Updates on the Care of Cloacal Exstrophy.

Cloacal exstrophy is the most severe congenital anomaly of the exstrophy-epispadias complex and is characterized by gastrointestinal, genitourinary, neurospinal, and musculoskeletal malformations. Individualized surgical reconstruction by a multidisciplinary team is required for these complex patients. Not infrequently, patients need staged surgical procedures throughout childhood and adolescence. Following significant improvements in medical care and surgical reconstructive techniques, nearly all patients with cloacal exstrophy now survive, leading to an increased emphasis on quality of life. Increased attention is given to gender identity and the implications of reconstructive decisions. Long-term sequelae of cloacal exstrophy, including functional continence and sexual dysfunction, are recognized, and many patients require ongoing complex care into adulthood.

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.

Surgical Considerations in the Management of Adolescent Endometriosis-An Expert Commentary.

Given the complexities and controversies that exist in diagnosing adult endometriosis, as well as optimizing medical and surgical management, it is not surprising that there is even more ambiguity and inconsistency in the optimal surgical care of endometriosis in the adolescent. This collaborative commentary aimed to provide evidence-based recommendations optimizing the role of surgical interventions for endometriosis in the adolescent patient with input from experts in minimally invasive gynecologic surgery, pediatric and adolescent gynecology, and infertility/reproductive medicine.

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.

Evaluation and Management of Endometriosis in the Adolescent.

Endometriosis is a chronic condition, with debilitating symptoms affecting all ages. Dysmenorrhea and pelvic pain often begin in adolescence, affecting school, daily activities, and relationships. Despite the profound burden of endometriosis, many adolescents experience suboptimal management and significant delay in diagnosis. The symptomatology and laparoscopic findings of endometriosis in adolescents are often different than in adults, and the medical and surgical treatments for adolescents may differ from those for adults as well. This Narrative Review summarizes the diagnosis, evaluation, and management of endometriosis in adolescents. Given the unique challenges and complexities associated with diagnosing endometriosis in this age group, it is crucial to maintain a heightened level of suspicion and to remain vigilant for signs and symptoms. By maintaining this lower threshold for consideration, we can ensure timely and accurate diagnosis, enabling early intervention and improved management in our adolescent patients.

Toward representative genomic research: the children's rare disease cohorts experience.

Background: Due to racial, cultural, and linguistic marginalization, some populations experience disproportionate barriers to genetic testing in both clinical and research settings. It is difficult to track such disparities due to non-inclusive self-reported race and ethnicity categories within the electronic health record (EHR). Inclusion and access for all populations is critical to achieve health equity and to capture the full spectrum of rare genetic disease. Objective: We aimed to create revised race and ethnicity categories. Additionally, we identified racial and ethnic under-representation amongst three cohorts: (1) the general Boston Children's Hospital patient population (general BCH), (2) the BCH patient population that underwent clinical genomic testing (clinical sequencing), and (3) Children's Rare Disease Cohort (CRDC) research initiative participants. Design and Methods: Race and ethnicity data were collected from the EHRs of the general BCH, clinical sequencing, and CRDC cohorts. We constructed a single comprehensive set of race and ethnicity categories. EHR-based race and ethnicity variables were mapped within each cohort to the revised categories. Then, the numbers of patients within each revised race and ethnicity category were compared across cohorts. Results: There was a significantly lower percentage of Black or African American/African, non-Hispanic/non-Latine individuals in the CRDC cohort compared with the general BCH cohort, but there was no statistically significant difference between the CRDC and the clinical sequencing cohorts. There was a significantly lower percentage of multi-racial, Hispanic/Latine individuals in the CRDC cohort than the clinical sequencing cohort. White, non-Hispanic/non-Latine individuals were over-represented in the CRDC compared to the two other groups. Conclusion: We highlight underrepresentation of certain racial and ethnic populations in sequencing cohorts compared to the general hospital population. We propose a range of measures to address these disparities, to strive for equitable future precision medicine-based clinical care and for the benefit of the whole rare disease community.

Racial and ethnic representation amongst general clinics, clinics that provide genetic testing, and genomic-based research at Boston Children's Hospital Background: Individuals who identify as belonging to a race or ethnicity that has been historically excluded from mainstream cultural, political, and economic activities ('historically marginalized') experience barriers to clinical care. These barriers are further complicated for families touched by rare genetic conditions. Obstacles can present as accessibility issues (transportation, financial, linguistic), low-quality medical care, or inadequate inclusion in research. It is important to have representation within rare disease research so that the full scope of these conditions is understood, leading to better patient care for all, and for health equity. Objective: We aimed to (1) to create new and inclusive race and ethnicity categories for the electronic health record (EHR) and (2) identify differences in racial and ethnic representation amongst patients generally seen at Boston Children's Hospital (general BCH), those who received genetic testing in a clinic at Boston Children's Hospital (clinical sequencing), and participants who enrolled in the CRDC research project at Boston Children's Hospital (CRDC). Design and Methods: We combined race and ethnicity categories to make more inclusive options than existing EHR categories. Differences in race and ethnicity representation were observed when looking at the three different patient groups (general BCH, clinical sequencing, and CRDC). Results: We observed a lower percentage of individuals who self-identify as Black or African American/African, non-Hispanic/non-Latine in the genetic testing groups (both research and clinical) than in the general BCH group. Individuals who self-identify as multi-racial, Hispanic/Latine are also under-represented in the CRDC research compared to the two other groups. The highest population percentage seen in all groups was that of patients who identify as White, non-Hispanic/non-Latine. This group was over-represented in the research CRDC group compared to the two others. Conclusion: Our study found that patients who are historically marginalized are underrepresented in clinical genetic testing and genomic research studies compared to their White counterparts. In order to benefit all patients with rare genetic conditions, these differences must be addressed by improving access to specialty physicians/researchers and incorporating inclusive language in the EHR, clinics, and research protocols.

Pelvic Ectopic Kidney Prevalence and Pressure Changes During Cloacal Exstrophy (Omphalocele-Exstrophy-Imperforate Anus-Spinal Defects Syndrome) Closure.

OBJECTIVES: To assess the prevalence of pelvic ectopic kidneys (PEK) and compare renal parameters of the PEK to the orthotopic kidney following pubic bone approximation. METHODS: In four Omphalocele-Exstrophy-Imperforate Anus-Spinal Defects Syndrome (OEIS) patients undergoing second-stage repair with known pelvic and orthotopic kidneys, changes in the renal pelvis pressure (RPP), peak systolic velocity (PSV), and resistive index (RI) were measured in the pelvic and orthotopic kidneys following pubic bone approximation. A paired t-test was performed for analysis. Prevalence was tabulated using a multi-institutional retrospective review. RESULTS: Mean rise in RPP was +26.0 mmHg in the PEK and +10.3 mmHg in the orthotopic kidney (P = .55). One patient had immediate increase in RPP of 66 mmHg in the PEK (7 mmHg in the orthotopic kidney) which decreased to +17 mmHg in the PEK upon release of the pubic approximation stitch. Mean change in PSV was +67.7 cm/s in the PEK compared to - 25.7 cm/s in the orthotopic kidney (P = .09). Mean change in renal RI was + 0.06 in PEK compared to - 0.01 in the orthotopic kidney (P = .29). Among 80 OEIS patients, 24 (30%) had a PEK. 3 (4%) had a solitary PEK. CONCLUSION: During second-stage OEIS closure, we witnessed patterns of higher change in RPP, PSV, and RI of the PEK compared to the orthotopic kidney. Furthermore, PEKs were found in nearly a third of our OEIS patients. Real-time RPP monitoring, especially those with solitary PEK, may aid in the management of OEIS patients during the perioperative period.

Exstrophy-Epispadias Complex.

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

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.

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.

Approaches and Barriers to Biomarker Discovery: The Example of Biomarkers of Renal Scarring in Pediatric Urology.

A noninvasive test that can longitudinally assess renal parenchymal status would be incredibly valuable for a wide range of conditions, including neurogenic bladder, renal transplantation, and upper and lower urinary tract anomalies. To address this need, enormous amounts of time, effort, and resources have been invested to identify biologic molecules that signal the pathologic processes of renal parenchymal defects. In this comprehensive narrative review, the authors summarize biomarkers that have previously been investigated while highlighting the key pitfalls and barriers that have impeded biomarker discovery and translation.

All-MEMS Lidar Using Hybrid Optical Architecture with Digital Micromirror Devices and a 2D-MEMS Mirror.

In a lidar system, replacing moving components with solid-state devices is highly anticipated to make a reliable and compact lidar system, provided that a substantially large beam area with a large angular extent as well as high angular resolution is assured for the lidar transmitter and receiver. A new quasi-solid-state lidar optical architecture employs a transmitter with a two-dimensional MEMS mirror for fine beam steering at a fraction of the degree of the angular resolution and is combined with a digital micromirror device for wide FOV scanning over 37 degree while sustaining a large aperture area of 140 mm squared. In the receiver, a second digital micromirror device is synchronized to the transmitter DMD, which enables a large FOV receiver. An angular resolution of 0.57°(H) by 0.23° (V) was achieved with 0.588 fps for scanning 1344 points within the field of view.

Abdominal Wall Endometriosis.

Abdominal wall endometriosis (AWE) is a rare type of endometriosis defined as endometrial glands and stroma located within the abdominal wall. Patients with a history of prior abdominal surgery classically present with cyclic abdominal pain and a palpable mass. Definitive diagnosis is made by pathologic tissue examination, but preoperative imaging with ultrasonography or MRI helps narrow the differential and informs surgical management. Surgical management is traditionally via an open approach; however, laparoscopic removal of AWE is recommended for subfascial or rectus lesions. Following surgical excision, more than 90% of patients experience complete symptom relief.

Normative values for ureteral diameter in children.

BACKGROUND: Assessment of the ureter is a fundamental part of the radiologic evaluation of the urinary tract. Abnormal ureteral dilation warrants further investigation to assess the etiology, which includes obstruction and/or reflux. Despite this fundamental need, there are no established normative values in children based on imaging. OBJECTIVE: To provide normative values for ureteral diameter in pediatric patients with age-related ranges. MATERIALS AND METHODS: We retrospectively reviewed all magnetic resonance (MR) urography studies and chose only normal ureters for assessment. The images were analyzed on commercially available software to assess maximum internal diameter. Manual measurements were done in cases where the images were below the resolution for automated assessment. Maximum intraluminal ureteral diameters were measured in upper, mid and lower thirds and the average of the three maximum ureteral diameters was used to obtain the average widest internal ureteral diameter. Multivariable linear regression was performed to test the association between the calculated diameter and gender. Differences in sizes between the left and right ureter were assessed using paired Wilcoxon signed rank test. RESULTS: One hundred twenty-one MR urography studies were selected, which included 160 ureter units. The diameter increases progressively with age, ranging from 3.2 mm during infancy to 5.0 mm in patients older than 16 years of age. After 9 years of age, the average widest internal ureteral diameter is slightly larger in males compared to females (odds ratio [OR]=1.91, 95% confidence interval [CI] [1.63, 2.25], P<0.0001). The right ureter was slightly larger than the left (3.9 mm vs. 3.7 mm, P=0.004) among 39 patients in whom both right and left ureter units were included. The average mid ureteral diameter is widest, followed by the distal third then proximal third. CONCLUSION: We present the normative values for the average widest internal ureteral diameter based on laterality and different segments. In the pediatric population, 3.8 mm should be considered the average widest internal ureteral diameter.

Endometriosis Classification Systems: An International Survey to Map Current Knowledge and Uptake.

OBJECTIVE: In the field of endometriosis, several classification, staging and reporting systems have been developed, but do clinicians routinely use these classification systems, which system do they use and what are the clinicians' motivations? DATA SOURCES: A cross-sectional study was performed to gather data on the current use of endometriosis classification systems, problems encountered and interest in a new simple surgical descriptive system for endometriosis. Of particular focus were three systems most commonly used: the Revised American Society for Reproductive Medicine (rASRM) classification, the Endometriosis Fertility Index (EFI), and the ENZIAN classification. Data were analysed by SPSS. A survey was designed using the online SurveyMonkey tool consisting of 11 questions concerning three domains-participants background, existing classification systems and intentions with regards to a new classification system for endometriosis. Replies were collected between 15 May and 1 July 2020. METHODS OF STUDY SELECTION: na TABULATION, INTEGRATION AND RESULTS: The final dataset included the replies of 1178 clinicians, including surgeons, gynecologists, reproductive endocrinologists, fertility specialists and sonographers, all managing women with endometriosis in their clinical practice. Overall, 75.5% of the professionals indicate that they currently use a classification system for endometriosis. The rASRM classification system was the best known and used system, the EFI system and ENZIAN system were known by a majority of the professionals but used by only a minority. The lack of clinical relevance was most often selected as a problem with using any system. The findings of the survey suggest that clinicians worldwide are open to using a new classification system for endometriosis that can achieve standardized reporting, and is clinically relevant and simple. The findings therefore support future initiatives for the development of a new descriptive system for endometriosis and provide information on user expectations and conditions for universal uptake of such a system. CONCLUSION: Even with a high uptake of the existing endometriosis classification systems (rASRM, ENZIAN and EFI), most clinicians managing endometriosis would like a new simple surgical descriptive system for endometriosis.

Endometriosis classification systems: an international survey to map current knowledge and uptake.

STUDY QUESTION: Which classification system for endometriosis do clinicians use most frequently, and why? SUMMARY ANSWER: Even with a high uptake of the three existing endometriosis classification systems, most clinicians managing endometriosis would like a new simple surgical descriptive system for endometriosis. WHAT IS KNOWN ALREADY: In the field of endometriosis, several classifications, staging and reporting systems have been developed and published, but there are no data on the uptake of these systems in clinical practice. STUDY DESIGN SIZE DURATION: A survey was designed using the online SurveyMonkey tool consisting of 11 questions concerning three domains-participants background, existing classification systems and intentions with regards to a new classification system for endometriosis. Replies were collected between 15 May and 1 July 2020. PARTICIPANTS/MATERIALS SETTING METHODS: A cross-sectional study was performed to gather data on the current use of endometriosis classification systems, problems encountered and interest in a new simple surgical descriptive system for endometriosis. The particular focus was on the three systems most commonly used: the Revised American Society for Reproductive Medicine (rASRM) classification, the endometriosis fertility index (EFI), and the ENZIAN classification. Data were analysed to detect statistically significant differences among user groups. MAIN RESULTS AND THE ROLE OF CHANCE: The final dataset included the replies of 1178 clinicians, including surgeons, gynaecologists, reproductive endocrinologists, fertility specialists and sonographers, all managing women with endometriosis in their clinical practice. Overall, 75.5% of the professionals indicate that they currently use a classification system for endometriosis. The rASRM classification system was the best known and used system, while the EFI system and ENZIAN system were known by a majority of the professionals but used by only a minority. The lack of clinical relevance was most often selected as a problem with using any system. The vast majority of respondents replied positively to the question on whether they would use a simple surgical descriptive system available for endometriosis, if available. LIMITATIONS REASONS FOR CAUTION: While the total number of respondents was acceptable, some regions/professions were not sufficiently represented to draw conclusions. WIDER IMPLICATIONS OF THE FINDINGS: The findings of the survey suggest that clinicians worldwide are open to using a new classification system for endometriosis that can achieve standardized reporting and is clinically relevant and simple. The findings therefore support future initiatives for the development of a new descriptive system for endometriosis and provide information on user expectations and conditions for universal uptake of such a system. STUDY FUNDING/COMPETING INTERESTS: The meetings and activities of the working group were funded by the American Association of Gynecologic Laparoscopists, European Society for Gynecological Endoscopy, ESHRE and World Endometriosis Society. A.W.H. reports grant funding from the MRC, NIHR, CSO, Roche Diagnostics, Astra Zeneca, Ferring, Charles Wolfson Charitable Trust, Standard Life, and consultancy fees from Roche Diagnostics, AbbVie, Nordic Pharma and Ferring, outside the submitted work. In addition, A.W.H. has a patent Serum biomarker for endometriosis pending. He is Chair of TSC for STOP-OHSS and CERM trials and Chair of RCOG Academic Board 2018-2021. M.A. reports being member of the executive board and vice president of AAGL. N.P.J. reports personal fees from Abbott, Guerbet, Myovant Sciences, Vifor Pharma, Roche Diagnostics outside the submitted work; he is also President of the World Endometriosis Society and chair of the trust board. S.M. reports grants from AbbVie, DoD, NIH and Marriot Family Foundation, honoraria from University British Columbia and WERF, support for speaking at conferences (ESHRE, CanSAGE, Endometriosis UK, UEARS, IFFS, IASP, National Endometriosis Network UK) participation on Advisory Boards from AbbVie and Roche, outside the submitted work. She also discloses having a leadership or fiduciary role in SWHR, WERF, WES, ASRM and ESHRE. C.T. reports grants, consulting and speakers' fees non-financial support and other from Merck SA, non-financial support and other consulting fees from Gedeon Richter and Nordic Pharma, and support for meeting attendance non-financial support from Ferring Pharmaceuticals, outside the submitted work and without private revenue. K.T.Z. reports grants from Bayer Healthcare, MDNA Life Sciences, Volition Rx, and Evotec (Lab282-Partnership programme with Oxford University), non-financial support from AbbVie Ltd, all outside the submitted work; and is a Board member (Secretary) of the World Endometriosis Society and World Endometriosis Research Foundation. J.P. reports personal fees from Hologic, Inc., outside the submitted work; he is also a member of the executive boards of ASRM and SRS. The other authors had nothing to disclose.

Risk Factors Associated with Adnexal Torsion after Hysterectomy.

STUDY OBJECTIVE: To identify preoperative and intraoperative risk factors for adnexal torsion after hysterectomy, and to estimate the incidence of the disease in the modern-day era of laparoscopic surgery. DESIGN: Retrospective nested case-control study. SETTING: Large urban medical system. PATIENTS: Eighty-nine female patients ages 17 to 51. INTERVENTIONS: Patients underwent ovarian-sparing hysterectomy. MEASUREMENTS AND MAIN RESULTS: The estimated incidence of ovarian torsion after hysterectomy was 0.5% (46/8538 ovarian-sparing hysterectomies). The following variables were found to be associated with adnexal torsion after hysterectomy in an adjusted logistic regression: laparoscopic or laparoscopic-assisted approach to hysterectomy vs any other approach (odds ratio [OR], 3.36; 95% confidence interval [CI], 0.86-13.23); younger age at the time of hysterectomy (17-40 years) vs older age (41-51 years) (OR, 3.45; 95% CI, 1.33-8.97); and a gynecologic history significant for endometriosis (OR, 4.07; 95% CI, 1.04-15.88). CONCLUSION: There is an association between laparoscopic approach to hysterectomy, younger age at time of hysterectomy, and a history of endometriosis with subsequent risk of adnexal torsion. Providers should have a heightened index of suspicion for adnexal torsion after hysterectomy in patients presenting with acute-onset abdominal pain who underwent laparoscopic hysterectomy at a younger age.

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.