformation despite normal neuroimaging. Although several reports of successful surgical treatment exist, controversy remains with respect to patient selection and efficacy. We present a large series with excellent clinical followup, neuroimaging and urodynamic characterization. MATERIALS AND METHODS: We present our experience with 36 patients at a single institution with preoperative clinical findings, neuroimaging and urodynamics available. Postoperative outcomes were assessed clinically and with urodynamics. We determined predictive parameters to improve patient selection. RESULTS: Approximately 0.04% of pediatric urology clinic visits resulted in neurosurgical referral for the potential of an occult tethered cord. They occurred after failure of a mean of 2 years of aggressive medical management. Daytime urinary incontinence was present in 83% of patients and 47% had encopresis. Preoperative urodynamics were markedly abnormal in all patients with mean bladder capacity 55% of expected capacity. Clinical improvement in urinary symptoms was seen in 72% of patients with resolution of incontinence in 42%. Bowel symptoms improved in 88% of cases, including resolution of encopresis in 53% within 3 months of surgery. Urodynamic improvements were demonstrated in 57% of cases. We were unable to determine preoperative factors that were more likely associated with surgical success. CONCLUSIONS: In a highly select population with severe urinary and fecal dysfunction sectioning a normal-appearing filum terminale can result in significant improvement. We were unable to identify factors that may increase the chance of surgical success.

Spontaneous bladder perforations: a report of 500 augmentations in children and analysis of risk.

PURPOSE: The spontaneous perforation of an augmented bladder is an uncommon but serious complication. To our knowledge our institution has the largest reported series of bladder augmentations. We examined our data to determine the incidence of spontaneous bladder perforation and to delineate associated risk factors. MATERIALS AND METHODS: We performed a retrospective chart review of 500 bladder augmentation procedures performed during the preceding 25 years with a minimum followup of 2 years. RESULTS: Spontaneous perforations occurred in 43 patients (8.6%), for a total of 54 events. The calculated risk was 0.0066 perforations per augmentation-year at risk. Approximately a third of the cases had perforated within 2 years of surgery, a third between 2 and 6 years postoperatively, and a third at more than 6 years after augmentation. Patients who underwent augmentation between 1997 and 2003 had a higher rate of perforation within 2 years of surgery than those operated on between 1978 and 1987. Increased risk of perforation was observed with the use of sigmoid colon and bladder neck surgery. A decreased risk was associated with the presence of a continent catheterizable channel. CONCLUSIONS: We believe that this large and comprehensive series gives valuable insight into this serious complication. The delineation of these potential risk factors serves as a guide for further discussion and investigation.

Partial urogenital mobilization: a limited proximal dissection.

INTRODUCTION: The treatment of urogenital sinus malformations is complex and controversial. Despite numerous and significant contemporary surgical advances, the dissection of the urogenital sinus remains technically challenging. METHODS: Based on total urogenital mobilization, we describe a technique whereby this dissection is limited to the pubourethral ligament. Our short-term results with partial urogenital mobilization (PUM) performed on 15 patients are retrospectively reviewed. RESULTS: There were no intraoperative complications and the short-term cosmetic results are excellent. No patients have developed voiding dysfunction or urinary tract complications. CONCLUSIONS: While total urogenital mobilization is a very effective procedure, we believe that the PUM approach limits potential morbidity in the reconstruction of these complex problems.

Experience with non-cycled artificial urinary sphincters.

OBJECTIVE: To report our experience in children and adolescents with a non-cycled artificial urinary sphincter. While some children with the AUS can void, others require clean intermittent catheterization (CIC) through the sphincter or an alternative site for catheterization; in some of the latter we have either not cycled (pumped) an activated AUS or the AUS has failed, and there is concern about ischaemia in some adults with a non-cycled AUS. PATIENTS AND METHODS: In all, 143 patients who had an AUS placed between 1980 and 2002 were reviewed retrospectively; 15 (10 boys and five girls) no longer cycled (pumped) their AUS. The mean age at AUS insertion was 11 years and the mean (range) follow-up after insertion was 10.4 (1.64-22.2) years. The diagnoses included myelomeningocele in 11, sacral agenesis in three and cloaca in one. Nine patients have an activated functioning AUS and in six the AUS does not function; in the first nine the sphincter has not routinely been cycled (pumped) for a mean (range) of 1.6 (0.6-2.9) years. In the other six with a nonfunctioning AUS the mean (range) observation period is 6.4 (1.5-10) years since the system has not functioned. RESULTS: All patients were completely continent, including the six with a nonfunctioning AUS. After inserting the AUS, two patients voided in combination with CIC (one each urethral and abdominal stoma) and 13 emptied by CIC (nine abdominal stoma, four urethral). There was a mechanical complication in eight patients; three had the AUS repaired and are dry, and five are dry with no repair. In one patient the AUS was never activated. To date there has been no erosion of the cuff in any of the 15 patients with a non-cycled AUS. CONCLUSION: The AUS remains an extremely reliable procedure to achieve continence in children and young adults. It is versatile and can be combined with other procedures that provide an alternative means for catheterization. While some have noted the need to routinely cycle the AUS to prevent erosion, this has not been our experience in these 15 patients.

Tap water and the Malone antegrade continence enema: a safe combination?

PURPOSE: The Malone antegrade continence enema provides independence and improved quality of life in patients with fecal incontinence or intractable constipation. However, isolated reports of fatal hypernatremia after irrigation with normal saline have raised safety concerns about frequent colonic irrigation in children. Significant electrolyte abnormalities have also been reported with hypertonic phosphate and high colonic tap water enemas. Because our patients routinely use tap water for Malone antegrade continence enema irrigations, we examined the safety profile of this practice MATERIALS AND METHODS: In the last 3.5 years 71 patients at our institution have used antegrade tap water enemas for managing fecal incontinence or intractable constipation. Standard serum electrolytes were measured RESULTS: We obtained 101 sets of serum electrolyte measurements in 71 patients at a mean of 8.4 months postoperatively (range 1 to 33). A girl who presented with severe hyponatremia and hypochloremia had not used the Malone antegrade continence enema for several days. The most interesting finding was significantly elevated sodium and chloride in 1 case 6 weeks after surgery that was associated with tap water treated with a home softening system. Electrolytes reverted to normal 1 week after using untreated tap water CONCLUSIONS: We did not detect significant hyponatremia or hypochloremia in any patient using tap water for Malone antegrade continence enema irrigation. Although dangerous electrolyte abnormalities are rare, potential morbidity in those cases warrants periodic evaluation. Due to the elevated sodium content in softened tap water families should be alerted to use untreated tap water for preparing enemas.

p38 MAPK mediates renal tubular cell TNF-alpha production and TNF-alpha-dependent apoptosis during simulated ischemia.

Ischemia causes renal tubular cell loss through apoptosis; however, the mechanisms of this process remain unclear. Using the renal tubular epithelial cell line LLC-PK(1), we developed a model of simulated ischemia (SI) to investigate the role of p38 MAPK (mitogen-activated protein kinase) in renal cell tumor necrosis factor-alpha (TNF-alpha) mRNA production, protein bioactivity, and apoptosis. Results demonstrate that 60 min of SI induced maximal TNF-alpha mRNA production and bioactivity. Furthermore, 60 min of ischemia induced renal tubular cell apoptosis at all substrate replacement time points examined, with peak apoptotic cell death occurring after either 24 or 48 h. p38 MAPK inhibition abolished TNF-alpha mRNA production and TNF-alpha bioactivity, and both p38 MAPK inhibition and TNF-alpha neutralization (anti-porcine TNF-alpha antibody) prevented apoptosis after 60 min of SI. These results constitute the initial demonstration that 1) renal tubular cells produce TNF-alpha mRNA and biologically active TNF-alpha and undergo apoptosis in response to SI, and 2) p38 MAPK mediates renal tubular cell TNF-alpha production and TNF-alpha-dependent apoptosis after SI.

Shunt infection and malfunction after augmentation cystoplasty.

PURPOSE: Maintenance of a sterile intraperitoneal environment is critical in patients with ventriculoperitoneal shunts. Recent series have reported a broad discrepancy in the rate of shunt infection (0% to 20%) following augmentation cystoplasty. The need for distal shunt revision has not been well defined. We report the incidence of shunt infection and revision at our institution after bladder augmentation. MATERIALS AND METHODS: We retrospectively reviewed the records of all patients with myelodysplasia and a ventriculoperitoneal shunt who underwent augmentation cystoplasty since August 1990. All patients included in the study had a minimum of 12 months of followup. RESULTS: A total of 55 patients with a ventriculoperitoneal shunt secondary to myelodysplasia required augmentation cystoplasty for management of a neuropathic bladder. Standard perioperative intravenous and oral antibiotic preparation, mechanical bowel preparation and intraoperative shunt isolation were used. Mean postoperative followup was 60.4 months (range 12 to 111). One patient presented with an extruded peritoneal shunt tip and positive cultures from cerebrospinal fluid and urine. Bladder perforation occurred in 2 patients and the shunt was empirically externalized. Revision was required for 5 (9%) distal shunt obstructions, including 1 cerebrospinal fluid pseudocyst. CONCLUSIONS: The incidence of shunt infection after augmentation cystoplasty is low (less than 2% in this large series), and presence of a ventriculoperitoneal shunt should not preclude bladder augmentation. Meticulous perioperative and intraoperative preparation contributes to the low rate of adverse events. Although the rate of distal revision after augmentation is significant, it does not exceed the reported distal failure rate for ventriculoperitoneal shunts in children without a history of urological surgery.

Symptomatic ureteropelvic junction obstruction in children in the era of prenatal sonography-is there a higher incidence of crossing vessels?

OBJECTIVES: To determine whether prenatal sonography and early detection and correction of ureteropelvic junction obstruction (UPJO) has changed the incidence of crossing vessels as the etiology of obstruction in older children presenting with symptomatic UPJO. METHODS: We reviewed the medical records of all children and adolescents who underwent pyeloplasty for symptomatic UPJO between 1986 and 1999, during the era of widespread use of prenatal sonography. Operative notes were used to determine which patients had obstruction due to lower pole crossing vessels. RESULTS: Thirty-eight patients were identified who underwent pyeloplasty for symptomatic UPJO. Lower pole vessels were identified in 22 (58%) of 38 patients (P <0.0001 compared with historical controls). All patients underwent dismembered pyeloplasty and remained asymptomatic after surgery, with renal scans demonstrating excellent drainage and preservation of function. CONCLUSIONS: Prenatal ultrasonography has increased the incidence of crossing vessels as the etiology of UPJO in young children and adolescents presenting with symptomatic UPJO compared with the historical incidence of 11% to 15%. This finding may impact treatment recommendations with respect to endourologic management of the obstruction in this patient population. Currently, we recommend open dismembered pyeloplasty for young children and adolescents presenting with symptomatic UPJO.

Abdominal wall hernia: an uncommon complication of in utero vesicoamniotic shunt placement.

Three cases are reported in which placement of a vesicoamniotic shunt in utero for the treatment of obstructive uropathy led to the rare complication of abdominal wall hernia. All 3 patients underwent vesicoamniotic shunt placement for severe oligohydramnios and a markedly dilated bladder in an effort to preserve renal function and to prevent pulmonary hypoplasia. All three shunts were initially placed at or above the umbilicus. The abdominal wall hernias were closed postnatally at the time of temporary vesicostomy for urethral obstruction. All 3 patients had sufficient pulmonary development, but 2 of 3 had renal failure, requiring dialysis. Management and potential etiology of this rare complication are discussed.

A long continent ileovesicostomy using a single piece of bowel.

PURPOSE: In 1981 Mitrofanoff presented a procedure to create a continent urinary stoma for intermittent catheterization. Since then, several other methods have been introduced, including the Yang-Monti ileovesicostomy. The length of these ileovesicostomies is limited by the circumference of the bowel segment used, which is inadequate in some cases. We developed a procedure to double the length of the Yang-Monti ileovesicostomy using a single section of bowel. MATERIALS AND METHODS: A 3.5 cm. section of ileum is isolated on its mesentery. The bowel is divided into 2 segments for 80% of its circumference, leaving the bowel intact over the mesentery. Each ring of bowel is then divided adjacent to the mesentery but on opposite sides, allowing the bowel to be unfolded and reconfigured in a single long strip that may then be tubularized. The blood supply to the tube is excellent and it is in the center of the reconfigured ileum. The ends may be trimmed or widely spatulated as necessary. RESULTS: We have performed this procedure in 8 patients. The resulting ileovesicostomy created from a 3.5 cm. section ofileum is 10 to 14 cm. long and accepts a 12F catheter. A larger tube may be created from a longer piece of ileum. All patients are dry and they perform catheterization easily. CONCLUSIONS: This form of ileovesicostomy allows the creation of a long bowel tube that is easily catheterized. The longer length of the tube increases application of the continent stoma principle to more patients and enables reconstruction to be performed with optimal placement and without tension.

Appendicovesicostomy and newer alternatives for the Mitrofanoff procedure: results in the last 100 patients at Riley Children's Hospital.

PURPOSE: We present our experience using the various Mitrofanoff techniques to create a continent catheterizable stoma as an adjunct to continent urinary tract reconstruction in children and young adults. MATERIALS AND METHODS: Between 1990 and 1998 a Mitrofanoff procedure was performed at our institution in 55 male and 45 female patients with a mean age of 10.5 years. The etiology of incontinence was diverse but more than 90% of the patients had neurogenic bladder, the epispadias-exstrophy complex or a cloacal anomaly. Surgery included appendicovesicostomy in 57 cases, a Yang-Monti ileovesicostomy in 21, continent vesicostomy in 21 and formation of a tapered ileal segment as a catheterizable channel in 1. Simultaneously bladder augmentation was performed in 52 patients, bladder neck reconstruction was done in 48 and a Malone antegrade colonic enema stoma was constructed for fecal incontinence in 17. RESULTS: The abdominal stoma is continent in 98 of our 100 patients. Mean followup is 2 years (range 2 months to 8 years) with the longer followup in the appendicovesicostomy group. One patient with stomal incontinence who underwent revision is now dry. Postoperative complications requiring an additional procedure developed in 20 patients, including stomal stenosis in 12. Continent vesicostomy was most prone to stomal problems (6 of 21 patients, 29%). CONCLUSIONS: The Mitrofanoff procedure is a reliable technique for creating a continent catheterizable urinary stoma. Appendicovesicostomy continues to be our first option for this procedure, although we have also had good results with the Yang-Monti ileovesicostomy and continent vesicostomy. These newer options have allowed preservation of the appendix for the Malone antegrade colonic enema stoma procedure in patients with urinary and fecal incontinence.

Repair of hypospadias complications using the previously utilized urethral plate.

OBJECTIVES: To review our results of patients who underwent repeat hypospadias surgery using local skin flaps with preservation of the urethral plate. METHODS: We retrospectively reviewed the medical records of all patients who underwent a reoperative hypospadias repair using the urethral plate between 1988 and 1996. A total of 53 patients were identified who developed either a large fistula (47 patients) or severe stricture (6 patients) after the initial repair. Seventeen patients underwent a repeat Mathieu flip-flap and 36 an onlay flap. The mean age at the time of surgery was 5.2 years (range 1 to 27). The mean duration of follow-up was 17 months (range 6 to 108). RESULTS: The initial failed hypospadias repair was corrected with a single procedure in 44 (83%) of the 53 patients. The 9 patients who had further complications required 17 additional procedures. A meatal-based flap had been used in 4 of these patients and an onlay flap in 5. Three of the 9 patients who had further complications presented with urethral strictures after the previous repair. CONCLUSIONS: Fistula and stricture are common complications of hypospadias surgery. The use of the urethral plate in primary repairs has helped decrease the complication rate. We report the effectiveness of using local skin flaps and preserving the urethral plate in complex reoperative hypospadias surgery. These techniques were successful in 83% of these challenging patients. Patients with stricture after hypospadias surgery are at increased risk of further complications.

alpha-Adrenergic blockade in children with neuropathic and nonneuropathic voiding dysfunction.

PURPOSE: Inadequate bladder emptying is a common urinary dysfunction in children. The role of alpha-blockers for managing bladder outlet obstruction remains relatively unexplored in children. Because of the well established impact of alpha-blocker therapy in men, we investigated its use for treating inadequate bladder emptying in the pediatric population. MATERIALS AND METHODS: We treated 17 children 3 to 15 years old with documented poor bladder emptying of various etiologies, including dysfunctional voiding, the Hinman syndrome, the lazy bladder syndrome, posterior urethral valves, myelomeningocele and the prune-belly syndrome, using the alpha-1 adrenergic receptor antagonist, doxazosin. The initial dose of 0.5 to 1.0 mg. nightly was increased according to patient response and as tolerated. Patients were followed weekly to monthly by symptomatic history, and urine flow and/or post-void residual urine volume measurement. Two patients with neurogenic bladder were also followed with cystometrography and leak point pressure determination. RESULTS: Bladder symptomatology and/or emptying improved in 14 patients (82%). Ten patients had decreased post-void residual urine during treatment and in 3 uroflowmetry showed increased maximum flow. Two patients with neuropathic bladder secondary to myelomeningocele had decreased leak point pressure on alpha-blocker therapy and in 2 with a history of posterior urethral valves new onset bilateral hydronephrosis completely resolved. Only 1 patient had mild postural hypotension, which resolved with dose reduction. CONCLUSIONS: Selective alpha-blocker therapy seems to be well tolerated in children and appears effective for improving bladder emptying in various pediatric voiding disorders at short-term followup. Long-term followup and further investigation are warranted to validate the potential role of alpha-blocker therapy in pediatric urinary dysfunction.

Concealed penis in childhood: a spectrum of etiology and treatment.

PURPOSE: Concealed penis is an uncommon condition due to poor skin fixation at the base of the penis, cicatricial scarring after penile surgery and excessive obesity. The condition varies in severity and several surgical options are available, such as excision of previous scarring, degloving the penile shaft, reconstructing the penile shaft skin with flaps, fixing the penile skin at the penopubic and penoscrotal angles, and removing excess suprapubic fat. MATERIALS AND METHODS: We reviewed the records of 43 patients treated for concealed penis from 1993 to 1998. We categorized the cases as type 1-congenital concealed penis, type 2-concealed penis due to scarring from previous surgery and type 3-complex cases involving excessive obesity. Cases were reviewed in regard to surgical techniques and outcomes. We identified 18 type 1, 18 type 2 and 7 type 3 cases. Mean age of type 1 patients at surgery was 12.4 months with 1 patient presented at age 7 years. None had previously undergone penile surgery. All patients underwent complete penile degloving. To reconstruct the penile shaft flaps or Z-plasties with penile skin were used in 12 patients and scrotal skin flaps were used in 2. In 12 patients the penile skin was fixed at the penoscrotal and penopubic angles to maintain penile length and in 2 excess fat was excised. Mean age of type 2 patients at surgery was 19.8 months. All had previously undergone surgery, including hypospadias in 1 and circumcision in 17. All patients underwent complete penile degloving and the cicatricial scar that trapped the penis was excised. Penile skin flaps and Z-plasties were used in 12 cases, scrotal skin flaps were used for reconstruction in 2 and skin grafting was done in 1. In 10 patients the penile skin was fixed with sutures to maintain penile length. Mean age of type 3 patients at surgery was 15.8 years. Of the 7 boys 6 had previously undergone penile surgery. All required extensive scar excision and complex reconstruction involving penile skin flaps in 3, scrotal flaps in 5 and penile skin fixation in 6. Excessive suprapubic fat was removed in 5 patients, of whom 3 underwent liposuction. RESULTS: Surgical results were uniformly good in type 1 patients except in 1 who was believed to have excessive suprapubic fat. Results were good in 14 of the 18 type 2 patients, although 2 retained excessive suprapubic fat and 2 had some unsightly scarring. No type 1 or 2 patient required additional surgery. Of the 7 type 3 patients 6 had a good result and required no additional surgery. One patient has recurrent concealed penis after 2 procedures and awaits additional surgery. CONCLUSIONS: Concealed penis has a varied etiology and requires a flexible surgical approach. The common surgical options in all cases include complete penile degloving, excising the scarring due to previous surgery, removing excess suprapubic fat, reconstructing the penile skin with local flaps, and fixing the penile skin at the penopubic and penoscrotal angles.

The Young-Dees-Leadbetter bladder neck repair for neurogenic incontinence.

PURPOSE: We review our experience with the Young-Dees-Leadbetter bladder neck repair among patients with neurogenic incontinence. MATERIALS AND METHODS: Between 1978 and 1997, 25 girls and 13 boys with a mean age of 10.5 years (range 5 to 25) underwent a standard Young-Dees-Leadbetter bladder neck repair for neurogenic incontinence. Of the 38 patients 26 had undergone prior urological surgery, including bladder neck surgery in 6 and bladder augmentation in 4. A primary Young-Dees-Leadbetter bladder neck repair was performed in 24 patients, a secondary Young-Dees-Leadbetter procedure in 6 and a primary Young-Dees-Leadbetter procedure with periurethral silicone sheath placement in 8. RESULTS: Of the 38 patients 30 (79%) are dry, 7 (18%) are partially dry and 1 remains incontinent. Total or partial dryness was achieved after the initial repair in 26 cases (68%), while 8 required an additional procedure and 3 required more than 2 procedures to achieve continence. All patients who underwent silicone sheath placement were initially dry but incontinence developed subsequently in 5 due to sheath erosion. Of the 38 patients 35 (92%) ultimately required bladder augmentation. CONCLUSIONS: The management of neurogenic incontinence remains difficult. Success with the Young-Dees-Leadbetter procedure in our experience nearly always requires augmentation cystoplasty. The majority of patients will achieve continence after the initial procedure, and persistent incontinence can frequently be cured with further bladder neck surgery.

Genitourinary anomalies in the CHARGE association.

PURPOSE: We identified the incidence and types of genital and urinary anomalies, and established a plan for evaluating the urinary system in the CHARGE association. MATERIALS AND METHODS: We retrospectively reviewed the charts of 32 patients in whom the CHARGE association was diagnosed. RESULTS: Of the 32 patients identified 22 (69%) had genitourinary abnormalities. Genital anomalies, including micropenis, penile agenesis, hypospadias, chordee, cryptorchidism, a bifid scrotum, atresia of the uterus, cervix and vagina, and hypoplastic labia majora, labia minora and clitoris, were present in 18 patients (56%). Of the 24 patients who underwent renal ultrasound 10 (42%) were diagnosed with urinary tract anomalies including a solitary kidney, hydronephrosis, renal hypoplasia and duplex kidneys. Further evaluation revealed vesicoureteral reflux, neurogenic bladder secondary to spinal dysraphism, nephrolithiasis, ureteropelvic junction obstruction and a nonfunctioning upper pole in both duplex kidneys. CONCLUSIONS: There is a high incidence of genitourinary anomalies in the CHARGE association. Because of this high incidence of anomalies, patients with this condition should undergo a careful genitourinary evaluation, including renal and bladder ultrasound, and voiding cystourethrography screening.