Therapeutic efficacy of extended release oxybutynin chloride, and immediate release and long acting tolterodine tartrate in children with diurnal urinary incontinence.

PURPOSE: We compare the tolerability and efficacy of extended release oxybutynin chloride, and immediate release and long acting tolterodine tartrate in children with nonneurogenic diurnal urinary incontinence and symptoms of overactive bladder. MATERIALS AND METHODS: Children with a history of diurnal urinary incontinence were arbitrarily assigned to extended release oxybutynin, immediate release tolterodine or long acting tolterodine. The dose was titrated until effective (onset of complete diurnal urinary continence), maximal recommended dosage was achieved or bothersome anticholinergic side effects developed. An independent observer recorded the dose used, anticholinergic side effects and efficacy of therapy (incidence of urinary frequency, urgency, posturing and urinary incontinence). RESULTS: The study included 86 girls and 46 boys. There were no statistically significant differences among the 3 treatment groups regarding the presence of peripheral or central nervous system anticholinergic side effects. Extended release oxybutynin and long acting tolterodine were significantly more effective at reducing daytime urinary incontinence than immediate release tolterodine (p <0.01 and 0 <0.05, respectively). Extended release oxybutynin was significantly more effective then long acting tolterodine for complete resolution of diurnal incontinence (p <0.05). CONCLUSIONS: Extended release oxybutynin and long acting tolterodine are more effective than immediate release tolterodine in decreasing diurnal urinary incontinence. Extended release oxybutynin chloride is more effective than either immediate or long acting tolterodine for control of daytime urinary incontinence and urinary frequency.

A region of human chromosome 9p required for testis development contains two genes related to known sexual regulators.

Deletion of the distal short arm of chromosome 9 (9p) has been reported in a number of cases to be associated with gonadal dysgenesis and XY sex reversal, suggesting that this region contains one or more genes required in two copies for normal testis development. Recent studies have greatly narrowed the interval containing this putative autosomal testis-determining gene(s) to the distal portion of 9p24.3. We previously identified DMRT1, a human gene with sequence similarity to genes that regulate the sexual development of nematodes and insects. These genes contain a novel DNA-binding domain, which we named the DM domain. DMRT1 maps to 9p24. 3 and in adults is expressed specifically in the testis. We have investigated the possible role of DM domain genes in 9p sex reversal. We identified a second DM domain gene, DMRT2, which also maps to 9p24.3. We found that point mutations in the coding region of DMRT1 and the DM domain of DMRT2 are not frequent in XY females. We showed by fluorescence in situ hybridization analysis that both genes are deleted in the smallest reported sex-reversing 9p deletion, suggesting that gonadal dysgenesis in 9p-deleted individuals might be due to combined hemizygosity of DMRT1 and DMRT2.

Failure to obtain durable results with collagen implantation in children with urinary incontinence.

PURPOSE: We studied the efficacy and durability of injection of glutaraldehyde cross-linked bovine collagen for treating urinary incontinence in children. MATERIALS AND METHODS: From April 1994 to July 1995, 12 boys and 8 girls 4 to 18 years old (mean age 9.5) underwent endoscopically directed collagen injections into the bladder neck. Followup ranged from 9 to 23 months (mean 15.2). The etiology of incontinence included myelodysplasia in 12 patients, exstrophy/epispadias in 4, and epidural abscess, sacral agenesis, imperforate anus and posterior urethral valves in 1 each. RESULTS: Collagen was injected once in 9 patients, twice in 10 and 3 times in 1. Injected volume ranged from 3 to 18 cc (mean 7.3). Followup urodynamic studies were available for 10 patients. Leak point pressure increased from 28.7 to 34.9 cm. water. One patient (5%) was dry, 5 (25%) had improvement, 10 (50%) had transient improvement for 2 to 90 days (mean 52) and 4 (20%) remained incontinent. Five children underwent bladder neck sling procedures. In 1 patient transient sciatic nerve irritation developed due to gluteal hematoma. CONCLUSIONS: The previously reported high success rate of collagen injection for urinary incontinence in children is unsupported by this study. Improvement in continence was temporary or inadequate in the majority of patients. Collagen therapy may only delay the need for surgery for managing organic urinary incontinence in children.

Intermittent self-catheterization by quadriplegic patients via a catheterizable Mitrofanoff channel.

PURPOSE: Neurogenic bladder due to cervical spinal cord injury is often best managed by clean intermittent catheterization. Limited upper extremity function makes patients with quadriplegia dependent on caregivers for clean intermittent catheterization. The Mitrofanoff umbilical appendicovesicostomy provides easy access to the bladder in patients with all types of access difficulty. We evaluated the Mitrofanoff umbilical appendicovesicostomy in 7 patients with quadriplegia. MATERIALS AND METHODS: Four men and 3 women with cervical spinal cord injury underwent the Mitrofanoff umbilical appendicovesicostomy with appendix (5) or ileum (2). RESULTS: All patients are continent and able to self-catheterize via the umbilical stoma. In 1 patient with an ileal Mitrofanoff umbilical appendicovesicostomy umbilical incontinence was repaired surgically and stomal stenosis developed in 1, which required stomal revision. All patients use the stoma regularly. Four patients have moved out of care facilities and are living more independently. CONCLUSIONS: The Mitrofanoff catheterizable channel is a valuable option for bladder management in patients with quadriplegia. Clean intermittent self-catheterization allows these patients independent function and facilitates their return to productive lives outside care facilities.

Successful treatment of giggle incontinence with methylphenidate.

PURPOSE: Giggle incontinence, the second most common type of childhood enuresis unrelated to disease, is notoriously difficult to treat. However, the association of laughter or emotion precipitated alteration of muscle tone is suggestive of a functional relationship to cataplexy, a part of the narcoleptic syndrome complex that may respond to stimulant medication. MATERIALS AND METHODS: Two boys and 5 girls (mean age 10.9 years) with giggle incontinence, a positive family history of giggle incontinence (4 patients) and no evidence of urological disease were treated with methylphenidate for 1 to 5 years. RESULTS: All patients responded positively with complete cessation of enuresis to varying dose schedules of methylphenidate. CONCLUSIONS: These results suggest that giggle incontinence is a centrally mediated and likely hereditary disorder that may share a common pathophysiological basis with the narcolepsy/cataplexy syndrome.

Bladder stimulation therapy improves bladder compliance: results from a multi-institutional trial.

PURPOSE: We examined data from multiple institutions to determine whether intravesical bladder stimulation therapy is effective in improving bladder compliance by increasing bladder capacity and lowering bladder storage pressures. MATERIALS AND METHODS: The charts of 568 patients from 11 institutions were evaluated. Of the 568 patients 335 had adequate and accurate pretreatment and posttreatment urodynamic studies, and were included in this study. A total of 155 patients was from Children's Memorial Hospital, while the remaining 180 were from 10 other institutions. Bladder capacity and bladder capacity pressure were determined for each patient before and after therapy. RESULTS: Overall, 53% of patients had increased bladder capacity of 20% or greater after treatment (average increase 105 cc), which represents a 63% increase from pretreatment values. This increase occurred in an average of 1.9 years. Further analysis of this subset of patients revealed that in 90% intravesical storage pressures were decreased or maintained within a safe range (less than 40 cm. water). Evaluation of patients who did not respond to bladder stimulation with a 20% or greater increase in bladder capacity revealed that they had nearly normal bladder capacity before therapy. When the data on bladder capacity and bladder capacity pressure from Children's Memorial Hospital were compared to results from the 10 other institutions, there were no appreciable differences. CONCLUSIONS: Bladder stimulation is effective in increasing bladder capacity without significantly elevating storage pressure in a majority of patients. We conclude that this technique is safe and effective in improving bladder compliance, and that it is reproducible elsewhere.

Synchronous bilateral Wilms' tumor in a neonate.

OBJECTIVES: Wilms' tumor is the most common solid abdominal tumor of childhood. Wilms' tumor is extremely rare in the neonate, with less than 20 well-documented cases. No bilateral lesions in neonates have been described in the literature. METHODS: We report on a neonate who presented with synchronous bilateral Wilms' tumors. RESULTS: Child underwent bilateral partial nephrectomy, followed by standard chemotherapy, and is now well 2 years after completion of the therapy without any evidence for recurrent disease. CONCLUSIONS: Wilms' tumor should be included in the differential diagnosis of the solid renal mass in the neonate.

Hematuria in a newborn infant caused by bladder hemangioma.

A newborn boy had gross hematuria caused by hemangiomas of the bladder. The sonographic examination and the voiding cystourethrogram showed an irregular and thick bladder wall with a lesion extending into the vesical lumen. Cystoscopic examination and biopsy of the lesions were diagnostic of hemangioma. Immediately after lesion fulguration, the hematuria disappeared, and 3 weeks later the follow-up ultrasound examination showed resolution of the lesion. At 1 year, the patient's urinalysis and ultrasound scan were normal. To our knowledge, this is the first reported case of hematuria in a newborn caused by a bleeding hemangioma of the bladder.

Seromuscular colocystoplasty lined with urothelium: experience with 16 patients.

OBJECTIVES: Report of initial clinical experience with seromuscular colocystoplasty lined with urothelium (SCLU), a new operation to increase bladder capacity and compliance without incorporating intestinal mucosa into the urinary tract. We have previously reported the technique and results of this procedure in a canine model. METHODS: Sixteen patients underwent SCLU at two institutions. Their charts were reviewed retrospectively. The patients have been observed for a mean of 12 months. RESULTS: Postoperatively, the bladder capacity increased an average of 2.4-fold from a mean of 139 mL (+/- 23.7 SEM) to 335 mL (+/- 38.9 SEM) in 14 patients (P < 0.001). Two patients required reaugmentation. Preoperative and postoperative bladder end filling pressures were available in 13 patients and decreased from a mean of 51.6 cm H2O (+/- 4.2 SEM) to 27.7 cm H2O (+/- 2.4 SEM) (P < 0.001). Of the 16 patients, 13 were incontinent preoperatively; 5 had renal insufficiency. Three patients have undergone renal transplantation following SCLU. After SCLU, 13 patients were dry day and night, 1 incontinent at night only, and 2 remained incontinent. Ten patients have undergone postoperative bladder biopsy. Seven demonstrate urothelium covering the augmented portion of the bladder, 2 have regrowth of colonic mucosa, and 1 shows a mixture of colonic mucosa and urothelium. CONCLUSIONS: The results presented suggest that SCLU is a viable alternative to the more conventional forms of bladder augmentation in selected patients.

Nephrectomy combined with lower abdominal extraperitoneal ureteral bladder augmentation in the treatment of children with the vesicoureteral reflux dysplasia syndrome.

Ureterocystoplasty is a novel procedure well suited for treating children with megaureters and small or noncompliant bladders. It combines the benefits of enterocystoplasty without many of its drawbacks. We performed nephrectomy through a posterior approach with extraperitoneal ureteral augmentation in 6 patients with bladder neck obstruction and reflux into a nonfunctioning kidney (the vesicoureteral reflux dysplasia/vesicoureteral reflux nonfunctioning kidney syndrome). In all patients capacity increased significantly. Bladder pressure at capacity decreased in patients who were evaluated by urodynamics postoperatively, and continence was achieved or preserved. There were no complications and hospital stay was short. Nephrectomy via a posterior approach with extraperitoneal ureteral augmentation is an appropriate procedure for children with the vesicoureteral reflux dysplasia syndrome. It should be considered for all such patients before nephroureterectomy is done and irreversible loss of valuable compliant native urothelium occurs.

Intraureteral metallic self-expanding endoprosthesis (Wallstent) in the treatment of difficult ureteral strictures.

Intractable and recurrent ureteral stricture presents a continuous challenge to the urologist. We report on 5 patients with severe ureteral stricture who were successfully treated with self-expanding metallic stents. Ureteral stricture occurred at ureteroileal anastomotic sites after neoplasm resection in 2 cases, multiple upper ureteral strictures were related to multiple surgical procedures for correction of bladder exstrophy in 1 and a ureteral kink developed in 1. Treatment with transluminal balloon dilation provided poor results but self-expanding metallic stents were used successfully with no major complications. In the last patient the stent and the overlying ureter were removed due to recurrent reflux; the gross and histological ureteral changes are discussed in detail. The technical approach is described, alternative therapeutic options are considered and pertinent literature is reviewed.

Distal hypospadias repair without stents: is it better?

We previously reported the results of the meatal based flap urethroplasty (Mathieu) for distal hypospadias repair. Of 49 patients in whom stents were left indwelling for 2 to 5 days meatal stenosis developed in 1 and there were no fistulas. In view of these good results and to decrease postoperative discomfort from bladder spasms, we performed 37 consecutive meatal based flap repairs without stents. Seven patients (19%) had urinary retention requiring catheterization several hours after surgery, of whom 3 had had a caudal block and 4 a penile block with 0.25% bupivacaine for postoperative pain control. In 5 patients (14%) a urethrocutaneous fistula developed, which required surgical repair. Of the patients with a fistula 2 were also among those who presented with urinary retention and 1 had concomitant meatal stenosis. One child had meatal stenosis only, requiring a meatoplasty after failed dilations. Subsequently, of another 16 children who underwent the Mathieu repair with stents a urethrocutaneous fistula and meatal stenosis developed in 1 (6.2%) and 15 patients had no complications. Overall of 65 patients in whom a stent was used 3 (4.6%) had complications, in contrast with a complication rate of 18.9% in the unstented group, representing a statistically significant difference (p < 0.05). The stent obviates urinary retention, which was unrelated to the type of anesthetic block used, and minimizes the incidence of fistula and stenosis. We conclude that the use of a multiperforated silicone urethral stent is advantageous for the outcome of this operation.

Scrotal ultrasound for evaluation of subacute testicular torsion: sonographic findings and adverse clinical implications.

There is an increased use of scrotal ultrasound in the clinician's office and emergency room for the investigation of scrotal pain. The use of real-time scrotal ultrasound for the diagnosis of testicular torsion has been described in the literature. A false-negative ultrasound examination can postpone the diagnosis of torsion and result in testicular loss. We examined 6 patients 1 day to 18 years old who had subacute testicular torsion with scrotal symptomatology (pain and/or swelling) for longer than 8 hours (range 12 hours to 6 days). Scrotal ultrasound was performed as 1 of the initial tests. A common sonographic pattern was an inhomogeneous testicle with hypoechoic areas alternating with hyperechoic areas and thickening of adjacent scrotal tissue. Another common finding was an edematous hyperechoic epididymis and a small hydrocele. In 4 of the 6 cases these nonspecific findings suggested a misleading diagnosis of tumor or epididymitis and resulted in delay of surgery and testicular loss. Treatment was not delayed in only 2 patients in whom the diagnosis of torsion was made initially by history and physical examination, and ultrasound was done for interest only. Misdiagnosis of intratesticular blood flow and some potential pitfalls of scrotal imaging by color Doppler ultrasound are discussed. We conclude that real-time scrotal sonography can be misleading in cases of subacute testicular torsion and, therefore, it should not be used in this clinical setting.

Renal rupture after the Credé maneuver.

We report a case of renal rupture with development of a large perinephric urinoma and deterioration of renal function after the Credé maneuver in a girl with neurogenic bladder and detrusor sphincter dyssynergia without vesicoureteral reflux. After percutaneous drainage of the urinoma, management was changed to clean intermittent catheterization, and renal function returned to normal. We recommend that the Credé maneuver not be used in children with neurogenic bladder and nondenervated urethral sphincter.

Urological aspects of sacrococcygeal teratoma in children.

Sacrococcygeal teratoma is the most common extragonadal germ cell tumor of infancy. Associated urological complications, most of which are reported in children with malignancy, include vesicoureteral reflux, ureteral and urethral obstruction, and neurogenic bladder. To evaluate the influence of tumor grade and type on adverse urological outcome we reviewed the charts of 29 children with sacrococcygeal teratoma and correlated urological problems to lesion type and grade. No correlation was noted between tumor grade and the incidence of urological complications. The most common urological complications were neurogenic bladder in 12% of the patients, ureteral obstruction in 10% and vesicoureteral reflux in 7%. The highest incidence of urological complications (81%) was seen in patients with type IV (presacral) disease. We recommend early radiographic and neurourodynamic evaluation in all children with sacrococcygeal teratoma.

Silicone shedding from artificial urinary sphincter in children.

Silicone is an inert material used in all genitourinary prosthetic devices. Silicone particle shedding has been documented in adults with penile prostheses and artificial urinary sphincters. We searched prospectively for silicone particles in the peri-prosthetic tissue and regional lymph nodes in 6 children who underwent removal or exchange of an artificial urinary sphincter. The peri-prosthetic fibrous capsule was biopsied and examined by light and polarizing microscopy. Regional lymph nodes were biopsied if clinically enlarged and evaluated in a similar manner. Lymph nodes from 2 children without an artificial urinary sphincter served as controls. An artificial urinary sphincter was in place from 3 to 10 years (mean 4.3 years) and none of the sphincters demonstrated infection or erosion. Silicone particles were found in the peri-sphincteric tissue of 3 patients, which induced foreign body giant cell reaction in 2 and eosinophilic infiltrate in 1. Focal histiocytic proliferation around the cuff was seen in another case without silicone migration. There was fibrosis around the sphincter in all cases. No silicone was found in the regional lymph nodes. Silicone shedding was documented in 50% of our patients. X-ray energy dispersive spectroscopy was not performed, raising the possibility that it was under-detected. Long-term effects of silicone in children with an artificial urinary sphincter are unknown and warrant further study.

Urethral atresia and the prune belly syndrome. Report of 6 cases.

An association between urethral atresia and the prune belly syndrome (PBS) has been recognised, but few reports discuss the outcome of treatment for these gravely ill patients. Of the 34 patients with prune belly syndrome evaluated at our institutions, 6 had urethral atresia (3 males and 3 females). Two of these patients died as neonates as a result of pulmonary insufficiency and 1 was stillborn. The common feature of the 3 surviving patients (2 males and 1 female) was the presence of a vesicocutaneous fistula. As neonates, 2 suffered from pulmonary insufficiency due to oligohydramnios. The mean length of follow-up for these 3 patients was 37 months (range 13-58). The urinary tract was decompressed in 2 patients by a formal vesicostomy performed early in life. The surviving girl has normal renal function following reconstructive surgery. Because both the bladder and urethra were absent in this patient, an ileocaecal bladder substitute and an appendiceal urethra were constructed. The 2 surviving boys both have renal insufficiency. One has received a renal transplant from a living relative and is doing well. In one of the boys, urethral atresia was initially managed by perineal urethrostomy and then by reconstructive surgery. Progressive catheter dilation was used on the other boy. Urethral atresia occurred in 18% of our patients with PBS; the incidence was equal in males and females. Survival correlated with the development of a spontaneous vesicocutaneous fistula. Two-thirds of the survivors developed end-stage renal failure.

Computerized tomography in the diagnosis of appendicovesical fistula.

Appendicitis and its complications are among the causes of an enterovesical fistula, with approximately 100 such cases reported. This condition is seldom diagnosed preoperatively and surgery is often delayed. We report 2 cases of an appendicovesical fistula that were diagnosed preoperatively by computerized tomography (CT). We describe a CT finding consistent with the diagnosis, namely calcification in the thickened bladder wall adjacent to the cecum on noncontrast CT, which is a fecalith in the lumen of the fistula.