The transition of young adults with lifelong urological needs from pediatric to adult services: An international children's continence society position statement.

INTRODUCTION: Children with urinary tract disorders managed by teams, or individual pediatricians, urologists, nephrologists, gastroenterologists, neurologists, psychologists, and nurses at some point move from child-centered to adult-centered health systems. The actual physical change is referred to as the transfer whilst the process preceding this move constitutes transition of care. Our aims are twofold: to identify management and health-service problems related to children with congenital or acquired urological conditions who advance into adulthood and the clinical implications this has for long-term health and specialist care; and, to understand the issues facing both pediatric and adult-care clinicians and to develop a systems-approach model that meets the needs of young adults, their families and the clinicians working within adult services. METHODS: Information was gleaned from presentations at an International Children's Continence Society meeting with collaboration from the International Continence Society, that discussed problems of transfer and transitioning such children. Several specialists attending this conference finalized this document identifying issues and highlighting ways to ease this transition and transfer of care for both patients and practitioners. RESULTS: The consensus was, urological patients with congenital or other lifelong care needs, are now entering adulthood in larger numbers than previously, necessitating new planning processes for tailored transfer of management. Adult teams must become familiar with new clinical problems in multiple organ systems and anticipate issues provoked by adolescence and physical growth. During this period of transitional care the clinician or team assists young patients to build attitudes, skills and understanding of processes needed to maximize function of their urinary tract-thus taking responsibility for their own healthcare needs. Preparation must also address, negotiating adult health care systems, psychosocial, educational or vocational issues, and mental wellbeing. CONCLUSIONS: Transitioning and transfer of children with major congenital anomalies to clinicians potentially unfamiliar with their conditions requires improved education both for receiving doctors and children's families. Early initiation of the transition process should allow the transference to take place at appropriate times based on the child's development, and environmental and financial factors. Neurourol. Urodynam. 36:811-819, 2017. © 2016 Wiley Periodicals, Inc.

Examining long-term outcomes of bladder exstrophy: a 20-year follow-up.

OBJECTIVE: To examine long-term quality-of-life, urinary continence and sexual function outcomes in patients diagnosed with bladder exstrophy (BE). PATIENTS AND METHODS: A total of 65 patients with BE and follow-up of at least 20 years were identified. After informed consent for inclusion in the study, the patients were asked to complete three validated questionnaires, the Short-Form 36 quality-of-life questionnaire (SF-36), the International Consultation on Incontinence Questionnaire (ICIQ) and the International Index of Erectile Function (IIEF), to assess quality of life, perceived urinary continence and sexual function. RESULTS: In all, 21 patients responded to the questionnaires, yielding a 32% response rate. High scores in each of the eight dimensions of the SF-36 reflected a positive perception of quality of life by respondents; calculated scores were compared with those of a normal control group. The only significant difference found between the groups was that the study population perceived their general health to be poorer than those in the control group. High scores on the ICIQ indicate high levels of subjective incontinence in patients, with scores ranging from 0 to 21. Reporting scores of 0 (continent), 12/21 patients perceived their continence to be normal, 9/21 patients had scores >2 (mild), with one patient scoring a 16, and 11 (severe incontinence), patients reported no identifiable leakage during normal activities. There was a 29% response rate for the IIEF (15 patients completed this). IIEF scores were broken down into five dimensions and mean scores were calculated. The mean scores showed mild to moderate dysfunction in each category, including overall satisfaction with sexual experience. CONCLUSIONS: The patient-reported quality of life in patients with BE was normal in all dimensions, with the exception of perception of general health. Half of the patients reported normal continence and had no complaints of urinary leakage. Sexual function in males was significantly affected across all dimensions, with mild to moderate dysfunction.

Destruction of the bladder by single dose Mitomycin C for low-stage transitional cell carcinoma (TCC)--avoidance, recognition, management and consent.

OBJECTIVES: To identify a cohort of patients under our care who have had significant and in some cases irreparable damage to their bladders after Mitomycin C (MMC) instillation. To highlight the importance of avoidance and recognition of bladder perforations during transurethral resection of bladder tumour (TURBT) and explore the issue of consent regarding MMC given the serious complications that may occur after its instillation. PATIENTS AND METHODS: Patients referred to our tertiary centre for a second opinion to manage their complications after a suspected MMC leak was identified from the departmental database between January 2000 and December 2010. After collection of all the records, we established a cohort of six patients. All patients had their initial tumour resection elsewhere and were referred for specialist management thereafter. Details of the operating surgeon and cystoscopic findings were known only in half of the cases. Retrospective analysis of their notes including documentation from the referring centre was undertaken. This included a review of all the histology and imaging. RESULTS: All patients had immediate severe pelvic pain on instillation of the MMC. Four of the six continue to have chronic pelvic pain. Two patients had urinary retention and three had severe lower urinary tract symptoms. One patient developed a frozen pelvis. Initial treatment was with an indwelling catheter for a period of 2-52 weeks to aid healing. Two patients had reconstructive surgery, one with success and the other with failure, as an intestinal patch failed to close the fistula and he continues with a catheter. One patient had an ileal conduit. No patient was warned of such complications. CONCLUSIONS: Although rare, prophylactic MMC can have devastating consequences. Patients should be aware of such major risks. Strong emphasis should be placed on the quality of the initial TURBT coupled with the judgement of an experienced surgeon before to MMC instillation. The real clinical benefit could be reviewed and intravesical MMC offered only to patients who have a good chance of benefit.

Evaluation of retained testes in adolescent girls and women with complete androgen insensitivity syndrome.

PURPOSE: To evaluate the magnetic resonance (MR) imaging appearance of the testes in women with complete androgen insensitivity syndrome (CAIS), including any benign or malignant changes. MATERIALS AND METHODS: This was a retrospective review of the testicular MR images and histologic reports from 25 patients with CAIS who chose to retain their testes beyond age 16 years and who were imaged between January 2004 and December 2010. Ethical approval was obtained, and informed consent was obtained from each subject to review the medical records, images, and histologic slides and reports. Imaging and histologic findings were compared. RESULTS: Twelve patients (mean age, 24 years; age range, 18-39 years) retained their testes and 13 (mean age, 22 years; age range, 17-37 years) eventually underwent gonadectomy. Review of the MR images showed that testicular parenchyma was heterogeneous in 30 of 46 testes (65%). The most common changes on MR images included simple-looking paratesticular cysts (34 of 46 testes, 74%) and low-signal-intensity, well-defined Sertoli cell adenomas (26 of 46 testes, 56%). Correlation of the histologic and MR imaging findings showed that MR imaging could correctly depict the presence or absence of Sertoli cell adenomas in 19 of 23 testes (83%). Paratesticular cysts were correctly detected in 22 of 23 testes (96%). Microscopic examination showed that the testes were composed of atrophic seminiferous tubules, whereas germ cells were found in 13 of 26 testes (50%). All paratesticular cysts were confirmed to be benign; however, a focus of intratubular germ cell neoplasia was found in a Sertoli cell adenoma. Premalignant foci were detected in three patients, two with intratubular germ cell neoplasia and one with sex cord tumor with annular tubules. No invasive cancers were found. CONCLUSION: MR imaging is accurate in the detection of testicular changes, including paratesticular cysts and Sertoli cell adenomas. Although these changes are usually benign, Sertoli adenomas can sometimes harbor premalignant lesions. MR imaging cannot depict premalignant changes; therefore, the standard of care for patients with CAIS should remain gonadectomy after puberty.

Persistent histological changes in the exstrophic bladder after primary closure-a cause for concern?

PURPOSE: We investigated bladder biopsies from patients with classic bladder exstrophy for the histological features and discuss the potential clinical significance of the findings. MATERIALS AND METHODS: Bladder tissues were collected from patients with bladder exstrophy between 2004 and 2011. These specimens were obtained at primary bladder closure (group 1, 29 patients), during secondary reconstructive procedures (group 2, 27) or during cystectomy for failed reconstruction (group 3, 15). All tissue specimens were investigated for inflammatory, proliferative, metaplastic and dysplastic changes. Expression of urothelial differentiation markers CK13 and CK20 was determined by immunohistochemical analysis. RESULTS: Inflammatory, proliferative and metaplastic changes were found in bladder specimens of all subgroups. Neither dysplasia nor neoplasia was present. Severe epithelial changes such as cystitis glandularis and intestinal metaplasia were observed in up to 62% of bladders several years after primary closure. Aberrant expression patterns of CK13 and CK20 suggesting abnormal urothelial differentiation were shown to be present in the urothelium of all subgroups. CONCLUSIONS: Our findings provide prima facie evidence that the epithelial changes observed in the unclosed bladder template persist or even progress in a subset of bladders after primary closure. Although the malignant potential of cystitis glandularis and intestinal metaplasia is controversial, some patients may be at increased risk for dysplasia/neoplasia in the long term. Since the natural history of these lesions in the exstrophic bladder is unknown, these patients require lifelong surveillance.

Adult care of children from pediatric urology.

PURPOSE: In this article we highlight the difference, from established adult urology, in required approach to the care of adolescents and young adults presenting with the long-term consequences of the major congenital anomalies of the genitourinary tract. We review some abnormalities of the kidneys, progressive renal failure and disorders of bladder function from which general conclusions can be drawn. MATERIALS AND METHODS: The published literature was reviewed and augmented with material from our institutional databases. For renal function the CAKUT (congenital abnormalities of the kidney and urinary tract) database at University College London Hospitals was used, which includes 101 young adult patients with CAKUT in whom the urinary tract has not been diverted or augmented. For bladder function some data are from patient records at Boston Children's Hospital. RESULTS: Adolescents who grow up with the burden of a major congenital anomaly have an overwhelming desire to be normal. Many achieve high levels of education and occupy a wide range of employment scenarios. Babies born with damaged kidneys will usually experience improvement in renal function in the first 3 years of life. Approximately 50% of these cases will remain stable until puberty, after which half of them will experience deterioration. Any urologist who treats such patients needs to test for proteinuria as this is a significant indicator of such deterioration. In its absence, the urologist must have a reasonable strategy for seeking a urological cause. The most effective management for nephrological renal deterioration is with angiotensin converting enzyme inhibitors, which slow but do not prevent end stage renal failure. Renal deterioration is generally slower in these patients than in those with other forms of progressive renal disease. The bladder is damaged by obstruction or by functional abnormalities such as myelomeningocele. Every effort should be made to stabilize or reconstruct the bladder in childhood. A dysfunctional bladder is associated with or causes renal damage in utero, but continued dysfunction will cause further renal damage. Bladder function often changes in puberty, especially in boys with posterior urethral valves who may experience high pressure chronic retention. Dysfunction is managed with antimuscarinic drugs, clean intermittent self-catheterization and intestinal augmentation. Adult urologists must be able to manage the long-term problems associated with these treatments. CONCLUSIONS: Pediatric conditions requiring management in adolescence are rare but have major, lifelong implications. Their management requires a broad knowledge of pediatric and adult urology, and could well be a specialty in its own right. Therefore, adult urologists must remain aware of the conditions, the problems that they may encounter and the special management required for these patients to live normal lives.

Adult care of children from pediatric urology: part 2.

PURPOSE: We describe the outcomes of undescended testes and sex development disorders in adolescence and young adulthood. We reviewed the requirements for the long-term care of children born with these and other major congenital anomalies of the genitourinary system. MATERIALS AND METHODS: The current English language literature was retrieved with a PubMed® search for articles on these subjects. Only articles covering outcomes at ages past puberty were included in analysis. The material was supplemented from the database of the clinic for adults with sex development disorders at University College London Hospitals. RESULTS: An undescended testis has impaired spermatogenesis. In men in whom a unilateral undescended testis was corrected before puberty the incidence of paternity is normal at around 90% of those who attempt it. The equivalent rate for those with bilateral undescended testes is about 65%. If surgery for bilateral undescended testes is delayed until after puberty, fertility is unlikely. The risk of testicular neoplasms is overestimated and the relative risk is between 2.5 and 8. Children born with a sex development disorder receive multidisciplinary treatment throughout childhood and require the same care as adults. Males who are under virilized likely have a micropenis (greater than 2 SD below the mean stretched length) but they may have normal sexual function. Fertility depends on the underlying condition. Virilized females, who most commonly have congenital adrenal hyperplasia, currently present to adult clinics with an inadequate vagina after infantile surgery. Reconstruction is required to allow intercourse. CONCLUSIONS: The care of adults born with abnormalities of the genitalia is complex. Early management may define upbringing in childhood but requirements for sexuality and fertility in adult life are different. Multidisciplinary care is essential and a case can be made to establish a subspecialty of urology to coordinate it.

The efferent segment in continent cutaneous urinary diversion: a comprehensive review of the literature.

OBJECTIVE: To critically assess the biophysical properties and current status of outlet formation in heterotopic intestinal urinary diversion. As despite three decades of clinical experience with continent cutaneous urinary diversion through bowel segments, no consensus has been reached for the optimal efferent segment although its function largely determines patient satisfaction. METHODS: A comprehensive Medline literature search using the Medical Subject Headings database (search terms: continent urinary diversion followed by either efferent segment, nipple, Mitrofanoff, Yang-Monti, Benchekroun, tapered ileum, intussuscepted ileum, Kock pouch, T-valve, or Ghonheim) was conducted to identify all full-length original articles addressing the various principles and techniques of outlet formation as well as their outcomes and complications. Examined series were published in English between 1966 and 2010. All studies were systematically evaluated using a checklist (study design, number of patients, etc.) and rated according to the Oxford Centre for Evidence-Based Medicine Levels of Evidence (LoE). RESULTS: While there was a continuous flow of publications over the last three decades, the vast majority of studies were retrospective case series with numerous confounding factors and poorly defined, non-standardized outcomes (LoE, 3). Only a few investigations compare different efferent segments (LoE, 2a). No randomized studies exist. The major biophysical principles are based on the use of flap, nipple, and hydraulic valves. Vermiform appendix, intussuscepted ileal nipple, and the Yang-Monti tube are the most popular techniques and have well-established data on outcomes, complications, and failure rates. Artificial sphincter systems and tissue engineering have provided disappointing results thus far. Most reconstructive strategies are subject to a process of on-going improvement. CONCLUSIONS: The continuous quest for optimization has not led to a single universally applicable efferent segment in continent cutaneous urinary diversion. While all techniques have their unique set of advantages and disadvantages, they will always remain a compromise. Success depends on selecting the optimal strategy for individual patients. A major change in principles in the near future is unlikely.

Animal models in urinary diversion.

We set out to critically assess the value of animal experimentation in urinary diversion through intestinal segments, as some authors question the effectiveness of animal research, criticising the methodological quality, lack of standardization, inadequate reporting and the few systematic reviews in this field. Based on a comprehensive MEDLINE literature search (MeSH database; search terms: urinary diversion, urinary reservoirs, continent, rat, dog, animal models) we retrieved and evaluated all full-length papers published in English, German, French, and Spanish languages from 1966 to 2011 reporting the use of animal models in the setting of urinary diversion. Studies were stratified according to the addressed research question. Within each category species, gender, number of animals, age at procedure, type of diversion, mortality, length of follow-up, experimental procedure and outcome were recorded and tabulated. In all, 159 articles were judged to be relevant and while there are numerous animal models only a few have been used in more than one study. Animals were used for the systematic study of new surgical techniques (93 articles) or metabolic and functional consequences of urinary reconstruction (66 articles). For the latter purpose, the most often used animal is the rat, whereas the dog model is preferred for technical experimentation. In many studies, the validity of the model is at least questionable. Animal experiments have repeatedly been conducted addressing the same question, often with striking discrepancies in outcome. Animal studies were even performed after a surgical technique had been pioneered in humans. The use of animal models in urinary diversion is far from standardized rendering the results less than ideal for comparison across studies. Due to differences in anatomy and physiology, the applicability of findings in animal experiments to clinical urology is limited. Continued effort is needed to optimise the use of animal models in experimental urology.

Genital prolapse in adult women with classical bladder exstrophy.

INTRODUCTION AND HYPOTHESIS: The aim of this paper is to study the incidence and risk factors for genital prolapse in adult women with bladder exstrophy and to describe the long-term outcome of the Gore-Tex wrap procedure for genital prolapse. METHODS: A retrospective observational study on adult women with classical bladder exstrophy in a tertiary referral centre for disorders of sex development, including complex urogenital anomalies. Medical notes were reviewed and a confidential postal questionnaire on urinary continence and symptoms of prolapse was carried out. RESULTS: Fifty-two women with bladder exstrophy and a mean age of 39 years (range 23 to 63) were identified, of whom 27 patients (52%) developed pelvic organ prolapse. Twenty-three out of 27 (85%) were treated surgically while only 4 patients were managed expectantly. Risk factors for prolapse included pregnancy in 10 (37%) patients and introitoplasty in 1 patient (4%). Of the 23 women treated surgically, 16 (70%) were treated using a Gore-Tex wrap, while 7 had other surgical procedures including colposuspension and hysterosacrocolpopexy. In the Gore-Tex wrap group, 12 out of 16 patients (75%) had had a successful result after the first repair compared with 2 out of the 7 patients (28%) in the group treated using other procedures. Mean follow-up was 8 years (range 1-15). One patient developed an infection after the procedure and another patient had Gore-Tex erosion. CONCLUSIONS: Prolapse is a common gynaecological complaint in adult women with bladder exstrophy and the majority will require treatment. At present the Gore-Tex wrap offers good results with a low rate of serious complications.

Guidelines for colorectal cancer screening and surveillance in moderate and high risk groups (update from 2002).

The British Society of Gastroenterology (BSG) and the Association of Coloproctology for Great Britain and Ireland (ACPGBI) commissioned this update of the 2002 guidance. The aim, as before, is to provide guidance on the appropriateness, method and frequency of screening for people at moderate and high risk from colorectal cancer. This guidance provides some new recommendations for those with inflammatory bowel disease and for those at moderate risk resulting from a family history of colorectal cancer. In other areas guidance is relatively unchanged, but the recent literature was reviewed and is included where appropriate.

Anorectal malformations: what happens in adulthood?

OBJECTIVE: To comprehensively evaluate the outcomes in adults who were born with anorectal malformations (ARMs), to assess the relationships between the major types of ARM and outcomes, and to examine the relationships between bowel and urinary function and sexual well-being, as advances in surgical treatment for ARMs have led to more patients surviving into adult life, and adult data on urinary and bowel function are scant, with even less known about the effect of diagnosis and treatment on sexual and reproductive function. PATIENTS AND METHODS: In all, 74 adult patients with a diagnosis of ARM confirmed by review of paediatric medical records completed a series of validated questionnaires on urinary and bowel function, body esteem, sexual well being, fertility and overall quality of life. RESULTS: There were high levels of urinary and bowel incontinence for both men and women. A high ARM was associated with a greater degree of incontinence. Both men and women scored more poorly than controls on the body esteem, sexual well-being and quality-of-life assessments. Urinary and bowel incontinence was associated with poorer sexual well-being. CONCLUSIONS: Bowel and urinary outcomes in adults appear to be worse than suggested in paediatric reports, with high levels of both urinary and fecal incontinence. Poorer outcomes appeared to affect sexual well-being adversely. To date, attention to issues during transition from childhood to adulthood, particularly concerns about sexual function and well-being, has been conspicuous by its absence. Patient-focused multidisciplinary care during this transition must address these issues, which are central to an acceptable quality of life.

Risk of bladder tumours after childhood cancer: the British Childhood Cancer Survivor Study.

OBJECTIVES: To estimate the risk of a second primary tumour (SPT) of the bladder in a cohort of childhood cancer survivors, investigate factors associated with a bladder SPT developing, and compare the risk observed with that expected from the general population. PATIENTS AND METHODS: The analysis included 17981 individuals diagnosed with childhood cancer, between 1940 and 1991 in Britain, and surviving for ≥5 years. Ascertainment of a bladder SPT was primarily through the National Health Service Central Registers (NHSCR). RESULTS: From the NHSCR, 17 bladder SPTs were ascertained; this corresponded to four times (95% confidence interval 2.5-6.4) the expected number of bladder tumours. Standardized incidence ratios (SIRs) varied significantly (P < 0.05) by first primary tumour (FPT) type, follow-up period, attained age and chemotherapy. The highest SIRs were in those: with heritable retinoblastoma (31.4); treated with chemotherapy (12.0); 0-9 years of follow-up (10.8); and aged 0-19 years (9.3). The absolute excess risk (AER) for a bladder SPT was 3.7 cases/100000 survivors per year. The AER varied significantly by FPT type, follow-up period, attained age and gender. The highest AERs were in those: diagnosed with heritable retinoblastoma (34.0); 20-29 years of follow-up (14.2); aged 40-49 years (13.0); and male (5.8). Using multivariable Cox regression, FPT and chemotherapy were significantly associated with the risk of a bladder SPT developing. By the age of 55 years, 0.4% of survivors developed a bladder SPT. CONCLUSION: Although the absolute risk of a bladder tumour within childhood cancer survivors was low, the risk was four times that expected from the general population. Specific groups, e.g. survivors of heritable retinoblastoma and those treated with chemotherapy, were at the highest risk.

Stabilization of renal deterioration caused by bladder volume dependent obstruction.

PURPOSE: Previously published data from our unit show the detrimental effect of excessive bladder filling at normal pressure on renal function in chronically dilated renal units. Synchronous cystometry and dynamic renography identified a critical volume of filling that prevents upper tract drainage. In this followup study we determined whether maintaining bladder volume below this critical level would halt renal deterioration. MATERIALS AND METHODS: Followup data were collected on 20 patients in the original study. All had progressive renal function deterioration for which no other cause was identified. Creatinine measured nearest to the time of the study renogram served as a baseline and subsequent values were used to monitor renal function. Data were analyzed by the paired Student t test. RESULTS: Complete data were obtained on 14 patients with a mean age of 34.4 years (range 22 to 70). The mean glomerular filtration rate at entry to this part of the study was 42 ml per minute per 1.73 m(2) (range 18 to 69). Four patients had a neuropathic bladder, 4 had posterior urethral valves, 4 had bladder exstrophy, 1 had radiation cystitis, 1 had a solitary pelvic kidney and detrusor failure, 5 had a native bladder and 9 underwent cystoplasty. Drainage was via the native urethra and a Mitrofanoff channel in 7 cases each. Mean followup was 27 months (range 3 to 39). There was no significant difference in mean +/- SD creatinine at baseline vs latest followup (168 +/- 72 vs 185 +/- 90 micromol/l, p >0.05). CONCLUSIONS: In patients with bladder volume dependent renal obstruction function can be stabilized by consistently maintaining bladder volume below the critical level.

Routine surveillance cystoscopy for patients with augmentation and substitution cystoplasty for benign urological conditions: is it necessary?

OBJECTIVE: To evaluate screening cystoscopy as the long-term follow up in patients with an enterocystoplasty for > or =10 years. PATIENTS AND METHODS: We performed a prospective analysis of 92 consecutive patients who attended our endoscopy suite for regular check cystoscopy as per standard follow-up. This is performed for all patients with cystoplasty performed at our institute after 10 years. The data were recorded on patient demographics, original diagnosis and type of cystoplasty. In all, 53 of these patients consented to undergo bladder biopsies at the same time. RESULTS: The median (range) follow-up was 15 (10-33) years. No cancer was identified with either surveillance cystoscopy or on routine biopsies. Chronic inflammation was identified in 25 biopsies (27%). Villous atrophy was present in 12 (55%) ileal patch and three (12.5%) colonic patch biopsies. During this study, the first and only case of malignancy in a cystoplasty at our institution was diagnosed in a symptomatic patient. She had intermittent haematuria and recurrent urinary tract infections (UTIs). She previously had a normal surveillance cystoscopy. CONCLUSIONS: We feel that it is not necessary to perform yearly check cystoscopies in patients with augmented bladders at least in the first 15 years, as cancer has not yet been detected with surveillance cystoscopy in this patient group. However, if the patient develops haematuria or other worrisome symptoms including suprapubic pain and recurrent unexplained UTIs a full evaluation, including cystoscopy and computerized tomography should be undertaken.

Vaginal restoration in survivors of childhood rhabdomyosarcoma.

Childhood genital rhabdomyosarcoma is a devastating diagnosis. Treatment is usually rigorous, comprising extensive surgery often with adjuvant chemotherapy and radiotherapy. This management is, however, increasingly successful, and survival into adolescent and adult life is becoming more commonplace. These young women will have sexual and reproductive expectations similar to those of their peers and will present to gynecologists for advice and treatment. This report describes the presenting problems seen in this group and highlights the complex nature of the investigations required and the treatments available.

Sexual function and genital sensitivity following feminizing genitoplasty for congenital adrenal hyperplasia.

PURPOSE: Female sex assignment followed by cosmetic genitoplasty to feminize the genitalia in infancy remains standard practice in the clinical management of ambiguous genitalia. The effects of surgery on genital sensitivity have never been objectively evaluated. To our knowledge the current study is the first to evaluate genital sensitivity and sexual function in women with congenital adrenal hyperplasia. MATERIALS AND METHODS: A total of 28 women with congenital adrenal hyperplasia and 10 normal controls were recruited. Details of prior genital surgery were obtained from medical records. Sensitivity thresholds for the clitoris and upper vagina were measured using a GenitoSensory Analyzer (Medoc, Ramat, Israel). Sexual function was assessed using a standardized measure. RESULTS: Of 28 women with congenital adrenal hyperplasia 24 had undergone feminizing genital surgery. In women who underwent surgery there was significant impairment to sensitivity in the clitoris compared to controls. No difference was observed for the sensitivity threshold in the upper vagina, where surgery had not been done in any of the women. Data on the 4 women with congenital adrenal hyperplasia who had not undergone surgery were similar to those in controls. Sexual function difficulties were more severe in women who underwent surgery, especially vaginal penetration difficulties and intercourse frequency. Linear relationships were observed for impairment to sensitivity and severity of sexual difficulties. CONCLUSIONS: Genital sensitivity is impaired in areas where feminizing genital surgery had been done and impairment to sensitivity are linearly related to difficulties in sexual function. The new information may help inform clinicians and parents making difficult decisions about genital surgery for infants with ambiguous genitalia.

Comparison of biochemistry and diet in patients with enterocystoplasty who do and do not form stones.

OBJECTIVE: To evaluate patients with a history of urinary stones in intestinal reservoirs and compare them with similar patients who have never formed stones. PATIENTS AND METHODS: One consequence of storing urine in an intestinal reservoir is urolithiasis, and there are several theories on its cause, all based on limited evidence. There are many factors predisposing to stone formation, and dietary and biochemical factors might be useful to distinguish those who form stones from those who do not. In a prospective study (with ethical committee approval) we identified 15 patients (six male and six female, mean age 29 years) who had formed stones after an enterocystoplasty (group 1), and another 10 (three male and seven female, mean age 44 years) with no history of urolithiasis after enterocystoplasty (group 2). The respective mean (range) follow-up was 14.6 (8-24) and 15.2 (6-23) years. They were investigated using our stone-screening protocol described previously and the results compared between the groups using an unpaired Student's t-test, with statistically significance indicated at P < 0.05. RESULTS: There was a statistically significant difference in almost all the variables assessed. The mean 24-h urine output was 41% higher (P = 0.009) and the mean citrate excretion 173% higher (P = 0.002) in group 2. The mean (range) urinary pH was 6.46 (6.0-7.0) and 6.93 (6.3-7.8) in groups 2 and 1, respectively (P = 0.005). Of the stone-forming elements, only the excretion of calcium was significant (2.78 vs 5.2 mmol/day, P < 0.001). The biochemical risk of stone formation was significantly higher for both calcium oxalate and calcium phosphate stones in group 1 than group 2 (P < 0.001 in both). From the dietary diaries there was a 24% higher fluid intake in group 2 (P = 0.04). The difference between group 2 and group 1 for the intake of magnesium (18.2 vs 12.38 mmol/day) and phosphate (49 vs 37.8 mmol/day) was statistically significant (P = 0.04 and 0.02, respectively). CONCLUSIONS: Apparently the use of bowel in the urinary tract does not alone increase the risk of urolithiasis. Patients in group 1 were more prone due to the constituents of urine and possibly their dietary habits. This implies that with adequate fluid intake and eating a healthy balanced diet, the risk of urolithiasis can be reduced in patients with enterocystoplasty, as with idiopathic stone formers.

Epithelial-stromal tumor of seminal vesicle in a patient with chromophobe renal cell carcinoma and small lymphocytic lymphoma.

Biphasic tumors of the seminal vesicle are rare. We report a further case in a 61-year-old man of a seminal vesicle epithelial-stromal tumor with focally atypical epithelial and stromal cells, the latter displaying a smooth muscle immunophenotype. In addition, this was associated with 2 synchronous malignant neoplasms, chromophobe renal cell carcinoma and small lymphocytic lymphoma, both of which were detected incidentally after clinical presentation because of the seminal vesicle mass.