["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.

LUMBAR Syndrome and Bladder Exstrophy: A Case Report.

LUMBAR syndrome is rare with a multitude of features that requires a high index of suspicion for timely diagnosis and appropriate management. We present on a newborn female whose untreated segmental infantile hemangioma lead to poor healing of her bladder exstrophy closure. The objective of this report is to describe bladder exstrophy as a urogenital anomaly in patients with LUMBAR syndrome and the importance of balancing management of infantile hemangioma and time to surgery.

Prenatal Presentation of a Covered Cloacal Exstrophy Variant; Early Diagnostic Challenges Within the Broad Spectrum of the Exstrophy-Epispadias Complex.

There is a broad range of variant phenotypes that can occur within the bladder exstrophy and epispadias complex spectrum. Accurate prenatal detection helps prepare families and to coordinate subspecialty resources. Here, we present the case of a patient with prenatally diagnosed patient with covered cloacal exstrophy variant along with four additional cases illustrating the nonlinear spectrum from isolated epispadias to cloacal exstrophy. Given the rarity of these variants overall and of each subtype within the spectrum, there is a need for long-term multi-institutional outcomes data to improve detection, characterization, and prognostication for these patients.

Presentations of bladder exstrophy in a resource-limited setting and the role of Mainz II continent diversion for late referrals or failed primary closures: a multicentric report.

Primary closure techniques that have been updated and longterm follow-up for CBE (classic bladder exstrophy) may be out of reach for many patients living in resource-limited settings. Late referrals to medical care and primary closures that lack the necessary skills and facilities for comprehensive treatment are still common. Alternative and long-term surgical solutions may improve the lives of these unfortunate patients. During surgical outreach missions, patients with CBE, either non-operated or with a previous unsuccessful bladder closure, who were referred from vast under-resourced rural areas to three Eastern African hospitals, were studied. The following information is provided: mode of presentation, clinical history, diagnostic workout, management, and outcome. There were 25 cases (M/F ratio 17/8) ranging in age from two days to twenty years. Five of the seventeen patients who were not treated (35%) were under 120 days old and eligible for primary closure in a qualified tertiary center when one was available in the country. There were twelve late referred cases (ranging from 120 days to 20 years). Between the ages of ten months and twelve years, eight children arrived following a failed primary closure. In all of them, the bladder plate was too altered to allow closure. Following a preoperative diagnostic workout, a Mainz II continent internal diversion was proposed to fourteen patients with acceptable bowel control and postponed in the other three. Three cases were lost before treatment because parents refused the procedure. Twelve cases ranging in age from three to twenty years (mean seven years) were operated on. Eight people were followed for a total of 53.87 months (range: 36-120). Except for three people who complained of occasional night soiling, day and night continence were good. The average voiding frequency during the day was four and 1.3 at night. There was no evidence of a metabolic imbalance, urinary infection, or significant upper urinary tract dilatation. Two fatalities could not be linked to urinary diversion. Four patients were not followed up on. Due to the limited number of specialist surgical facilities, CBE late referral or failed closure is to be expected in a resource-limited context. In lieu of the primary closure, a continent internal diversion will be proposed and encouraged even at the level of a non-specialist hospital to improve the quality of life of these unfortunate patients. It is recommended that patients be warned about the procedure's potential long-term risks, which will necessitate a limited but regular follow-up.

Omphalocele, Exstrophy of Cloaca, Imperforate Anus, and Spinal Defects Complex: A Case Report.

 Omphalocele, exstrophy of cloaca, imperforate anus, and spinal defects complex is a rare malformation complex that includes omphalocele, cloacal exstrophy, imperforate anus and spinal defects with the incidence of 1 in 200,000 to 400,000 pregnancies and is even rarer in twin gestation. The etiology of this complex is still unclear. Most cases are sporadic. Prenatal screening must be done for diagnosis and appropriate multidisciplinary management of cases. In severe cases, termination of pregnancy is considered. We present a 4-day first twin child with underdeveloped ambiguous genitalia delivered via emergency lower section cesarean section at 32+3 weeks of gestation with giant liver containing omphalocele, cloacal exstrophy, imperforate anus and meningocele with severe pulmonary artery hypertension and non-visualization of right kidney and ureter, absence of uterus, fallopian tubes and right ovary. Separation and repair of the cecum and bladder were done. The ladd procedure was performed. Ileostomy was created and single-stage repair of the abdominal wall was done. Keywords: anorectal malformations; bladder exstrophy; case reports; neural tube defects; umbilicus.

False-positive urine pregnancy screening tests are uncommon in the hospital setting among patients with bowel-containing urinary tract reconstruction.

PURPOSE: False-positive urine pregnancy screening tests (UPST) have been reported among patients with bowel-containing urinary reconstruction (BCUR). However, the true frequency of such inaccurate results, which have been attributed to urinary mucous or other proteins interfering with or mimicking the binding of beta-HCG in the assay, is unknown in this population. We sought to determine the incidence of false-positive pregnancy screening tests among this patient population at our institution. MATERIALS AND METHODS: Using existing databases of patients with spina bifida, bladder exstrophy, and genitourinary rhabdomyosarcoma, we identified female patients with BCUR who had UPST over a 10-year period as screening prior to procedures or imaging. Patient and test result information was recorded. RESULTS: A total of 120 patients with a history of BCUR were identified: 33 with spina bifida, 73 within the exstrophy-epispadias complex (EEC), and 14 with genitourinary rhabdomyosarcoma. Of this group, 46 patients (38%) had at least one UPST during the study period; 15 had 1 UPST, 6 had 2 UPSTs, 4 had 3 UPSTs, and 21 had greater than 3 UPSTs, for a total of 244 UPST in this cohort. UPSTs used at our institution included Sure-Vue brand and Alere brand (HCG sensitivity 20 mIU/ml). Types of BCUR included ileal enterocystoplasty in 25 patients, colon enterocystoplasty in 6, stomach enterocystoplasty in 5, composite enterocystoplasty in 7, and continent catheterizable channel alone (e.g. Yang-Monti, appendicovesicostomy) in 3 patients. Of the 244 UPSTs in patients with BCUR, zero (0%) were positive. CONCLUSIONS: Despite reports in the literature that false-positive UPST are common among patients with bowel-containing urinary diversions, we found no positive UPST among patients with BCUR in the healthcare setting. False-positive UPST in the home setting may be due to variability in sensitivity thresholds, binding agents, technical errors in test technique, kit quality control, or other factors.

Prenatal Diagnosis of Bladder Exstrophy and OEIS over 20 Years.

OBJECTIVE: To examine the prenatal diagnosis rates of bladder exstrophy (BE) and Omphalocele-Exstrophy-Imperforate anus-Spinal Defect Syndrome (OEIS) in a large cohort of patients over a 20-year period. We hypothesized that prenatal diagnosis rates improved over time due to evolving techniques in fetal imaging. METHODS: A multi-institutional database was queried to identify BE or OEIS patients who underwent primary closure between 2000 and 2020. We retrospectively determined prenatal or postnatal diagnosis. Those with unknown prenatal history were excluded. Multivariable logistic regression was used to investigate temporal pattern in rate of prenatal diagnosis while adjusting for sex and treating institution. RESULTS: Among 197 BE and 52 OEIS patients, 155 BE and 45 OEIS patients had known prenatal history. Overall prenatal diagnosis rates of BE and OEIS were 47.1% (73/155) and 82.2% (37/45), respectively. Prenatal diagnosis rate was significantly lower in BE compared to OEIS (P <.0001). The prenatal diagnosis rate for BE significantly increased over time (OR 1.10; [95%CI: 1.03-1.17]; P = .003). Between 2000 and 2005, the prenatal diagnosis rate of BE was 30.3% (10/33). Between 2015 and 2020, prenatal diagnosis rate of BE was 61.1% (33/54). Prenatal diagnosis rate for OEIS did not change over time. Rates of prenatal diagnosis did not differ by sex or treating institution. CONCLUSION: Rates of prenatal diagnosis of BE and OEIS are higher than previously reported. Prenatal diagnosis rate of BE doubled in the last 5 years compared to the first 5 years of the study period. Nonetheless, a significant proportion of both BE and OEIS patients remain undiagnosed prior to delivery.

Prenatal diagnosis of diphallia in association with bladder exstrophy: a case report.

BACKGROUND: Penile duplication or diphallia is a rare congenital anomaly with unclear pathophysiological cause. Most cases of diphallia are reported postnatally; however, today with the use of a high-resolution ultrasound device, in-uterine diagnosis of many congenital anomalies is possible. CASE PRESENTATION: Herein we report a multiparous mother at 25 weeks of gestation who referred due to an abnormal cystic structure protruding from a large abdominal wall defect located below the umbilicus that was noted during a routine exam. Target scan revealed two penile-like protrusions with an empty scrotal sac and double bladder in an otherwise normal fetus, which was confirmed postnatally. Neonatal microarray study and karyotype were normal. CONCLUSION: Diphallia could be detected prenatally as an isolated anomaly, associated with caudal duplication syndrome, or as an exstrophy-epispadias complex. As this is a rare congenital anomaly, all sonographers should be familiar with prenatal ultrasound features and associated anomalies, an important issue in prenatal counseling with parents, delivery planning, psychological support of the family, and postnatal management.

Bladder exstrophy with exstrophic rectal duplication in an infant: An extremely rare case.

Exstrophic rectal duplication and its association with bladder exstrophy and anorectal malformation is an extremely rare clinical entity. This is a report of the second case of an exstrophic rectal duplication associated with bladder exstrophy in English literature. However, it is the first case, where all these anomalies were accompanied by an anorectal malformation.

The role of anatomic pelvic dissection in the successful closure of bladder exstrophy: an aid to success.

INTRODUCTION: Classic bladder exstrophy is one of the rarest congenital anomalies compatible with life. Surgical treatment of bladder exstrophy has progressed, but the goal of surgery remains a successful primary bladder closure. Several factors have been identified to decrease the risk of failed closure, including appropriate use of osteotomy and adequate postoperative immobilization and analgesia. However, the role of the radical anatomic pelvic dissection, including dissection of the urogenital diaphragm fibers, in a successful closure has not yet been extensively explored. OBJECTIVE: The objective of this study was go examine the role of radical anatomic pelvic dissection, including dissection of the urogenital diaphragm fibers, in patients with classic bladder exstrophy. STUDY DESIGN: This was a retrospective study based on an institutional database. METHODS: A retrospective review from an institutional approved database of more than 1,300 patients with epispadias-exstrophy complex was performed. The inclusion criteria included patients with classic bladder exstrophy with at least one failed bladder closure and a reclosure at the authors' institution with a single senior surgeon. Data collection included demographics, clinical variables, and status of urogenital diaphragm fibers. Magnetic resonance imaging (MRI) scans, if available, were reviewed with a pediatric radiologist to identify urogenital diaphragm fibers. RESULTS: From the database, 93 patients met inclusion criteria. Of these patients, 74 had urogenital diaphragm fibers completely intact at the time of repeat closure, whereas 19 patients did not. There was no association with age or gender and status of urogenital diaphragm fibers. There was no association with osteotomy, the type of primary bladder closure, surgeon subspecialty, and the status of the urogenital fibers. Fourteen patients had at least two prior closures; surprisingly, 11 of these repeat closure patients still had intact urogenital fibers even after two prior closures. DISCUSSION: The recent development and application of 3D MRI-guided pelvic dissection in a large group of patients led the authors to investigate whether adequate pelvic floor dissection had been accomplished at primary or secondary closure. Several patients had MRI scans performed before repeat closure in which the urogenital diaphragm fibers were identified to be intact on imaging; this was corroborated with surgical findings. Approximately 80% of patients had their urogenital diaphragm fibers completely intact and, therefore, did not have an adequate pelvic dissection during their primary or secondary bladder closure, putting the success of their previous closures at risk. CONCLUSION: Inadequate pelvic diaphragm dissection, defined as intact urogenital diaphragm fibers, demonstrated in a large group of patients with failed exstrophy closure, may be a decisive factor in bladder closure failure. The use of 3D intra-operative image guidance may aid in a safer and more successful pelvic dissection.

Impact of pelvic immobilization techniques on the outcomes of primary and secondary closures of classic bladder exstrophy.

INTRODUCTION: A potential determinant of successful bladder closures in patients with classic bladder exstrophy (CBE) is the postoperative pelvic immobilization technique. This study investigates the success rates of primary and secondary bladder closures based on various immobilization techniques from a high-volume exstrophy center. METHODS: A prospectively maintained institutional exstrophy-epispadias complex database of 1336 patients was reviewed for patients with CBE who have undergone primary or secondary closures between 1975 and 2018 and subsequently had a known method of pelvic immobilization. Patients were divided into two groups: primary and secondary closures. Associations between closure outcomes and immobilization techniques were determined. RESULTS: A total of 476 patients with primary closures and 101 patients with secondary closures met the inclusion criteria. In total, 343 (72.1%) primary closures were successful. As shown in the table, the success rates of primary closures were highest in patients immobilized with modified Buck's and Bryant's traction (95.0% and 79.3%, respectively) and lowest in those with spica cast (49.6%). A propensity score-adjusted logistic regression (adjusting for osteotomy status, period of closure, location of closure, and closure type) revealed that modified Buck's traction had a 5.60 (95% confidence interval 1.74-23.1, p = 0.008) greater odds of success compared to spica casting during the primary closure. For the secondary closure group, there were 92 (92.1%) successful secondary closures. Success rates were highest in modified Buck's traction (97.3%) and lowest with spica casting (66.7%). DISCUSSION: This study confirms previous findings of better outcomes when patients are immobilized with external fixation and Buck's traction after adjusting for potential confounding factors. Immobilization with modified Buck's or Bryant's traction yielded significantly higher primary closure success rates when compared to spica casting. It is the authors' belief that despite a longer hospital length of stay, external fixation with Buck's traction provides the best chance of a successful closure and, thus, a financially responsible method to care for these children in the postoperative period. CONCLUSIONS: Success rates for primary closures were highest when using modified Buck's traction with external fixation and lowest for spica casts. Similarly, for secondary closures, the best outcomes were achieved using modified Buck's traction with external fixation and the lowest success rates were associated with spica casts.

Isolated bladder exstrophy in prenatal diagnosis.

PURPOSE: Isolated classic bladder exstrophy (CBE) is the most common variant of the bladder-exstrophy-epispadias complex (BEEC). The BEEC represents a spectrum ranging from isolated epispadias over CBE to the most severe form, cloacal exstrophy. We report on a series of 12 cases with CBE diagnosed prenatally and illustrate the spectrum of prenatal ultrasound findings with comparison to prior published reports on this entity. METHODS: This was a retrospective study involving 12 fetuses with CBE at two large tertiary referral centers in Germany over a 14-year period (2004-2018). RESULTS: Median diagnosis was made with ultrasound in 24 + 5 (IQR25,75: 21 + 2, 29 + 0) weeks of gestation. All fetuses presented with the pathognomonic findings non-visualization of the fetal bladder and protruding abdominal mass below the umbilical cord insertion. All fetuses showed normal kidney anatomy and normal amniotic fluid throughout pregnancy. Epispadia was visible prenatally on ultrasound in 6/8 male fetuses. 1/12 Parents opted for termination of pregnancy, 11/12 fetuses were live born and received reconstructive surgery. CONCLUSIONS: Isolated CBE is an extremely rare prenatal sonographic finding. Prenatal diagnostics should exclude additional malformations within the spectrum of cloacal malformations.

22q11.2 duplications in a UK cohort with bladder exstrophy-epispadias complex.

The bladder exstrophy-epispadias complex (BEEC) comprises of a spectrum of anterior midline defects, all affecting the lower urinary tract, the external genitalia, and the bony pelvis. In extreme cases, the gastrointestinal tract is also affected. The pathogenesis of BEEC is unclear but chromosomal aberrations have been reported. In particular, duplications of 22q11.2 have been identified in eight unrelated individuals with BEEC. The current study aimed to identify chromosomal copy number variants in BEEC. Analyses was performed using the Affymetrix Genome-wide SNP6.0 assay in 92 unrelated patients cared for by two UK pediatric urology centers. Three individuals had a 22q11.2 duplication, a significantly higher number than that found in a control group of 12,500 individuals with developmental delay who had undergone microarray testing (p < .0001). Sequencing of CRKL, implicated in renal tract malformations in DiGeorge syndrome critical region at 22q11, in 89 individuals with BEEC lacking 22q11 duplications revealed no pathogenic variants. To date, 22q11.2 duplication is the genetic variant most commonly associated with BEEC. This is consistent with the hypothesis that altered expression of a single, yet to be defined, gene therein is critical to the pathogenesis of this potentially devastating congenital disorder.

Variant Presentations of the Exstrophy-Epispadias Complex: A 40-Year Experience.

OBJECTIVE: To investigate the diagnosis, surgical management, and outcomes in patients with variant EEC. Variant presentations of the exstrophy-epispadias complex (EEC) span a wide range of abnormalities. The rarity and diversity of EEC variants can lead to challenges in the diagnosis and subsequent management of this population. METHODS: The authors reviewed an institutional database of 1336 EEC patients from 1975 to 2018 for variant presentations of EEC. Variant presentations included those with skin covered bladder exstrophy (BE), duplicate bladders, superior vesical fistula, and epispadias with major bladder prolapse. Surgical management and outcomes were assessed. RESULTS: In total, 44 EEC variants were identified. Nineteen (43%) presented with a skin-covered BE variant. Five patients presented with duplicate BE, while 6 presented with superior vesical fistula. Fourteen patients (32%) presented with epispadias with major bladder prolapse. Overall, 36 (82%) EEC variants underwent primary bladder closure, at a median of 135 days after birth (range 1-2010), with 21 (58%) undergoing pelvic osteotomy. Primary closures were successful in 89% of cases. Continence procedures were performed in 17 patients. This includes 5 patients who underwent bladder augmentation. However even without a continence procedure, continence with volitional voiding was found in 8 patients. CONCLUSION: The most common EEC variant is the skin-covered form of BE. In order to expedite appropriate management, accurate diagnosis upon initial presentation is crucial. Still, successful surgical reconstruction often results in continence that is similar to, or better than, nonvariant EEC presentations.

Inequalities in healthcare access: how a man with exstrophy in rural India coped.

We report a case of a 22-year-old man with adult exstrophy. The patient made a self-made urine collection device, which helped him to lead a normal life, carrying out his routine as well as occupational activities smoothly. This patient is a prime example of inequalities in healthcare distribution in low-income and middle-income countries. He was never taken to a proper medical centre to correct his condition nor was his mother ever given proper antenatal healthcare access. His background of being a poor person from rural India highlights the problems of inequalities in healthcare access.

A Borderline Ovarian Tumour in a Patient with Classic Bladder Exstrophy; a Case Report.

A 37-year-old Romanian lady presented with a large pelvic mass, urosepsis and deteriorating renal function. She had undergone separation from her conjoined twin. Imaging revealed grossly abnormal anatomy and a suspicious pelvic mass. Examination was consistent with classic bladder exstrophy. Postoperative histology showed borderline ovarian tumour (BTO).

Early versus delayed closure of bladder exstrophy: A National Surgical Quality Improvement Program Pediatric analysis.

INTRODUCTION: Delayed closure of bladder exstrophy has become more popular; however, there is limited the evidence of its success. Existing literature focuses on intermediate and long-term outcomes, and short-term postoperative outcomes are limited by the small number of cases and varying follow-up methods. OBJECTIVE: The objectives of the current study were to: 1) compare 30-day complications after early and delayed closure of bladder exstrophy, and 2) evaluate practice patterns of bladder exstrophy closure. STUDY DESIGN: The National Surgical Quality Improvement Program Pediatric (NSQIPP) database from 2012 to 2015 was reviewed for all cases of bladder exstrophy closure. Early closure was defined as surgery at age 0-3 days, and delayed closure was defined as age 4-120 days at time of surgery. Demographic, clinical, and peri-operative characteristics were collected, as were postoperative complications, readmissions, and re-operations up to 30 days. Descriptive statistics were performed, and multivariate linear and logistic regression analyses were performed for salient complications. RESULTS: Of 128 patients undergoing bladder exstrophy closure, 62 were included for analysis, with 44 (71%) undergoing delayed closure. Mean anesthesia and operative times were greater in the delayed closure group, and were associated with more concurrent procedures, including inguinal hernia repairs and osteotomies. The delayed closure group had a higher proportion of 30-day complications, due to a high rate of blood transfusion (57% vs 11%). Wound dehiscence occurred in 6/44 (14%) delayed closures, as compared with 0/18 (0%) early closures. When compared with prior published reports of national data from 1999 to 2010, delayed closure was performed more frequently in this cohort (71% vs 27%). DISCUSSION: The NSQIPP provides standardized reporting of peri-operative characteristics and 30-day complications, allowing a comparison of early to delayed closure of bladder exstrophy across multiple institutions. Assessing short-term risks in conjunction with long-term follow-up is crucial for determining optimal management of this rare but complex condition. CONCLUSION: Delayed closure of bladder exstrophy is performed frequently, yet it carries a high rate of 30-day complications worthy of further investigation. This can be useful in counseling patients and families, and to understand practice patterns across the country.

Gradual bone transfer for the correction of the pubic diastasis using the Ilizarov technique in closure of bladder and cloacal exstrophy.

BACKGROUND: In the patient of the cloacal exstrophy, cloaca with local abdominal wall is disrupted and exposed inner surface of the bladder needs early closure. Pelvic osteotomies are required for severe cases whose bladder cannot be closed by the suture of soft tissue only. We developed a technique involving the gradual positioning of bone fragments using a light, Ilizarov external fixator. The usefulness of the technique was assessed. METHODS: We enrolled 3 patients with cloacal exstrophy and 1 with bladder exstrophy as a gradual transfer group and 6 patients who were treated by other osteotomies as a control group. The patients aged 6.7-8.4 months at the time of surgery were followed up for 4.0-8.6 years. An external fixator with carbon fiber half-rings was placed to internally rotate and anteriorly move the distal bone fragment over 2 weeks. Then, the bladder was closed. Computed tomography (CT) images were used to assess the pelvis form. Wound dehiscence and number of the surgeries after the osteotomies are also compared between the two groups. RESULTS: CT analysis of correction of the pelvic deformity achieved more and less decreasing its volumetric capacity in the gradual transfer group. No patients had wound dehiscence after the primary closure with pelvic osteotomy in the gradual transfer group but all had them in the control group. The mean number of the surgeries after the osteotomies were 2.25 in the gradual transfer group whereas 5.5 in the control group. CONCLUSIONS: Sufficient closure of the abdominal wall and bladder was achieved in all cases in the gradual transfer group. The correction of pelvic bones were more with less decreasing of their pelvic capacities, no patients had wound dehiscence after the closure and there was an effect to decrease the number of the surgeries after the treatment by this method.

Bladder exstrophy closure in the newborn period with external pelvic fixation performed without osteotomy: A preliminary report.

BACKGROUND: Successful primary bladder closure is the most crucial element for urinary continence in patients with classic bladder exstrophy (CBE). In the newborn period, bladder closure can be performed in the first 48 h without pelvic osteotomy or external fixation, but requires postoperative lower extremity immobilization (i.e., spica cast, Bryant's or Buck's traction). OBJECTIVE: To present a novel surgical approach for primary bladder closure for CBE using two-pin external fixation without pelvic osteotomy, and without postoperative lower extremity immobilization. STUDY DESIGN: A retrospective chart review of patients with CBE was performed at the current institution from 2000 to 2016, including all primary bladder closures with external fixation and without osteotomy or lower extremity immobilization. Patients were discharged with the external fixator in place, which was later removed in clinic. Baseline clinical and demographic variables, and follow-up data were recorded. RESULTS: Thirteen patients were analyzed; eight (61.5%) were male. Pre-operative intersymphysial distance was 3.68 ± 1.0 cm (2.0-5.0). Mean follow-up was 56.8 ± 40.3 months (10-131). One patient had a partial bladder neck dehiscence, due to pin displacement on postoperative day 1: he had the lowest gestational age of 34 weeks (Summary table). DISCUSSION: This approach used external fixation to bring the pubic bones together intra-operatively, and to decrease the tension in closing the pelvic ring and abdominal wall without osteotomy. External fixation with osteotomy and long-term immobilization, or using a spica cast without osteotomy offered the added advantage of improved wound care, due to lack of lower limb immobilization, less patient discomfort, and facilitation of mother/caregiver and newborn bonding. CONCLUSION: The two-pin external fixator without osteotomy as an adjunct to primary bladder closure in CBE patients was technically feasible. At the current institution this approach had an equivalent success rate to previous reports in the literature for primary bladder closure, decreased the length of hospital stay, and precluded the need for lower extremity immobilization. Early data for bladder capacity were encouraging.