External urethral sphincter dilation for the management of high risk myelomeningocele: 15-year experience.

PURPOSE: We investigate the long-term outcome using external urethral sphincter dilation for high risk myelomeningocele. MATERIALS AND METHODS: Since 1984 external urethral sphincter dilation was performed in 25 patients with myelomeningocele who demonstrated passive leak point pressure greater than 40 cm. H2O and/or poor bladder compliance. Mean followup from the first dilation was 8.4 years. Overall 2.4 dilations were performed per patient (range 1 to 8). Cystometrography, imaging study and continence status were evaluated retrospectively. RESULTS: Overall external urethral sphincter dilation produced durable improvements in mean leak point pressure (60.9 versus 34.4 cm. H2O), capacity (119.8 versus 233.3 ml.), initial compliance (11.5 versus 28.4 ml./cm. H2O) and terminal compliance (1.1 versus 7.7 ml./cm. H2O). Categorical analysis revealed 3 groups in terms of outcome. Group 1 consisted of 11 patients (44%) who demonstrated durable improvements in urodynamic parameters as well as preservation of the upper tracts. These patients demonstrated a 2-step compliance pattern on pre-dilation cystometrography, in which elevated leak point pressure was associated with excellent initial compliance. Group 2 consisted of 5 patients (20%) who failed to maintain safe leak point pressure and whose upper tracts deteriorated, including 4 who eventually underwent augmentation cystoplasty. This group demonstrated a 1-step hypertonicity in which elevated leak point pressure was associated with a steep pressure increase during early filling. Group 3 consisted of 9 patients (36%) who responded minimally in terms of leak point pressure reduction but whose upper tracts remained well preserved. They demonstrated a high pressure instability pattern associated with excellent baseline compliance. CONCLUSIONS: External urethral sphincter dilation provides an effective long-term solution for select high risk myelomeningocele cases. Those who demonstrate elevated leak point pressure and poor bladder compliance at the time of external urethral sphincter dilation are less likely to respond, suggesting that the bladder may have already undergone irreversible changes due to high outlet resistance. Patients who demonstrate instability patterns are less likely to respond to external urethral sphincter dilation in terms of leak point pressure reduction but the upper tracts appear to be well preserved.

Nephrolithiasis associated with the ketogenic diet.

PURPOSE: The ketogenic diet has been used for treating seizure disorders for more than 70 years. Nephrolithiasis is a known complication of this diet with a reported stone rate as high as 10% but there is sparse literature detailing the nature and treatment of these stones. We report on 4 children with nephrolithiasis on this diet. MATERIALS AND METHODS: We describe stone treatment and analysis as well as metabolic and urine abnormalities in 4 children with nephrolithiasis on the ketogenic diet who presented to our institution. RESULTS: All patients were treated with shock wave lithotripsy, fluid liberalization and oral citrate. One child was ultimately withdrawn from the diet due to persistent stone formation. Analysis revealed 3 calcium and 1 ammonium urate stones. Three patients had hypercalciuria, 2 elevated urinary uric acid and 1 hypocitruria. Serum studies revealed acidosis in 3 cases. CONCLUSIONS: The ketogenic diet induces several metabolic abnormalities that increase the propensity for stone formation. Urologists should be aware of this potential complication. Fluid liberalization and bicitrate are recommended as prophylaxis.

Barometers and bladders: a primer on pressures.

PURPOSE: We develop a "consilient" (unified) view of pressure as a physical phenomenon and "clinimetric" tool, making a connection between barometers and bladders. MATERIALS AND METHODS: The philosophy, physics and clinical applications of pressure during the last 2 millennia were examined from Lucretius to the modern medical subspecialties. RESULTS: A variety of units and systems of pressure quantification developed as the physics of pressure became understood. Applications of pressure in clinical medicine with distinct physiological relevance have been created for organ systems across the subspecialties. Some measurements have become useful for management of urinary tract and other diseases. CONCLUSIONS: Despite a broad range of units, systems and applications, a consilient view of pressure in medicine can be approached. This perspective is fundamental to understanding the significance of pressures in the expanding clinimetric arena and should mitigate against misplaced concreteness that is tempting in modern medical practice, whereby laboratory tests become virtual realities and are mistaken for patients.

Delayed presentation of posterior urethral valves: a not so benign condition.

PURPOSE: Posterior urethral valves are usually detected during infancy by prenatal sonography. Rarely they may be diagnosed during later childhood, adolescence or even adulthood. Less is known about presentation and outcome in these older patients. We reviewed our experience at 4 institutions with the late presentation of posterior urethral valves. MATERIALS AND METHODS: A 13-year retrospective review revealed the late presentation of posterior urethral valves in 47 patients 5 to 35 years old (mean age 8). Data collected included presenting symptomatology, radiographic findings and renal function. Statistical analysis determined the relationships among presenting symptoms, patient age at diagnosis and renal function. RESULTS: The most common presenting symptoms were diurnal enuresis in 60% of the cases, urinary tract infection in 40% and voiding pain in 13%. Other presenting symptoms in less than 10% of the cases included poor stream, gross hematuria and proteinuria. At diagnosis hydronephrosis and vesicoureteral reflux were present in 40 and 33% of the patients, respectively, while serum creatinine was elevated in 35% and end stage renal disease had developed in 10%. The severity of presenting signs and symptoms was significantly associated with renal impairment, while patient age at diagnosis was not. CONCLUSIONS: Posterior urethral valves is not merely a disease of infancy. Voiding cystourethrography should be considered in boys older than 5 years who have voiding complaints, especially in association with diurnal enuresis or urinary tract infection. Patients who present late with posterior urethral valves are at risk for progression to end stage renal disease.

Two good reasons: women's and men's perspectives on dual contraceptive use.

In the US, continued high rates of unintended pregnancy, combined with increases in heterosexual transmission of HIV to women, have sharply magnified concern about the factors leading to or barring the use of contraceptive methods to protect concurrently against both risks. This paper reports on results of focus group research among African-American women participating in a longitudinal study and African-American men who are either partners of the women or are of similar socio-economic status as their partners. We found a high level of agreement between men and women on the issues and problems that both sexes face. People felt that regardless of a woman's use of other contraceptive methods, a condom should always be used for protection. This belief, however, differed markedly from actual practice. Although we attempted to discern the relative salience of concern about pregnancy versus STIs, we conclude that people may not separate these two concerns in their resolve to use two methods. Furthermore, they recognized the need for dual protection, but expected conflict with their partners from using condoms as a second method because of high levels of distrust regarding sexual fidelity. Thus people are caught in a bind: distrust further increases the sense of a need for dual methods, but using condoms exacerbates the problems people have with achieving trust in relationships.

Beyond the Trendelenburg position: Friedrich Trendelenburg's life and surgical contributions.

Friedrich Trendelenburg (1844-1924) was a giant figure in the formative years of modern surgical practice. His name lives on in the Trendelenburg position, a pelvis-up, head-down position that is of great use in surgical practice. That position, however, was certainly well known before Trendelenburg and the linkage of his name was by no means the greatest of Trendelenburg's achievements. Trendelenburg was a world class surgeon, innovator, and educator who made novel advances that spanned the entire range of surgical practice.

Lower ureteral reconstruction.

Lower ureteral reconstruction is commonly performed in instances of congenital anomalies and ureteral injury. Keys to a successful outcome are an understanding of ureteral anatomy, familiarity with the principles of tissue handling, and the knowledge of various techniques of repair. This article presents some important and useful reconstructive techniques that can be considered in the management of patients with congenital anomaly or lower ureteral injury.

Renal transplantation in children with severe lower urinary tract dysfunction.

PURPOSE: Renal transplantation in children with end stage renal disease due to congenital urological malformations has traditionally been associated with a poor outcome compared to transplantation in those with a normal urinary tract. In addition, the optimal urological treatment for such children remains unclear. To address these issues, we retrospectively reviewed our experience with renal transplantation in this population. MATERIALS AND METHODS: Between 1986 and 1998, 12 boys and 6 girls a mean age of 8.4 years with a severe dysfunctional lower urinary tract underwent a total of 15 living related and 6 cadaveric renal transplantations. Urological anomalies included posterior urethral valves in 8 cases, urogenital sinus anomalies in 4, the prune-belly syndrome in 2, and complete bladder duplication, ureterocele, lipomeningocele and the VATER syndrome in 1 each. In 11 children (61%) bladder augmentation or continent urinary diversion was performed, 2 (11%) have an intestinal conduit and 5 (28%) have a transplant into the native bladder. RESULTS: In this group patient and overall allograft survival was 100 and 81%, respectively. These values were the same in all children who underwent renal transplantation at our center during this era. In the 17 children with a functioning transplant mean serum creatinine was 1.4 mg./dl. Technical complications occurred in 4 patients (22%), including transplant ureteral obstruction in 2 as well as intestinal conduit stomal stenosis and Mitrofanoff stomal incontinence. CONCLUSIONS: Renal transplantation may be successfully performed in children with end stage renal disease due to severe lower urinary tract dysfunction. Bladder reconstruction, which may be required in the majority of these cases, appears to be safe when performed before or after the transplant. A multidisciplinary team approach to surgery is advantageous.

Temporal expression of elastic fiber components in bladder development.

Fetal and postnatal bovine bladders were examined for expression of elastic fiber components by immunohistochemistry as well as by measurement of steady state mRNA levels. Expression of fibrillin-1, microfibril-associated glycoprotein (MAGP) and elastin during the fetal period were compared with that of postnatal two year old animals (heifers) and adults. Each bladder was separated into two distinct tissue samples: 1) the outer smooth muscle layer (detrusor) and 2) the inner epithelium (urothelium) lined lamina propria (urotherial-lamina propria). Each of these samples was analyzed separately. Distribution of the elastic fiber components, determined by immunohistochemistry with matrix-specific antibodies, was different depending upon the region of the bladder wall examined and its developmental stage. In particular, MAGP and fibrillin-1 were conspicuously present in the urothelium during the later fetal stages. RNA products of elastic fiber genes were detectable both in the detrusor smooth muscle and urothelial-lamina propria fractions. The highest level of expression occurred in the urothelial-lamina propria fraction during the late second-early third trimester. Elastin expression was different from that of MAGP and fibrillin-1. The highest levels of steady-state elastin mRNA occurred at the earliest developmental stages examined and then progressively decreased through term. A high level of elastin expression occurred within the inner or lamina propria layer of the bladder. Since this layer is the functional capacitance layer within the bladder, its flexibility is likely related to the structural integration of elastin and associated microfibrillar components.

Laparoscopy for the nonpalpable testis.

Cryptorchidism represents one of the most common childhood disorders with the incidence of testicular maldescent being 0.8% by 1 year of age. Nonpalpable testis occurs in approximately 20% of patients with cryptorchidism. Laparoscopy for diagnosis and management of nonpalpable testis has shown to be highly effective with few complications. This article reviews some of the special considerations in performing laparoscopy in children with descriptions about the unique features of a child's anatomy and physiology. The role of laparoscopy in the evaluation and management of nonpalpable testis is discussed, along with some of the controversies about how laparoscopy may be changing our approach to a child with a nonpalpable testis.

Comments on pediatric elimination dysfunctions: the Whorf hypothesis, the elimination interview, the guarding reflex and nocturnal enuresis.

AIMS OF STUDY: This paper addresses pediatric elimination disorders including nocturnal enuresis from the perspectives of terminology (the Whorf hypothesis), the elimination interview, and the guarding reflex. METHODS: The elimination interview and a modern model of normal voiding function, including the guarding reflex, are explained. RESULTS: The language of voiding dysfunction influences our perception of it. Nocturnal enuresis, and all other voiding dysfunctions, should be evaluated by a careful elimination interview. The guarding reflex may fit into an evolving etiologic paradigm for nocturnal enuresis. CONCLUSIONS: (1) Words and phrases of eliminology should aim for simplicity, clarity and accuracy. (2) Urologists need to perfect their eliminology skills vis-à-vis the elimination interview. (3) The role of the guarding reflex in nocturnal enuresis and all other voiding dysfunctions should be critically examined.

Role of type III collagen in bladder filling.

The function of the urinary bladder is to store urine at low pressure and expel it periodically. To accomplish this, it must have the appropriate structural properties to accommodate slow but continuous volume changes. While much is presently known about the functional measurements of compliance, relatively little is known about the structural basis of compliance. In the present study, immunohistochemistry has been used to localize type III collagen fibers in the bladder wall at different intravesical volumes. To improve the resolution of these fibers, confocal microscopy was utilized to determine the changes in type III collagen fiber orientation and correlate them with the degree of mechanical distension of the bladder wall at partial and full capacity. We demonstrate that there were significant changes in both the orientation and conformation of type III collagen fibers during bladder filling. These observations support the view that volume accommodation in the bladder is achieved by changes in the arrangement of type III collagen. These data suggest that abnormal deposition or arrangement of type III collagen fibers can have an impact on normal bladder function.

Developmental expression of interstitial collagen genes in fetal bladders.

Bovine bladders at 3 stages during fetal development were examined for expression of collagens by immunohistochemistry as well as by measurement of steady state mRNA levels. Expression of type I and type III collagens during the fetal period was compared with that of adult cows as well as a young animal (heifer). Each bladder was separated into a detrusor and a urothelial-lamina propria sample which were then analyzed separately. Distribution and fiber arrangement of types I and III collagens were different depending upon the region of the bladder wall examined. Type III collagen, in particular, has a "coiled" appearance which is especially prominent within the lamina propria. Collagen gene expression showed a distinctive pattern which was different for both type I and type III collagen. While type I collagen gene expression peaked during the late second to early third trimester, type III collagen expression progressively decreased throughout the fetal period. In addition, expression of both collagens was greater in the urothelial-lamina propria fractions. These data demonstrate that the pattern of collagen gene expression in the developing bladder is developmentally regulated and is unique to each of the two major structural layers.

Long-term results of ureterosigmoidostomy in children with bladder exstrophy.

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.

The long-term outcome in men with exstrophy/epispadias: sexual function and social integration.

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.