Cloacal Exstrophy Research Articles

The clinical features of patients who underwent bladder augmentation of cloacal exstrophy and their functional outcomes: the results of a nationwide survey in Japan.

Cloacal exstrophy (CE) patients may need bladder reconstruction after initially undergoing surgery to obtain continence and improve their quality of life. This study attempts to clarify the clinical features of CE patients who underwent bladder augmentation (BA) and their urinary functional outcomes based on a nationwide survey in Japan. A questionnaire survey was conducted, and 150 CE patients were enrolled. Their clinical characteristics and urinary outcomes were reviewed. BA was performed in 52 patients (34.7%). Most cases underwent early bladder closure at initial surgery in neonate period. The age at the BA was performed 6.4 [6-9.0] years. Among them, the most used organ for BA was ileum (n = 30, 57.7%). Regarding the outcomes, the age when the renal function was evaluated was 14.0 [10.0-20.5] years and the serum creatinine level was 0.44 [0.36-0.60] (mg/dl). Clean intermittent catheterization was required in 37 (71.2%) patients. On the other hand, no dialysis or kidney transplantation was necessary in any of these patients. The renal function and conditions of patients who underwent BA were relatively well preserved. Individualized management with a stepwise surgical approach for CE patients should thus be considered in the future.

Urethral Plate Grafting for Deficient Urethral Plate in Exstrophy: An Alternative Method to Augment Urethral Plate

IntroductionShort urethral plate remains a challenge in exstrophy management. We report our experience with urethral plate grafting in cases of exstrophy with deficient urethral plate. MethodsAmong the exstrophy patients treated at the authors’ institutions (2018–2022), those with a short urethral plate were prospectively included. A short urethral plate was defined as a distance between the verumontanum and the base of the glans of less than 10 mm. Urethral plate grafting was performed electively before the exstrophy closure. The urethral plate was divided just distal to verumontanum, and a thin inner preputial or para-exstrophy skin graft was harvested and deployed to cover the defect. Exstrophy closure was subsequently performed. The following parameters were recorded: age at grafting, type of graft and age at exstrophy closure. Reported outcomes include success of closure, complications, and follow up. ResultsSix male patients were included in the study: 3 classic bladder exstrophy (CBE) and 3 cloacal exstrophy (CE). Median age at grafting was 9 (3–18) months. Inner preputial grafts were utilized in the 3 CBE patients, and para-exstrophy skin grafts were used for the 3 CE patients. There was no graft loss, and longer and wide urethral plate was seen in all cases. Median time to bladder exstrophy closure was 3 (3–13) months after grafting. ConclusionPre-closure urethral plate grafting represents a safe and effective option for exstrophy patients with a short or inadequate urethral plate.

Mucosal Violations and Their Effect on Successful Bladder Neck Closure in Cloacal Exstrophy

BackgroundCloacal exstrophy (CE) is rare and challenging to reconstruct. In the majority of CE patients voided continence cannot be achieved and so patients often undergo bladder neck closure (BNC). Prior mucosal violations (MVs), a surgical event when the bladder mucosa was opened or closed, significantly predicted failed BNC in classic bladder exstrophy with an increased likelihood of failure after 3 or more MVs. The aim of this study was to assess predictors for failed BNC in CE. MethodsCE patients who underwent BNC were reviewed for risk factors for failure including osteotomy use, successful primary closure, and number of MVs. Chi-squared and Fisher's exact tests were used for comparing baseline characteristics and surgical details. ResultsThirty-five patients underwent BNC. Eleven patients (31.4%) failed BNC including a vesicoperineal fistula in nine, vesicourethral and vesicocutaneous fistula in one each. The fistula rate in patients with 2 or more MVs was 47.4% (p = 0.0252). Two patients subsequently developed a vesicocutaneous fistula after undergoing repeated cystolithotomies. A rectus abdominis or gracilis muscle flap were used to close the fistula in 11 and 2 patients, respectively. ConclusionsMVs have a greater impact in CE with an increased risk of failed BNC after 2 MVs. CE patients are most likely to develop a vesicoperineal fistula while a vesicocutaneous fistula is more likely after repeat cystolithotomy. A prophylactic muscle flap should be considered at time of BNC in patients with 2 or more MVs. Levels of EvidencePrognosis Study, Level III.

A Single-Institutional Experience With Prenatal Diagnosis of Cloacal Exstrophy: Room for Improvement

IntroductionA single institutional study characterizes the rate of prenatal diagnosis of cloacal exstrophy (CE) and examines its role on successful primary closures. Materials and MethodsAn institutional database of 1485 exstrophy-epispadias patients was reviewed retrospectively for CE patients with confirmed presence/absence of prenatal diagnostics, primary exstrophy closure since 2000, institution of closure, and at least 1 year of follow up following closure. ResultsThe cohort included 56 domestic patients and 9 international patients. Overall, 78.6% (n = 44) of domestic patients were prenatally diagnosed while 21.4% (n = 12) were diagnosed postnatally. A positive trend was observed in the rate of prenatal diagnosis across the study period, 56.3%, 84.2%, 88.9% respectively (p = 0.025). Confirmatory fMRI was obtained in 40.9% (n = 18) of prenatally diagnosed cases. Patients diagnosed prenatally were found to be more likely to undergo treatment at exstrophy centers of excellence (72.1% v 33.3%, p = 0.020). Prenatal diagnosis was not predictive of increased rate of successful primary closure (75.6% vs 75.0%; p = 1.00; OR: 1.03, 95% CI: 0.23–4.58). Primary closures undertaken at exstrophy centers of excellence were significantly more likely to be successful compared to outside hospitals (90.9% v 50.0%, p = 0.002). ConclusionsThe rate of prenatal diagnosis of CE in patients referred for management to a high-volume exstrophy center is improving. Despite this improvement, patients continue to be missed in the prenatal period. While prenatal diagnosis offers the ideal opportunity to educate, counsel, and prepare expectant families, patients diagnosed at birth are not disadvantaged in their ability to receive a successful primary closure. Further research should investigate the benefit of patient referral to high-volume exstrophy centers of care to ensure optimal care and outcomes.

Bladder exstrophy-epispadias complex related litigation: A legal database review.

To review the nature and extent of bladder exstrophy-epispadias related malpractice litigation in the United States. Two legal databases (Nexis Uni, WestLaw) were reviewed for state and federal cases using the terms "bladder exstrophy", "cloacal exstrophy", "epispadias", in combination with "medical malpractice", or "negligence", or "medical error", or "complication", or "malpractice", or "tort". Databases were queried from 1948 to 2022 and reviewed for medical and legal details. Our search yielded 16 unique legal cases with 6 fitting established criteria for analysis. Urology and paediatric urologists were named in 50% of cases as were community medical systems. Cause for lawsuit included negligence in surgical performance (50%), primary closure of exstrophy (33%), and post-operative care (50%). Settlement agreement was reached in one case (17%). Outcomes favoured the physician in 60% of trials. Lawsuits alleging negligent surgical performance and/or post-operative care exclusively named urologists with outcomes favouring the surgeon in 66% of cases. The settlement payment (n = 1) was $500,000 and monetary damages (n = 1) equated to $1.3 million. Malpractice litigation related to BEEC treatment is rare. Trial outcomes favour the medical provider. Cases that resulted in financial liability successfully alleged avoidable negligence resulting in irreversible physical damage. The authors recommend families with BEEC seek board-certified paediatric urologists experienced in treating this complex and/or Bladder Exstrophy Centers of Excellence. Further, we recommend surgeons treating BEEC properly educate patients and families on the severity of this major birth defect including its lifelong implications and need for surgical revisions.

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

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. 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. 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). 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. 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.

Spinal dysraphism in exstrophy: a single-center study of a 39-year prospective database.

The two main objectives of this study were to explore the rate of spinal dysraphism within bladder and cloacal exstrophy and to analyze the relationship between spinal dysraphism surgery, including timing of spinal dysraphism surgery, with urological and neurological outcomes. A prospectively maintained IRB-approved database of pediatric exstrophy patients treated from 1982 to 2021 was retrospectively reviewed for patients with spinal dysraphism. Spinal dysraphism was categorized into the following 7 subtypes: lipoma-based closed defect, myelomeningocele, meningocele, diastematomyelia, myelocystocele, low-lying conus with tethered cord/fatty filum, and sacral bony defect. Other factors assessed included patient demographic characteristics, type of spinal dysraphism procedure, reoperation, complication, presence of other neurological problems (e.g., hydrocephalus, Chiari malformation), neurological status, and urological function. Analysis revealed that 114/1401 patients had coexisting spinal dysraphism. Of these 114, sufficient records including type of dysraphism were available for 54. Spinal dysraphism was most common within cloacal exstrophy (83.3% [45/54 patients]), followed by cloacal exstrophy variants (9.3% [5/54]), classic bladder exstrophy (3.7% [2/54]), and classic bladder exstrophy variants (3.7% [2/54]). Within spinal dysraphism, lipoma-based closed defects (63.0% [34/54]) and low-lying conus with tethered cord/fatty filum (11.1% [6/54]) were most common. Hydrocephalus and Chiari malformation occurred in 24.1% (13/54) and 11.1% (6/54) of patients. All 13 patients with hydrocephalus underwent shunt placement. Among those who underwent neurosurgical intervention, the complication rate for spinal dysraphism was 14.6% (7/48). Motor function data were available for 41 patients and revealed that motor function declined for 2/41 (4.8%) patients and improved for 6/41 (14.6%) after neurosurgery. There was no statistical difference in lower-extremity motor outcome related to timing of neurosurgery and exstrophy closure. The authors have reported the surgical management and outcomes of patients with exstrophy and coexisting spinal dysraphism (n = 54). In 54 patients, spinal dysraphism was most common in the subset of patients with cloacal exstrophy (83.3%). Lipoma-based closed defects (63.0%) and low-lying conus with tethered cord/fatty filum (11.1%) were the most common, and the rates of hydrocephalus and Chiari malformation were 24.1% and 11.1%, respectively. There was no difference in lower-extremity motor outcome related to timing of neurosurgery and exstrophy closure.

Urinary Bladder Cancer in Bladder Exstrophy and Epispadias Complex: A Swedish Register Study and a Systematic Review of the Literature

Abstract The congenital malformation bladder exstrophy and epispadias complex involves multiple organs and includes 3 subtypes: epispadias, classic bladder exstrophy, and cloacal exstrophy. The risk of urinary bladder cancer is known to be higher in individuals with classic bladder exstrophy and appears at a younger age compared with the general population. Purpose: The purpose of this study was to evaluate urinary bladder cancer in individuals with bladder exstrophy and epispadias complex. Materials and Methods: We performed 2 studies, a Swedish register-based case series reporting 12 novel cases and a systematic review summarizing published cases thus far. MEDLINE, EMBASE, Web of Science, and Google Scholar were searched in January 2022 following the Preferred Reporting Items for Systematic Review and Meta-Analysis statement. Publications reporting at least 1 case of bladder exstrophy and epispadias complex with urinary bladder cancer were eligible. Ninety-seven publications were included, reporting 165 cases. Results: The main result from the register study was the tumors being predominantly urothelial. Conversely, earlier published cases from the literature had a nonurothelial tumor type in 95%, whereof the majority were adenocarcinomas. Both substudies consistently indicate a young age at cancer diagnosis, with the majority being younger than 65 years. Conclusions: Urinary bladder cancer affects individuals with bladder exstrophy and epispadias complex at a young age. The most common tumor type in the register study is urothelial. The divergence between the 2 substudies in tumor types could reflect a slightly older age in our case series, likewise a possible publication bias.

Omphalocele, Exstrophy of Cloaca, Imperforate Anus and Spinal Defect – An Autopsy Report

Omphalocele, Exstrophy of Cloaca, Imperforate Anus and Spinal Defect – An Autopsy Report

Clinical features of patients who underwent anoplasty for cloacal exstrophy and their functional outcomes: the results of a nationwide survey in Japan.

This study was performed to clarify the clinical features of cloacal exstrophy (CE) patients who underwent anoplasty and their functional outcomes based on a nationwide survey in Japan. A questionnaire survey was conducted and data were obtained from 229 CE patients. After the exclusion of non-surviving patients and unknown data, 195 patients were enrolled. We compared the patient characteristics of the no anoplasty group (NAP group) to the anoplasty group (AP group). The defecation function of patients who underwent anoplasty was evaluated. The AP group had significantly lower rates of omphalocele (p = 0.045) and spinal defects (p = 0.003) than the NAP group. Of the 195 patients enrolled in this study, only 17 (8.7%) underwent anoplasty. Of the 17 patients who underwent anoplasty, 7 (41.1%) eventually had a permanent stoma after anoplasty [after ileostomy: n = 3 (60.0%), after colostomy: n = 4 (44.4%)]. Regarding soiling, 4 patients (66.7%) showed the absence of soiling, 2 (33.3%) showed accidental soiling, and no patients showed frequent soiling. In our study, spinal defects may have affected the determination of anoplasty. Anoplasty may improve the quality of life, because defecation can be kept clean, but indications, including colon function, may be carefully considered.

Fetal Tethered Spinal Cord: Diagnostic Features and Its Association with Congenital Anomalies

Objective We assessed the frequency and type of associated congenital anomalies encountered with fetal tethered spinal cord (TSC) determined prenatally. Method A retrospective review was conducted based on the associated fetal abnormalities following diagnosis of low-lying fetal conus medullaris during the prenatal ultrasound. Results Of the 26 fetuses with low-lying conus medullaris, four were solitary TSC and 22 had TSC combined with associated congenital malformations, including four cases with spina bifida occulta, four cases with spina bifida aperta, one case with severe hydrocephalus, and 13 cases with multisystem congenital malformations. Among all the 13 cases with combined multisystem congenital malformations, four cases had vertebral defects, anal anomalies, cardiac defects, trachea-esophageal fistula, renal anomalies, and limb anomalies (VACTERL) syndrome, two cases had combined kidney development abnormalities, one case had cloacal exstrophy (OEIS syndrome), and six cases had chromosomal abnormalities (one case of chromosome 7q deletion, two cases of trisomy 13 syndrome, one case of trisomy 18 syndrome, one case of trisomy 9 syndrome, and one case of chromosome 4p deletion). Conclusions Low-lying conus medullaris found during prenatal ultrasound examination were often associated with neural tube malformations or multi-systemic complex developmental malformations. The frequency of chromosomal abnormalities was 23.1%.

Bladder exstrophy-epispadias complex

A 2-year-old male, with the bladder exstrophy-epispadias complex since birth presents to the department of surgical pediatrics for surgical management. Exposed, everted bladder template is clearly obvious immediately below umbilicus; left and right corpora cavernosa are visible beneath and alongside urethral plate; the scrotum is caudally displaced. A spectrum of congenital defects epispadias, typical bladder exstrophy, and cloacal exstrophy, make up the exstrophy-epispadias complex (EEC). Nelson et al. calculated the total incidence of EEC at 2.15 per 100,000 live births, with an equal male-to-female ratio. Normal invagination of the urogenital system begins at the end of the third week of pregnancy in the intermediate layer of mesoderm, while the lateral plate mesoderm contributes to the formation of the primitive gut tube. EEC develops because of disruption in this interaction, which may be caused by an overgrowth of the cloacal membrane that prevents mesenchymal tissue from migrating medially. The severity of the resulting condition depends on the point at which the interaction between the mesodermal layers is disturbed. Depending on the form and degree of the defect, different surgical procedures are required to rectify it; most neonates, however, require bladder and abdominal wall closure, epispadias repair, ureteral reimplantation, and bladder neck repair.

Unique Evaluation and Management Considerations for Adolescents with Late Gynecologic and Colorectal Issues in the Setting of Anorectal Malformations

Study ObjectiveThere is little guidance for managing pubertally identified Mullerian anomalies in patients with anorectal malformations (ARMs). We sought to assess these unique issues. DesignRetrospective review SettingSingle-institution study ParticipantsNatal female patients aged 10-25, with an ARM, cloaca, or exstrophy, who presented from 2009 to 2019 with a gynecologic concern were included. InterventionData collection was performed and included the presenting problem, psychological evaluation, fertility and sexuality concerns, and management strategies for these problems. Main Outcome MeasuresThe main outcome was unique needs that had to be addressed in the young adult population and the type of colorectal and gynecological procedures needed on representation. ResultsTwelve patients were identified; all had gynecologic concerns. Ten had ARMs, including cloaca (n = 3) and cloacal exstrophy (n = 5). Median age at representation was 14.6 years (IQR = 12.7, 15.3). Colorectal revisions included posterior sagittal anorectoplasty (n = 1), resection of bowel attached to urogenital sinus (n = 1), and appendicostomy revision (n = 1). Gynecologic issues included dysmenorrhea (n = 8), obstructed Mullerian anomaly (n = 6), and introital stenosis (n = 5). Behavioral health concerns (n = 9) and fertility/sexuality concerns (n = 4) were identified. Median time from first visit to reconstruction was 1.5 years (IQR = 0.5, 1.5), providing multiple visits to achieve consensus among patients and providers before intervention, including vaginal or introital repair (n = 5) and hysterectomy of obstructed uterine horns (n = 3). ConclusionsGoal-directed follow-up is required before surgical management to identify psychological and reproductive issues in patients with ARMs who have gynecologic concerns. Patient input and psychologic consultation are helpful for patients requiring staged reconstruction.

Association between Genitourinary and Congenital Heart Defects: A 52-year Case-Control Study of the Latin American Collaborative Study of Congenital Malformations (ECLAMC)

Abstract Background Congenital urological anomalies are present in 4.3/10 thousand newborns, and their association with other anomalies may increase the overall mortality and disability. The present study establishes the risk of having congenital urological anomalies presenting associated cardiopathies. Methods We conducted a retrospective case-control study using the Latin American Collaborative Study of Congenital Malformations (Estudio Colaborativo Latino Americano de Malformaciones Congénitas, ECLAMC, in Spanish). The analysis included all registered cases of congenital urological malformation from 1967 to 2019. Patients with or without associated heart defects were included for the statistical analysis. Odds ratios (ORs) were calculated using a 95% confidence interval (95% CI). We compared the variables with the Chi-squared test and analysis of variance (ANOVA). The statistical analysis was performed using the Statistical Package for the Social Sciences (IBM SPSS Statistics for Windows, IBM Corp., Armonk, NY, United States) software, version 27.0. Values of p &lt; 0.05 were considered statistically significant. Results A total of 7,767,161 newborns were evaluated, and 17,834 genital and upper urinary tract malformations were identified. Of these, 64.2% were genital anomalies, and 35.8% were abnormalities of the upper urinary tract. Genitourinary malformations and concomitant congenital heart defects (GU + C) were observed in 3.5% of the cases. Subjects with GU + C had a higher number of malformations (4.59 ± 2.3) than patients without heart defects (1.53 ± 1.58) (p &lt; 0.000). The OR was of 3.61 (range: 1.86–7.00) for cloacal exstrophy, of 4.01 (range: 3.14–5.12) for imperforate anus, of 5.52 (range: 3.92–7.78) for horseshoe kidney, and of 13.7 (range: 6.65–28.22) for trisomy 21 (Down syndrome) with malformations of the upper urinary tract. Conclusion The association of congenital heart defects with urological anomalies is higher for complex congenital anomalies such as imperforate anus, cloacal exstrophy, and horseshoe kidney. Patients with urological abnormalities and Down syndrome have the highest likelihood.

The exstrophy experience: A national survey assessing urinary continence, bladder management, and oncologic outcomes in adults

The bladder exstrophy-epispadias complex (BEEC) is a rare spectrum of congenital genitourinary malformations with an incidence of 1:10,000 to 1:50,000. Advances in reconstructive surgical techniques have improved clinical outcomes, but there is a paucity in data about disease sequela in adulthood. This is the largest survey to date in the United States exploring the urinary continence, bladder management, and oncologic outcomes in adults with BEEC. Respondents were over the age of 18 with a diagnosis of bladder exstrophy, cloacal exstrophy, or epispadias. They were treated at the authors' institution, included in the Association for the Bladder Exstrophy Community (A-BE-C) mailing list, and/or engaged in A-BE-C social media. A survey was created using uniquely designed questions and questionnaires. Survey responses between May 2020 and July 2020 were processed using Research Electronic Data Capture (REDCap). Quantitative and qualitative statistics were used to analyze the data with significance at p<0.05. A total of 165 patients completed the survey. The median age was 31.5 years (IQR 25.9-45.9). Many patients considered themselves continent of urine, with a median satisfaction score of 74 (IQR 50-97) on a scale from 0 (consider themselves to be completely incontinent) to 100 (consider themselves to be completely continent). There was less leakage among those with a continent urinary diversion compared to those who void or catheterize per urethra (p=0.003). Patients with intestinal-urinary tract reconstruction, such as augmentation cystoplasty or neobladder creation, were more likely to perform bladder irrigations (p=0.03). Patients with continent channels were more likely to report UTI than all other forms of bladder management (89.0% vs. 66.2%, p=0.003). Three (1.9%) patients were diagnosed with bladder cancer. A small portion of patients (27.2%) were given bladder cancer surveillance recommendations by a physician. Most patients achieved a satisfactory level of urinary continence, with the highest continence rates in those with a continent urinary diversion. Those with intestinal-urinary tract reconstruction were more likely to perform bladder irrigations, perhaps to avoid complications from intestinal mucous production. The rates of self-reported UTI and were higher in patients with continent channels, but recurrent UTIs were not affected by the type of genitourinary reconstruction. Bladder cancer exists in this population, highlighting the need for long-term follow-up. Most BEEC patients achieve a satisfactory level of urinary continence, with the best outcomes in those with a continent urinary diversion. This population requires long-term follow-up with a transitional urologist to ensure adequate oncologic care.

Patient with treatment resistant peristomal and scalp dermatitis

A 39-year-old male with history of cloacal exstrophy with colostomy and ileal conduit presented with a rash that started around his ostomy and on his scalp and spread to involve his gluteal cleft and groin gradually over 1 year. Physical examination revealed erythematous scaly papules coalescing into plaques with petechiae surrounding his ostomy and on his scalp (Figs 1 and 2). Previous biopsies from peristomal skin and scalp showed spongiotic and psoriasiform dermatitis, respectively, and he had failed topical steroids, fluconazole, dupilumab, and guselkumab.

Application of Tunica Vaginalis Flap for Epispadias Repair in the Epispadias-Exstrophy Complex

To describe long-term outcomes and experience with the tunica vaginalis flap (TVF) as a tissue augment for complex epispadias repair. A prospectively maintained institutional database was reviewed for patients who underwent epispadias repair with TVF from 2010 to 2021. Evaluation of patient characteristics between those who developed dehiscence or UCF and those who did not was performed via Mann-Whitney U, Kruskal-Wallis, or Fisher's exact test, as appropriate. A total of 47 male patients were identified, of whom, 1 (2.1%) had cloacal exstrophy, 38 (80.9%) had classic bladder exstrophy or a variant, and 8 (17.0%) had epispadias as their primary exstrophy-epispadias complex diagnosis. The median age at epispadias repair was 13 months (IQR 11 - 19). The overall rate of fistula or dehiscence development was 19.1% (9 patients), of whom, 7 developed urethrocutaneous fistulae, 1 dehiscence, and 1 both. Notably, 0 of the 5 patients who had undergone prior epispadias repair and 0 of the 8 patients with a diagnosis of isolated epispadias developed a UCF or dehiscence after repair with TVF. Differences in age at repair, primary EEC diagnosis, prior epispadias repair, pre-repair testosterone, fibrin sealant utilization, closure layers, stent removal time, and bladder capacity at repair were not statistically significant (P>.05) CONCLUSION: Our expanded evaluation indicates that utilization of the tunica vaginalis as an adjunct to epispadias repair may provide durable protection against fistula development in EEC patients who have undergone prior epispadias repair and in primary repair of patients with isolated epispadias.

A Case Report Baby Born with Multiple Congenital Anomalies Including Ruptured Omphalocele at a Primary Healthcare

A baby was born at a primary healthcare with multiple congenital anomalies, which included ruptured omphalocele, cloacal exstrophy, and congenital talipes equinovarus. IV line was obtained, and the baby was stabilized. The baby was referred to a tertiary health care and underwent surgery to close the defect, ileostomy, and mucous fistula. He survived the surgery but is currently in intensive care.&#x0D;

The Y-Pelvic Osteotomy in Treating Bladder Exstrophy: A Surgical Technique.

Bladder exstrophy (BE) is a rare congenital anomaly caused by an embryological defect in the closure of the abdominal wall.It comprises a spectrum of defects about severity, including epispadias in the mildest form and cloacal exstrophy in the worst. Surgical correction is required to achieve urinary continence, maintain normal renal function, achieve secured abdominal wall closure, and create cosmetically and functionally satisfactory genitalia. Iliac bone osteotomy is considered essential to achieve the above goals in most patients by reducing the tension of the closed abdominal walllayers, particularly when present late in infancy. Several types of pelvic iliac bone osteotomy have been described to aid bladder and cloacal exstrophy closure. They can be grouped into posterior iliac osteotomy, anterior iliac osteotomy, oblique (also called diagonal) iliac osteotomy, and a combination of posterior and anterior iliac osteotomy. We described here the Y-pelvic osteotomy, which was developed by the Manchester Orthopaedic Group in the United Kingdom. It has the advantage of anterior and posterior osteotomiesbut also has less risk to the neurovascular structures, less blood loss, and ease of surgical technique. The osteotomy was named the Y-pelvic osteotomy due to the morphological shape it resembles.

Early Outcome of Patient Born with Gastroschisis and Omphalocele

Background: Gastroschisis and omphalocele are the most common congenital defects of the anterior abdominal wall which need urgent and prompt intervention post-operative time outcomes of gastroschisis and omphalocele differ between different countries, especially between developed and developing countries. Objectives: Is to determine the early outcome of patients born with gastroschisis and omomphalocele Patients and Methods: An analytical study including 30 patients (11 gastroschisis and 19 omphaloceles) were treated in Erbil city during 2015 -2018. Patients with associated bladder and cloacal exstrophies were excluded. Results: Female to male ratio was 1.3:1, and the mean age at presentation was 11.7 hours. The Mean weight was 2.86 kg. the Majority of patients25 (83.3%) were term. In Nineteen cases (63.3%)were delivered vaginally (9 gastroschisis and 10comphalocele), prenatally diagnosis was done in 9(30%). The Mean size of the defect was 3.7cm, and the content was mixed in 7(23.3%). Primary repair has performed in 18(60%), a silo in 8(26.7%), and 4(13.3%) treated none surgically (all were omphalocele). Operations have been done within 24 hours of arrival in 16(61.5%). Associated anomalies have been found in 11(57.9%) patients with omphaloceles and only 2(18.2%) patients with gastroschisis. The mean period of hospital stays was6 days. Mortality among the gastroschisis patients were 8(72%), but only 4(21.1%) for omphalocele patients. Conclusion: Gastrochisis and omphalocele constitute a considerable part of neonatal surgical problems and their management is still challenging. Omphalocele cases were more likely to have associated congenital anomalies and gastroschisis, if the associated anomalies were confined to the gastrointestinal tract. There was a high mortality rate, especially in patients with gastroschisis and ruptured omphalocele due to a lack of intensive care units, facilities, and trained personnel to look after such high-risk patients.