Redo surgery to improve urinary function, sexual function and cosmesis in male patients with exstrophy-epispadias complex complications. Technical principles and pearls based on case scenarios.

The bladder exstrophy-epispadias complex includes some of the most challenging conditions treated by pediatric urologists. They are associated with the need for multiple intricate reconstructive procedures, aimed at restoring the anatomy and function of the bladder, urethra and external genitalia. These patients often endure multiple redo reconstructive procedures to improve urinary function, sexual function and cosmesis throughout the first two decades of life. In this article, we present the 30-year experience of a single surgeon performing redo surgery for males born with epispadias and bladder exstrophy. Through detailed documentation of 6 clinical cases, we highlight technical aspects that may contribute to a successful surgical reconstruction in these patients. The article is focused specifically on patients undergoing redo epispadias repair with or without concomitant continence procedures. We make the case for complete penile disassembly with external rotation of the corpora to correct recurrent dorsal curvature; this approach also allows the surgeon to have access to the proximal urethra and bladder neck after opening the intersymphiseal scar/band. This is useful when additional procedures on the bladder, such as bladder neck tailoring, are necessary. We also highlight the importance of avoiding reverse Byars' flaps when performing skin closure, due to the resulting midline scar. Besides being associated with a poor cosmetic outcome, it can also contribute to recurrent dorsal curvature. The authors advocate for rotational skin flaps to cover the penile shaft. Correction of dorsal curvature and improved cosmesis obtained with complete penile disassembly sometimes comes at the expense of the urethra being left as a hypospadias (figure). This will require further surgeries (usually a 2-stage buccal mucosa graft), much like the treatment of proximal hypospadias. Redo epispadias surgery in males remains a challenge. The systematic approach offered by the case scenarios may help guide surgeons dealing with this difficult condition. Patient with complications after repair of classic bladder exstrophy. A) Stone retrieved from posterior urethra after complete penile disassembly. B) After opening the inter-symphiseal scar, the bladder has been opened and the bladder neck tailored. C) Complete penile disassembly has been completed with corporal bodies and urethra individualized. D,E,F) Final appearance of the repair; abdominal wall was closed with anterior rectus sheath flaps, penile skin was closed with rotational flaps and urethra ended up as a hypospadias.

Bladder exstrophy-epispadias complex: The effect of urotherapy on incontinence.

INTRODUCTION: Achieving urinary continence is a key goal in children born with the bladder exstrophy-epispadias complex (BEEC). Unfortunately, this goal is only moderately achieved despite sometimes extensive surgical treatment. Undergoing repeated hospitalization and operations may consequently have a negative impact on quality of life. We therefore believe that other, conservative treatment options should be explored in an earlier stage of incontinence treatment in BEEC patients. As part of this, an intensive urotherapy program based on was offered to patients with persistent incontinence after reconstructive surgery for BEEC. OBJECTIVE: The aim of this study is to evaluate the benefits of intensive urotherapy on incontinence after reconstructive surgery in children with BEEC. STUDY DESIGN: A retrospective chart study was performed including all children who were enrolled in an intensive urotherapy program because of persistent incontinence after reconstructive surgery for BEEC. Urotherapy consisted of a ten-day inpatient training program based on cognitive behavioral therapy, with intensive follow-up by experienced urotherapists. Main outcome measurement was continence, expressed as the percentage of children that achieved complete continence (good result; 100% dry) or 50-99% decrease of wet days a week (improved result) after treatment. RESULTS: Data of 33 patients with a mean age of 10.6 years were analyzed. In 61% of cases (20/33) an improved or good result was reported on incontinence after urotherapy. Children with classic bladder exstrophy more often achieved a good or improved result (13/16; 81%), compared to children with epispadias (6/16; 38%). The only patient with a cloacal exstrophy completed treatment with an improved result. From the group of patients with persistent incontinence, 75% (12/16) reported that the complaints were socially acceptable at the end of follow-up. DISCUSSION: By following our intensive urotherapy program the majority of patients achieved complete continence or improved incontinence. In addition, our results show that the inpatient training program has a positive impact on acceptance in cases of persistent incontinence. The urotherapists offer individualized care and clear guidance, which we deem essential elements of successful treatment. Considering that repeated surgery may impede progress and offers no guarantee of continence, we recommend giving preference to conservative treatment options. CONCLUSION: Our results show that 37% (12/33) of patients with BEEC who were enrolled in our intensive urotherapy program because of persistent incontinence after reconstructive surgery, achieved complete continence after urotherapy and 63% (21/33) still experienced some degree of incontinence. 75% of patients who did not achieve complete continence, described the remaining incontinence as socially acceptable. These findings strongly support counselling patients with BEEC to consider conservative treatment before opting for further surgery.

Isolated Male Epispadias Repair: Long-Term Outcomes.

Isolated male epispadias is one of the most severe congenital genital anomalies that require surgical correction. The goals of the surgery are to reach good aesthetic and functional outcomes. The aim of this retrospective study was to analyze the long-term outcomes of surgical reconstruction of male epispadias. A total of 31 patients with a mean age of 17 years, who underwent surgical repair of isolated male epispadias from January 2000 to January 2015, were involved. The main outcome measures were defined as: aesthetic outcome, continence, postoperative complications, sexual function, and quality of life. The follow-up period ranged from 8 to 23 years, with an average of 14.4 years. Each patients underwent an average of 2.2 surgical procedures in this period. The most common postoperative complications were urethral fistula and residual curvature, in 22.6% and 12.9%, respectively. Satisfactory aesthetic outcome was reported in 71.4% of cases. The repair of male epispadias usually includes more than two procedures with satisfactory aesthetic outcome. Unsolved urinary incontinence remains a significant issue and has a high impact on the quality of life. Follow-up should be extended even after complete sexual maturity. Comprehensive long-term evaluation is necessary for proper treatment of isolated epispadias.

Urothelial carcinoma occurring in a defunctionalized bladder after urinary diversion due to the bladder exstrophy-epispadias complex.

Introduction: The bladder exstrophy-epispadias complex is a rare congenital disease. Urothelial carcinomas rarely occur in patients with this disease, and there have been few reports on its treatment. Case presentation: We report the case of a 44-year-old man with a hemorrhage from the external urethral meatus. He was diagnosed with bladder exstrophy-epispadias complex and underwent urinary diversion with substitution cystoplasty and Mitrofanoff appendicovesicostomy. Because computed tomography and magnetic resonance imaging suggested invasive bladder carcinoma in the defunctionalized bladder, we performed a cystectomy. The patient was diagnosed with urothelial carcinoma with glandular differentiation. One month after the surgery, nivolumab adjuvant chemotherapy was administered. The patient showed no signs of recurrence or metastasis after the treatment. Conclusion: This is the first case of adjuvant nivolumab therapy for urothelial carcinoma with the bladder exstrophy-epispadias complex.

Innervation pattern of the unclosed detrusor muscle in classic bladder exstrophy: a study of patients with urothelial overexpression of nerve growth factor.

PURPOSE: An overexpression of nerve growth factor (NGF) in the urothelium is discussed to lead to neuronal hyperinnervation of the bladder detrusor. The aim was to assess the sensory and sympathetic innervation of the detrusor in unclosed exstrophic bladders patients with known overexpression of NGF in the urothelium. METHODS: Full-thickness bladder biopsies were prospectively obtained from 34 infants at delayed primary bladder closure between 01/2015 and 04/2020. The bladder biopsies were immunohistochemically stained with antibodies against S100, calcitonin gene-related peptide (anti-CGRP), Neurofilament 200 (anti-NF200), and tyrosine-hydroxylase (anti-TH). Specimens from 6 children with congenital vesicoureterorenal reflux (VUR) served as controls. RESULTS: There was no statistically significant difference in nerve fiber density in any of the immunohistochemical assessments (anti-S100 [p = 0.210], anti-CGRP [p = 0.897], anti-NF200 [p = 0.897]), and anti-TH [p = 0.956]) between patients with BE and patients with VUR. However, we observed a trend toward lower nerve fiber densities in exstrophic detrusor. CONCLUSION: Overall our results showed an unharmed innervation pattern in this cohort but a lower density of nerve fibers in the detrusor compared to controls. Further studies in patients after successful primary closure are needed to clarify the potential impact of the urothelial overexpression of NGF modulating the innervation pattern in exstrophic bladders.

A catheterizable serous-lined urinary outlet associated with the ileal bladder augmentation Abol-Enein and Ghoneim procedure: a safe and reliable procedure in children.

Purpose: This study aims to evaluate the long-term outcome of the serous-lined extramural continent catheterizable outlet procedure (SLECCOP) associated with ileal bladder augmentation in children. Methods: This was a monocentric and retrospective study (2002-2021) that included children (<18 years) undergoing the SLECCOP associated with W-shaped ileocystoplasty with a catheterizable channel (Abol-Enein and Ghoneim procedure). Patients who received other types of bladder augmentation or W-shaped ileocystoplasty without a catheterizable channel were excluded. Patient records were reviewed for demographic information, surgical data, and long-term outcomes. Results: This study included 52 children [33 boys, median age: 8.5 (0.8-18) years]. Pathological conditions included 28 children with the bladder exstrophy and epispadias complex (BEEC), 11 with neurogenic bladders, and 13 with other pathologies. Two patients underwent total bladder substitution. Thirty-four (65%) patients had bladder neck reconstruction (BNR), with 23 undergoing the SLECCOP and ileocystoplasty and 11 having prior BNR. All stomas, except for two, were umbilical, and were associated with omphaloplasty in 28 patients with the BEEC. A total of 40 stomas were created using the appendix (77%) and 12 with a Monti tube (23%). Stoma-related complications included cutaneous strictures (n = 2, 4%) and leaks (n = 10, 19%), all treated by dextranomer/hyaluronic acid copolymer injection (n = 10). A redo surgery was required in three patients: extraserosal wrapping was performed for persistent leakage (n = 2, 4%), and surgical revision was required for the Monti tube procedure (n = 1, 2%). Three patients (6%) underwent dilatation for transient stoma stenosis. Leakage occurred in 20% of appendix channels (n = 8/40) and 17% of Monti tubes (n = 2/12). Strictures were reported in 3% of appendix channels (n = 1/40) and 8% of Monti tubes (n = 1/12). Bladder stones developed in four patients (8%). Channel leakage persisted in one patient (2%) at a median follow-up of 4.4 years (IQR 1.4-9.7). Conclusion: W-Ileal bladder augmentation with the SLECCOP is an efficient technique for treating children with incontinence caused by different etiologies. The rate of channel complication is very low, specifically for strictures, in this complex population of patients.

Male epispadias repair: Outcomes at three sites prior to the establishment of a multi-institutional collaboration.

INTRODUCTION: Epispadias, which occurs on the more mild end of the Bladder Exstrophy Epispadias Complex (BEEC) spectrum, presents still with a wide range of severity in boys, from mild glanular epispadias to penopubic epispadias with severe urethral and bladder neck defects. Surgical management ranges from isolated epispadias repair to epispadias repair with bladder neck reconstruction (BNR) with or without pelvic osteotomies. OBJECTIVES: We aimed to evaluate outcomes in epispadias treated at three institutions prior to formation of a formal collaboration. In addition, we sought to delineate outcomes based on anatomic severity at time of diagnosis, and initial procedure performed in cases of penopubic epispadias. METHODS: IRB approved databases were retrospectively queried at three institutions for patients who underwent repair of epispadias between 1/1993 and 1/2013. Degree of epispadias, age and technique at initial repair, and self-reported continence status at last follow-up were recorded. Continence was categorized as: wet, intermediate (dry 2-3 h), or dry, while also distinguishing those who void and those who require clean intermittent catheterization (CIC). Those not seen since 1/1/2015, younger than 10 years at last follow up, or in whom continence data were not recorded were excluded. RESULTS: A total of 48 boys were identified; 36 met inclusion criteria. The epispadias cohort consisted of 8 glanular epispadias (GE) (22%); 8 penile epispadias (PE) (22%), and 20 penopubic epispadias (PPE) (56%) with a median follow-up of 11.3 years (3.2-26.2 years). Overall, 33 of 36 (92%) boys void per urethra. Within the group that voids, 19/33 (58%) are completely dry, while 6/33 (18%) are wet. Among patients who underwent initial epispadias repair without concurrent or subsequent bladder neck reconstruction, continence rates were: GE 63% (5/8); PE 75% (6/8); PPE 71% (5/7). Among the 9 boys with PPE who underwent initial epispadias repair with concurrent BNR, 22% (2/9) were dry with no further surgeries. Overall, 8/20 (40%) of boys with PPE void with complete dryness. DISCUSSION: This multi-center retrospective review of continence in epispadias demonstrates that even some boys with glanular and penile epispadias can have challenges with continence, and boys with penopubic epispadias may remain wet despite careful preoperative assessment of bladder neck functionality and concurrent BNR. CONCLUSION: Continence outcomes in boys with all degrees of epispadias can be variable. Even boys with more distal defects may have significant bladder neck deficiency. And those with the most severe form of epispadias may require bladder neck reconstruction to achieve continence.

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

BACKGROUND: This 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. METHODS: Patients 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. RESULTS: Thirty-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. CONCLUSIONS: Most 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 EVIDENCE: Treatment study - level IV.

Ensuring our exstrophy-epispadias complex patients and families thrive.

Individuals with bladder exstrophy-epispadias complex (EEC) need long-term integrated medical/surgical and psychosocial care. These individuals are at risk for medical and surgical complications and experience social and psychological obstacles related to their genitourinary anomaly. This care needs to be accessible, comprehensive, and coordinated. Multiple surgical interventions, reoccurring hospitalizations, urinary and fecal incontinence, extensive treatment regimens for continent diversions, genital differences, and sexual health implications affect the quality of life for the EEC patient. Interventions must include psychosocial support, medical literacy initiatives, behavioral health services, school and educational consultation, peer-to-peer opportunities, referrals to disease-specific camps, mitigation of adverse childhood events (ACEs), formal transition of care to adult providers, family and teen advisory opportunities, and clinical care coordination. The priority of long-term kidney health will necessitate strong collaboration among urology and nephrology teams. Given the rarity of these conditions, multi-center and global efforts are paramount in the trajectory of improving care for the EEC population. To achieve the highest standards of care and ensure that individuals with EEC can thrive in their environment, multidisciplinary and integrated medical/surgical and psychosocial services are imperative.

["Epidemiology, diagnosis, treatment and follow-up of the bladder exstrophy-epispadias complex"]. / "The making of " ­ die neue S3-Leitlinie "Epidemiologie, Diagnostik, Therapie und Nachsorge des Blasenekstrophie-Epispadie Komplexes".

The development of the new S3 guideline "Epidemiology, diagnosis, treatment and follow-up of the bladder exstrophy-epispadias complex" was funded by the German Innovation Fund of the Federal Joint Committee (G-BA). Despite the relatively low level of evidence of the identified literature, a systematic approach and consistent evaluation of the literature enabled the formulation of a large number of evidence-based recommendations across a variety of topics. In addition, a patient guideline is under development in order to disseminate the guideline recommendations and to enhance self-management and understanding among patients and their relatives. A needs analysis had been carried out in order to adequately assess the topics that are most important for patients and relatives. Upon completion of the German guideline, an English translation in cooperation with the e­UROGEN network is planned.

Course of an unplanned and unexpected pregnancy in a 39 year-old patient with Complex bladder extrophy: a case report.

BACKGROUND: With improved operative techniques pregnancy rates have been rising in patients with anomalies of the extrophy-epispadias-complex, including also female patients with bladder extrophy. Specific risks around pregnancy need to be addressed sufficiently beforehand. CASE PRESENTATION: An unplanned pregnancy was detected at 34 weeks in a 39-year old White female patient with former complex bladder extrophy. Decades after her operation she had not received any follow-up medical care and believed to be unable to conceive due to her anomaly. Thus no contraceptive matters were taken. The patient had lived in a stable relationship with regular sexual intercourse for many years. Until 34 weeks the pregnancy was uncomplicated, but then uterine prolapse and signs of beginning pre-eclampsia appeared, and a healthy girl was born with cesarean section. CONCLUSION: As patients with bladder extrophy and other anomalies from the extrophy-epispadias-complex reach adolescence/adulthood, they need continuous medical follow-up and transition of care to adult surgery and gynecology in order to address specific aspects of sexual health, reproduction, contraception, and also cancer screening. In the presented case lack of transition of care resulted in an unplanned and complicated pregnancy.

An observational study on the sexual, genital and fertility outcomes in bladder exstrophy and epispadias patients.

INTRODUCTION: Bladder exstrophy and epispadias complex (BEEC) is a spectrum of congenital malformations ranging from an isolated epispadias to a full exstrophy. It is an uncommon disease and little is known on how patients cope with its implications later in life. OBJECTIVE: The goal of this study is to assess the sexual, continence and fertility outcomes of BEEC patients, who had reconstructive bladder surgery during childhood. Considering the sensitive nature of these topics, they are not easily spoken about in the doctor's office. Our aim is to shed some light on possible points of improvement in follow-up. STUDY DESIGN: 63 patients between 18 and 45 years old were sent an electronic questionnaire based on previous existing standardized questionnaires. They were asked about sexual and psychosexual wellbeing, urinary incontinence and fertility. Data from their medical files (medical history on previous surgeries). and questionnaire answers are linked through an anonymous subject number and put into an Excel file for descriptive representation. RESULTS: 22 men and 8 women filled in the questionnaire. All but 2 are sexually active. Reasons to avoid sexual activity are equally divided as BEEC-related and non-BEEC-related. Sexual satisfaction is lower in the male group due to problems with erection, ejaculation, condom usage and embarrassment about physical appearance. In females problems concerning pain and reaching orgasm are mentioned. 30% report depressive feelings. There is a clear correlation between number of reconstructive surgeries and sexual satisfaction. 90% of patients urinate via catheterization, mostly through a Mitrofanoff connection. This leads to complications such as foul odors, infection, embarrassment and sexual dysfunction. 8 out of 13 men conceived a child (with the use of their own sperm), 2 out of 4 women did. DISCUSSION: A strength of this study is the use of standardized questionnaires which allow comparison to a control patient group. Our study is one of the first to show how patients cope with the challenges of BEEC by the use of open questions. We see an overall high quality of life yet an important impact on mental health. CONCLUSION: BEEC is associated with many challenges in the adult life of patients. A more holistic and interdisciplinary approach is needed to include sensitive topics in long term follow-up.

A Rare Case of Isolated Male Epispadias.

Epispadias is a congenital malformation marked by the failure of the urethral bulb to tubularize dorsally. This results in a wide-open urethral plate dorsally. Epispadias is frequently associated with bladder exstrophy-epispadias complex.  We report the case of a five-year-old patient who presented in the outpatient department with isolated epispadias without any other associated abnormality. This report aims to document this rare case of isolated male epispadias with incontinence and the success of using the modified Cantwell-Ransley technique for its treatment.

Assessment of Anorectal Function and Related Quality of Life of 27 Patients with Bladder Exstrophy or Epispadias After Kelly Radical Soft Tissue Mobilisation.

INTRODUCTION: The Radical soft-tissue mobilisation (RSTM) described by J.H. Kelly for bladder exstrophy repair implies a detachment of levator ani muscle insertions from the pelvic wall. The aim of this controlled study was to evaluate the impact of this procedure on subsequent anorectal function. METHODS: Monocentric controlled study of prospectively collected data of children who underwent RSTM for BEEC from 2010 to 2017. Patients born after 2017 were not included, as they were below the theoretical age of continence acquisition at the time of the study. Anorectal function was assessed using the Childhood Bladder and Bowel Dysfunction Questionnaire, and quality of life (QoL) related to fecal continence using the CINCY FIS questionnaire. The control group was paired on age and sex with a 1:3 patient/control ratio. Answers to questionnaires were collected from September 2021 to January 2022. Univariate statistical analysis comparing two groups and subgroup analysis following age were also performed. RESULTS: During the period of study, 55 children with BEEC underwent Kelly RSTM. Twenty-seven (49%) were included and paired with 81 healthy children on age and sex. Median age at surgery was 15 months [0.5-93] and median follow-up was 10 years [4-13]. Patient's group median age at evaluation was 11 years [5-19]. There was no difference between patients and control group in anorectal function for both incontinence and constipation items. No significant difference was found in QoL related to fecal incontinence assessment. Subgroup analysis did not show difference. CONCLUSION: This study suggests that the levator ani detachment during Kelly procedure, realised in a paediatric population under the age of 8, did not impact anorectal function with a mid-term follow-up. LEVEL OF EVIDENCE: III.

Classical bladder exstrophy in an adolescent: A case report on management, challenges and outcome.

Classical bladder exstrophy is a congenital anomaly whose management and outcome has advanced over years. Management and outcome are better when management starts at the newborn period. This was the management of a neglected bladder exstrophy in a male presenting at 16 years of age. We report our challenges, management and outcome to highlight the rarity of this presentation, and the adaptation to the usual protocol of care. The patient presented at 16 years of age with classic bladder exstrophy. The bladder plate was contracted and had cystitis. The patient had a modification of complete primary repair of exstrophy (CPRE) with bilateral pelvic osteotomy stabilised with a 7-hole plate and 4 screws, then bladder neck reconstruction + bladder augmentation + cross-trigonal neocystoureterostomy in a 12-h procedure. He had surgical site infection, superficial wound breakdown and vesicocutaneous fistula that all healed with dressing and prolonged suprapubic cystostomy drainage. He achieved some degree of urinary continence and ability to void, though he still has stress incontinence and frequency at 6 months of follow-up. He has a micturition interval of 60-120 min, and is expected to improve. Presentation and repair of classic bladder exstrophy in the adolescent is very rare in the literature and therefore no known standard of care. This report adds to the body of knowledge. Again, this experience lends credence to the proponents of CPRE in reducing the number of procedures required to treat exstrophy.

Exstrophy-Epispadias Complex.

Exstrophy-epispadias complex encompasses a spectrum of disorders with lower abdominal midline malformations, including epispadias, bladder exstrophy, and cloacal exstrophy, also known as Omphalocele-Exstrophy-Imperforate Anus-Spinal Anomalies Complex. In this review, the authors discuss the epidemiology, embryologic cause, prenatal findings, phenotypic characteristics, and management strategies of these 3 conditions. The primary focus is to summarize outcomes pertaining to each condition.

Surgical correction of epispadias associated with buried penis.

INTRODUCTION AND IMPORTANCE: Isolated male epispadias is a rare entity with incidence of approximately 1 in 120,000 live births. Epispadias usually presents with a phimotic preputial orifice where glans is not visible and hence is also known as concealed epispadias. Buried penis in children is defined as a congenital insufficient penile skin with an unretractable foreskin that keeps the penis deep inside the pre-pubic fat. This congenital malformation of the penile envelopes is usually isolated. However, in some cases the concealed penis hides an underlying penile anomaly. We present surgical repair of a very rare case with concealed epispadias. CASE PRESENTATION: A nine-month-old infant had buried his penis, and his mother was seeking his circumcision. Local examination revealed concealed penopubic epispadias. A pediatric surgeon operated on this patient using the modified partial penile disassembly technique. The patient was doing well at follow-up visits at one, three, and six months. There were no urethral stricture or obstructive urinary symptoms. The parents were satisfied with the cosmetic outcome. CLINICAL DISCUSSION: The embryogenesis and development of the urethra and the prepuce are linked. Urethral development defects (as in hypospadias or epispadias) are frequently coupled with faulty prepuce on the same side. The goal of surgical management for epispadias is to correct the dorsal chordee and reconstruct the epispadiac urethra and glans. Based on the cosmesis of the penis reconstruction, preservation of erectile function, and achieving urine continence, the outcome is evaluated. CONCLUSION: Concealed epispadias is frequently ignored because patients appear with buried penis, non-retractile prepuce, and a normal urine stream. Preoperative diagnosis and parent counseling are critical for the effective treatment of this uncommon entity. The modified partial penile disassembly procedure, in which the tunica albuginea is stitched to the pubic periosteum at 3 and 9 o'clock, can be used to correct buried epispadias.

Marked Metabolic Acidosis Due to a Transverse Stoma after Urethroplasty for Congenital Epispadias.

A 58-year-old woman was admitted to our hospital. At 10 years old, she had undergone bilateral uretero-sigmoid anastomosis for congenital epispadias, and at 57 years old, she had received transverse colostomy. Biochemical tests showed marked metabolic acidosis. Computed tomography showed urine stagnation in the sigmoid colon, leading to a diagnosis of metabolic acidosis associated with transverse stoma after bilateral uretero-sigmoid anastomosis. Her bone mineral density was below normal, and the bone metabolic marker levels were high, indicating high-turnover osteoporosis. Both metabolic acidosis and bone metabolism were stabilized by treatment with a transanal urinary catheter, sodium bicarbonate, and vitamin D.

Prevalence of opioid and benzodiazepine use in adult patients with the exstrophy-epispadias complex.

INTRODUCTION: Bladder exstrophy (BE), cloacal exstrophy (CE), and epispadias (E) are variants of the exstrophy-epispadias complex (EEC). These children require opioids and benzodiazepines to achieve pain management and immobilization for a lifetime of surgeries. It is hypothesized that these children would be sensitized to opiates and benzodiazepines as adults. The objective was to identify incidence of opiate and benzodiazepine use in adult EEC patients. METHODS: A US Health network, TriNetX Diamond was queried from 2009 to 2022. Incidence of prescriptions for benzodiazepines and opioids were calculated for adults aged 18-60 years with a diagnosis of BE, CE, or E. RESULTS: A total of 2627 patients were identified: 337 with CE, 1854 patients with BE, and 436 with E. Of these, 55.5% of CE, 56.4% of BE, and 41.1% of E had received any opioid prescription. Non-EEC controls had lower rates of opioids at 0.3%. E had a lower likelihood than BE or CE of receiving opioids (p < 0.0001, p < 0.0001). Benzodiazepines were prescribed in 30.3% of CE, 24.4% of BE, 18.3% of E, and 0.1% of controls. CE had a higher likelihood of benzodiazepines than both BE and E (p = 0.022, p < 0.001, respectively). E group had the lowest likelihood of benzodiazepine prescription (p = 0.007 when compared to BE) and all groups were significantly higher than controls (p < 0.0001 for all comparisons). For BE, females were more likely to be prescribed opioids (p = 0.039) and benzodiazepines (p = 0.027) than males. Sub-analyses revealed BE females had higher rates of surgical procedures (general, cardiac, gastrointestinal, and maternity) and chronic diagnoses (generalized anxiety disorder, major depressive disorder, chronic pain) compared to males with BE. Older age was associated with higher likelihood of opioid or benzodiazepine prescriptions in BE (p < 0.001), CE (p = 0.004), and E (p = 0.002). DISCUSSION: Across the EEC, adult patients with the most severe anomalies of CE were more likely to have received opioids and benzodiazepines. Females with BE were prescribed more opioid and benzodiazepines than males with BE. Mirroring the US population, female sex and increasing age were associated with higher rates of prescriptions, chronic diagnoses, and surgical procedures. Limitations include the lack of granular data and ability to correlate results with childhood surgeries. CONCLUSION: Adult EEC patients have higher rates of opioid and benzodiazepine prescriptions, with a high percentage of co-prescribing when compared to healthy controls. Across the spectrum, those with more severe anomalies, female sex, and increasing age were more likely to have received prescriptions.

"We the BE": An educational mobile health application for children and families affected by bladder exstrophy-epispadias-cloacal exstrophy complex.

GOALS: Despite the proliferation of over 45 000 smartphone mobile health applications (MHAs), as far as we know, there is no MHA for those living with rare diseases such as Bladder Exstrophy-Epispadias-Cloacal Exstrophy complex (BEEC). We hypothesized that an MHA could provide similar "on-demand" information and connectivity within health communities for patients with BEEC as they do for more common diseases. Thus, our primary goal was to create an MHA for patients and families affected by BEEC to provide them with important information about the condition and a format for them to connect with other affected patients and families. A secondary goal was to develop an adaptable MHA template for other rare diseases in the future. METHODS: We began our app development by examining existing common-disease MHAs for thematic structure. We conducted an extensive literature search of PubMed and Google scholar for MHA development and existing MHAs related to BEEC, utilizing these search terms: mobile health applications, rare diseases, bladder exstrophy, and online health communities. Our app development team began with our clinical multidisciplinary team of pediatric urologists; a child psychiatrist; a patient/family mental health therapist; and a certified nurse practitioner. We hired a website engineer and a production team. All clinical members have extensive experience caring for children and families affected by BEEC. Additionally, clinical team members compiled lists of themes deemed relevant from these reviews and themes gleaned from their clinical experience that appear with some frequency or urgency and from the myriad of themes discussed within the literature for MHAs. RESULTS: We found no existing rare disease MHAs in the literature or our search of app stores online. However, we derived basic app categories from existing MHA formats and the thematic content of all sources reviewed. These categories aligned with the groupings of our lists of clinical themes. Thus, we could subsume diverse themes within a broad categorical format: for example, child development (as "Psychological Development" in the app) or various clinical care options (as "Treatment"). This app structure became nine sections, as shown in. This format allows diverse information to be retrieved efficiently from broader categories. This app is being offered to affected families, healthcare providers, and individuals unrelated to where care is offered. CONCLUSION: "We the BE" is the first MHA developed for a rare disease, BEEC. It has been published in a downloadable format for the general public at no cost. Further research is required to determine its efficacy for the BEEC community members; preliminary, unsolicited feedback from multiple users has been positive.