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ABSTRACT: 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.© RSNA, 2013.
Radiology 03/2013; · 5.73 Impact Factor
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ABSTRACT: 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.
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.
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.
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.
International Urogynecology Journal 03/2012; 23(9):1201-5. · 1.83 Impact Factor
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ABSTRACT: 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.
BJU International 09/2011; 109(1):6-23. · 2.84 Impact Factor
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ABSTRACT: 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.
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).
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.
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.
BJU International 06/2011; 109(2):288-97. · 2.84 Impact Factor
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Christopher R J Woodhouse
Current opinion in urology 11/2009; 19(6):555. · 2.50 Impact Factor
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ABSTRACT: 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.
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.
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.
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.
BJU International 11/2009; 106(3):398-404. · 2.84 Impact Factor
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ABSTRACT: 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.
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.
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).
In patients with bladder volume dependent renal obstruction function can be stabilized by consistently maintaining bladder volume below the critical level.
The Journal of urology 08/2009; 182(4 Suppl):1973-7. · 4.02 Impact Factor
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ABSTRACT: To evaluate screening cystoscopy as the long-term follow up in patients with an enterocystoplasty for > or =10 years.
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.
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.
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.
BJU International 03/2009; 104(3):392-5. · 2.84 Impact Factor
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Christopher R J Woodhouse
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ABSTRACT: The commonest cause of neurogenic bladder in children is myelomeningocele. Survival of children is much improved in the Western world, but by 35 years old, about 50% will have died. In adults, the commonest causes of death are lung and heart diseases. All physical aspects deteriorate with age, especially in those with thoracic lesions. Those who walk in childhood have a 20-50% chance of becoming wheelchair dependent as adults. Immobility, poor respiratory reserve, obesity, latex allergy and worsening kyphoscoliosis contribute to the increased risks of surgery. It is essential that safe and manageable urine drainage is established in childhood: the bladder never improves with time, and surgical reconstruction becomes progressively more difficult. Independence in adult life will only be possible with intense preparation in childhood. Children must be allowed to join in with family chores and events. Education, both academic and practical, must be encouraged. Skills such as driving, shopping and birth control must be taught. However, even with the best support, less than 40% will have gainful employment. Children who are continent and have lesions below L2 are likely to have normal sexual function. Sexual activity in adolescents, especially in those with hydrocephalus, is limited (but not absent). However, by adult life, about two thirds will have established a regular partnership. All females and those males who are naturally potent are likely to be fertile. There is a high risk of neural tube defects in their offspring unless the female partner takes prophylactic folic acid for 3 months before pregnancy and for first trimester.
Pediatric Nephrology 09/2008; 23(8):1223-31. · 2.52 Impact Factor
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ABSTRACT: To evaluate patients with a history of urinary stones in intestinal reservoirs and compare them with similar patients who have never formed stones.
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.
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).
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.
BJU International 07/2008; 101(11):1427-32. · 2.84 Impact Factor
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ABSTRACT: 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.
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.
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.
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.
The Journal of urology 03/2008; 179(2):634-8. · 4.02 Impact Factor
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ABSTRACT: To examine the experiences of young people living with bladder exstrophy.
Qualitative interviews with 16 adolescents aged 16-21 years, exploring the psychological and social impact of bladder exstrophy.
Participants described difficulties, solutions, and practical assistance needed to manage living with bladder exstrophy. Participants identified practical difficulties, their identities, and relationships as the three main areas of importance. They discussed challenges associated with disclosing the nature of their condition, being bullied, and sexual relationships. Despite the difficulties and challenges described, young people repeatedly expressed a determination to live their lives as normally as their peers did.
Many examples of difficulties experienced in the past and the present are described. Young people report a range of creative coping strategies used to respond to the demands of living with a chronic health condition.
Journal of Adolescent Health 12/2007; 41(5):504-8. · 3.33 Impact Factor
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ABSTRACT: To examine the relevance of bladder volume in patients with chronic hydronephrosis and abnormal bladder function who lose renal function even though the bladder or reservoir pressure is normal (<40 cmH(2)0).
In all, 20 patients (16 male; age range 17-67 years) were studied prospectively; 12 had a reconstructed bladder. All had progressive loss of renal function with a glomerular filtration rate (GFR) of >15 mL/min. The study was in three parts: a baseline (99m)Tc-mercapto-acetyltriglycine (MAG3) scan was performed with an empty bladder, then a combined full bladder cystometrogram (CMG) with (99m)Tc-MAG3 study was done supine, and finally repeated in the sitting position. The pressure and volume changes together with the (99m)Tc-MAG3 scintigraphic variables with and without a full bladder were compared.
Of the 17 patients with normotensive bladders, 13 (77%) had functional obstruction of the kidneys with a full bladder. In seven this was threshold dependent and occurred at a total mean (range) bladder volume of 348 (135-720) mL for both positions. In the other six patients there was minimal drainage of isotope from the kidneys even with the bladder empty. When the sitting position was compared with supine, gravity was more important for upper tract drainage in the reconstructed bladders. In five patients the detrusor pressure at which drainage began was close to zero.
In 13 of 17 patients (77%) the kidneys failed to drain with a full bladder despite 'normal' detrusor pressures. It remains to be seen whether bladder emptying designed to keep the urine volume below the obstructing threshold volume will prevent further loss of renal function.
BJU International 11/2007; 100(5):1131-6. · 2.84 Impact Factor
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ABSTRACT: Authors from the UK present a prospective study into the effect of the full abnormal bladder on upper tract drainage, using a combined cystometrography procedure.A new transportable ESWL machine for managing urinary stones is presented by authors from Italy, and its use in 233 patients is described. Its merits and demerits are outlined.OBJECTIVE
To examine the relevance of bladder volume in patients with chronic hydronephrosis and abnormal bladder function who lose renal function even though the bladder or reservoir pressure is normal (<40 cmH20).PATIENTS AND METHODS
In all, 20 patients (16 male; age range 17–67 years) were studied prospectively; 12 had a reconstructed bladder. All had progressive loss of renal function with a glomerular filtration rate (GFR) of >15 mL/min. The study was in three parts: a baseline 99mTc-mercapto-acetyltriglycine (MAG3) scan was performed with an empty bladder, then a combined full bladder cystometrogram (CMG) with 99mTc-MAG3 study was done supine, and finally repeated in the sitting position. The pressure and volume changes together with the 99mTc-MAG3 scintigraphic variables with and without a full bladder were compared.RESULTSOf the 17 patients with normotensive bladders, 13 (77%) had functional obstruction of the kidneys with a full bladder. In seven this was threshold dependent and occurred at a total mean (range) bladder volume of 348 (135–720) mL for both positions. In the other six patients there was minimal drainage of isotope from the kidneys even with the bladder empty. When the sitting position was compared with supine, gravity was more important for upper tract drainage in the reconstructed bladders. In five patients the detrusor pressure at which drainage began was close to zero.CONCLUSIONS
In 13 of 17 patients (77%) the kidneys failed to drain with a full bladder despite ‘normal’ detrusor pressures. It remains to be seen whether bladder emptying designed to keep the urine volume below the obstructing threshold volume will prevent further loss of renal function.
BJU International 10/2007; 100(5):1131 - 1136. · 2.84 Impact Factor
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ABSTRACT: In the last 10 years there have been many reports of children and adolescents with varicocele, but virtually none from the UK. There is an increasing incidence with age, to 19% by the age of 19 years. There is some evidence that a varicocele impairs the development of the affected testis; 9.3% of boys of 19 years old have a small testis as a result. Some aspects of testicular function are compromised. Treatment of the varicocele allows compensatory growth and testicular function. The surgical technique must be meticulous to avoid complications, particularly the formation of a hydrocele. Despite much research, the effect on fertility is unknown. The incidence of varicocele is much higher than that of male factor infertility. It is not known whether varicocele in adolescence impairs fertility or whether surgery restores fertility. At present the main indications for surgery are persistent delay in growth of >20%, bilateral varicocele and impaired spermatogenesis persisting beyond 18 years old.
BJU International 10/2007; 100(4):744-7. · 2.84 Impact Factor
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ABSTRACT: In the last 10 years there have been many reports of children and adolescents with varicocele, but virtually none from the UK. There is an increasing incidence with age, to 19% by the age of 19 years. There is some evidence that a varicocele impairs the development of the affected testis; 9.3% of boys of 19 years old have a small testis as a result. Some aspects of testicular function are compromised. Treatment of the varicocele allows compensatory growth and testicular function. The surgical technique must be meticulous to avoid complications, particularly the formation of a hydrocele. Despite much research, the effect on fertility is unknown. The incidence of varicocele is much higher than that of male factor infertility. It is not known whether varicocele in adolescence impairs fertility or whether surgery restores fertility. At present the main indications for surgery are persistent delay in growth of >20%, bilateral varicocele and impaired spermatogenesis persisting beyond 18 years old.
BJU International 09/2007; 100(4):744 - 747. · 2.84 Impact Factor
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BJU International 08/2005; 96(1):22-7. · 2.84 Impact Factor
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ABSTRACT: To assess urinary symptoms in adult women with congenital adrenal hyperplasia (CAH), as feminizing surgery in infancy is current standard practice for CAH and one of the indications for surgery is to reduce urinary symptoms.
In a case-control study, 19 women with CAH, of whom 16 had had childhood feminizing genital surgery, and age-matched women with no CAH, were evaluated. Subjects and controls completed the Bristol Female Lower Urinary Tract Symptoms (BFLUTS) questionnaire.
Urge incontinence was reported in 13 (68%) patients and three (16%) controls (P = 0.003); stress incontinence was present in 47% and 26%, respectively (P = 0.31). Results from the controls were comparable with those documented in larger studies on normal populations. Nine of the patients felt that their urinary symptoms had an adverse effect on their lives, compared with only one of the controls (P = 0.008).
Patients with a diagnosis of CAH are more likely to have significant urinary symptoms than normal controls. At present it is not clear whether this is a result of surgery or an effect of CAH. In at least two-thirds of patients surgery did not achieve the objective of reducing urinary symptoms.
BJU International 07/2005; 95(9):1263-6. · 2.84 Impact Factor
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ABSTRACT: To evaluate a group of young women undergoing complex vaginoplasty, which may be defined as where there has already been previous complex pelvic surgery and the patient is unsuitable for conservative treatment or minor surgical revision.
All patients who had undergone a complex vaginoplasty between 1997 and 2004 were reviewed retrospectively. Information was recorded on the underlying condition, previous surgery and preoperative assessment. The operative technique and complications were noted, and the outcome in terms of menstruation and sexual activity. In all, 15 patients fulfilled the criteria (mean age 22.2 years, range 17-36) and the mean follow-up was 4.75 (0.75-7) years.
Fourteen patients had complex congenital anomalies of the lower genital tract and 11 of these had associated anomalies of the urinary and gastrointestinal system; four were XY females. The 15 patients had had 31 vaginal procedures, including ileal vaginoplasty, mobilization of perineal skin flaps and split-skin grafting. Three patients required osteotomies to increase pelvic outlet diameter. The commonest complications were stenosis (six patients) and fistula formation (four). Eight patients are now sexually active; the remaining seven have not attempted sexual intercourse.
Complications are common and can be major; most patients require many repeat operations to achieve a patent vagina. Unfortunately at present, this is the only option for this group of young women to achieve a functional vagina. Patients with complex congenital anomalies of the reproductive and genital tracts are increasingly surviving into adulthood, and the numbers of these patients is likely to increase in the future.
BJU International 07/2005; 95(9):1293-8. · 2.84 Impact Factor
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BJU International 06/2005; 96(1):22 - 27. · 2.84 Impact Factor
