Experiences of surgical continence management approaches for cloacal anomalies: a qualitative analysis based on 6 women.

STUDY OBJECTIVE: The aim of this qualitative study was to gain insight into health care experiences of young women diagnosed with cloacal anomalies, with a special focus on continence management. DESIGN: Qualitative analysis of one-to-one interviews. SETTING: A tertiary center for congenital anomalies of the urogenital tract in London. PARTICIPANTS: Six women aged 16 to 24 with cloacal anomalies. INTERVENTIONS: Tape-recorded one-to-one semi-stuctured interviews with a skilled interviewer. MAIN OUTCOME MEASURE(S): The taped interviews were transcribed and analyzed verbatim using interpretative phenomenological analysis according to the research question. Organizing themes across all of the accounts were identified. RESULTS: Two organizing themes concerning our research interests are summarized. The first theme Personal Agency in the Hands of Experts focuses on the interviewees' appreciation of their life-saving surgical care and their involvement in treatment decisions. The second theme Compromises and Trade-Offs focuses on what it was like to live with the more traditional versus the more advanced continence methods. Reliability emerged as a key priority in terms of continence treatment outcome. Gratitude may have interfered with the women's honest communications during treatment decision and evaluation consultations. CONCLUSIONS: A more developed approach to communication about the complex interventions proposed, founded on a nuanced understanding of users perspectives, can enhance informed decision making about continence management approaches. Despite these specific gaps, the interviewees were appreciative of their care and optimistic about life.

[Patients' acceptance of urinary diversion. The pouch of Sisyphus]. / Aspekte der Akzeptanz von Harnableitung: Der Pouch des Sisyphos.

It is important that any patient with a urinary diversion can accept the psychological impact alongside the surgical and physical aspects. However, there are currently no validated methods or instruments available to allow direct measurement of this phenomenon in these patients. Health-related quality of life (HRQoL) is often high following different types of urinary diversion-this may suggest a high acceptance level and thus may act as a secondary end point. Such an assessment is a retrospective validation of successful patient selection, allowing us to redirect the nihilistic misinterpretation that urologists should return to offering ileal conduits as a standard. In modern urinary diversion, high patient acceptance develops from comprehensive counselling providing a realistic expectation, careful patient-to-method-matching, strict adherence to surgical detail during the procedure and a meticulous lifelong follow-up. Coping strategies, disease-related social support and confidence in the success of treatment are among other factors which contribute to acceptance of urinary reconstruction as either independent or combined factors. Significant experience is required in every respect, as misjudgement and mistakes in any of these issues may be detrimental to the patients' health. It should be acknowledged that there is no 'best' urinary diversion in general terms. A reconstructive surgeon must have all techniques available and choices need to be tailored to the individual patient.

Supra-vesical urinary diversion and ureteric re-implantation for malignant disease.

Supra-vesical diversion or ureteric reconstruction is indicated for fistulae from the bladder or ureter, urinary incontinence, painful frequency and for end-stage renal failure due to obstructive uropathy. In a palliative setting, conservative measures, such as an indwelling catheter or ureteric stents, should be tried first. Open or laparoscopic surgery should be considered if these measures fail. For a patient who is leaking urine or has a very painful bladder, such surgery may well be justified, even very close to the end of life, as the symptoms are so unpleasant. When the problem is of end-stage renal failure that may be symptomless, the decision is more difficult; the patient may only gain a few months of life with no change in symptoms in return for the major surgery. The options available include cutaneous diversion either by ureterostomy or conduit and reconstruction either by re-implanting a ureter into the bladder or transuretero-ureterostomy. A laparoscopic approach may be possible in many cases.

Adolescent urology: A challenge for adult urologists.

The medical care of adolescents has become a growth area in many disciplines. There are two major aspects. Firstly, adolescents have specific medical and emotional needs which are not fulfilled either by paediatric or by adult specialists. Secondly, some childhood problems, particularly the congenital deformities, have no equivalent in adult life and so lifelong care is mandatory. Renal damage, especially dysplasia and scarring, leads to a substantial risk of early onset hypertension and, occasionally, to renal failure. Bladder outlet obstruction in utero, such as from a posterior urethral valve, causes irreversible changes to the wall that will act adversely on the kidneys in adolescence or early adulthood. The incidence of renal failure in early adulthood is about 36%. Bladder reconstruction with bowel has been very beneficial in preventing renal failure and improving continence. Life long follow-up is needed because of the high incidence of complications. These include stones, hyperchloraemic acidosis, perforation, anastomotic stenosis and, possibly, cancer. Patients have a normal expectation of sexuality and fertility. Their desires cannot always be achieved but they require considerable emotional and surgical support.

99mTechnetium-mercaptoacetyltriglycine scintigraphy with full bladder in patients with severe bladder dysfunction.

PURPOSE: We evaluated 99mtechnetium-mercaptoacetyltriglycine scintigraphy for detecting threshold bladder volume at which upper tract obstruction occurs in patients with bladder dysfunction. MATERIALS AND METHODS: A total of 24 patients 19 to 74 years old with severe bladder dysfunction who underwent 99mtechnetium-mercaptoacetyltriglycine scintigraphy and videocystometrogram in a 4-year period were selected for retrospective study. 99mTechnetium-mercaptoacetyltriglycine scintigraphy was done with a full bladder with a mean instilled volume of more than 850 ml saline. In patients in whom an obstructed renal outflow pattern was observed saline was drained at a rate of 100 ml every 5 minutes while dynamic imaging was performed. If results were abnormal, the study was repeated with an empty bladder. Differential function, parenchymal transit time index and outflow efficiency were calculated. RESULTS: Of the 24 patients 15 had an obstructed outflow pattern with a full bladder, which was relieved at a bladder volume of less than 390 ml (median 300, range 250 to 600). Only 2 of these 15 patients had a normal vesical end filling pressure of less than 20 cm H2O. There was no obstruction in 9 patients, of whom 5 had increased vesical end filling pressures. Followup in patients who had normal tracer outflow on a full bladder showed no decrease in renal function, while a small decrease was seen in patients who had obstructed outflow on a full bladder. CONCLUSION: This novel, full bladder 99mtechnetium-mercaptoacetyltriglycine scintigraphic technique provides the ability to detect bladder volumes at which obstructive outflow patterns develop in patients with severe bladder dysfunction.

Standing the test of time: long-term outcome of reconstruction of the exstrophy bladder.

The surgical management of classic bladder exstrophy has evolved over time. Different techniques are used to address the challenge of reconstructing these patients. We review the long-term outcomes of bladder exstrophy treatment from the published literature with regard to urinary continence, voiding and secondary complications. Continence now can be achieved in up to 80% of children in specialist centres. Whether such success can sustained into adult life is uncertain. About 40% of adults are dry in the best hands. Up to 84% of children can void, but there is some evidence that this function is lost with time in 70%. The need for bladder augmentation is widely variable between series, reported in 0-70% of children. This reduces the ability to void spontaneously to about 50% of children. It brings with it the later risk of metabolic disturbance and stone formation. Adults with exstrophy have a 694-fold increase in the risk of bladder cancer by the age of 40 years.

Metabolic factors in the causation of urinary tract stones in patients with enterocystoplasties.

Stones are a common complication of the storage of urine in intestinal reservoirs. Previous studies have identified predisposing physical characteristics in the reservoirs. Biochemical and dietary factors have been little investigated. Fifteen patients (6 males and 9 females) who had undergone various enterocystoplasty operations and who had subsequently formed either upper or lower urinary tract stones were investigated. The programme has been previously described and included stone, blood and urine analysis and dietary review. Comparison was made with 15 age- and sex-matched idiopathic stone formers with normal bladders. Stones were infective in origin in 86% of cases, and 14% were sterile. Metabolic screen showed that 80% of enterocystoplasty patients had risk factors for at least three different types of stone. All patients had raised pH (mean 6.93) and hypocitraturia. Five had a raised alkaline phosphatase. Raised serum and urinary calcium, hyperoxaluria and hyperuricosuria were found in 33% of patients. Five had a 24-h urine volume below 1.6 l/day. All patients had a high risk index (PSF) for phosphatic stones and 12 also for calcium oxalate stones. Compared to age-and sex-matched idiopathic stone-formers, the urine had a higher pH, sodium and protein excretion and a lower calcium and citrate excretion. Although the patients were already selected as stone-formers, the data show that metabolic and dietary factors are present. They may be as important in the aetiology of the stones, as the already recognised factors of infection and poor reservoir drainage. Investigation should include such factors, the presence of which may be taken into account in a prophylactic regime.

Modified ureterosigmoidostomy (Mainz II): a long-term follow-up.

OBJECTIVE: To assess the long-term results in patients treated using a modified ureterosigmoidostomy (Mainz II). PATIENTS AND METHODS: Between 1994 and 1999, 17 patients had their lower urinary tract reconstructed by a ureterosigmoidostomy, modified by reconfiguring the rectum to make a low-pressure reservoir (Mainz II). All patients were followed on a standard protocol. Data were extracted from the database and from a review of the case-notes. In 12 patients the procedure was with a radical cystectomy for carcinoma. Five had a failed conventional ureterosigmoidostomy for bladder exstrophy and therefore proceeded to a Mainz II. The data on continence and complications were retrieved for a retrospective analysis; the mean (range) follow-up was 6.4 (4-8.6) years. RESULTS: Ten of those with bladder cancer and one in the revision group were continent. Two patients in the revision group had sufficiently severe nocturnal incontinence to require conversion to a colonic conduit. Seven of the 17 patients had hyperchloraemic acidosis, one had pyelonephritis and one had renal stones. There were no anastomotic neoplasms. CONCLUSION: The Mainz II has a good outcome if used as the primary procedure. In patients with an existing ureterosigmoidostomy who are incontinent, detubularization of the rectosigmoid alone is unlikely to restore continence.

Long-term outcome of Fowler-Stephens orchiopexy in boys with prune-belly syndrome.

PURPOSE: Intra-abdominal testes in boys with prune-belly syndrome have been conventionally managed by 1 or 2-stage orchiopexy with division of the gonadal vessels. We reviewed a series of adults with prune-belly syndrome to assess the morphological and functional outcome of orchiopexy in childhood with specific reference to the spontaneous onset of puberty, hormonal profiles and sexual function. MATERIALS AND METHODS: A total of 41 boys were divided into 3 groups depending on the type of orchiopexy performed, namely group 1-20 with bilateral 1-stage orchiopexy, group 2-10 with unilateral 1-stage and contralateral 2-stage orchiopexy, and group 3-11 with bilateral 2-stage orchiopexy. RESULTS: In group 1 9 of 20 patients had good scrotal testes bilaterally, 6 had a good scrotal testis on 1 side and 3 had small testes on each side. Two boys required testosterone supplementation but 18 had normal hormonal and sexual function. In group 2 6 of 10 patients had good scrotal testes bilaterally and 4 had a good scrotal testis on 1 side. All patients underwent spontaneous puberty with good sexual function. In group 3 7 of 11 boys had good scrotal testes bilaterally and 3 had 1 good testis with normal puberty and sexual function. These 10 patients underwent spontaneous puberty with good sexual function. CONCLUSIONS: The majority of boys with prune-belly syndrome had a satisfactory outcome after orchiopexy with division of the gonadal vessels with testicular function sufficient to induce puberty and maintain satisfactory sexual function in adult life.

Genital sensation after feminizing genitoplasty for congenital adrenal hyperplasia: a pilot study.

OBJECTIVE: To assess sensation in the clitoris and vagina in women with congenital adrenal hyperplasia (CAH) who have previously had genital surgery, and to evaluate sexual function in this group as the latter, and particularly the experience of orgasm, appear to be closely related to sensitivity. PATIENTS AND METHODS: Six women were recruited from a multidisciplinary clinic specialising in intersex conditions, and representing an initial cohort from a larger ongoing study. The patients were asked to complete a postal questionnaire with a specialized sexual function assessment. Thermal, vibratory and light-touch sensory thresholds were assessed in the clitoris and vagina using a genito-sensory analyser and Von Frey filaments. RESULTS: All six women had highly abnormal results for sensation in the clitoris. Only three of them had an introitus capable of admitting the vaginal probe, and the vaginal sensory data of all three were within the validated ranges. A self-administered sexual function assessment was completed by the five women who were sexually active. The scores indicated sexual difficulties, particularly in the areas of infrequency of intercourse and anorgasmia. CONCLUSIONS: The sensory data for all six women were outside the normal range for the clitoris. The results for the upper vagina, which had not had surgery, were within normal ranges. These findings suggest that genital surgery may disrupt sensory input. Sexual function also appears to be impaired and this may relate to the compromised sensitivity and restricted introitus. The possibility that women with CAH have deficient clitoral sensation ab initio cannot be excluded. These striking findings must be evaluated further in the light of the controversy about the issue of genital surgery in children with CAH.

Pregnancy after lower urinary tract reconstruction for congenital abnormalities.

OBJECTIVE: To evaluate the effect of pregnancy on renal function, and the effect of congenital urinary tract abnormality and reconstruction on pregnancy and delivery. PATIENTS AND METHODS: The case notes were reviewed of 20 women (median age 32.5 years) who had had 29 live babies. Data collected included patient demographics, congenital urological abnormality, urological reconstructive procedure(s) and any subsequent urological complications. Pregnancy details, including urological and obstetric complications, presentation and mode of delivery, were obtained via a postal questionnaire from the relevant obstetrician. RESULTS: Seven patients had exstrophy-epispadias, seven spinal dysraphism, two sacral agenesis, and one each cerebral palsy, epispadias, imperforate anus and small bladder with vesico-ureteric reflux and congenital incontinence. They had had a mean (range) of 5.7 (1-12) urological reconstructive procedures each. Patients with exstrophy-epispadias had significantly more operations (mean 7.8) than those with spinal dysraphism (mean 4.14) or other diagnoses (mean 2.6) (P < 0.01). At the last follow-up 13 patients had an enterocystoplasty, six a neobladder and one an ileal conduit. Pregnancy-related urological complications were urinary tract infection in 15, upper tract obstruction requiring nephrostomy and stent in three, Mitrofanoff difficulties in two and pyelonephritis in one. There was no significant deterioration in glomerular filtration rate or serum creatinine after pregnancy. Only 10 of the births were normal or assisted vaginal deliveries. Seven patients had emergency and 12 had elective Caesarean sections for obstetric indications, including four breech births in the seven patients with vesical exstrophy. CONCLUSIONS: Pregnancy has no long-term effect on renal function and does not compromise reconstruction. Although there is a substantial complication rate and an increased need for Caesarean section, pregnancy in women with lower urinary tract reconstruction for congenital urological abnormalities is ultimately safe for both mother and baby. Interdisciplinary co-operation is desirable for a successful outcome.

The effect of enterocystoplasty on bone strength assessed at four different skeletal sites in a rat model.

The objective of the study was to investigate bone strength at four different skeletal sites in a chronic animal model of urinary diversion. Young male Wistar rats (120) were allocated randomly to four groups undergoing ileocystoplasty; ileocystoplasty and resection of the ileocecal segment; colocystoplasty; or sham operation (controls). After 8 months the lumbar vertebrae, femora, and tibiae were harvested at necropsy. Bone strength was assessed biomechanically at four different skeletal sites: vertebra L3, femoral middiaphysis, femoral neck, and distal femoral metaphysis. Bone mass and architecture were assessed using standard static histomorphometry of the proximal tibial metaphysis (trabecular bone volume [BV/TV]; trabecular number [Tb.N]) and ash weight. Statistically significant differences of biomechanical parameters between groups were observed at three skeletal sites with corresponding changes in tibial histomorphometry. Isolated ileocystoplasty resulted in decreased maximum load values of L3 (-16.4%; p < 0.0035) and a substantial reduction in tibial BV/TV (-34.7%; p < 0.05). Ileocystoplasty combined with resection of the ileocecal segment led to a significant loss of bone strength of L3 (-32.4%; p < 0.0015) and a dramatic reduction of tibial BV/TV (-45.9%; p < 0.01). Loss of tibial metaphyseal bone mass was predominantly caused by a decrease in Tb.N. (p < 0.01). Colonic augmentation had no significant effect on bone strength or histomorphometric values. In conclusion, this is the first experimental study to demonstrate the relevance of histomorphometrically proven bone loss after enterocystoplasty in terms of biomechanical variables.

[The effect of enterocystoplasty in childhood on linear growth]. / Längenwachstum nach Harnblasenaugmentation mit Darmsegmenten.

OBJECTIVE: The assumption that enterocystoplasty in children has a detrimental effect on linear growth has been based almost exclusively upon a chance finding in a retrospective study 10 years ago. We re-evaluated the same research question in a larger cohort and with a longer follow-up. PATIENTS AND METHODS: Between 1982 and 1997, 242 children and adolescents underwent enterocystoplasty. Patients with conditions involving organ systems apart from the urinary tract, and those with myelomeningocele, malignant diseases, reduced glomerular filtration rate and incomplete notes were excluded. In the definitive study cohort (123; mean age at operation 8.6 years; mean age at investigation 16.8 years), enterocystoplasty had been undertaken using colon in 70, ileum in 37, a combination of both in 11, ileocaecal segments in three and stomach in two patients. RESULTS: In all, 1215 height and weight measurements had been recorded. The distribution of percentile positions before and after enterocystoplasty showed a normal configuration, with 83 % and 80 % of patients growing within two standard deviations of the 50th percentile. After surgery, 85 % either remained the same or reached a higher percentile. Nineteen (15.5 %) were in a lower position, with a similar tendency in the weight percentile. A clinically relevant growth disorder was recognized in four patients with a complete endocrinological evaluation; in none of these was enterocystoplasty thought to be a causal factor. CONCLUSIONS: It is very unlikely that loss of the preoperative percentile position on the growth curve in 15 % of children after enterocystoplasty is a consequence of that particular surgery. Rather, it is a non-specific phenomenon that has to be considered in any clinical population of the same size and age distribution after the same length of time.

Skeletal growth and long-term bone turnover after enterocystoplasty in a chronic rat model.

OBJECTIVE: To investigate skeletal growth and bone metabolism in a chronic animal model of urinary diversion. MATERIALS AND METHODS: Young male Wistar rats (120) were allocated randomly to four groups undergoing: ileocystoplasty, ileocystoplasty and resection of the ileocaecal segment, colocystoplasty, and controls. All animals received antibiotics for 1 week after surgery; half of each group remained on oral antibiotics. Bone-related biochemistry was measured in serum and urine. Dual-energy X-ray absorptiometry and peripheral quantitative computed tomography (pQCT) were used to determine bone mass ex vivo. RESULTS: Most (90%) of the rats survived the study period (8 months); six rats died from bowel obstruction at the level of the entero-anastomosis and four had to be killed because of persistent severe diarrhoea. Vital intestinal mucosa was found in all augmented bladders. There were no differences in bone length and volume. Loss of bone mass was almost exclusively in rats with ileocystoplasty and resection of the ileocaecal segment (-37.5%, pQCT, P < 0.01). There was no hyperchloraemic metabolic acidosis or gross impairment of renal function. Hypomagnesaemia, hypocalcaemia and decreased insulin-like growth factor-binding protein 3 were the only significant findings on blood analysis. Deoxypyridinoline crosslinks in urine were higher in rats with an enterocystoplasty than in controls. CONCLUSIONS: Enterocystoplasty in rats neither impairs skeletal growth nor bone quantity, but leads to significant loss of bone mass when combined with resection of the ileocaecal segment. Rarefaction of the trabecular network is confined to the metabolically highly active cancellous compartment, most likely as a consequence of intestinal malabsorption.

Enterocystoplasty in childhood: a second look at the effect on growth.

OBJECTIVE: To re-evaluate the assumption that enterocystoplasty in children has a detrimental effect on linear growth (which is almost exclusively based upon a chance finding in a retrospective study 10 years ago) in a larger cohort and with a longer follow-up. PATIENTS AND METHODS: The original 12 children who had impaired linear growth in a previous study 10 years earlier were re-measured. A larger cohort was identified from the 242 children and adolescents who had undergone enterocystoplasty between 1982 and 1997. Patients with conditions involving organ systems apart from the urinary tract, and those with myelomeningocele, malignant diseases, reduced glomerular filtration rate and incomplete notes were excluded. In the definitive study cohort (123; mean age at operation 8.6 years; mean age at investigation 16.8 years) enterocystoplasty had been undertaken using colon in 70, ileum in 37, a combination of both in 11, ileocaecal segments in three and stomach in two patients. RESULTS: Of the original 12 patients, six had regained or surpassed their preoperative position on their growth charts. In all patients with a known target centile range the final height was within their genetic growth potential. In the cohort of 123 patients, 1215 height and weight measurements had been recorded. The distribution of percentile positions before and after enterocystoplasty showed a normal configuration, with 83% and 80% of patients growing within two standard deviations of the 50th percentile. After surgery, 85% either remained on the same or reached a higher centile. Nineteen (15.5%) were in a lower position, with a similar tendency in the weight centile. A clinically relevant growth disorder was recognized in four patients with a complete endocrinological evaluation; in none of these was enterocystoplasty thought to be a causal factor. CONCLUSIONS: It is very unlikely that the loss of the preoperative percentile position on the growth curve in 15% of children after enterocystoplasty is a consequence of the surgery. Rather it is a non-specific phenomenon that has to be considered in any clinical population of the same size and age distribution after the same length of time.

Current approaches to assessing the quality of life in children and adolescents.

Urologists are well aware of the importance of the quality of life (QoL) in determining the success of their treatments. The public are increasingly aware of this aspect of medicine. The advent of fetal ultrasonography has made knowledge of QoL in long-term survivors of congenital anomalies essential in counselling about pregnancy termination. It is becoming mandatory that clinical trials involving adults include an assessment of QoL. For children, measuring QoL has been restricted to life-threatening conditions such as cancer or transplantation medicine. Measuring QoL is difficult in children and adolescents, and this is reflected in the few suitable instruments available. The development of sexuality contributes a further difficulty as many anxieties typical of those with genital anomalies may be common to all teenagers. Several instruments rely on the opinions of a proxy (parent or carer) but self-assessment by the child is preferable where possible. The need for child self-assessment is supported by finding little concordance between child and proxy assessments. While measuring QoL is challenging, we provide a substantial case for greater consideration of QoL in paediatric urology.