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Summary Background The radical soft-tissue mobilization (RSTM, or Kelly repair) is an anatomical reconstruction of bladder exstrophy generally performed as a second part of a two-step strategy, following successful neonatal bladder closure. Objective The objective of this study is to determine the feasibility of a combined procedure of delayed bladder closure and RSTM in one stage without pelvic osteotomy, in both primary and failed initial closure. Design, setting, and participants From 11/2015 to 01/2018, 27 bladder exstrophy patients underwent combined bladder closure with RSTM by the same surgical team at four cooperating tertiary referral centers for bladder exstrophy, including 20 primary repairs (delayed bladder closure, median age 3.0m [0.5–37m]) and seven secondary repairs after failed attempt at neonatal closure, median age 10m [8–33m]. Intervention RSTM included full mobilization of the bladder plate, urogenital diaphragm, and corpora cavernosa from the medial pelvic walls, followed by anatomical reconstruction with antireflux procedure, bladder closure, urethrocervicoplasty, muscle sphincter approximation, and penile/clitoral reconstruction. Outcome measurements The main criteria were bladder dehiscence or prolapse. Secondary outcomes included bladder neck fistula or urethral fistula, urethral stenosis, and parietal hernia. Continence and voiding have not been addressed at this stage. Results and limitations All bladder exstrophy cases were successfully closed without osteotomy, with no case of bladder dehiscence after 12 m [3–30] follow-up. Complications Urethral fistula or stenosis occurred in eight patients: 4/5 fistulae closed spontaneously in less than 3 months; four urethral stenoses were successfully treated with 1–3 sessions of endoscopic high-pressure balloon dilatation or meatoplasty; one patient with persistent bladder neck fistula is currently awaiting repair. Although the follow-up is short, it does allow examination of the main outcome criterion, namely bladder dehiscence, which is usually expected to happen very early after surgery. Conclusion The Kelly RSTM can be safely combined with delayed bladder closure without osteotomy in both primary and redo cases in classic bladder exstrophy. Table . Primary delayed closure + Kelly Redo closure + Kelly n 20 7 Males/females 13/7 5/2 Age (mo.) 3.0 [0.5–37] 10 [8–33] Bladder dehiscence 0 0 Urethral stenosis 1/20 3/7 Urethral fistula 3/20 2/7

Objective In a retrospective review, to analyse the long-term outcomes of vaginal reconstruction, comparing techniques and timing. Patients and methods We identified 63 patients who underwent a total of 71 vaginoplasties in 1985–2000. The techniques used were posterior skin flap (42), intestinal replacement (21) and pull through (8). The majority of operations were performed before puberty (63%) and as primary procedures (79%). Presenting diagnoses were congenital adrenal hyperplasia (23), cloacal exstrophy (8), true persistent cloaca (12), androgen insensitivity (8), urogenital sinus anomaly (6), mixed gonadal dysgenesis (3), true hermaphroditism (2) and sacrococcygeal teratoma (1). Results The mean age at operation was 83.9 (2–235) and mean follow up was 116.8 (48–232) months. Strictures and discharge were the most common problems. Nine patients underwent revision surgery and a fistula repair was required in two patients. Mucosal prolapse was observed in one ileo- and one colovaginoplasty patient, and diversion colitis occurred after colovaginoplasty in one patient. More complications occurred in operations performed prior to than following puberty. Conclusion If vaginoplasty is the only indicated operation, delaying until puberty may minimize complications. When other genital surgery is indicated or in the presence of symptoms pre-pubertal vaginoplasty should be performed, since the second procedure usually involves simple dilatations and is associated with good results.

OBJECTIVE: To present our experience with the use of injectable polydimethylsiloxane (Macroplastique, Uroplasty, Minneapolis, MI, USA) for treating incontinence in children with the exstrophy-epispadias complex (EEC), as incontinence continues to be a challenging problem in such children, and although the primary management of EEC has developed over the last few decades, with early closure and reconstruction of the penis, achieving satisfactory continence status remains elusive. PATIENTS AND METHODS: We retrospectively reviewed the hospital records of 52 patients (41 boys and 11 girls, mean age at first injection 6.6 years, range 3.6-16.7) with EEC who had injections with Macroplastique between January 1991 and February 2004; 34 had bladder exstrophy and 18 primary epispadias. For this study we defined success as complete dryness with no use of pads or nappies. Improvement was defined as being occasionally wet but with dry intervals lasting >or= 4 h. RESULTS: The mean (range) follow-up was 4.6 (0.5-9) years. Twenty patients had one injection, 10 had two, 13 had three, six had four, two had six and one had seven injections. In most patients a maximum of three injections predicted the outcome. The injection of Macroplastique was successful in nine patients (17%; with an annual follow-up, two at 1-2 years, three at 2-5 years and four at >5 years), whilst 17 (33%) improved significantly (one at 5 years). Those patients comprised five of 18 (27%) with epispadias and four of 34 (12%) with exstrophy. A history of previous surgery and gender had no significant effect on the outcome. Overall half the patients benefited from the procedure. CONCLUSIONS: This series confirms that injection with Macroplastique is minimally invasive, durable in significantly many patients and has a reasonable success rate. A history of previous surgery and gender had no significant effect on the outcome. Patients with epispadias are more likely to benefit from an injection with Macroplastique than those with bladder exstrophy. A maximum of three injections is predictive with reasonable certainty of any benefit from the procedure

OBJECTIVE: To present a single series of Y-type duplication with an analysis of the presenting features, the management and outcome, as complete Y-type urethral duplication in the male is a rare congenital anomaly that presents many challenges. PATIENTS AND METHODS: We retrospectively reviewed patient charts, identifying those with urethral duplication, and then those with complete Y-type duplication. The age at presentation, investigations, management and outcomes were reviewed. RESULTS: Of 49 patients with a diagnosis of urethral duplication, 13 had the complete Y-type. Three were lost to follow-up and were therefore excluded from the study. The median (range) age at presentation was 2 months (birth to 10 years). All patients had other comorbidities, with anorectal malformations in seven, renal agenesis in four, sacral agenesis/partial agenesis in two, and unilateral/bilateral cryptorchidism in four. Presenting features included passage of urine perineally or rectally in seven, and dribbling from the orthotopic urethral meatus in one. The diagnosis was confirmed by micturating cysto-urethrography. A mean of 14 procedures was required to achieve a final complete repair, and included urethral reconstruction, repair of fistulae, urethral dilatation, and evaluative cystoscopy. Excluding cystoscopy and urethral dilatation, a mean (range) of 3 (1-5) reconstructive procedures was required in each patient. The median follow-up was 8 years. Five boys developed urethral strictures and one developed multiple fistulae. The best outcomes were in boys who had a staged urethral reconstruction. CONCLUSION: Due to the strong association with other congenital anomalies we recommend that all patients should be evaluated thoroughly, including a detailed physical examination, renal tract ultrasonography and spinal radiography. Surgical management remains a significant challenge, requiring many procedures with unpredictable outcomes, the goals of which should be to maintain continence and reconstruct the urethra with good cosmesis. The optimum management scheme must be individualized in this rare condition

Authors from the USA and UK evaluate female patients’ perception of genital anatomy, and estimate the implications of this for genitoplasty. There are several important observations by the people who completed the questionnaire which should lead surgeons who perform genitoplasty to remember the functional aspects as well as the anatomical appearance after this operation. OBJECTIVE To assess the perceptions of healthy women of their genital anatomy and sexual sensitivity, and to provide suggestions for genitoplasty based on this information, as the success of genitoplasty has historically relied upon the surgeon's perception of the patient's anatomy and function, rather than the patient's perception of outcome in terms of appearance and erotic sensitivity. SUBJECTS AND METHODS Fifty healthy, sexually active, adult women (aged 20–56 years) with no history of genital surgery completed the female version of the Self-Assessment of Genital Anatomy and Sexual Function. This self- report questionnaire comprises written text and images enabling women to rate the appearance, size and position of clitoris and vagina, as well as the intensity of orgasm and effort required for achieving orgasm in specified areas around the clitoris and within the vagina. Anatomical locations were compared for these ratings by repeated-measures analysis of variance. RESULTS Anatomically, 46% of women described their clitoris as ‘moderate-sized and raised’, 42% as ‘small and raised’, and 78% reported that their vaginal opening was adequate for sexual penetration. The women reported the strongest orgasm and least effort to obtain an orgasm with stimulation of the area on and above the clitoris. For vaginal sensitivity, scores for orgasm intensity increased, and for orgasm effort decreased, with increasing vaginal depth, and they indicated less sexual sensitivity for the vagina than for the external genitalia. CONCLUSION The skin above the clitoris, and the clitoris itself, appeared to be the most sexually sensitive. During genitoplasty, attention to preserving skin-flap integrity in this area seems appropriate.

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.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.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.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.

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.

Purpose: Persistent cloaca is a complex malformation with variable presentation that remains a difficult reconstructive challenge. Previous reviews have concentrated on surgical technique, and data on long-term outcome are scarce. We evaluate long-term outcome and when possible correlate outcome with anatomy at presentation.Materials and Methods: The records and radiographs of 64 patients 0.5 to 25 years old with persistent cloaca treated at 1 institution from 1975 to 2000 were retrospectively reviewed to determine the outcome for urinary and fecal continence. Outcome data were available in 61 girls as 3 died. Of these patients 11 were younger than 3 years, making evaluation of continence difficult, and so the remaining 50 patients constitute the study population. Mean patient age at review was 11.3 years (range 4 to 25).Results: Of the 50 patients 10 (20%) are incontinent of urine (4 with urinary diversion) and 40 (80%) are socially continent, including 11 (22%) who void spontaneously, 6 (12%) wh...

Purpose: Cloaca is a complex malformation in which the rectum, vagina and urinary tract open into a single common channel. Functional results after reconstructive surgery have been documented but the renal outcome is less clearly understood.Materials and Methods: The records of all patients with cloacal malformation treated at our institution from 1980 to 2000 were retrospectively reviewed to determine the renal outcome. All patients underwent serial ultrasound of the urinary tract, voiding cystography, nuclear renography, lumbosacral radiography, and measurement of serum creatinine and glomerular filtration rate when appropriate.Results: We identified 64 patients 0.5 to 19 years old (mean age 11.2) at the time of the study. Of the 64 patients 53 (83%) were born with a structural abnormality of the urinary tract, including renal dysplasia in 17 (27%), ectopic kidney in 9 (14%), solitary kidney in 8 (13%), duplex kidneys in 6 (9%) and ureteropelvic junction obstruction in 3 (5%). Vesicoureteral reflux was ...

Objective To describe the clinical features, treatment and outcome of functioning adrenocortical tumours in children, and to assess the value of tumour size as a marker for malignant potential. Patients and methods Twenty-one children (mean age at presentation 4.9 years, range 0.6–11; 12 girls and nine boys) who presented with a functioning adrenal mass over a 29-year period (1972–2000) were assessed. Clinicopathological features and outcome information were obtained from a structured review of the case-notes. Before 1980 the diagnosis and staging was mainly obtained by intravenous pyelography and plain X-ray. Since 1980, ultrasonography, computed tomography and more recently magnetic resonance imaging were the methods of choice. Results The mean (range) duration of symptoms before diagnosis was 31 (2–108) months. The symptoms and signs at presentation comprised virilization (18 children), Cushingoid features (three), precocious puberty (three) and hypertension (four). Three children had malignant neoplasms, one presented with metastasis and of the other two, one died from the disease within 6 months, despite adjuvant chemotherapy and radiotherapy. The third girl is disease-free 11 years after complete excision of the neoplasm. Eighteen children had a benign neoplasm and all are alive and free of recurrence. In most of the children surgical extirpation was relatively simple, blood loss minimal, recovery uneventful and the hospital stay short (mean 5 days). The virilizing effects of all the neoplasms gradually resolved after surgery within a median (range) of 27 (6–108) months. Conclusions Functioning adrenocortical neoplasms in children are rare, with a peak incidence in the first decade, are predominantly unilateral and more common in girls. Most are benign, but both benign and malignant lesions can present with virilizing and Cushingoid features. The malignant neoplasms have an extremely poor prognosis, especially if excision is incomplete, despite adjuvant chemotherapy and radiotherapy. Although there is an association between tumour size and malignancy, this cannot be used as a reliable individual discriminator.

Objective To compare the differences in the quality of Mitrofanoff channels created using appendix and re-tubularized small bowel (the Yang–Monti ileovesicostomy). Patients and methods The case-notes were reviewed retrospectively for all patients who underwent a Mitrofanoff procedure using either appendix or small bowel, over a 5-year period from June 1994 to July 1999. Results In all, 92 patients underwent 94 Mitrofanoff procedures; the appendix was used in 69 and small bowel in 25. The underlying diagnoses were exstrophy-epispadias complex (38), neuropathic bladder (21), anorectal malformations and cloacal anomalies (15), posterior urethral valves (nine) and miscellaneous (nine). The mean (range) age at operation was 9.2 (1.1–18.3) years. The mean (range) follow-up for the appendix group was 37 (6.7–65) months and for the Monti group 25 (6–66) months. Catheterization problems occurred in 18 (27%) patients from the appendix group; two needed an adjustment of technique, six dilatation and 10 revision. Stomal stenosis occurred in 10 (15%) patients, bladder level stenosis in four (6%) and conduit necrosis in two. Catheterization problems were reported in 15 (60%) patients from the Monti group; five needed revision, three dilatation and seven are being managed conservatively. The incidences of stomal stenosis (four, 16%) and bladder level stenosis (two, 8%) were comparable with the appendix group. In addition, two patients had distal channel (sub-stomal) stenosis and two had mid-channel stenosis. The problem unique to the Yang–Monti channel was a pouch-like dilatation in seven patients (28%), all of whom presented with catheterization problems; five are being managed conservatively and two have needed pouch resection. Stomal prolapse occurred in five (7%) patients in the appendix group, but in none of the Monti group. Conclusions The appendix is the conduit of choice for a Mitrofanoff procedure. Re-tubularized small bowel conduits have a considerably higher incidence of catheterization problems. Anatomical factors may contribute to the unique incidence of pouch formation.

Objective To examine, in infants presenting with vesico-ureteric reflux (VUR), the relationship between the presence of initial renal abnormalities with the outcome of VUR and bladder function at 16 months of age. Patients and methods The study group comprised 40 infants (32 boys) presenting consecutively (29 after prenatal detection) with VUR grade III or greater (bilateral in 29) on the initial micturating cystogram (median age 8 weeks). The initial presence of abnormal kidneys was determined from isotopic renography and/or ultrasonography. These data were correlated with the outcome of VUR, from direct isotope cystography, and bladder function assessed by natural filling urodynamics, examined at age 16 months (mean 16.4 months, sd 2.1). Results Three groups were identified. Group 1 (eight boys and six girls) had normal kidneys bilaterally; initially grade III VUR was common. At 16 months bladder function was normal in 10 children and none had VUR (complete resolution). Group 2 (14 boys and two girls) had unilateral renal abnormalities; initially VUR was predominantly grade IV or grade V. At 16 months bladder function was normal in eight children and VUR resolved in eight, five of these with normal bladder function. Group 3 (10 boys) had bilateral renal abnormalities. Initially grade V VUR predominated; at 16 months the bladder function was normal in only one, and in the rest the emptying dynamics were abnormal. All 10 boys had persisting VUR (no resolution). Conclusions In infants with moderate or severe VUR, resolution at 16 months old is associated with normal kidneys in a similar proportion of boys and girls. Resolution also correlates well with normal bladder function. Presentation in infancy with bilateral abnormal kidneys, associated with severe VUR in boys, is a poor prognostic sign for the early outcome of VUR and for bladder function.

BACKGROUND: Nephropathy associated with vesicoureteric reflux (VUR) and urinary tract infection can result in end-stage renal failure, hypertension, or both. Whether long-term VUR contributes to these outcomes is unknown. We compared, in a randomised trial, medical with surgical management of children with bilateral severe VUR and bilateral nephropathy. METHODS: We stratified by age and glomerular filtration rate (GFR) 25 boys and 27 girls aged 1-12 years and randomly assigned them to medical or surgical management. At enrolment and 4 years' follow-up we estimated GFR from the plasma clearance of 51Cr-labelled edetic acid (EDTA), and did intravenous urography. We also did a metastable 99mTc-labelled dimercaptosuccinic acid (DMSA) assay and contrast cystography. The change in GFR at 4 years, expressed as a percentage change between enrolment and 4 years, was available for 26 of 27 patients in the medical and 24 of 25 in the surgical group. We assessed GFR in 48 patients 10 years after enrolment. FINDINGS: Mean GFR at enrolment was 72.4 mL/min per 1.73 m(2) (SD 24.1) in the medical and 71.7 mL/min per 1.73 m(2) (22.6) in the surgical group. The mean percentage change in GFR at 4 years was 2.4% (SE 4.5) versus 4.7% (5.0) in the medical and surgical groups, respectively. The difference in change in GFR at 4 years between the two groups was not significant (7.1%, 95% CI 6.4% to 20.6%). INTERPRETATION: Our data do not lend support to the view that the outcome for renal function is improved by surgical correction of VUR in children with bilateral disease.

Fecal soiling or intractable constipation frequently occurs in association with urinary incontinence in children undergoing major reconstructive urological operations. To treat double incontinence or the combination of wetting and severe constipation, we constructed a Mitrofanoff conduit and a channel for antegrade continence enemas in 18 patients between 1989 and 1995. We review the underlying pathological conditions, various surgical techniques and outcomes of these operations.Underlying abnormalities mainly included spinal lesions, bladder exstrophy, imperforate anus and various cloacal anomalies. Patient age ranged from 2 to 18 years (average 8.4). In 13 patients both procedures were done simultaneously. The appendix was used to construct the antegrade continence enema channel in 8 cases and the Mitrofanoff channel in 5. It was long enough to be divided and used for both procedures in 2 cases but it was missing or unsuitable in 3. Alternative antegrade continence enema conduits were cecal flap in 7 patients and ileum in 1, while the ureter, ileum and detrusor tube were used to establish Mitrofanoff channels in 5, 5 and 1, respectively. Stomas were constructed according to the V-flap or V. Z. Q. technique and situated in close proximity in the right lower abdominal quadrant in 13 cases.Convalescence was uneventful except for 1 abscess near an antegrade continence enema stoma. Ten patients needed dilation or minor revisions due to difficulty in catheterizing the antegrade continence enema (5), Mitrofanoff (3) or both conduits (2). Subsequently 3 patients underwent repeat operations for reconstruction of 2 antegrade continence enema channels (cecal flap and ileum) and 1 detrusor tube Mitrofanoff channel. Currently 15 patients are dry on regular clean intermittent catheterization using 10 to 12F catheters. Outcomes of the antegrade continence enema channels are satisfactory in 15 patients who are clean or rarely soil. Failure occurred in 1 patients with severe constipation necessitating colostomy and 2 (1 noncompliant who stopped catheterizing regularly) in whom the channels subsequently closed.Synchronous construction of antegrade continence enema and Mitrofanoff channels is successful in the majority of doubly incontinent patients. Selection of patients with high motivation is important to obtain satisfactory results.

Purpose: In 1986 we reported the placement of a silicone sheath sandwiched between layers of omentum around a newly reconstructed bladder neck. We now present long-term followup of 94 cases of silicone sheath bladder neck reconstruction.Materials and Methods: A total of 94 silicone sheaths was placed in 84 patients between August 1983 and October 1992. We retrospectively reviewed our results and divided the reconstructions into 3 groups according to modifications in surgical technique. We report the results of each modification and current recommendations for use.Results: Each sequential modification of silicone sheath bladder neck reconstruction significantly reduced the risk of erosion from 100 percent, 32 percent 7 percent, respectively (p less than 0.05). Erosion was independent of patient age, sex, pathological condition or whether bladder neck reconstruction was a repeat procedure. Patients who had silicone sheath erosion did not have different continence or loss of urethral continuity than ...

We describe a novel, double lumen, intravesical, suprapubic catheter designed to meet the requirements of pediatric urodynamics that is easy to use and has minimal complications.A commercially available 10Fr pediatric suprapubic pigtail catheter forms the outer lumen for instilling filling media. A 16 gauge epidural catheter is inserted through the outer catheter providing an inner lumen for measuring intravesical pressures. The resultant double-lumen catheter is inserted suprapubically using a peel away needle supplied with the 10Fr catheter, with the patient under general anesthetic.The catheter has been used for 15 years in more than 700 patients with good reliability and few complications. The concentric construction of the double lumens and the rigidity of the inner intravesical pressure channel ensure there is no transmission of pressure from the filling channel to the inner lumen. The catheter has a circular cross section and a pigtail distal end which help to retain it within the bladder. There is low resistance to filling that allows adequate filling rates to be achieved by gravity rather than necessitating a pump. The catheter is easily made from readily available components and is less expensive than other double-lumen catheters suitable for suprapubic use.A reliable, double lumen catheter that fulfills criteria not found in commercially available alternatives can be inexpensively made for urodynamics.

Objective To review retrospectively the value of the human chorionic gonadotrophin (hCG) test in the evaluation of prepubertal boys with bilateral impalpable testes. Subjects and methods The study comprised 31 boys investigated between 1974 and 1990 at the Hospital for Sick Children, London. All boys had an hCG test consisting of three intramuscular injections of hCG on successive days at a daily dose dependent on their age ( 10 years, 1500 units). Blood samples were taken before the first dose and 24 h after the last dose and the level of plasma testosterone assessed and expressed as a pre/post ratio. Results Eight boys had no response to hCG, due to anorchia. One boy had no response to hCG but had bilateral atrophic intra-abdominal testes. Twenty-two boys responded to hCG and had testes whose size was related to the degree of testosterone elevation after this stimulatory test. The hCG test therefore had a positive predictive value of 89% and a negative predictive value of 100%. There was a quantitative difference in testosterone response between 14 boys who had bilateral intra-abdominal testes of ‘normal’ volume (median pre/post ratio, 11.4) and nine boys who had an otherwise reduced volume of testes (dysplastic or unilateral intra-abdominal) (median pre/post ratio of 4; P = 0.02). Conclusion The hCG test is a valid indicator of the presence of functioning testicular tissue. It is predictive of anorchia and a good response to hCG suggests the presence of testes sufficiently large for orchidopexy.

Objectives To evaluate normal bladder function and micturition patterns in infants. Patients, subjects and methods Twenty-one infants (16 boys, five girls; mean age 5.9 months) with no lower urinary tract pathology underwent natural filling cystometry. Micturition patterns were also observed simultaneously with polysomnography in 26 healthy neonates (16 boys, 10 girls; mean age 7.4 days). Results In infants, cystometry showed (95% CI) a capacity of 42–53 mL, a maximum rise in detrusor pressure during voiding of 95–120 cmH2O and a voiding efficiency (voided volume/capacity) of 0.86–0.91. On micturition, urinary flow was discoor-dinated from peak detrusor pressures in 10 infants. Detrusor instability occurred in one of 21 infants. Micturition was observed only during wakefulness or on arousal from sleep. In neonates, 17 of 61 recorded voids (28%) were during full wakefulness and 44 (72%) during arousal from sleep. Notably, none of the recorded voids occurred during quiet sleep. Conclusions The normal infant's bladder was stable and emptied almost completely. Voiding with incomplete co-ordination between detrusor contraction and urinary sphincter relaxation could be normal. Micturition never occurred during quiet sleep. There was cortical arousal in response to a full bladder even in new-born infants. This contradicts the traditional concept of a totally uninhibited bladder in infancy. There are potential implications for the management of children with nocturnal enuresis.

From 1978 to 1993, 180 boys with epispadias (85) and the exstrophy/epispadias complex (95) underwent epispadias reconstruction at our institution. The 180 patients were divided into 4 groups: group 1–2-stage epispadias reconstruction with dissection of the corpora (30 patients), group 2–pedicled preputial tube urethroplasty either alone or with a lyophilized human dural patch to the corpora for correction of penile deformity (35), group 3–pedicled preputial tube urethroplasty and corporeal rotation (40), and group 4–a modified Cantwell epispadias repair incorporating complete tubularized urethroplasty, cavernocavernostomy and corporeal rotation.In 46 patients from groups 1 to 3 the primary outcome was not considered satisfactory and they underwent radical penile revision with the modified Cantwell technique. Followup ranges from 1 to 15 years (mean 6 years). We conclude that a good cosmetic result can be achieved in almost all cases using the modified Cantwell technique as a primary procedure in e...

The ideal timing and nature of surgical reconstruction in individuals with Disorders of Sex Development (DSD) is highly controversial. Despite the increasing number of publications on this topic, evidence-based recommendations still cannot be made. However it is generally accepted that optimal care for DSD requires an experienced multidisciplinary team. This means that surgical decisions are now made within the context of a multidisciplinary team and all members of the team – and not just specialist surgeons – may be called upon to discuss choices for surgery with patients and parents. To do this well, every clinician in the team should have an understanding of the range of techniques available for genital surgery, the risks and benefits of procedures and the controversies surrounding timing of surgery. The aim of this paper is to give an overview of the variety of surgical procedures in current use and in what situation a particular technique would be indicated. The short-term risks and benefits are described and where available long-term outcome data is discussed. To date, discussions surrounding genital surgery have been led primarily by surgeons. Some non-surgical clinicians have expressed unease about decision making in genital surgery but have felt ill equipped to comment on an area with which they are unfamiliar. This review gives a detailed explanation of current surgical practice offered in a specialized center for DSD and such information should facilitate a more balanced discussion.

Seven boys (mean age 38 months) with posterior urethral valves underwent renal transplantation between June 1988 and August 1991. Urodynamic studies were performed before transplantation in 6/7 patients. In 4 the investigation indicated bladders of capacity and compliance which were deemed suitable for transplantation. Two patients had poorly compliant bladders; one of these underwent bladder augmentation before engraftment and the other proceeded to transplantation without bladder surgery. Six patients have functioning renal allografts with a mean follow-up of 1.3 years and a mean plasma creatinine of 51.6 mumol/l. Mean glomerular filtration rate (ml/min/1.73 m2 SA) 6 months after transplantation was 76.8 and at 1 year it was 84.5. In one patient early rejection was followed by transplant nephrectomy. Careful pre-operative evaluation is mandatory for a successful outcome of renal transplantation in young boys with posterior urethral valves.

The antenatal histories of 42 patients with posterior urethral valves diagnosed between June 1987 and September 1990 were reviewed. The mothers of all patients had at least one ultrasound scan during pregnancy. Despite this, fetal uropathy was diagnosed in only 19 cases. The remaining 23 undiagnosed children presented acutely, all within the first 6 months of life. In 33 of 36 pregnancies scanned before 24 weeks' gestation, fetal urological pathology was undetected. Mean plasma creatinine (pCr) at presentation in the group antenatally diagnosed was 139 mumol/l and in those presenting acutely was 238 mumol/l. All pCr analysed were taken after at least 48 h of life. Renal function as measured by follow-up pCr was better in the antenatally diagnosed group during the first year of life. It would appear that a routine second ultrasound scan at 26 weeks' gestation or later would reveal more cases of posterior urethral valves and this information may improve the outcome in terms of renal function.

Summary— We report our experience of gastrocystoplasty in 8 children (mean age 10.1 years) with compromised renal function (mean creatinine 186 mmol/l, mean glomerular filtration rate 39 ml/min/ 1.73 m2). Current follow-up ranges from 11 to 35 months (mean 21). The physiological outcome of the procedure has been excellent, with improved biochemical and urodynamic parameters in all cases. Six patients are off H2-receptor blockers and are asymptomatic. In 2 children we have encountered significant symptoms related to acid secretion in the bladder. Detailed investigations suggest that the excess aciduria is related to the size of the gastric patch in 1 patient. The second child underwent renal transplantation 5 months after gastrocystoplasty and it is postulated that his immunosuppressive regimen (which includes prednisolone) may be responsible for the increased acid secretion. It is concluded that gastrocystoplasty is a very satisfactory alternative to intestinal segment bladder enhancement in children with compromised renal function, but the size of the gastric patch is critical in determining the resultant acid secretion. Post-transplantation immunosuppressive therapy may increase acid production from the gastric patch; since the children most suitable for gastrocystoplasty are also those likely to require transplantation, this aspect requires further study.

A total of 112 patients (142 kidneys) presented with hydronephrosis consistent with ureteropelvic junction obstruction that had been diagnosed by prenatal ultrasound. The kidneys were classified as having poor, moderate or good function based on isotope imaging at 3 months after birth. Of 9 kidneys that showed poor function 3 recovered sufficient function on pigtail drainage to justify preservation and these patients underwent pyeloplasty. Of 27 kidneys with moderate function 23 also underwent pyeloplasty and 14 of these demonstrated improvement in function postoperatively. Of the 100 kidneys in the good function group that were followed conservatively 23 underwent pyeloplasty during followup primarily because of an observed decrease in function. We propose that there is no indication for immediate pyeloplasty in infants with prenatally diagnosed hydronephrosis who demonstrate good function postnatally.

Buried penis is a congenital abnormality in which the phallus is concealed within the subcutaneous tissue. The corporal bodies and glans have developed normally. A deficiency of penile shaft skin in association with abnormal mobility of the skin gives rise to the phenotypic appearance. The skin defect may be related to an abnormal attachment of dartos muscle during embryogenesis. Correction of this condition requires separation of these abnormal muscular attachments and provision of skin coverage of the penile shaft. It is imperative not to circumcise the patient as this will worsen the skin deficiency. We describe a novel technique for correcting buried penis performed on 12 patients at the Great Ormond Street Hospital for Sick Children. A satisfactory cosmetic result was obtained in all patients.

The aim of this study was to determine whether redo bladder neck reconstruction is effective in achieving continence after a failed bladder neck reconstruction procedure.We retrospectively reviewed the hospital records of patients with bladder exstrophy who had undergone redo bladder neck reconstruction. There were 30 patients in the study, including 20 boys and 10 girls. Mean patient age at redo bladder neck reconstruction was 9.3 years (range 3.2 to 15.5). The patients were divided into 3 groups on the basis of the preoperative pattern of incontinence--incomplete wetters, complete wetters and those on continuous suprapubic drainage. Of the patients 15 already had undergone bladder augmentation, 12 had undergone a Mitrofanoff procedure and 12 had been treated with bulking agents injected in the bladder neck in an attempt to achieve continence. Four patients had undergone more than 1 bladder neck procedure. The patients were investigated with a combination of noninvasive urodynamics, cystoscopy, cystogram and ultrasound. All patients underwent Mitchell's modification of Young-Dees-Leadbetter bladder neck reconstruction. Additional procedures performed included augmentation cystoplasty and Mitrofanoff formation.Mean followup was 6.9 years (range 1.2 to 15.5). Postoperatively 28 patients were using clean intermittent catheterization to empty the bladder (5 per urethra, 23 via Mitrofanoff). Two patients remained on continuous suprapubic catheter drainage. A total of 18 patients (60%) were dry postoperatively (80% of girls and 50% of boys). Among dry patients only 3 were performing clean intermittent catheterization per urethra and 15 via a Mitrofanoff channel. No patient was able to void per urethra without the need for clean intermittent catheterization. The 2 patients on continuous suprapubic catheter drainage continued to remain so. At night only 50% of the patients were dry (5 on free drainage, 4 on clean intermittent catheterization, 6 not on any drainage). Those patients who did not respond satisfactorily to redo bladder neck reconstruction underwent subsequent additional procedures, which included injection of bulking agents (3 patients), insertion of an artificial urinary sphincter (1), Mitrofanoff formation (2) and bladder augmentation plus Mitrofanoff channel (1). Postoperative complications included difficulty with clean intermittent catheterization (8 patients), perivesical leak (1), recurrent epididymo-orchitis (1), upper urinary tract dilatation (2) and incisional hernia (1). Bladder neck closure was being considered in 5 patients.In our experience redo bladder neck reconstruction cannot achieve continence with volitional voiding per urethra. Although redo bladder neck reconstruction can render a significant number of patients dry, it is only effective if performed in conjunction with augmentation. Failure of the initial bladder neck reconstruction may be a reflection of a bladder that is of inadequate capacity and/or compliance. Therefore, bladder augmentation should be considered in all patients requiring redo bladder neck reconstruction. Bladder neck closure may be a better alternative to redo bladder neck reconstruction.

Purpose: Since 1980 numerous variations of the Mitrofanoff principle have been described. We report on 22 children in whom a ureteral Mitrofanoff channel was created.Materials and Methods: Between 1986 and 1995 a ureteral Mitrofanoff channel was constructed as a catheterizable conduit in 22 children 2 to 15 years old (average age 6.5) with various abnormalities of the lower urinary tract, mainly exstrophy and neurogenic bladder. Indications included unavailability or unsuitability of the appendix, preference for appendix as a catheterizable colonic stoma for antegrade washouts or concomitant removal of a nonfunctioning kidney, leaving the ureter available for use. Surgical technique was based on the principles of appendicovesicostomy and in 9 cases the ureteral Mitrofanoff channel was reimplanted.Results: Followup ranged from 1 to 72 months (average 30.5). Complications included stenosis of the conduit that caused difficult catheterization in 3 patients, necessitating dilation or minor revision in...

We report on the long-term followup of a mixed dissociative (conversion) disorder after circumcision in childhood.

Purpose: We evaluated functional outcome following heminephrectomy in duplex kidneys. Materials and Methods: A total of 101 patients were identified who underwent upper or lower pole heminephrectomy between January 1995 and December 2000. We retrospectively reviewed 60 case notes with complete data. A total of 37 cases (62%) were diagnosed prenatally, while 20 (33%) presented as single or recurrent urinary tract infection and 3 (5%) as urinary incontinence. Of the patients 52 (87%) had unilateral duplex and 8 (13%) had bilateral duplex kidneys. In addition, 20 of the 60 patients (33%) had ureterocele. Mean age at surgery was 46 months (range 3 to 10 years). Results: Mean function as assessed by nuclear renography of the duplex kidney before heminephrectomy was 39.9% (range 22% to 61%) and following heminephrectomy was 33.1% (13% to 60%). Mean decrease in function was 6.8%, with a decrease in function of less than 5% in 18 patients, 5% to 10% in 13 and more than 10% in 5. The functional values were increased by 1% to 5% in 9 patients and unchanged in 15 patients after heminephrectomy. Conclusions: Following heminephrectomy there was a significant decrease in 8% of patients and a small decrease in function in the remaining moiety in 51%.

Purpose: We assessed the role and long-term outcome of lower pole heminephrectomy in the treatment of nonfunctioning lower renal moieties in children with duplex kidneys.Materials and Methods: Between 1979 and 1994, 54 lower pole heminephrectomies were performed in 53 patients 1 to 192 months old (mean age 54) with duplex systems. A total of 15 patients was prenatally diagnosed, while the others presented with a urinary tract infection (36), orchiepididymitis (1) and failure to thrive (1). The surgical technique was essentially similar to that of upper pole heminephrectomy.Results: Operative course was uneventful except for intraoperative bleeding in 5 cases, which necessitated blood transfusion, and a postoperative urinary tract infection in 1. Followup ranged from 3 to 168 months (mean 56.9). Postoperative renograms available in 34 cases showed unchanged differential function in 12 and an ipsilateral 2 to 14 percent decrease (mean 5.65 percent) in 22. No late complications were detected except i...

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.

The outcome and quality of life in children with cloacal exstrophy are greatly influenced by small intestine residual length. We report 2 cases of almost complete midgut deletion in newborns with duodenal atresia in addition to cloacal exstrophy. To our knowledge this combination of anomalies has not been reported previously CASE HISTORIES Case 1. A male neonate born at 36 weeks of gestation with a birth weight of 2,500 gm. had cloacal exstrophy. Prenatal ultrasound had demonstrated an anterior abdominal wall defect. Magnetic resonance imaging of the spine showed a low lying tethered cord with lipoma and sacral hypoplasia. Bilious nasogastric aspirates were observed on day 3 of life and the classic double bubble appearance was evident on plain abdominal x-ray (see figure). Duodenal obstruction was confirmed by an upper gastrointestinal contrast study. Laparotomy revealed atresia of the second part of the duodenum with only 21 cm. of residual intestine. Atresia was corrected but after discussion with the parents other active treatment was withdrawn. The neonate died at age 17 days. Case 2. A male infant born at 32 weeks of gestation with a birth weight of 2,060 gm. had classic cloacal exstrophy. Prenatal scan had shown an anterior abdominal wall defect. Ultrasound of the spine demonstrated sacral agenesis, a tethered cord and myelomeningocele. Abdominal x-ray revealed a double bubble appearance. Laparotomy confirmed atresia of the second part of the duodenum with only 12 cm. of residual small intestine. No further surgery was performed. The neonate died at age 6 days. DISCUSSION

Bladder exstrophy and epispadias are congenital abnormalities of the urinary bladder. Evaluation of bladder function before and after bladder-neck reconstruction has not been done in patients with these conditions. We report the preliminary results of a prospective study of lower-urinary-tract function in 36 children (10 girls, 26 boys; age range 1·5-16 years) with bladder exstrophy and epispadias. Children's bladder function was measured by cystometry and cystography, and their upper urinary tracts were examined by ultrasound imaging. Normal bladder function was seen in children with primary epispadias but this was severely impaired in children who had had conventional bladder-neck surgery for continence, implying that this may not be the optimal treatment. Contrary to current assumption, children with closed exstrophy bladders are not capable of normal function, and the abnormalities we identified may be a major cause of upper-urinary-tract damage and may impair development of bladder capacity. Although unrecognised in the past, involuntary bladder contractions are a primary cause of urine leakage persisting in children with exstrophy and epispadias after continence surgery. Detailed functional testing should become a routine part of the evaluation of children with exstrophy and epispadias.

We report the death of a 17-year-old male exstrophy patient from adenocarcinoma arising at the ureterosigmoidostomy. This was detected at reconstructive surgery at the age of 16 years, 13 years after ureterosigmoidostomy. This case highlights the importance of including children in endoscopic surveillance. We recommend annual sigmoid-colonoscopy commencing 10 years after ureterosigmoidostomy irrespective of age.

Transverse retubularization of small ileal segments has been described as a new time and labor saving variation of the Mitrofanoff principle in a dog model with good functional results. We report our initial clinical experience with this technique.From May 1996 through January 1997 a new technique of channel formation for intermittent catheterization was applied in 9 children (1 to 16 years old) and 7 adults (18 to 56) with various abnormalities of the lower urinary tract. The new method was used in primary reconstruction of the lower urinary tract and in revision procedures. An ileal segment 2 cm. long was excised. The bowel wall was opened longitudinally about 1 cm. from the mesentery. The resulting rectangle was retubularized over a 14F catheter in transverse direction. The longer portion of the tube was implanted submucosally into the native bladder, the augmented bladder or an intestinal reservoir. The shorter portion was used to form the stoma. In 4 patients we created a double tube.Of the patients 13 (81%) are completely continent day and night with easy catheterization postoperatively. In 2 cases of tunnel failure continuous leakage required reimplantation of the intact ileal tube to achieve continence. Minor leakage with bladder fullness in an 11-year-old boy could be obviated by adjusting the interval of catheterization.With the advantage of constant availability, minimal loss of bowel, relative simplicity (no mesentery interfering with implantation, high tube mobility), minimized risk of stone formation (no staples), reliable continence (no leak point) and easy catheterization (longitudinal folds), this straightforward technique is an excellent second choice use of the Mitrofanoff principle.

The syndrome of posterior urethral valves, persistent unilateral reflux and renal dysplasia (VURD) is said to be protective of the contralateral nonrefluxing kidney and the outcome for renal function is reported to be excellent. We tested this hypothesis in our patients by replicating previous studies but with longer followup and glomerular filtration rate data.We retrospectively reviewed the records of 183 boys presenting with posterior urethral valves between 1980 and 1989, including 12 who underwent nephrectomy for ipsilateral nonfunction and fulfilled all criteria for the VURD syndrome. Mean age at the most recent followup was 8.5 years. Serial serum creatinine levels and glomerular filtration rates were analyzed and compared to age matched normal values.Histological evaluation revealed dysplasia in all kidneys, confirming the VURD syndrome. Followup plasma creatinine was normal 67% of the patients during year 2 of life, 50% between ages 4 and 5 years, and only 30% between ages 8 and 10 years. Glomerular filtration rate was within the normal range in 25% of boys tested in year 2 of life, and between ages 5 and 8 years.Our data do not support the protective effect of the VURD syndrome on long-term renal function. All patients with posterior urethral valves require diligent long-term followup.

Objective To examine the characteristics of primary vesico-ureteric reflux (VUR) in young infants following prenatal hydronephrosis. Patients and methods The study comprised 155 consecutive infants with VUR detected at a mean age of 8.7 weeks (sd 6.3). Reflux units (n=236) were analysed for relationships between gender, severity of reflux, exposure to urinary tract infection (UTI) and the presence of focal and generalized types of kidney damage on imaging. Bladder wall thickness (from ultrasonography) was examined in comparison with a further group of 29 males without VUR. Results Male infants predominated (117 of 155, 75%); bilateral VUR affected the same proportion (52%) of males and females. Most kidneys exposed to VUR (158 of 236, 67%) were normal and of the 78 abnormal kidneys (57 without UTI), 53 showed generalized damage (only eight exposed to UTI) and 71 (91%) were associated with severe (grades IV and V) reflux that predominantly affected males (P

Objective To evaluate natural filling cystometry in infants and young children. Patients and methods The study group comprised 57 infants and young children (mean age. 4.1 years) with various urological conditions. Suprapubic catheters were used in all patients with urethral sensation. Natural filling urodynamic (NFU) studies were performed using an ambulatory recorder and with an observer present throughout. For comparison. 17 of the 37 patients also had slow filling conventional cystometry (CMG). Results All NFU studies were successfully completed and the great majority of patients were unaffected by the investigation procedures. In comparison with conventional cystometry there were significant differences. For NFU, there was a lower bladder capacity (means. NFU 122 mL vs CMG, 188 mL. P

Objective To assess urinary concentrating ability, urine production and glomerular filtration rates in a cohort of boys with previously treated posterior urethral valves. Patients and methods Urinary concentrating capacity was assessed in 51 boys aged 5.4–9.9 years with previously treated posterior urethral valves. They all completed urinary frequency/volume charts, permitting calculation of 24 h urine volumes. The osmolal-ity of an overnight urine collection was measured and, if

Summary— Congenital obstruction of the posterior urethra was first systematically classified by Young in 1919. Since then, no-one has seriously challenged the presence of both Type I and Type III “valves”, although the presence of Type II lesions has often been disputed. A review of Young's papers and more recent anatomical studies, together with endoscopic findings in our own patients, indicates that most congenital posterior urethral obstructions are anatomically similar. Consequently, Young's classification now seems redundant.

The management of 58 patients born with an imperforate anus who presented to the urology service and who are currently 18 years old or older is discussed. Of the 44 patients with a high imperforate anus 43 were treated by a rectal pull-through and 1 by an anal cut back procedure, while an anal cut back procedure was used in all 14 children with a low imperforate anus. A total of 32 children (55%) had a neuropathic bladder (hyperreflexic in 29 and atonic in 3). A spinal deformity was present in 72% of the hyperreflexic group but not in the atonic group. Vesicoureteral reflux occurred in 41% of the patients, two-thirds of whom had a neuropathic bladder. A total of 30 children had an associated genital anomaly and 27 had an upper tract anomaly. Nineteen children underwent an operation for the neuropathic bladder, with modern reconstructive methods involving substitution or augmentation cystoplasty, supplemented with self-catheterization, being superior to older methods with regard to continence. The incidence of a neuropathic bladder in these children is high in both operative groups but it is usually associated with a spinal deformity and unlikely to be iatrogenic in nature unless proved to be of lower motor neuron origin. Early operative management is advised to achieve continence and minimize renal impairment.

Increasing recognition of bilateral renal involvement in Wilms' tumor (nephroblastoma) has led to a less radical approach to surgical treatment. Experience with bilateral nephrectomy and subsequent dialysis or transplant has been unsatisfactory, and unilateral nephrectomy with contralateral radiotherapy carries the morbidity of radiation in addition to the risk of recurrent disease. Advances in chemotherapy have facilitated preoperative shrinkage of tumors and subsequent local excision of remaining tumor nodules with preservation of functioning renal tissue. We have treated 10 children with bilateral (stage V) Wilms' tumor in this manner. Two-year disease-free survival in this group is 60%, and renal function is satisfactory in all survivors. This experience suggests that nephron-sparing surgery may be possible in the majority of children with Wilms' tumor. Conservative surgery is essential in certain special situations such as solitary kidney or the horse-shoe abnormality.

The pedicle patch urethroplasty provides an alternative single-stage vascularised graft procedure for the repair of moderately severe hypospadias. Over a 7-year period, 38 primary hypospadias repairs were performed employing this technique. The fistula rate was 18% in the 34 patients reviewed, but there were few other significant complications. The operation is recommended when the urethral plate can be preserved and the penis straightened in boys who are unsuitable for a meatal-based flap.

Purpose Mixed Gonadal Dysgenesis (MGD) is one of the more frequent intersex disorders. The most common genotype is 45XO/46XY mosaicism and these patients typically present with a streak gonad on one side and a palpable gonad on the other with hypospadias. Debate exists over the nomenclature and management of MGD. Many would advocate bilateral gonadectomy and a female sex of rearing. However little is known about the outcomes of these patients. Recent reports have demonstrated success with the male sex of rearing. Material and methods Retrospective analysis revealed 13 patients with MGD and a proven 45XO/46XY with complete records and documented follow-up. Case notes were evaluated for clinical presentation, investigation (biochemical, radiological, and histological) operative procedures and outcomes. Results Median age at presentation was 60 months (range; birth-11 years), and gender was assigned at a median of 2 days (range; 0-35 days). Nine cases presented with ambiguous genitalia at birth and referred for intersex evaluation. Four were initially assigned as males at birth but overall 9 were reared as males. Eight cases had unilateral palpable gonad and 4 had bilateral impalpable gonads. Three patients were gender reassigned to the female sex of rearing on the basis of an inadequate phallus. The median XO:XY ratio was 80:20 respectively. All patients had removal of streak gonads. Bilateral gonadectomy was performed in 5 cases and 8 had contralateral orchidopexy. The follow-up ranged from 10-144 months (median 63 months). Conclusions We are witnessing a paradigm shift in the management of children with MGD. Modern reconstructive surgical techniques are allowing more patients to be successfully reared as males.

Purpose Isolated female epispadias, without bladder exstrophy involvement, is an exceedingly rare congenital malformation with a reported incidence varying between 1/160.000 to 1/480.000. The diagnosis is often overlooked due to failure to recognise the cardinal features of a bifid clitoris and patulous urethral opening. Material and methods Classically, female epispadias is associated with urinary incontinence with an incompletely formed bladder neck and a short, wide urethra. One strategy for repair is to combine external cosmetic correction with urethral reduction and angulation performed from the perineum. In some cases this may be all that is required for achieving satisfactory voiding continence. In others further surgical intervention is required at the bladder neck, but this initial procedure increases outflow resistance and stimulates the development of bladder capacity which in turn facilitates any continence surgery at the bladder neck which may be required. Results In this video we demonstrate the primary surgical repair of an isolated epispadias in a 9 month old female, including: 1. Urethral reduction. 2. Repair of the “urogenital diaphragm”. 3. Urethral angulation. 4. Clitoroplasty and labioplasty.