Re: Quality of life among women in Bangladesh following ileal conduit urinary diversion operations for irreparable vesicovaginal fistula and bladder exstrophy; observational study.

COMPLETE PRIMARY REPAIR OF EXSTROPHY

Bladder exstrophy and epispadias complex is a rare congenital anomaly with significant medical and psychosocial implications. We employed a dual approach to evaluate the prevalence and risk factors of psychiatric morbidity in bladder exstrophy and epispadias patients, by combining a systematic review with a retrospective national registry study. To evaluate the prevalence and risk factors of psychiatric morbidity in bladder exstrophy and epispadias complex. We conducted a systematic literature review following Preferred Reporting Items for Systematic Reviews and Meta-analyses guidelines, including studies on psychiatric disorders or symptoms and quality of life in bladder exstrophy and epispadias patients of all ages. Article quality was assessed using the Newcastle-Ottawa Scale. Prevalence data for psychiatric conditions and scores from psychiatric and quality-of-life questionnaires were extracted. Additionally, all bladder exstrophy and epispadias patients born in Finland 2001-2006 were identified from national registries. A matched and randomized control group without congenital malformations was selected from the same population. We evaluated the prevalence and risk factors for psychiatric diagnoses in this cohort. Of the 3850 retrieved results, 30 studies with 1179 participants were included. The overall prevalence of psychiatric morbidity was 31 %, [95 % CI 17 %-47 %]. Psychiatric morbidity was highest in adolescents, while morbidity in adults was comparable to the general population. In the Finnish cohort, 80 % (n = 16/20) of bladder exstrophy and epispadias patients had a psychiatric diagnosis compared to 26 % (n = 21/80) of the controls (OR 11.2, [95 % CI 3.37-37.4], p < 0.001). The severity of the anomaly, number of surgeries, maternal unemployment, or maternal psychiatric diagnosis were not significantly associated with increased psychiatric morbidity. Bladder exstrophy and epispadias is associated with an elevated risk of psychiatric morbidity. Our dual approach highlights the need for systematic mental health assessment and support in this population.

To examine long-term quality-of-life, urinary continence and sexual function outcomes in patients diagnosed with bladder exstrophy (BE). A total of 65 patients with BE and follow-up of at least 20 years were identified. After informed consent for inclusion in the study, the patients were asked to complete three validated questionnaires, the Short-Form 36 quality-of-life questionnaire (SF-36), the International Consultation on Incontinence Questionnaire (ICIQ) and the International Index of Erectile Function (IIEF), to assess quality of life, perceived urinary continence and sexual function. In all, 21 patients responded to the questionnaires, yielding a 32% response rate. High scores in each of the eight dimensions of the SF-36 reflected a positive perception of quality of life by respondents; calculated scores were compared with those of a normal control group. The only significant difference found between the groups was that the study population perceived their general health to be poorer than those in the control group. High scores on the ICIQ indicate high levels of subjective incontinence in patients, with scores ranging from 0 to 21. Reporting scores of 0 (continent), 12/21 patients perceived their continence to be normal, 9/21 patients had scores >2 (mild), with one patient scoring a 16, and 11 (severe incontinence), patients reported no identifiable leakage during normal activities. There was a 29% response rate for the IIEF (15 patients completed this). IIEF scores were broken down into five dimensions and mean scores were calculated. The mean scores showed mild to moderate dysfunction in each category, including overall satisfaction with sexual experience. The patient-reported quality of life in patients with BE was normal in all dimensions, with the exception of perception of general health. Half of the patients reported normal continence and had no complaints of urinary leakage. Sexual function in males was significantly affected across all dimensions, with mild to moderate dysfunction.

During the last 20 years 50 children with exstrophy of the bladder were treated in the Department of Paediatric Surgery of the Children's Hospital of Cologne employing besides other surgical methods such as primary closure, ureterosigmoideostomy, ureterocutaneostomy etc., in 15 cases an ileal conduit and in 12 cases a colonic conduit. These children could be followed up on the average 8.5 or 3 years after the operation, clinically, roentgenologically and in some cases via scintigraphy. Late complications requiring surgical correction, such as stomatostenoses, conduit elongation, stenoses of the ureterointestinal anastomosis, calculus formation in the conduit, or complications like ureteral reflux, recurring infections of the urinary passages with pyelonephritis, occurred only with ileal conduits, whereas no late complications requiring surgery were seen with the colonic conduits. Similar results in respect of late complications were found among the patients in Munich from 1955 to 1983 with 35 exstrophies of the bladder (13 ileal conduits, 1 colonic conduit) in which additionally an adeno-carcinoma was seen after ureterosigmoideostomy with fatal outcome. Hence, we are of the opinion that the method of choice is the preparation of a colonic conduit in patients with exstrophy of the bladder where primary closure is not possible because the bladder lamina is too small or already epithelialised. This approach offers the safest possible long-term protection of the primary normally positioned upper urinary tract.

Bladder exstrophy (BE) is a rare congenital malformation of the genitourinary tract, as well as surrounding structures, within the spectrum of the exstrophy-epispadias complex. This review aims to summarize the current literature on repair procedures, specifically the modern staged repair and complete primary reconstruction, along with their associated complications and complication rates. A particular focus is placed on long-term management strategies to address these complications as patients transition into adulthood. Additionally, this review highlights the management of sexual health for both males and females, fertility considerations, and overall quality of life, emphasizing that patient care extends beyond voiding function. By incorporating these broader aspects, the review provides a global perspective on patient management, which is crucial for the entire multidisciplinary care team. Due to the rarity of BE, the available data is inherently limited, and findings may not be generalizable to every patient. Despite these challenges, this review synthesizes the existing literature through comprehensive searches conducted on PubMed and Google Scholar, thematically organizing findings to identify well-recognized complications and their management. The importance of multidisciplinary care, extending beyond pediatric urology and including adult urologists, is critically emphasized to ensure a seamless transition of care as patients age. Finally, this review underscores the need for ongoing research to establish standardized long-term management protocols, particularly as patients with BE are living longer lives due to advances in surgical techniques and medical care.

Half Century of Followup After Ureterosigmoidostomy Performed in Early Childhood

Bladder exstrophy is difficult and rare congenital malformations. Occurs in three clinical forms: incomplete, complete or classic (exstrophy-epispadias complex) and third, cloacal exstrophy which is a much more severe abnormality, with significant involvement of other organ systems. The modern ultrasound devices allow great advances in the diagnosis, a large number of controls and adequate monitoring of pregnant women. However, there are still special circumstances without appropriate visualisation of the fetus. It is important to keep delivery without additional obstetric complications as well as neonatology care . The final outcome of operation depends of child vitality and the anomaly itself. The case shows of a female child born in Gynecology- Obstetrics clinic in Kragujevac with classic bladder exstrophy and epispadias that we followed until third month of life. Team of gynecologist - neonatologist - pediatric surgeon - pediatrician can provide successful surgical outcome and prevent complications. The most important is that the child has preserved psychomotor development and the possibility of certain quality of life.

An Initial Evaluation of Pelvic Floor Function and Quality of Life of Bladder Exstrophy Patients After Ureterosigmoidostomy

Background: Urinary diversion continues to present considerable challenges in urological surgery. The Heitz-Boyer-Houvelac uretero-rectostomy, first described in 1912 and later validated experimentally by Shaw in 1937, remains a foundational approach. Bladder exstrophy, a severe congenital anomaly affecting the lower abdominal wall, urinary tract, genitalia, and pelvic bones, is a principal indication for urinary diversion. Despite advances in reconstructive techniques, approximately 10% of exstrophic bladders are of inadequate capacity and 30% of patients remain incontinent following staged reconstruction. This study aimed to evaluate the efficacy and safety of urinary diversion using a modified Duhamel uretero-rectostomy technique in the management of urinary incontinence associated with bladder exstrophy, focusing on renal preservation, continence outcomes, cosmetic results, and long-term complications. Patients and Methods: A retrospective review (2009–2014) followed by a prospective analysis (2014–2021) was conducted on 27 patients (19 males, 8 females; mean age 6.8 years) treated by a single surgeon at two centers in Duhok, Iraq. Postoperative assessments included renal function, serum electrolytes, urinary pH, ultrasound, proctography, and annual proctoscopy with rectal biopsy. Results: Mean operative duration was four hours, with uneventful recovery in all cases. Rectal pouch capacity increased significantly to a mean of 330 mL at one year. Continence improved progressively, and urinary tract infection occurred in four patients, with one requiring surgical correction. No histopathological abnormalities were observed. Conclusion: The modified Duhamel uretero-rectostomy provides a reliable, technically straightforward, and functionally effective option for urinary diversion in bladder exstrophy, demonstrating durable long-term outcomes and a low complication rate.

Ureterorectostomy as a continent urinary diversion for complicated bladder exstrophy in children by using a modified Duhamel procedure: A case series

Quality of life of children operated for bladder exstrophy.

Exstrophy of bladder is a rare congenital malformation of the bladder with the lack of its front wall and, consequently, the anterior abdominal wall, which is often accompanied by epispadias, inguinal hernia, prolapse of the rectum, anomalies of the upper urinary tract. Its exstrophy of bladder frequency ranges on average from 1:10 000 to 1:50 000 newborns, in boys 3-6 times more often than girls. Without surgical treatment half of children with this disorder do not live to the age of 10, and 75% die in age 15.This article describes a clinical case of a patient M., 18 years old, who was admitted to the gynecological department number 1 of the Donetsk Regional center for maternal and child health for forming of vagina. The patient had a congenital malformation of the genitourinary system in the form of bladder exstrophy, at age 2 and 5 she had reconstructing operations to form the front abdominal wall of local fabrics, made at age 7 reconstruction of bladder neck has not led to a controlled urinate. Internal genital organs with no pathology except atresia of the vagina. After careful examination, using modern laboratory, instrumental and apparatus research methods patient M. was performed laparotomy in a volume: old postoperative scar excision; formation of rectal bladder, ureterorectostomia and bringing down the sigmoid colon by Duhamel; vaginoplasty.Surveillance for 75 days after the operation showed the following results: control urination and defecation, lack of urine leakage from the anterior abdominal wall and the presence of formed vagina, improving the quality of life and patient’s reproductive plans.This considered a clinical case illustrates the high efficiency of treatment of bladder exstrophy with individual methodological approach to the selection of an adequate surgical tactics.

The latissimus dorsi flap for the repair of an eventration on bladder exstrophy in an adult patient: A case report

To conduct a prospective comparison of quality-of-life (QoL) outcomes in patients who underwent ileal conduit (IC) urinary diversion with those who underwent orthotopic neobladder (ONB) reconstruction after radical cystectomy for invasive bladder cancers. Between January 2007 and December 2012, 227 patients underwent radical cystectomy and either IC urinary diversion or ONB (sigmoid or ileal) reconstruction. Contraindications for ON were impaired renal function (serum creatinine >2 mg/dL), chronic inflammatory bowel disease, previous bowel resection and tumour involvement at the bladder neck/prostatic urethra. Patients who did not have these contraindications chose to undergo either IC or ONB reconstruction, after impartial counselling. Baseline characteristics, including demographic profile, body mass index, comorbidities, histopathology of the cystoprostatectomy (with lymph nodes) specimen, pathological tumour stage, postoperative complications, adjuvant therapy and relapse, were recorded and compared. The European Organization for Research and Treatment of Cancer QoL questionnaire C30 version 3 was used to analyse QoL before surgery and 6, 12 and 18 months after surgery. Of the 227 patients, 28 patients in the IC group and 35 in the ONB group were excluded. The final analysis included 80 patients in the IC and 84 in the ONB group. None of the baseline characteristics were significantly different between the groups, except for age, but none of the baseline QoL variables were found to be correlated with age. In the preoperative phase, there were no significant differences in any of the QoL domains between the IC or the ONB groups. At 6, 12 and 18 months in the postoperative period, physical functioning (P < 0.001, P < 0.001 and P = 0.001, respectively), role functioning (P = 0.01, P = 0.01 and P = 0.003, respectively), social functioning (P = 0.01, P = 0.01 and P = 0.01, respectively) and global health status/QoL (P < 0.001, P < 0.001 and P = 0.002, respectively) were better in patients in the ONB group than in those in the IC group and the differences were significant. The financial burden related to bladder cancer treatment was significantly lower in the ONB group than in the IC group at 6, 12 and 18 months of follow-up (P = 0.05, P = 0.05 and P = 0.005, respectively) ONB is better than IC in terms of physical functioning, role functioning, social functioning, global health status/QoL and financial expenditure. ONB reconstruction provides better QoL outcomes than does IC urinary diversion.

We describe a case of a postmenopausal woman with a corrected bladder exstrophy (BE) who presented with a uterovaginal prolapse complicated by calcified fibroids and discuss its surgical management. A 51-year-old woman presented with a uterovaginal prolapse. She had previously had one full term delivery by caesarean section 20years ago. She had an ileal conduit performed for her BE at birth. A computed tomography scan revealed significant pelvic anomaly and the uterus contained multiple calcified fibroids. Due to the complexity of her condition the surgical management was planned in two stages: initial examination under anaesthetic in conjunction with a consultant urologist and then the definitive planned reconstructive surgery. This case describes the need for careful evaluation and planning of complex surgery and the successful surgical management option for correction of uterovaginal prolapse in patients with BE. A vaginal approach seemed to be a most sensible route to correct the prolapse in view of her previous multiple abdominal surgeries.