Simultaneous augmentation cystoplasty and cuff only artificial urinary sphincter in children and young adults with neurogenic urinary incontinence.

PURPOSE: We review our experience with artificial urinary sphincter and augmentation cystoplasty in patients with neurogenic bladder. This is the largest known series to specifically evaluate cuff only artificial urinary sphincter at augmentation cystoplasty. MATERIALS AND METHODS: A total of 18 males underwent simultaneous artificial urinary sphincter and augmentation cystoplasty at our institution between 1982 and 2012, of whom 13 (72%) underwent cuff only artificial urinary sphincter. Outcomes included urinary continence, emptying modality, artificial urinary sphincter status, complications and additional procedures. RESULTS: Of the patients undergoing augmentation cystoplasty and cuff only artificial urinary sphincter 10 (77%) were initially continent. Average time of continence was 52.9 months. Four patients (31%) required no additional procedures and remained continent. Urinary incontinence developed in 3 patients (23%) immediately postoperatively and in 6 (46%) subsequently. Ultimately 9 patients (69%) required conversion to complete artificial urinary sphincter at a mean of 36.9 months postoperatively. Overall 12 patients (92%) were continent at followup. There were no artificial sphincter specific complications in patients undergoing the cuff only procedure with conversion to complete artificial urinary sphincter. After conversion to complete artificial urinary sphincter 3 patients (23%) experienced artificial sphincter specific complications. Reoperation was performed in 10 patients (77%), for 13 total procedures (1.3 per patient). There were no complications with cuff only artificial urinary sphincter and 6 complications with complete artificial urinary sphincter (p = 0.025). Finally, patients undergoing cuff only artificial urinary sphincter requiring revision were younger than those not requiring revision (15.6 vs 30.8 years, p = 0.026). CONCLUSIONS: Simultaneous cuff only artificial urinary sphincter and augmentation cystoplasty appears safe and efficacious in patients with neurogenic bladder, with fewer complications than complete artificial urinary sphincter, and may provide definitive urinary continence in up to a third of patients. This procedure is technically easy, allows for outpatient revision, provides time for the child to mature and may be cost effective in avoiding placement of additional components in this select patient population.

The 2-global flash mfERG in glaucoma: attempting to increase sensitivity by reducing the focal flash luminance and changing filter settings.

PURPOSE: To test a new 2-flash multifocal electroretinogram (mfERG) paradigm in glaucoma using a reduced light intensity of the m-frame flash as opposed to the global flash, as it has been suggested that this may increase the responses induced by the global flash, which has been the part of the mfERG response where most changes have been noted in glaucoma. METHODS: A mfERG was recorded from one eye of 22 primary open angle glaucoma (POAG) patients [16 normal tension glaucoma (NTG), 6 high tension glaucoma (HTG)] and 20 control subjects. A binary m-sequence (2^13-1, Lmax 100 cd/m2, Lmin<1 cd/m2), followed by two global flashes (Lmax 200 cd/m2) at an interval of 26 ms (VERIS 6.0™, FMSIII), was used. The stimulus array consisted of 103 hexagons. Retinal signals were amplified (gain=50 K) and bandpass filtered at 1-300 Hz. For each focal response, the root mean square was calculated. We analyzed 5 larger response averages (central 15° and 4 adjoining quadrants) as well as 8 smaller response averages (central 10° and 7 surrounding response averages of approximately 7° radius each). Three epochs were analyzed: the direct component at 15-45 ms (DC) and the following two components induced by the effects of the preceding focal flash on the response to the global flashes at 45-75 ms (IC-1) and at 75-105 ms (IC-2). Statistical analysis was performed using linear mixed effects models adjusted for age. RESULTS: Responses differed significantly between POAG patients and controls in all central response averages. This difference was larger for the central 10° than for the response average of the central 15°. While these observations held true for all response epochs analyzed, the DC differed least and the IC-1 most when POAG was compared to control. For POAG, the most sensitive differential measure was IC-1 of the central 10° with an area under the ROC curve of 0.78. With a cutoff value of 12.52 nV/deg2, 80% of the POAG patients (100% HTG, 69% NTG) were correctly classified as abnormal, while 77% of the control subjects were correctly classified as normal. When the results of the mfERG were compared to the visual fields, there was a tendency for the mfERG to decrease as the mean defect increased. However, this correlation was only significant in the superior nasal quadrant when the IC-1 of the mfERG was compared to the corresponding area of the visual field. CONCLUSION: When compared to findings from previous studies, reducing the luminance of the m-frame flash in the 2-global flash paradigm did not increase the sensitivity and specificity of the mfERG to detect glaucoma further.

Intermittent testicular torsion.

Testicular torsion is the most common cause of acute scrotal pain in prepubertal and adolescent boys and should be foremost in the minds of primary care physicians evaluating these children. Intermittent testicular torsion is a separate entity that should be considered in all young males with a history of scrotal pain and swelling. Acute and intermittent sharp testicular pain and scrotal swelling, interspersed with long intervals without symptoms, are characteristic. Physical findings may include horizontal or very mobile testes, an anteriorly located epididymis, or bulkiness of the spermatic cord from partial twisting. Awareness of this entity and early elective orchiopexy will improve testicular salvage in patients with intermittent testicular torsion.

Administration of growth hormone to pigs alters the relative amount of insulin-like growth factor-I mRNA in liver and skeletal muscle.

The relative amount of insulin-like growth factor-I (IGF-I) mRNA was determined in the liver and skeletal muscle of market weight crossbred barrows (castrated male pigs) using a solution hybridization-nuclease protection assay. Pigs were given either 50 micrograms recombinant porcine GH per kg body weight or vehicle daily for 24 days i.m. They were fed corn-soybean meal diets containing either 140 or 200 g crude protein/kg (low or high protein). The percentage of muscle in the carcasses of pigs given GH was greater (P less than 0.01) than that of controls. Relative to controls, GH increased (P less than 0.05) the amount of liver IGF-I mRNA by 2.7-fold in pigs fed the low protein diet and 3.0-fold in pigs fed the high protein diet. The amount of IGF-I mRNA in the muscles of GH-treated pigs was 77% and 84% of control pigs in those fed the low and high protein diets respectively (P less than 0.08). GH increased (P less than 0.001) the serum concentration of IGF-I 1.6-fold in pigs fed the low protein diet and 2.0-fold in those fed the high protein diet. These results indicate that the administration of GH to pigs influences the relative amount of liver IGF-I mRNA. The increased amount of liver IGF-I mRNA and the increased serum IGF-I concentrations suggest that IGF-I plays an endocrine role in mediating GH-induced muscle hypertrophy in pigs.

Testicular yolk sac and embryonal carcinomas in pediatric patients: comparative immunohistochemical and clinicopathologic study.

Twelve Mayo Clinic patients less than 17 years of age were identified who had yolk sac tumors of the testis. Seven additional pediatric patients were identified who had embryonal carcinoma of the testis. These 19 patients form the basis for this study correlating histopathology and immunocytochemical patterns with clinical outcome. Immunoperoxidase studies were done with antibodies to alpha-fetoprotein, beta-subunit of human chorionic gonadotropin, alpha-1-antitrypsin, and human albumin. The immunocytochemical staining pattern in these neoplasms did not appear to correlate with prognosis.

Genitourinary polyps in children.

OBJECTIVES: This study was undertaken to review the presenting complaints, diagnostic evaluation, treatment, and natural history of children with genitourinary polyps seen at the Mayo Clinic during the past 35 years. METHODS: We retrospectively reviewed the charts of all children less than 16 years of age with symptomatic genitourinary polyps who were seen at the Mayo Clinic between 1957 and 1992. The age of each patient, clinical presentation, anatomic location of the polyp, diagnostic evaluation, and treatment were reviewed. Long-term follow-up data, including complications, were recorded, and the literature was reviewed. RESULTS: The most common presenting symptoms were hematuria in 9 patients, (gross 7, microscopic 2) and urinary tract obstruction in 9 patients (upper tract 3, lower tract 6). Four children had ureteral polyps. Excretory urography showed hydronephrosis and filling defects in 3 patients and a filling defect without hydronephrosis in 1 child. Two patients underwent segmental ureterectomy, 1 had open excision of the polyp, and 1 had ureteroscopic excision. Urethral polyps were identified in 12 children, most often as a filling defect of the posterior urethra on voiding cystourethrography (5 patients) or during cystourethroscopy (7 patients). All 12 patients were managed successfully by transurethral resection. CONCLUSIONS: Genitourinary polyps in children require a high degree of alertness and can be diagnosed with excretory urography, with voiding cystourethrography, or endoscopically. The biologic activity of these polyps is uniformly benign, and there have been no recurrences following complete excision.

Genitourinary neurofibromatosis mimicking posterior urethral valves.

A 12-year-old boy, examined after an episode of acute urinary retention, was found to have neurofibromatosis of the bladder neck and prostatic urethra. His symptoms of bladder outlet obstruction and radiographic findings of a dilated prostatic urethra mimicked posterior urethral valves. Complete urologic investigation, including cystourethroscopy, revealed that the dilatation of the prostatic urethra was secondary to neural involvement of the external sphincter and posterior urethra without mechanical obstruction or posterior urethral valves.

Identification of ureteroileal obstruction by retrograde loopography.

Retrograde loopography in 106 patients (212 renal units) with ileal conduit urinary diversion was assessed to define the accuracy of this study in identifying ureteroileal obstruction. In 171 renal units (81%), the radiographic findings demonstrated by retrograde loopography correlated appropriately with the findings on excretory urography. However, in 41 renal units (19%) with absent ileoureteral reflux, there was no evidence of ureteroileal obstruction by excretory urography. This potential disparity between excretory urography and retrograde loopography has limited our use of the latter study for selected patients in whom the excretory urogram demonstrates (1) evidence of progressive upper tract deterioration, (2) anastomotic obstruction, or (3) patients with chronic renal failure or iodide sensitivity in whom the intravenous administration of contrast material may be contraindicated.

Multilocular cysts of kidney. A study of 29 patients and review of literature.

Multilocular renal cyst is a distinct renal tumor whose gross external appearance and absence of normal renal tissue within the septa of loculi distinguish it clearly from other renal cystic lesions. Interlocular septa may contain either (1) fibrous tissue alone or (2) embryonic tissue separating adjacent loculi. Of 29 patients with multilocular renal cysts, 24 underwent a renal-sparing procedure, and only 5 had radical nephrectomy. None of the histologic specimens showed evidence of immature renal tissue or neoplasia. Patients were followed from three months to eight years (mean, 39 months), and no evidence of local recurrence or metastatic disease was found. Because it is difficult to distinguish multilocular renal cyst from cystic Wilms tumor and multicystic clear cell carcinoma on the basis of imaging studies alone, surgical intervention is the only effective method to differentiate multilocular renal cyst from a malignant lesion of the kidney.

Modified pudendal-thigh flap for correction of penoscrotal transposition.

OBJECTIVES: In patients with penoscrotal transposition, an occasional postoperative problem has been a deficiency of skin on the proximal penile shaft that results in penoscrotal fusion and tethering. METHODS: We describe a new operation using a modified neurovascular pudendal-thigh flap for correction of incomplete penoscrotal transposition. RESULTS: This procedure has been used in 6 children, and an excellent cosmetic and functional result has been achieved in each patient. CONCLUSIONS: The flaps provide a reliable blood supply, maintain normal innervation, and correct the problem of postoperative penoscrotal fusion and tethering. This technique preserves sufficient penile skin for a tension-free second-stage urethroplasty.

Experience with AS 800 artificial sphincter in pediatric and young adult patients.

Congenital or acquired sphincteric urinary incontinence in children and young adults is a severe social and physical burden. As a therapeutic option, we implanted 61 AS 800 artificial sphincters in 59 patients (46 males and 13 females). The mean age of the patients was 17.5 years (range, 6 to 34 years), and mean follow-up was forty-three months (range, 5 to 84 months). In addition to artificial sphincter implantation, intestinal cystoplasty was required in 22 patients to obviate problems of increased detrusor contractility or decreased detrusor compliance, or both. Continence was good in 47 patients (80%) and fair in 8 (14%). Four patients (7%) were awaiting sphincter modification or cystoplasty, or both. Careful and prolonged follow-up is mandatory in all patients managed in this fashion to preclude the deleterious effects of subtle changes in detrusor or ureteral function on the integrity of the upper urinary tracts.

Periumbilical pain secondary to persistent urachal band.

An eight-year-old boy had periumbilical pain associated with micturition; the disorder was secondary to a persistent urachal band. Surgical excision of the urachus resulted in resolution of the symptoms. Although urachal anomalies are unusual, they should be considered in the differential diagnosis of periumbilical pain with micturition.

Complications of Small-Carrion penile prosthesis.

Seventy-six patients with impotence have undergone insertion of the Small-Carrion penile prosthesis at Duke University Medical Center. Twenty patients experienced postoperative complications. Seven of these 20 patients lost one or both prostheses either by spontaneous extrusion or surgical removal. Although success with this procedure is well documented, complications may be significant when they occur and awareness of these problems is essential for proper management. The benefits of this procedure are reviewed, and reported complications and their management are discussed.

Transitional cell carcinoma of renal pelvis: rare occurrence in young male.

Transitional cell carcinoma of the renal pelvis was encountered in a twenty-one-year-old white man. Review of the literature shows this tumor to be rare in individuals less than thirty years of age.

Flutamide-induced cryptorchidism in the rat is associated with altered gubernacular morphology.

OBJECTIVES: One of the major controversies regarding descent of the testes is whether androgenic regulation of the gubernaculum testes exists. To determine if antiandrogens can alter the development of the gubernaculum within the fetus, the following experiment was performed. METHODS: Timed pregnant Sprague-Dawley rats were treated with either flutamide, dihydrotestosterone (DHT), or vehicle alone (controls) from gestational day (GD) 15 to 17. Fetal specimens were removed via cesarean section on GD 18 and 20. Serial coronal sections were obtained, and digital microscopy with computer-assisted reconstruction was used to ascertain the morphology of the three components of the gubernaculum, that is, the gubernacular cord and the mesenchymal and muscular components of the gubernacular bulb. RESULTS: Flutamide significantly prevented and DHT significantly enhanced gubernacular cord regression compared with controls (P < 0.01). Flutamide also resulted in a significant inhibition of the gubernacular bulb outgrowth, with diminution of both the mesenchymal and muscular components of the gubernacular bulb. CONCLUSIONS: These data suggest that androgens play an active role in gubernacular cord regression and gubernacular outgrowth within the fetal rodent.

Alterations in maternal epidermal growth factor (EGF) effect testicular descent and epididymal development.

OBJECTIVE: (1) To determine the ability of epidermal growth factor (EGF) to reverse antiandrogen-induced cryptorchidism and epididymal abnormalities; (2) to evaluate whether alterations in maternal EGF would result in abnormal testicular descent or mal development of the epididymis. METHODS: Experiment 1: Timed pregnant ICR mice were treated with either flutamide, flutamide plus EGF, or vehicle alone on gestational days 11 through birth. Experiment 2: Maternal EGF was abolished by removing the submandibular glands. Following timed mating, dams were treated with either flutamide, anti-EGF, DHT, or vehicle alone on gestational days 11 through birth. RESULTS: Experiment 1: Treatment with flutamide resulted in a 36 percent (26/72) incidence of undescended testes (UDT), and a 43 percent (31/72) incidence of abnormal epididymides. Rats treated simultaneously with flutamide plus EGF had a reduced incidence of UDT (14%, 6/42) and epididymal anomalies (19%, 8/42); p < 0.01. Experiment 2: The absence of maternal EGF resulted in a significant incidence of cryptorchidism in 11/50 (22%) testes, and epididymal anomalies in 19/50 (38%); p < 0.01. CONCLUSIONS: Our findings suggest that EGF stabilizes the wolffian duct system and partially mediates testicular descent.

Impact of pelvic lymphadenectomy in patients with prostatic adenocarcinoma.

The clinical course of 75 males subjected to radical prostatectomy after pelvic lymphadenectomy were compared with that of 29 males receiving radical without lymphadenectomy. The adverse survival impact of positive pelvic nodes and the impact of pelvic lymphadenectomy are discussed.

Pediatric urologic oncology.

Effective chemotherapy combined with surgery has produced excellent disease-free survival rates in most children with genitourinary malignancy. For patients with Wilms' tumor, the overall 2-year survival rate with no evidence of disease is greater than 90 per cent, and the interval of chemotherapy continues to be reduced for those children with low-stage lesions and favorable histologic patterns. In children with pelvic rhabdomyosarcoma, primary cytoreductive chemotherapy has proved to be an effective alternative to exenterative surgery. Patients with yolk-sac tumor who have localized disease need not be subjected to the potential morbidity of retroperitoneal lymph-adenectomy or the toxicity of adjuvant chemotherapy. Conversely, patients with advanced disease can be salvaged with recently developed combination chemotherapeutic regimens.

Wilms' tumor in a 'lump' kidney associated with sacral agenesis.

Many cases of Wilms' tumor developing in a horseshoe kidney have been previously reported. We present a case of Wilms' tumor developing in a "lump" kidney, associated with sacral agenesis. We discuss the implications for staging and make recommendations for follow-up in these cancer-prone children.

Bilateral rhomboid flaps for reconstruction of the external genitalia in epispadias-exstrophy.

In ten young males with the epispadias-exstrophy complex, a new technique of bilateral rhomboid flaps was used for penile elongation and genital reconstruction. This approach offers less chance of injury to the verumontanum and ejaculatory ducts and accurately defines the penopubic angle. Cosmetic and functional results were satisfactory in all patients.