Primary Epispadias Research Articles

Bladder Neck Surgery is not Routinely Needed to Achieve Urinary Continence in Patients with Primary Epispadias

BackgroundThis study aims at investigating the continence outcome in primary epispadias patients treated at a tertiary center. The authors hypothesized that additional continence procedures following primary epispadias repair is not routinely needed. MethodsPatients treated for primary epispadias at the authors’ institution between 2007 and 2019 and toilet trained, were identified from a prospective maintained database. Males underwent chordee correction, urethroplasty and glanuloplasty. Females underwent genitoplasty with reduction urethroplasty. If continence was not achieved by 4–5 years of age, pelvic floor muscle (PFM) biofeedback therapy was performed. Other continent procedures were discussed with family/patient if still incontinent. Primary outcome: urinary continence. Secondary outcomes: PFM biofeedback therapy, continence surgery, hydronephrosis. Type of epispadias, age at repair and follow-up presented as median was also reported. ResultsThirty-three patients (29 males) were included. Twelve had penopubic epispadias, 13 glanular/penile, 4 duplicated urethra, 4 females. Median age at repair: 2 years (IQR 1–3), at follow-up: 8 years (IQR 6–10). Daytime continence: 100 % in penile/glanular; 33 % in penopubic and 75 % in duplicated urethra. Nighttime continence: respectively 92 %, 50 % and 100 %. 24 % of males were intermittently incontinent. All patients except one voided urethrally. One patient underwent bladder neck closure, ileocystoplasty and Mitrofanoff. One girl achieved daytime continence, 2 were intermittently incontinent, one continuously incontinent. All were enuretic. 38 % of boys and 100 % of girls had biofeedback therapy. None had hydronephrosis/renal impairment. ConclusionsMost children with primary epispadias can achieve social urinary continence spontaneously or with the support of PFM biofeedback therapy. Other continence procedures should be reserved for patients who do not attain satisfactory continence. Level of EvidenceTreatment study – level IV.

Epispadias: recent techniques

Aim: This paper presents the latest surgical approaches for epispadias treatment in the pediatric population, as well as those for adolescent and adult populations after initial failed repair in childhood. Methods: The retrospective study was conducted between March 2005 and May 2020 and included 18 patients with the mean age of 21 months (range 11-48 months) (Group A), who underwent primary epispadias repair and 15 patients with the mean age of 18 years (range 13-29 years) (Group B), who underwent redo surgery after failed epispadias repair in childhood. In Group A, the surgery was performed as a one-stage procedure using complete penile disassembly technique, while, in Group B, the surgery was done as a two-stage procedure and included complete straightening and lengthening of the penis, followed by urethral reconstruction. Penile straightening and lengthening were achieved by tunica albuginea incision and grafting. In Group A, the urethral plate was mobilized, transposed ventrally, and tubularized and augmented with vascularized preputial skin flap where needed. In Group B, the urethra was reconstructed either using the buccal mucosa graft and genital skin flaps or with tubularization of genital skin flaps. Successful treatment was defined as a functional and esthetically acceptable penis without complications. Results: The mean follow-up was 88 months (range 15-197 months). Satisfactory results were achieved in 26/33 patients. Urethral fistula occurred in 4/18 patients from Group A and in 3/15 patients in Group B and was surgically repaired after four months. Skin dehiscence occurred in eight patients, five from Group A and three from Group B. Recurrent penile curvature was observed in 2/18 patients from Group A and required surgical correction and in 2/15 patients from Group B and was mild and did not need surgical repair. Eleven patients from Group B who filled out the International Index for Erectile Function reported satisfying erectile function, sexual desire, intercourse, and overall satisfaction. Conclusion: Primary or redo epispadias repair is challenging even for experienced reconstructive urologists. Only radical surgical approach can lead to complete correction of all deformities and provide successful outcome.

Modified partial penile disassembly repair for improved functional and cosmetic outcome in isolated male epispadias

Introduction and objectiveThe objective of the study was to evaluate the functional and cosmetic outcome of single-stage modified partial penile disassembly repair in isolated male epispadias. Materials and methodsA retrospective analysis of 15 cases of primary epispadias repair, from June 2015 to December 2018, was performed. Patients were classified by the type of epispadias, urinary incontinence, chordee, and rotation. Surgical techniquePenile degloving with the mobilization of the urethral plate from the ventral to the dorsal aspect with the preservation of blood supply at both ends, distally up to the level of mid-glans and proximally up to the pubic symphysis is done. Tubularization of urethral plate followed by spongioplasty, corporoplasty with medial rotation of corporeal bodies, and glanuloplasty with meatoplasty was done to bring the meatus ventrally. The skin cover is done by the rotation of the ventral flaps and the z-plasty whenever required. ResultsAge of the patients varied from 4 months to 21 years with a mean of 11 years. Thirteen patients had excellent cosmetic outcome while two patients had minimal residual chordee but did not require any surgery in a follow-up. Five patients with partial incontinence in the study group achieved continence after surgery. None of the patients developed complications such as fistula or stricture. All five male patients in the post-pubertal group reported normal erections and successful ejaculations after the surgery. Follow-up ranged from 3 months to 18 months. ConclusionsModified partial penile disassembly incorporates all the benefits of Cantwell Ransley repair and needs less extensive dissection than total penile disassembly. Both functional and cosmetic results are good with a low complication rate.

Long-term outcomes in primary male epispadias

The authors present a case series of 30 male patients who presented with primary epispadias between 1989 and 2002 and looked at their long-term outcomes. (1) What procedures and operations did these patients require following their original surgery? (2) What were their outcomes as adults in terms of continence, cosmesis, and sexual function? Primary male epispadias patients who had gone through transition into Adolescent and Adult Urology services were identified retrospectively from electronic patient records. The authors identified 30 male patients with a median follow-up of 18.5 years. Twenty-four had penopubic epispadias, and six had penile epispadias. All initial surgery took place between 9 and 48 months. Twenty-eight patients needed further surgery over the follow-up period, 26 had surgery for continence, and 12 required revision surgery. At follow-up, 15 patients were continent voiding per urethra, nine patients reported stress leakage with volitional urethral voiding, six patients were using a Mitrofanoff to void, and four of these had an ileocystoplasty. Nineteen patients had documentation on their feelings towardcosmetic outcome; 17 expressed concern. Twenty-one patients had documentation about sexual function; 20 had normal erections with six reporting chordee and nine reporting retrograde ejaculation. No patients were recorded to have fathered any children. There are only a few published studies looking at long-term outcomes of genitourinary reconstruction in primary male epispadias and their sexual function in adulthood. The majority of this patient cohort required surgery to improve their continence and had more than one continence procedure. There is limited data on continence outcomes in the literature with small cohorts and rates varying between 40 and 100% at 10-year follow-up. Despite corrective surgery, nearly all the patients were concerned about their genital appearance. Other studies have shown similar outcomes in terms of patient satisfaction and sexual function. The limitations of this work are that the information was gathered retrospectively from the electronic patient record and validated instruments on outcomes were not used. The study outcomes will be useful for clinicians who are counseling patients and parents regarding what to expect as adults.

Functional and cosmetic outcome of partial penile disassembly repair in isolated male epispadias patients

IntroductionEpispadias is a rare congenital anomaly and requires a carefully constructed and well-planned approach for the management. Modified Cantwell-Ransley technique and Mitchell's complete penile disassembly are commonly used technique and these may require multiple surgeries in majority of the patients to achieve the goals of cosmesis and continence. ObjectiveTo evaluate the functional and cosmetic outcome of single stage partial penile disassembly repair in isolated male epispadias. Patients and methodsA retrospective analysis of 43 cases of primary epispadias repair, performed during July 1998 to March 2013. Patients were classified on the basis of type of epispadias, urinary incontinence, presence/degree of chordee and penile rotation. TechniquePenile de-gloving with mobilization of urethral plate from ventral to dorsal aspect with preservation of blood supply at both ends, distally up to the level of mid-glans and proximally up to pubic symphysis with division of peno-pubic ligament to lengthen the penis and position the urethra ventrally. Tubularization of urethral plate followed by spongioplasty, corporoplasty with medial rotation of corporeal bodies (without any corporotomy) and glanuloplasty with meatoplasty is done to bring the meatus ventrally. Skin cover with rotation of ventral flaps and z-plasty when required. ResultsAge of the patients varied from 6 months to 26 years with a mean of 9 years. Ninety three percent of the patients had excellent cosmetic outcome while seven percent had minimal residual chordee/torque but did not require any surgery. None of the patients developed complications like fistula or stricture. All the 12 patients in the postpubertal group reported normal erections and successful ejaculations after the surgery. Postoperative follow up ranged from 2 to 10 years with a mean of 4 years. ConclusionsThe technique incorporates all the benefits of Cantwell-Ransley repair, can be done with less extensive dissection than total penile disassembly. Both functional and cosmetic results are good with low complication rate. Spongioplasty reconstructs near normal urethra and corporoplasty with spongioplasty also helps in prevention of urethral fistula.

Epispadias repair with tunica vaginalis flap

Primary epispadias is a rare congenital malformation that afflicts about 1 in 80,000 children. The surgical repair, originally described by Cantwell over 100 years ago, is fundamentally sound and incorporates several important steps to achieve an optimal result. A 9-month-old male with penopubic epispadias presented for surgical repair. Pre-operative imaging included a normal renal ultrasound and voiding cystourethrogram that demonstrated a moderate-sized bladder, partially competent bladder neck, and no vesicoureteral reflux. A modified Cantwell-Ransley approach to the repair was performed without complete disassembly of the penis. Established surgical principles of identification of laterally placed neurovascular bundles, preservation of the ventral mesentery to the urethral plate, mobilization of the urethral plate off the corporal bodies with ventral relocation, and dorsal medial rotation of the corporal bodies are demonstrated in this video. This procedure was performed as an outpatient. The patient had an unremarkable postoperative course. The #6-French urethral stent was removed on postoperative day 10. This video demonstrates a modified Cantwell-Ransley technique for epispadias repair, which employs a tunica vaginalis flap to reinforce the urethral repair. The adherence to surgical principles described by Cantwell in the late 1800s for children with primary epispadias continues to lead optimal surgical outcomes.

Kelly procedure for male primary epispadias

Primary epispadias is a rare congenital malformation involving the urogenital system. In patients with epispadias, bladder closure enhances bladder growth and continence. Several steps were carried out to bring the bladder neck to the midline and allow tension-free bladder neck reconstruction and recreation of the natural angulation of urethra. The urethral plate and penile shaft were dissected and the corpora cavernosa separated, and then the bladder neck repair was performed. The urethral plate was tubularised and brought ventrally. The separated corpora were reapposed, avoiding torsion. The skin was reoriented to provide cover to the penis. The Kelly procedure improves cosmesis and continence by reconstructing a tension-free bladder neck repair and lengthening the penis. The video demonstrates the Kelly procedure for primary epispadias in a male child.

The Tunica Vaginalis Flap as an Adjunct to Epispadias Repair: A Preliminary Report

To report our preliminary institutional experience of incorporating a tunica vaginalis flap (TVF) as an adjunct into primary or secondary epispadias repair. A prospectively maintained institutionally approved database of exstrophy-epispadias complex patients was used to identify and retrospectively review male patients who underwent epispadias repair from September 2010 to October 2014 at the authors' institution. Patients who underwent epispadias repair with TVF were identified and their clinical outcomes were measured. A total of 49 male patients were identified as meeting inclusion criteria, of which 15 (2 isolated epispadias, 13 classic bladder exstrophy) underwent epispadias repair incorporating a TVF. Median age at time of repair was 12 months (interquartile range [IQR] 10-15.5). A median of 4 layers (IQR 4-5) was incorporated into each repair closure, applying EVICEL Fibrin Sealant as an additional layer in all patients. All patients received preoperative testosterone injection therapy of 2 mg/kg 5 and 2 weeks before surgery for penile growth. There were no intraoperative complications. Median follow-up of 19 months (IQR 12-23) revealed 5 patients who underwent epispadias revision, 3 (20%) of which developed a urethrocutaneous fistula. All patients had a successful repair without recurrence. The authors have found the utilization of a TVF with epispadias repairs to be beneficial but no better than the repair with our routine soft-tissue coverage in primary epispadias repair. However, in patients presenting with complex anatomy and limited tissue reserves, a TVF is an important adjunct to epispadias repair and/or revision and may lead to improved outcomes.

Prospective study on the incidence of bladder/cloacal exstrophy and epispadias in Europe

On behalf of the European Society of Paediatric Urology (ESPU), a prospective study was designed with the aim of defining the actual number of babies born with bladder exstrophy, cloacal exstrophy, and epispadias in Europe over a 12-month period, and verifying the distribution of the exstrophy patients born during the study period among the different paediatric urology centres in Europe. The study was structured with a chief investigator and one national investigator for each country enrolled in the study. The national investigators nominated one local investigator for each European centre of paediatric surgery/paediatric urology and urology where the exstrophy complex could potentially be treated. The local investigators were responsible for reporting babies treated in their institutions for bladder/cloacal exstrophy and/or epispadias. During 2010, every 3 months, an electronic survey (Figure) was e-mailed to the local investigators asking them to report babies treated or referred for treatment during the previous 3 months. One-hundred and sixteen centres in 27 European counties were enrolled in the study. The overall response rate for the four online surveys was 79%. Two-hundred and thirty-eight babies were reported to be born with a condition within the bladder exstrophy epispadias complex (BEEC): 71 primary epispadias (66 males), 146 classic bladder exstrophy (97 males) of which two were female bladder exstrophy variant, and 21 cloacal exstrophy (17 males). Two of 67 (3%) male epispadias, 24/146 (16.4%) bladder exstrophy, and 6/21 (28%) cloacal exstrophy were antenatally diagnosed. Associated anomalies were reported in 2/71 (2.8%) epispadias patients, 8/146 (5.5%) bladder exstrophy patients, and 15/21 (71.4%) cloacal exstrophy patients. One-hundred and forty-seven (62%) of the 238 babies born in Europe with a condition within the exstrophy spectrum during 2010 were transferred from other institutions for treatment (36 male epispadias, 97 bladder exstrophy, and 14 cloacal exstrophy). Only 12 centres treated six or more exstrophy and or epispadias patients during the study period; 52 treated between one and five patients, of which 22 treated only one case in 12 months. This study provides a contemporary incidence of the BEEC in Europe. It demonstrates also that only a minority (19%) of the European centres involved in the treatment of exstrophy can be considered "high volume" exstrophy centres. There is a case for proposing a rationalisation of the treatment of this group of conditions in a small number of exstrophy units around Europe.

Psychosocial screening at paediatric BEEC clinics: A pilot evaluation study

Bladder Exstrophy and Epispadias Complex (BEEC) is associated with an increased risk of impaired mental health, quality of life, and psychosocial functioning. Therefore, screening patients to help identify and evaluate potential psychosocial difficulty is arguably an important consideration for BEEC Services. To screen paediatric BEEC patients for a range of general psychosocial difficulties in a multi-disciplinary out-patient clinic setting. This cross-sectional evaluation was conducted between April 2012 and July 2013. Families attending BEEC multi-disciplinary out-patient clinics were asked to complete a range of standardised psychosocial questionnaires, including the Paediatric Quality of Life Inventory (PedsQL 4.0 Generic Core and Family Impact Module), the Strengths and Difficulties Questionnaire (SDQ), the Paediatric Index of Emotional Distress (PI-ED), and the Hospital Anxiety and Depression Scale (HADS). 108 children attended clinic of which 80 (74.1%) patients and their parents/carers completed some or all of the questionnaires. The mean patient age was 8.41 years (SD = 4.46, range = 1-18 years). There were more boys (N = 50, 62.5%) and the majority had a diagnosis of classic bladder exstrophy (N = 51, 63.8%), followed by primary epispadias (N = 22, 27.5%) and cloacal exstrophy (N = 7, 8.7%). Mean total scores fell within the average/normal range on all questionnaires used (See table below). However, variation around these means was high. Age, gender and diagnosis were found to significantly influence certain questionnaire responses with older-age groups, males, and those with classic bladder exstrophy particularly at risk across some domains. The children/adolescents self-reported better health related quality of life (HRQoL) scores than published results for a range of paediatric chronic health conditions. Differences between parent and child responses on both the PedsQL and SDQ favoured a more positive response on the child self-report questionnaire but were not statistically significant. Mean scores on the measures used suggest a relatively optimistic picture of general psychosocial well-being, especially for HRQoL, in the BEEC population studied. Positive HRQoL outcomes have recently been reported for BEEC paediatric populations. Our results reflect this trend with better mean HRQoL scores than paediatric patients with a range of other chronic health conditions. However, this optimism is cautious given the limitations of this evaluation study and the high variation around the means. Limitations included the small sample size (especially for patients with cloacal exstrophy), the lack of a control group, the limited sensitivity of generic questionnaires in respect of BEEC-specific issues, and the low mean age of patients in the study. Future screening programmes may wish to consider measuring BEEC-specific variables (e.g. satisfaction with genital appearance/function); collecting information on medical aspects, such as continence, pubertal stage and frequency/timing of medical intervention; and asking both parents/carers (where possible) to complete the questionnaires. Screening questionnaire responses were used in conjunction with clinical psychology consultations to evaluate a range of psychosocial aspects in BEEC paediatric patients. Whilst mean scores on the measures used suggest a relatively optimistic picture, certain individual scores did fall within the clinical ranges, highlighting the potential need for further assessment. Developmentally tailored consultations with a clinical psychologist can provide detailed information around questionnaire responses and further assess BEEC specific aspects.

Modified Cantwell-Ransley epispadias repair in children our experience

Introduction: We retrospectively evaluated our experience with modified Cantwell-Ransley epispadias repair at our center to determine the complications and long-term results. Materials and Methods: We retrospectively reviewed the case records of 43 male children with a mean age of 9.13 ± 1.94 years who underwent primary epispadias repair at our center. The results of epispadias repair were assessed by both physical and endoscopic examination. All children who were old enough to opine as well as all parents/guardians were interviewed during the follow-up visits. Results: Urethrocutaneous fistulae occurred in 17.85% (5/28) children of the classic bladder exstrophy group and in 13.33% (2/15) children with penopubic epispadias. Postoperative cystoscopy done 12 weeks after repair revealed a smooth urethral tube in 81.39% (35/43) of children. With the patient in a standing position, the penis was dangling downward or in a horizontal position in 88.37% (38) of children, 85% of the patients ≥18 years of age were satisfied with both the functional and cosmetic outcome, as assessed by short form 36 and 93.02% (40/43) of the patients were continent during the daytime with voided volumes of more than 200 ml. Conclusion: In our experience, Cantwell-Ransley repair creates a functionally and cosmetically acceptable penis and produces a reliably tubularized neourethra with acceptable complication rates.

Self-Esteem and Satisfaction with Penis Appearance in Adolescent Males with Bladder Exstrophy: A Pilot Study

PURPOSE Self-esteem can play an important role in satisfaction with body appearance. The present pilot study was designed to examine the relationship between self-esteem and satisfaction with penis appearance in adolescent males with bladder exstrophy and/or epispadisas. MATERIAL AND METHODS Nine adolescent males aged 11-16 with bladder exstrophy or primary epispadias were identified in the Manchester Bladder Exstophy database. Six took part to the study completing a ‘satisfaction with penis appearance’ questionnaire and the Harter Self-Perception Profile to provide a global measure of self-worth. Standarised photographs were taken of each participant's genital area. Two Paediatric Urologists independently rated participants’ penis appearance based on the anonymised photographs using a penis appearance rating scale. Each participant's genital developmental stage was assessed using the Tanner Rating Scale. RESULTS Discrepancy scores were calculated between objective/professional assessments of penis appearance and subjective/young people's assessments of penis appearance. Lower self-worth scores on the Harter Self-Perception Profile were noted for participants who had a larger discrepancy between subjective and objective ratings of penis appearance. 4 of the 6 participants had self-worth ratings within the normal range. Developmental stage as assessed using the tanner rating scale did not seem to affect satisfaction with penis appearance in this small group. CONCLUSIONS The limited numbers in this study prevent generalisation of the results, however it is relevant that 4 of our 6 patients showed normal self-worth during adolescence despite having visibly different penises. Further research is required to examine the link between self-esteem and satisfaction with penis appearance.

Self-Esteem and Satisfaction with Penile Appearance in Adolescent Males with Bladder Exstrophy: A Pilot Study

PURPOSE Self-esteem can play an important role in satisfaction with body appearance. The present pilot study was designed to examine the relationship between self-esteem and satisfaction with penile appearance in adolescent males with bladder exstrophy and/or epispadisas. MATERIAL AND METHODS Nine adolescent males aged 11-16 with bladder exstrophy or primary epispadias were identified in the Manchester Bladder Exstophy database. Six took part in the study completing a ‘satisfaction with penile appearance’ questionnaire and the Harter Self-Perception Profile to provide a global measure of self-worth. Standarised photographs were taken of each participant's genital area. Two Paediatric Urologists independently rated participants’ penile appearance based on the anonymised photographs using a penile appearance rating scale. Each participant's genital developmental stage was assessed using the Tanner Rating Scale. RESULTS Discrepancy scores were calculated between objective/professional assessments of the penile appearance and subjective/young people's assessments of the penile appearance. Lower self-worth scores on the Harter Self-Perception Profile were noted for participants who had a larger discrepancy between subjective and objective ratings of penile appearance. 4 of the 6 participants had self-worth ratings within the normal range. Developmental stage as assessed using the tanner rating scale did not seem to affect satisfaction with the penile appearance in this small group. CONCLUSIONS The limited numbers in this study prevent generalisation of the results, however it is relevant that 4 of our 6 patients showed normal self-worth during adolescence despite having visibly different penises. Further research is required to examine the link between self-esteem and satisfaction with the penile appearance.

The Kelly Operation for Epispadias

PURPOSE To review the results of Kelly soft tissue reconstruction in incontinent primary epispadias. MATERIAL AND METHODS 22 patients are reviewed from our database at least 1 year after their Kelly operation. Non-invasive bladder function assessments and clinical histories were used to evaluate continence. Half (11 patients -10m, 1f) had a primary Kelly operation at a median age of 2 years (10 m - 8 y, 3 m). Half (11 patients - 9m, 2f) had a secondary Kelly procedure following previous epispadias repair, at a median age of 10 years (3 y, 3 m - 16 y). RESULTS In the Primary group 2 patients remain too young to assess reliably. Of the remainder 4/9 are dry by day and 2 of these also at night. 5/9 are judged to have no control. In the secondary group 8/11 patients are dry by day and 5 of these are also dry at night. 3 remain wet with inadequate control and capacity. All females are dry by day, 1 also at night. CONCLUSIONS As in patients with bladder exstrophy, continence relies on a combination of bladder outlet resistance and capacity. Whilst the results from secondary cases are comparable with our data in classic exstrophy (70% daytime continence) those in primary cases are inferior. Reasons for this may include: 1. patients with primary operations are younger and so their continence may continue to improve. 2. radical primary dissection may have a negative effect on native continence mechanisms in epispadias. 3. primary epispadias is not simply a more minor variant of the exstrophy-epispadias complex. (supported by anatomical observations of our cases).

Injectable polydimethylsiloxane for treating incontinence in children with the exstrophy‐epispadias complex: long‐term results

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. 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. 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 <1 year, two at 1-2 years, eight at 2-5 years and six 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. 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.

Perimesenteric detubularization of ileum for ileocystoplasty improves compliance and increases capacity.

To describe a modified form of detubularization using a perimesenteric incision of the ileal segment, which increases the capacity of the neobladder and reduces the length of the ileal segment required. From February 1993 to November 1999, ileocystoplasty was undertaken in 20 patients (mean age 8.4 years, range 4-16). Six had myelodysplasia, four had primary epispadias, five had bladder exstrophy, three had post-traumatic lesions and two had cloacal exstrophy. The patients were divided into two groups; in the first (eight patients, mean age 9.6 years, range 5-15) the ileal segment was opened along the antemesenteric border and in the second (12 patients, mean age 7.7 years, range 4-16) the ileal segment was opened adjacent to the mesentery, perimesenterically. Each segment was then folded and the perimesenteric edges sutured to form the pouch. The patients were assessed at 1 year after surgery; the neobladder capacity was evaluated as ((neobladder capacity - bladder capacity)/body weight), which shows the relative increase of bladder capacity after enterocystoplasty per unit of weight. There was a statistically significant increase in neobladder capacity in group 2 (perimesenteric transection; Kruskal-Wallis chi-square, P = 0.005; Mann-Whitney U-test, P = 0.006). These results indicate that augmentation can be carried out by perimesenteric transection of the intestinal segment, which improves compliance and increases capacity of the neobladder.

Total urogenital complex mobilization in female patients with exstrophy.

Female bladder exstrophy/epispadias has traditionally been approached in a staged fashion. This approach results in a vagina that remains in an abnormal position on the anterior abdominal wall. We present a surgical correction of the female exstrophy/epispadias urogenital complex with total mobilization that returns the vagina to its proper anatomical position. Since 1997, 7 female patients presenting with variants of the exstrophy/epispadias complex have undergone surgical repair using total urogenital complex mobilization. Of the patients 1 newborn and 2 school-age children had classic bladder exstrophy, 2 school-age children had cloacal exstrophy and 2 school-age children had primary epispadias. Total urogenital complex mobilization involved treatment of the urethra and vagina as a single unit. Complete disassembly of the pelvic diaphragm or floor anterior to the rectum was required to reposition the urethra and vagina to their proper anatomical positions in the perineum. The pelvic diaphragm was then reconstructed anterior to the urogenital complex to recapitulate the normal female pelvic floor anatomy. All patients have an anatomically correct position of the urogenital complex. All the vaginas reached the perineum without the need for skin flaps. All patients have adequate vaginal caliber without evidence of stenosis. The female with exstrophy/epispadias has unique anatomical defects in the urogenital complex that require special attention. Anterior displacement of the bladder, urethra and vagina with concomitant lack of development of the anterior pelvic floor musculature make a single stage, total urogenital complex mobilization repair ideal for this population. The results of this technique have been functionally and cosmetically pleasing. Whether repositioning the urogenital complex into the normal anatomical position will improve bladder dysfunction and urinary continence rates, and decrease or eliminate the need for future surgery will only be known after further long-term followup has been completed.

TOTAL UROGENITAL COMPLEX MOBILIZATION IN FEMALE PATIENTS WITH EXSTROPHY

Purpose: Female bladder exstrophy/epispadias has traditionally been approached in a staged fashion. This approach results in a vagina that remains in an abnormal position on the anterior abdominal wall. We present a surgical correction of the female exstrophy/epispadias urogenital complex with total mobilization that returns the vagina to its proper anatomical position.Materials and Methods: Since 1997, 7 female patients presenting with variants of the exstrophy/epispadias complex have undergone surgical repair using total urogenital complex mobilization. Of the patients 1 newborn and 2 schoolage children had classic bladder exstrophy, 2 schoolage children had cloacal exstrophy and 2 schoolage children had primary epispadias. Total urogenital complex mobilization involved treatment of the urethra and vagina as a single unit. Complete disassembly of the pelvic diaphragm or floor anterior to the rectum was required to reposition the urethra and vagina to their proper anatomical positions in the perineum. Th...

INITIAL EXPERIENCE WITH THE TRANSURETHRAL SELF-DETACHABLE BALLOON SYSTEM FOR URINARY INCONTINENCE IN PEDIATRIC PATIENTS

No AccessJournal of UrologyReconstruction1 Sep 2000INITIAL EXPERIENCE WITH THE TRANSURETHRAL SELF-DETACHABLE BALLOON SYSTEM FOR URINARY INCONTINENCE IN PEDIATRIC PATIENTS DAVID A. DIAMOND, STUART B. BAUER, ALAN B. RETIK, and ANTHONY ATALA DAVID A. DIAMONDDAVID A. DIAMOND More articles by this author , STUART B. BAUERSTUART B. BAUER More articles by this author , ALAN B. RETIKALAN B. RETIK More articles by this author , and ANTHONY ATALAANTHONY ATALA More articles by this author View All Author Informationhttps://doi.org/10.1016/S0022-5347(05)67222-9AboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract Purpose: A new endoscopic technique to treat urinary incontinence in children using a self-detachable balloon device was studied. Materials and Methods: The study includes 11 patients with a mean age of 14.6 years and all of whom had intrinsic sphincter deficiency due to myelomeningocele in 9, spinal artery bleed in 1 and cloacal exstrophy in 1. All patients were on clean intermittent catheterization preoperatively and postoperatively. Endoscopic balloon treatment was performed on an outpatient basis. A mean of 5 balloons (range 2 to 8) were placed per patient. All patients underwent formal urodynamic study preoperatively and at 6 weeks and 6 months following balloon placement. Results: Of the 9 patients without prior bladder neck surgery 7 had improvement in urodynamic parameters, including urethral pressure profile in all 7 and functional bladder capacity in 6, 4 were markedly improved clinically and 2 were dry. Two patients with prior bladder neck surgery were clinically unchanged following balloon placement, although 1 had urodynamic improvement. Conclusions: Our initial experience with the transurethral self-detachable balloon system as a minimally invasive outpatient procedure to treat urinary incontinence in children has been encouraging. To date this procedure appears most applicable to the patient who has not undergone surgery and has a neurogenic etiology for urinary incontinence. References 1 : Exstrophy-epispadias complex in bladder anomalies. In: . Philadelphia: Saunders1998. Google Scholar 2 : Urethral lengthening with anterior bladder wall flap for urinary incontinence: a new approach. J Urol1994; 152: 803. Google Scholar 3 : Management of urinary incontinence by bladder tube urethral lengthening and submucosal implantation. J Urol1990; 144: 559. Link, Google Scholar 4 : Experience with the Kropp anti-incontinence procedure. J Urol1989; 141: 1160. Link, Google Scholar 5 : Use of the AS800 device in exstrophy and epispadias. J Urol1988; 140: 1202. Link, Google Scholar 6 : Detachable self-sealing membrane system for the endoscopic treatment of incontinence. J Urol1997; 158: 1045. Link, Google Scholar 7 : The long-term results of artificial sphincters in children. J Urol1991; 146: 396. Link, Google Scholar 8 : Polytetrafluoroethylene injection for urinary incontinence in children. J Urol1985; 133: 248. Link, Google Scholar 9 : Glutaraldehyde cross-linked bovine collagen in exstrophy/epispadias complex. J Urol1993; 150: 631. Link, Google Scholar 10 : Endoscopic treatment of vesico-ureteric reflux and urinary incontinence: technical problems in the paediatric patient. Br J Urol1995; 75: 538. Google Scholar 11 : Treatment of urinary incontinence in children by endoscopically directed bladder neck injection of collagen. J Urol1996; 156: 637. Link, Google Scholar 12 : Endoscopic treatment for urinary incontinence in children with a congenital neuropathic bladder. Br J Urol1998; 82: 694. Crossref, Medline, Google Scholar 13 : Failure to obtain durable results with collagen implantation in children with urinary incontinence. J Urol1997; 157: 2306. Link, Google Scholar 14 : Endoscopic treatment of urinary incontinence in children with primary epispadias. Br J Urol1998; 81: 309. Crossref, Medline, Google Scholar 15 : Migration and granulomatous reaction after periurethral injection of polytef (Teflon). JAMA1984; 251: 32. Google Scholar From the Department of Urology, Children’s Hospital, Harvard Medical School, Boston, Massachusetts© 2000 by American Urological Association, Inc.FiguresReferencesRelatedDetails Volume 164Issue 3 Part 2September 2000Page: 942-946 Advertisement Copyright & Permissions© 2000 by American Urological Association, Inc.Keywordsurethrapediatricsurinary incontinenceendoscopyMetricsAuthor Information DAVID A. DIAMOND More articles by this author STUART B. BAUER More articles by this author ALAN B. RETIK More articles by this author ANTHONY ATALA More articles by this author Expand All Advertisement PDF downloadLoading ...

Urodynamic findings in children with isolated epispadias.

The exstrophy-epispadias complex represents a spectrum of anomalies with variable implications for upper and lower urinary tract function. Successful treatment of incontinence in this population is challenging and often elusive. To date few studies have focused on urodynamic findings specific to primary epispadias. We retrospectively reviewed the records of patients presenting with primary epispadias in the absence of bladder exstrophy. We identified 18 boys and 12 girls, of whom 16 underwent formal urodynamic evaluation before (5), after (6) or before and after (5) surgical narrowing of the bladder neck. Bladder capacity was recorded as percent of expected capacity for age. Bladders were considered hypertonic when end filling pressure was greater than 20 cm. water. End filling pressure was defined as the pressure at which urinary leakage was noted or the patient had discomfort. Uninhibited contractions were considered significant when the amplitude was greater than 15 cm water. The ability of the detrusor to generate a voiding contraction at the end of the filling phase was also recorded. Urodynamic studies were performed with a balloon catheter occluding the incompetent bladder neck or with a standard urodynamic catheter after formal bladder neck repair. Before bladder neck repair average bladder capacity was 157 ml (range 55 to 450), corresponding to 52% (range 22 to 100) of expected capacity. Significant uninhibited contractions were noted in 2 patients. In 7 of the 9 evaluable patients (78%) a voiding contraction was generated. In children in whom urodynamic studies were done after surgery mean bladder capacity was 260 ml (range 77 to 660), corresponding to 76% (range 36 to 147) of expected capacity. Hypertonicity and hyperreflexia were noted in 3 and 2 patients, respectively. In 5 of the 11 cases (46%) a voiding bladder contraction was generated. Of the 5 patients who underwent urodynamic evaluation before and after bladder neck repair the percent increase in bladder capacity was significantly greater in the 2 boys (67 and 110%, respectively) than in the 3 girls (-20, 10 and 20, respectively). The most frequently noted urodynamic pattern before surgical correction of the bladder neck in patients with isolated epispadias is a low capacity, highly compliant bladder with minimal detrusor dysfunction. Postoperatively capacity increases, albeit to a greater extent in boys than in girls, and the incidence of detrusor dysfunction increases as well. In some patients classic bladder neck repair may result in detrimental bladder dynamics due to as yet poorly elucidated mechanisms.