Search

Objective: To determine the rate of stone formation amongst patients of the exstrophy-epispadias complex with augmentation cystoplasty. We hypothesize that bowel segment choice influences the rate of stone formation after bladder augmentation and the rate of complications from bladder stone surgery., Methods: An IRB-approved institutional database of 1512 exstrophy-epispadias patients was reviewed retrospectively. Patients that had a history of bladder augmentation and were seen at our institution between 2003 and 2023 were included., Results: Out of 259 patients, bladder stones developed in 21.6% (56), of which the bowel segment used was colon in 147 patients and ileum in 100. Stones formed in 19% of colon augments compared to 29% ileal augments, however, this was not statistically significant (P = .07). The most common primary stone component was dahllite, followed by struvite for all augments (Table 1). The median time to stone treatment after augmentation was 4.14 years (0.75-31). Seventy-four percentage of patients had a recurrence that required a second surgery. The median time from first to second surgery and second to third surgery was 1.4 years and 2.22 years, respectively. Bladder stone surgery complications occurred in 14% of patients, vesicocutaneous fistula being the most common, and complications did not differ by augment type. Median follow-up after first stone intervention was 6.07 years (0-19.5)., Conclusion: The treatment of bladder stones in the exstrophy-epispadias complex remains challenging. Interventions to prevent recurrence are crucial as the majority of patients will require 2 or more stone surgeries in their lifetime., Competing Interests: Declaration of Competing Interest The authors declare that they have no conflict of interest., (Copyright © 2024. Published by Elsevier Inc.)

Objectives: To outline the management strategies applied to the adolescent patient population with exstrophy/epispadias and incontinence at our institution. These patients present a difficult management problem. At the same time they are dealing with difficult issues, including body image and sexual awareness, the added burden of ongoing incontinence causes major anxieties and lifestyle restrictions. In many, incontinence has persisted despite numerous operations., Methods: A total of 25 (19 male and 6 female) patients who remained incontinent into adolescence or early adulthood were reviewed. Of the 25 patients, 19 had exstrophy, 4 had cloacal exstrophy, and 1 male and 1 female had epispadias. Six patients had undergone eight prior attempts at continent reconstruction, one had undergone cutaneous diversion, and one had problems after ureterosigmoidostomy., Results: The mean age at continence surgery was 12.9 years. Of the 25 patients, 18 underwent bladder augmentation, with a continent stoma in 17 and an artificial sphincter in 1. An additional 5 patients underwent bladder neck transection, with a new continent stoma in 3. The ureterosigmoidostomy was converted to a Mainz II pouch. One patient underwent continent neobladder formation. Nine patients (36%) developed complications during follow-up. Three required stoma revision for stenosis and one for prolapse. Pouch stones occurred in 4 patients, and vesicocutaneous fistula developed in 1. All achieved full urinary continence. The mean follow-up was 72.4 months., Conclusions: Some children with exstrophy/epispadias reach adolescence and remain incontinent. For these patients, modern reconstructive techniques provide hope of continence. With careful preoperative assessment, exact surgical precision, and regular follow-up, a successful outcome can be expected in virtually all cases without the need for external urine collection devices.

Objectives: To identify whether nutritional abnormalities, specifically vitamin B12 deficiency, folate deficiency, and low carotene levels, are long-term sequelae of the use of ileum and/or colon for lower urinary tract reconstruction in pediatric patients., Methods: Serum levels of vitamin B12, methyl malonic acid (MMA), carotene, and folate were measured 3 to 13 years (mean 6) after surgery in patients less than 18 years old at the time of operation., Results: Thirteen (44.8%) of 29 patients had abnormal nutritional serum levels, with 5 (38.5%) of 13 having more than one abnormal value. Vitamin B12 was low in 4 (14%) of 29 patients; MMA was abnormally high in 7 (26%) of 27; and both folate and carotene levels were low in 4 (14.8%) of 27. No patient with reconstruction with colon alone (n = 3) had abnormal values, and no patient had any clinical signs or symptoms of vitamin B12 deficiency or fat malabsorption., Conclusions: Nutritional abnormalities can occur in patients after lower urinary tract reconstruction with ileum. No patient in this study had symptomatic abnormalities; however, long-term clinical effects may be significant. The implications of low folate levels in women of childbearing age must also be taken into consideration because of the potential association with congenital anomalies.

A 66-year-old woman presented with clitoromegaly since childhood, primary amenorrhea, no breast development, and a large right inguinal hernia. A mosaic karyotype was identified containing a predominant 45,X cell line and a cell line with 46 chromosomes, one X chromosome, and a small dicentric Y chromosome with a breakpoint in band qII.2. The patient underwent hysterectomy, bilateral gonadectomy, inguinal hernia repair, clitoral recession, and formation of a neointroitus. A dysgerminoma was identified in the right dysgenetic gonad. This report demonstrates the natural history of untreated mixed gonadal dysgenesis and the importance of early evaluation and treatment, as well as the molecular characterization of a dicentric Y chromosome.

A family is presented in which 4 of 4 (100%) siblings demonstrate vesicoureteral reflux on voiding cystogram. Mechanisms of inheritance are reviewed.

Testicular neoplasms constitute 1 percent of all childhood malignancies and rank eighth in childhood cancer mortality. From 1970 to 1988, 25 testicular tumors in children eighteen years and under were seen. The majority of the patients were white (88%). Pathologic analysis of the tumors revealed that 68 percent were germinal and 32 percent were nongerminal. Staging was undertaken in all patients with serum markers, chest x-ray film, and computerized tomography scans or lymphangiography. All patients underwent radical orchiectomy, and further therapy was given dependent on tumor type and stage. The survival among this cohort was excellent, with only 3 patients succumbing to their disease. Detailed results of treatment, and approaches to avoid excess treatment morbidity are reviewed.

Two patients with ileal conduits underwent planned urinary undiversion; in both patients, the previously constructed ileal conduit was utilized to augment the continent urinary reservoir. Both patients are totally continent with stable upper urinary tracts; neither individual suffers from diarrhea or intestinal absorption abnormalities. This variation in the construction of the continent urinary reservoir allows for the usage of less terminal ileum, and as a result, the risk of intractable diarrhea as well as folate and vitamin B12 malabsorption should be diminished. The criteria that must be met to utilize the ileal conduit in the construction of the continent urinary reservoir include: (1) adequate remaining conduit length after mobilization, and (2) a normal-appearing conduit with sufficient mesenteric length after mobilization.

To determine which form of urinary diversion is most effective for each type of hypospadias, we polled all full-time practicing pediatric urologists in the United States and Canada. Each was asked to complete a questionnaire, specifying the method of urinary diversion used, based on the severity of the hypospadias. Ninety-eight per cent (84 of 86) of the urologists contacted replied to the survey. Our findings suggest that: Urinary diversion is usually not necessary for very mild types of hypospadias. Intraurethral drainage is the preferred form of diversion for moderate types of hypospadias. Suprapubic drainage with an intraurethral stent is the favored means of diversion for severe types of hypospadias. Perineal urethrostomy is rarely used today in hypospadias surgery.

Thirty patients with previously untreated Stage C and D carcinoma of the prostate were treated with subcutaneously implanted estradiol pellets in an effort to determine the efficacy of this mode of therapy in the control of advanced prostatic carcinoma. Serum levels of gonadotropins, testosterone, and estradiol were obtained prior to institution of therapy and at monthly intervals thereafter. Twenty-eight patients were followed for at least one year. Sixteen patients were hormonally castrate at six months following 1-pellet implantation. In these 16 patients the means serum testosterone level was 43.6 ng./dl. with a serum luteinizing hormone (LH) of 13.3 mlU/ml. and a serum estradiol of 19.2 ng./dl. Twenty-two patients were hormonally castrate at twelve months following 1 or more pellet implantations. In this group the mean serum testosterone level was 51.5 ng./dl. with a serum LH of 16.2 mlU/ml. and a serum estradiol of 22 ng./dl. Fifteen patients who were taking narcotics for pain relief either became pain free or had a marked decrease in need for analgesics. Increased weight improvement in over-all well-being was noted in 86 per cent of patients. pellet implantation intervals ranged from four to ten months, with a mean interval of 5.85 months between implantations. Complications attributable to this mode of therapy were no more apparent than with oral estrogens. In addition to better compliance, subcutaneously implanted estrogen pellets appear to provide a safe, reliable, and effective method of tumor control and palliation in advanced carcinoma of the prostate.