Multi-Institutional Bladder Exstrophy Consortium After 8 Years: The Short- and Intermediate-Term Outcomes.

PURPOSE: Bladder exstrophy (BE) poses challenges both during the surgical repair and throughout follow-up. In 2013, a multi-institutional BE consortium was initiated, which included utilization of unified surgical principles for the complete primary repair of exstrophy (CPRE), real-time coaching, ongoing video capture and review of video footage, prospective data collection, and routine patient data analysis, with the goal of optimizing the surgical procedure to minimize devastating complications such as glans ischemia and bladder dehiscence while maximizing the rate of volitional voiding with continence and long-term protection of the upper tracts. This study reports on our short-term complications and intermediate-term continence outcomes. MATERIALS AND METHODS: A single prospective database for all patients undergoing surgery with a BE epispadias complex diagnosis at 3 institutions since February 2013 was used. For this study, data for children with a diagnosis of classic BE who underwent primary CPRE from February 2013 to February 2021 were collected. Data recorded included sex, age at CPRE, adjunct surgeries including ureteral reimplantations and hernia repairs at the time of CPRE, osteotomies, and immobilization techniques, and subsequent surgeries. Data on short-term postoperative outcomes, defined as those occurring within the first 90 days after surgery, were abstracted. In addition, intermediate-term outcomes were obtained for patients operated on between February 2013 and February 2017 to maintain a minimum follow-up of 4 years. Outcomes included upper tract dilation on renal and bladder ultrasound, presence of vesicoureteral reflux, cortical defects on nuclear scintigraphy, and continence status. Bladder emptying was assessed with respect to spontaneous voiding ability, need for clean intermittent catheterization, and duration of dry intervals. All operating room encounters that occurred subsequent to initial CPRE were recorded. RESULTS: CPRE was performed in 92 classic BE patients in the first 8 years of the collaboration (62 boys), including 46 (29 boys) during the first 4 years. In the complete cohort, the median (interquartile range) age at CPRE was 79 (50.3) days. Bilateral iliac osteotomies were performed in 89 (97%) patients (42 anterior and 47 posterior). Of those undergoing osteotomies 84 were immobilized in a spica cast (including the 3 patients who did not have an osteotomy), 6 in modified Bryant's traction, and 2 in external fixation with Buck's traction. Sixteen (17%) patients underwent bilateral ureteral reimplantations at the time of CPRE. Nineteen (21%) underwent hernia repair at the time of CPRE, 6 of which were associated with orchiopexy. Short-term complications within 90 days occurred in 31 (34%), and there were 13 subsequent surgeries within the first 90 days. Intermediate-term outcomes were available for 40 of the 46 patients, who have between 4 and 8 years of follow-up, at a median of 5.7 year old. Thirty-three patients void volitionally, with variable dry intervals. CONCLUSIONS: Cumulative efforts of prospective data collection have provided granular data for evaluation. Short-term outcomes demonstrate no devastating complications, that is, penile injury or bladder dehiscence, but there were other significant complications requiring further surgeries. Intermediate-term data show that boys in particular show encouraging spontaneous voiding and continence status post CPRE, while girls have required modification of the surgical technique over time to address concerns with urinary retention. Overall, 40% of children with at least 4 years of follow-up are voiding with dry intervals of > 1 hour.

Is Immobilization Technique Associated With Postoperative Pubic Rami Diastasis Following Bladder Exstrophy Closure?

OBJECTIVE: Pelvic osteotomies relieve tension of the bladder and fascial closures during bladder exstrophy repair. Multiple techniques for postoperative immobilization of the pelvis and lower extremities have been described. The primary aim of this study was to assess differences in short and long-term changes in pubic rami diastasis when comparing Bryant traction to spica cast immobilization. Secondary aims included a comparison of length of stay, skin-related complications, and urologic outcomes. METHODS: We performed a single-institutional retrospective review of bladder exstrophy patients younger than 18 months of age who underwent posterior pelvic osteotomy and bladder exstrophy closure from April 2005 to April 2020. Short-term and long-term pubic rami diastasis were defined as postoperative measurements ≤6 months and ≥12 months, respectively. Secondary outcomes included length of stay, pressure ulcer, skin rash/abrasion, urethrocutaneous fistula, and bladder or fascial dehiscence rates. Multivariable logistic regression assessed for an association between immobilization type and degree of diastasis while controlling for age at the time of diastasis measurement and sex. RESULTS: Fifteen patients underwent Bryant traction and 36 patients underwent spica cast immobilization. In both the short-term and long-term, there was a greater reduction in pubic diastasis in the spica cast group ( P = 0.002 and P = 0.05, respectively). After adjustments, there were higher odds of having a greater reduction in pubic rami diastasis in both the short-term (odds ratio: 2.71, 95% CI: 1.52-4.86, P = 0.001) and long-term (odds ratio: 2.41, 95% CI: 1.00-5.80, P = 0.05). Length of stay was significantly higher in Bryant's traction group (26 vs 19 d, P < 0.001). Rates of pressure ulcers were higher in the Bryant traction group (26.7% vs 0%, P = 0.005). Rates of skin rash/abrasions, urethrocutaneous fistula, and bladder/fascial dehiscence did not differ. CONCLUSIONS: Spica cast immobilization is a safe and effective immobilization method. Compared with Bryant traction, spica cast immobilization was associated with a greater reduction in postoperative pubic diastasis both short and long-term, along with a shorter length of hospitalization and reduced rate of pressure ulcers. LEVEL OF EVIDENCE: Level III-therapeutic study.

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.

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.

Enhanced Recovery After Surgery for an Uncommon Complex Urological Procedure: The Complete Primary Repair of Bladder Exstrophy.

PURPOSE: ERAS (enhanced recovery after surgery) protocols are designed to optimize perioperative care and expedite recovery. Historically, complete primary repair of bladder exstrophy has included postoperative recovery in the intensive care unit and extended length of stay. We hypothesized that instituting ERAS principles would benefit children undergoing complete primary repair of bladder exstrophy, decreasing length of stay. We describe implementation of a complete primary repair of bladder exstrophy-ERAS pathway at a single, freestanding children's hospital. MATERIALS AND METHODS: A multidisciplinary team developed an ERAS pathway for complete primary repair of bladder exstrophy, which launched in June 2020 and included a new surgical approach that divided the lengthy procedure into 2 consecutive operative days. The complete primary repair of bladder exstrophy-ERAS pathway was continuously refined, and the final pathway went into effect in May 2021. Post-ERAS patient outcomes were compared with a pre-ERAS historical cohort (2013-2020). RESULTS: A total of 30 historical and 10 post-ERAS patients were included. All post-ERAS patients had immediate extubation (P = .04) and 90% received early feeding (P < .001). The median intensive care unit and overall length of stay decreased from 2.5 to 1 days (P = .005) and from 14.5 to 7.5 days (P < .001), respectively. After final pathway implementation, there was no intensive care unit use (n=4). Postoperatively, no ERAS patient required escalation of care, and there was no difference in emergency department visits or readmissions. CONCLUSIONS: Applying ERAS principles to complete primary repair of bladder exstrophy was associated with decreased variations in care, improved patient outcomes, and effective resource utilization. Although ERAS has typically been utilized for high-volume procedures, our study highlights that an enhanced recovery pathway is both feasible and adaptable to less common urological surgeries.

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.

Repurposing tools: A peña stimulator as a tool to map the limits of dissection and continence zone during bladder exstrophy repair.

INTRODUCTION: The complete primary repair of bladder exstrophy (CPRE) aims to restore normal anatomy through complete mobilization and reapproximation of the bladder neck and proximal urethra. METHODS: The Peña stimulator has previously been used to identify musculature in the pelvis. The device is now used to distinguish intersymphyseal bands from pelvic floor and urethral sphincteric musculature during CPRE. The ability to distinguish the levator ani muscles from fibrotic bands assists the surgeon in identifying the appropriate extent of deep pelvic dissection during CPRE. RESULTS: During the anatomic bladder neck recreation, the muscle stimulator demonstrated the striated muscle at the level of the bladder neck and urethral sphincter. CONCLUSION: Localization of the muscles at the bladder neck and proximal urethra corroborates the concept that pelvic floor physical therapy could enhance the strength of the pelvic floor in children with bladder exstrophy.

Hindgut Duplication in an Infant with Omphalocele-Exstrophy-Imperforate Anus-Spinal Defects (OEIS) Complex.

Introduction The congenital anomaly of omphalocele, cloacal exstrophy, imperforate anus, and spinal abnormalities (OEIS complex) is rare but well recognized. Hindgut duplications are also uncommon and are not known to be associated with OEIS. We describe a neonate with OEIS who was found to have fully duplicated blind-ending hindguts. Case Report A premature infant boy with OEIS underwent first-stage closure on day of life 6, which included excision of the omphalocele sac, separation of the cecal plate and bladder halves, tubularization of the cecal plate, hindgut rescue with end colostomy, and joining of the bladder halves. Cecal plate inspection revealed two hindgut structures that descended distally, one descended midline into the pelvis along the sacrum and the second laterally along the left border of the sacrum. Both lumens connected to the cecal plate and had separate mesenteries. In an effort to maximize the colonic mucosal surface area, the hindgut segments were unified through a side-to-side anastomosis, creating a larger caliber hindgut. The cecal plate was tubularized and an end colostomy was created. Bowel function returned and he was discharged home on full enteral feeds. Discussion This case represents a cooccurrence of two extremely rare and complex congenital anomalies. The decision to unify the distinct hindguts into a single lumen was made in an effort to combine the goals of management for both OEIS and alimentary duplications. The hindgut is abnormal in OEIS and should be assessed carefully during repair.

Prostatic metaplasia of the vagina in transmasculine individuals.

PURPOSE: To determine the prevalence of prostatic metaplasia in an expanded cohort of transmasculine individuals undergoing gender-affirming resection of vaginal tissue. METHODS: Institutional Review Board approval was obtained. Clinical records were reviewed for all transmasculine individuals undergoing vaginal tissue resection at our institution between January 2018 and July 2021. Corresponding pathology specimens were examined grossly and microscopically, including immunohistochemical stains for NKX3.1, prostate-specific antigen (PSA), and androgen receptor (AR). Vaginal specimens from three patients without androgen supplementation were used as controls. RESULTS: Twenty-one patients met inclusion criteria. The median age at surgery was 26.4 years (range 20.6-34.5 years). All patients had been assigned female gender at birth and lacked endocrine or genetic abnormalities. All were on testosterone therapy; median duration of therapy at surgery was 4.4 years (range 1.4-12.1 years). In the transmasculine group, no gross lesions were identified. Microscopically, all specimens demonstrated patchy intraepithelial glandular proliferation along the basement membrane and/or nodular proliferation of prostate-type tissue within the subepithelial stroma. On immunohistochemical staining, performed for a subset of cases, the glandular proliferation was positive for NKX3.1 (16/16 cases; 100%), PSA (12/14 cases; 85.7%), and AR (8/8 cases; 100%). Controls showed no evidence of prostatic metaplasia. CONCLUSION: One hundred percent of vaginal specimens obtained from transmasculine individuals on testosterone therapy (21/21 cases) demonstrated prostatic metaplasia. Further investigation is warranted to characterize the natural history and clinical significance of these changes. Patients seeking hormone therapy and/or gender-affirming surgery should be counseled on the findings and their yet-undetermined significance.

Bilateral ureteral reimplantation at complete primary repair of exstrophy: Post-operative outcomes.

BACKGROUND AND STUDY OBJECTIVE: The value of bilateral ureteral reimplant (BUR) at the time of complete primary repair of bladder exstrophy (CPRE) has been suggested, however, outcomes are poorly characterized in current medical literature. We hypothesize that BUR at time of CPRE will decrease the rate of recurrent pyelonephritis, post-operative vesicoureteral reflux (VUR), and the need for subsequent ureteral surgery. STUDY DESIGN: We analyzed 64 consecutive patients with a diagnosis of classic bladder exstrophy (BE) who underwent CPRE at three institutions from 2013 to 2019.15 patients underwent cephalotrigonal BUR-CPRE and 49 patients underwent CPRE alone. Our primary outcome was >1 episode of pyelonephritis as documented in the medical record. Secondary outcomes were persistent vesicoureteral reflux (VUR), with a sub-analysis of number of refluxing renal units and presence of dilating VUR, and the need for subsequent ureteral surgery. Descriptive statistics in addition to standard, two tailed univariate statistics, were used to compare the groups where appropriate. RESULTS: BUR-CPRE was associated with a significant decrease in the rates of post-operative VUR, number of refluxing renal units, and need for subsequent ureteral surgery (p = 0.002, p = 0.001, and p = 0.048 respectively). There was a reduction in the rates of recurrent pyelonephritis and dilating reflux in patients undergoing BUR-CPRE, though it did not reach significance. Female gender was significantly associated with recurrent pyelonephritis regardless of BUR-CPRE status (p = 0.005). There were no reports of distal ureteral obstruction or other complications following BUR-CPRE. The mean post-operative follow up for the BUR-CPRE group was 46.33 (10.26) months vs. 53.76 (26.05) months for CPRE (p = 0.11). DISCUSSION: Recurrent pyelonephritis following bladder closure in patients with BE is a well-documented surgical complication, with centers performing CPRE reporting rates of post-operative pyelonephritis from 22 to 50%. Our series demonstrates similar efficacy of BUR-CPRE compared to other contemporary series and provides additional detail about need for subsequent ureteral surgeries and increased long term follow-up of these complex patients. Limitations of the study include male predominance of the cohort and lack of randomization of BUR-CPRE. CONCLUSIONS: BUR-CPRE decreases postoperative VUR and the need for additional ureteral surgery in select BE patients; it should be considered when technically feasible. While results continue to suggest a trend toward decreased recurrent pyelonephritis and dilating reflux, further longitudinal follow-up in our cohort will be needed.

The Richard Grady Monsplasty: A vertical Z-plasty technique.

Classic bladder exstrophy in the female results in an exstrophic bladder and urethra, an anterior introitus with a bifid clitoris and short labia minora. During closure, the lower abdominal wall is closed and the bifid clitori are brought into close apposition, but are often not completely closed to prevent injury to the clitoral bodies, thus leaving a persistent gap between the clitoral bodies that grows over time. We demonstrate a vertical z plasty closure to provide a 2 layer closure of the mons that decreases tension and improves cosmetic appearance by recreating a clitoral hood that provides a more normal appearance of the external genitalia for girls with bladder exstrophy.

Timing of inguinal hernia following complete primary repair of bladder exstrophy.

INTRODUCTION/BACKGROUND: Bladder exstrophy patients have a high prevalence of inguinal hernia that often become clinically evident following bladder closure. Understanding when the bladder exstrophy patient is under greatest risk of developing an inguinal hernia following bladder closure is important, since incarceration resulting in strangulation of intra-abdominal contents can lead to significant morbidity if not addressed in a timely fashion. Although the incidence and risk factors of inguinal hernia have been reported, the timing of occurrence is not well understood. OBJECTIVE: The primary objective of this study was to assess the timing of inguinal hernia following complete primary repair of bladder exstrophy (CPRE). In addition, we aimed to evaluate possible risk factors associated with inguinal hernia, including sex, age at bladder closure and iliac osteotomy status. STUDY DESIGN: A multi-institutional retrospective review identified patients with bladder exstrophy repaired by CPRE under 6 months of age while excluding those who underwent inguinal hernia repair before or during bladder closure. Timing of inguinal hernia following bladder closure was evaluated using Kaplan-Meier methods. Cox proportional hazards model was used to investigate association of sex, age at bladder closure, and osteotomy on the risk of developing of inguinal hernia while clustering for institution. RESULTS: 91 subjects were included in our analysis with median follow-up time of 6.5 years. 34 of 53 males (64.2%) and 2 of 38 females (5.3%) underwent inguinal hernia repair. The median time to inguinal hernia was 4.7 months following closure. The greatest hazard of inguinal hernia was within the first six months following closure. In multivariate analysis, male sex was strongly associated with inguinal hernia (HR = 19.00, p = 0.0038). Osteotomy and delay in closure were not significantly associated with inguinal hernia. 7 of 36 patients (19.4%) who underwent inguinal hernia repair presented with recurrence on the ipsilateral side. DISCUSSION: Our results suggest that the greatest risk of inguinal hernia is within the first six months following bladder closure. The decreased risk of inguinal hernia after one year of follow-up may reflect anatomic stability that is reached following major reconstruction of the pelvis. While male bladder exstrophy patients are significantly more susceptible to inguinal hernias following CPRE, osteotomy and delayed bladder closure do not appear to be protective factors for inguinal hernia development following initial bladder closure. CONCLUSIONS: There is a heightened risk of inguinal hernia in the first six months following closure. The rate of recurrence following inguinal hernia repair is significantly elevated compared to the general pediatric population.

Safety of delayed surgical repair of omphalocele-exstrophy-imperforate anus-spinal defects (OEIS) complex in infants with significant comorbidities.

PURPOSE: Management of infants with OEIS complex is challenging and not standardized. Expeditious surgery after birth has been recommended to limit soilage of the urinary tract and optimize intestinal function. However, clinical instability secondary to comorbidities is common in this population and early operation carries risk. We sought to define the risk/benefit profile of delaying repair. METHODS: All newborn patients with OEIS managed by our institution between Sep 2017 and Oct 2019 were reviewed. Comorbidities were evaluated, including cardiopulmonary pathologies and associated malformations. RESULTS: Ten patients with OEIS were managed. Patients underwent early (2 patients, repair at 0-2 days) or delayed (6 patients, repair at 6-87 days) first-stage exstrophy repair. Two patients died prior to repair (progressive respiratory failure, severe genetic anomalies). Repairs were delayed secondary to cardiac conditions, neurosurgical interventions, medical disease, and/or delayed transfer. Delayed repair patients had longer lengths of stay and use of parenteral nutrition. No patients experienced urinary tract infections prior to repair. CONCLUSIONS: Delaying first-stage exstrophy repair to allow physiologic optimization is safe. All repaired patients were discharged home, without parenteral nutrition or supplemental oxygen.

A New Approach to Cloaca: Laparoscopic Separation of the Urogenital Sinus.

Introduction: Cloaca malformation repair strategy is strongly dictated by common channel and urethral lengths. Mid to long common channel cloacas are challenging and often require laparotomy for dissection of pelvic structures. The balance of common channel and urethral lengths often dictates the approach for reconstruction. Laparoscopy has been utilized for rectal dissection but not for management of the urogenital (UG) structures. We hypothesized that laparoscopy could be applied to UG separation in reconstruction of cloaca malformations. Methods: Records were reviewed for 9 children with cloaca who underwent laparoscopic rectal mobilization and UG separation. Clinical parameters reviewed included demographics, relevant anatomic lengths, operative duration, transfusion requirements, and perioperative complications. Results: Repair was perfomed at a median (interquartile range) age of 12 (7, 15) months. Common channel length as measured by cystoscopy was 3.5 (3.3, 4.5) cm. There were no intraoperative complications. Transfusion requirements were minimal. Postoperative length of stay was 6 (5, 11) days. One patient developed a urethral web and 2 developed vaginal stenosis. One patient later underwent a laparotomy for obstruction due to a twisted rectal pull-through. Conclusions: Laparoscopic rectal mobilization and UG separation in long common channel cloaca are safe and well tolerated. Laparoscopy affords full evaluation of Mullerian structures and enables separation of the common UG wall, which may ultimately enhance long-term urinary continence.

Key anatomic findings on fetal ultrasound and MRI in the prenatal diagnosis of bladder and cloacal exstrophy.

INTRODUCTION: Prenatal determination of bladder exstrophy (BE) or cloacal exstrophy (CE), known also as the omphalocele-exstrophy-imperforate anus-spinal anomaly complex (OEIS), is challenging. Distinguishing between BE and CE is important because children with CE have many more challenges initially and during their lifetime. An accurate diagnosis is critical when counselling expectant parents. We hypothesized that there are key imaging features that can distinguish BE from CE, and that there are areas of diagnostic concordance and discordance between fetal ultrasound (fUS) and fetal MRI (fMRI) among these entities. MATERIALS AND METHODS: We queried a single institutional IRB-approved registry of children with BE and CE to identify those with accessible fetal imaging from 2000 to 2018, and formal interpretations were collected. Two pediatric radiologists performed independent retrospective blinded review of the images. Criteria evaluated included: genitalia, kidneys, bowel appearance, presence of anal dimple, location of insertion of umbilical cord into the abdomen relative to the abdominal wall defect, umbilical vessels, bladder protuberance, presence of omphalocele, and spine/neural cord abnormalities. We evaluated concordance between radiologic interpretations and postnatal diagnosis, as well as between specific findings in the two diagnostic modalities. RESULTS: Twenty-one infants born between 2000 and 2018 with BE or CE had fetal imaging for review: 15 had both fUS and fMRI, 2 had fUS alone, and 4 fMRI alone. There was 100% concordance between fUS and fMRI in evaluating kidneys, presence of anal dimple, location of abdominal insertion of umbilical cord relative to the defect, number of umbilical vessels, and spine abnormalities/level of neural cord termination. The following discrepancies were observed: 1) genitalia and bowel appearance, and bladder protuberance in 1/15 (6.7%); 2) presence of an omphalocele in 2/15 (13.3%). Of the initial radiologic interpretations, 4/17 (23.5%) of fUS and 2/19 (10.5%) of fMRI erroneously were interpreted as on the OEIS spectrum when the post-natal diagnosis was BE. Errors in diagnosis were due to a protuberant bladder plate extending beyond the plane of the abdominal wall with bowel loops posteriorly mimicking an omphalocele. In all of these BE cases, the abdominal wall defect was located inferior to the umbilical cord insertion on the abdominal wall. CONCLUSION: An everting bladder plate with bowel loops posterior to the plate in classic BE may be misdiagnosed as CE. Identification of the location of umbilical cord insertion relative to the abdominal wall defect, with fetal US or MRI, results in the correct differentiation between BE and CE.

Evaluation of outcomes following complete primary repair of bladder exstrophy at three individual sites prior to the establishment of a multi-institutional collaborative model.

PURPOSE: The Multi-Institutional Bladder Exstrophy Consortium (MIBEC) was established in 2013 to refine technical aspects of the complete primary repair of bladder exstrophy (CPRE), to decrease complications, and to improve outcomes. In order to place outcomes from the consortium into context of historic outcomes, we evaluated continence and dry intervals in children who were repaired prior to the beginning of the consortium at these institutions. We hypothesized that continence (voiding with dryness) is rarely achieved after primary CPRE and surgery following CPRE improves dryness but may hinder voiding per urethra. MATERIALS AND METHODS: We reviewed prospectively maintained IRB approved databases of children who underwent CPRE for classic bladder exstrophy (BE) between 5/1993 and 1/2013 at 3 institutions. Exclusion criteria included: lack of continence documentation, and lack of follow up subsequent to January 2014. We recorded age at closure, method of bladder emptying, bladder capacity and surgical history. We used a 3 part dryness scale for both patients who void volitionally and those on CIC. Children were considered dry if they could hold urine for over 3 h. An intermediate group was defined as having a dry interval of 2-3 h, with minimal dampness in between voids. If dry intervals were <2 h with frequent leakage, children were considered wet. RESULTS: A total of 54 of 73 (38 M) children met inclusion criteria. 35 of 54 (64.8%) children void per urethra, while 18 (33.3%) perform clean intermittent catheterization (CIC) and 1 underwent a vesicostomy due to incomplete emptying and UTI's. 25/35 (71.4%) of those voiding per urethra underwent CPRE only, while the remaining 10 underwent secondary continence procedures. In total, 26 of 54 (48.1%) are dry, that is either continent or not wet for > 3 h, while only 11/54 (20.4%) are truly continent, i.e., voiding per urethra. Only 9/54 (16.7%) are continent after a single surgery (CPRE). 14/54 children who are dry (25.9%) underwent a bladder neck procedure with or without augmentation and empty with CIC. DISCUSSION AND CONCLUSION: Granular detail about the specifics of emptying, surgical history, and dry intervals is crucial to understand the true outcomes from the repair of BE. Children with BE undergoing CPRE prior to the institution of MIBEC experienced variable results, with only 17% achieving continence while spontaneously voiding per urethra without additional reconstruction. We are now engaged in MIBEC to identify factors that contribute to continence and to attempt to render such findings reproducible.

Necrotizing Fasciitis Following Routine Genitourinary Surgery in Healthy Infants.

Morbidity and mortality associated with pediatric necrotizing fasciitis are strongly dependent on early diagnosis and timely intervention. Yet, the lack of early cutaneous findings and nonspecific symptoms may result in initial delayed diagnosis or misdiagnosis. Infants may be particularly at risk of missed or delayed diagnosis due to inherent barriers in communication and rarity of the condition, especially among healthy patients. We describe 2 cases of necrotizing fasciitis following routine genitourinary surgery in healthy infants.