Uterovaginal Anomalies: A Guide for the Generalist Obstetrician-Gynecologist.

Congenital gynecologic anomalies result from interruption of embryologic development of the female reproductive tract. The anomalies may be hymenal, vaginal, cervical, or uterine. The impact of these anomalies is variable: some are asymptomatic, incidental findings that require no intervention, others require simple surgical management, while some complex anomalies may require a multidisciplinary approach with extensive surgical expertise for optimal outcomes. Uterovaginal anomalies may occur in isolation or in association with other malformations, such as renal anomalies. The origin, presentation, evaluation and treatment of these conditions are reviewed here.

Unexpected ethical dilemmas in sex assignment in 46,XY DSD due to 5-alpha reductase type 2 deficiency.

Sex assignment at birth remains one of the most clinically challenging and controversial topics in 46,XY disorders of sexual development (DSD). This is particularly challenging in deficiency of 5-alpha reductase type 2 given that external genitalia are typically undervirilized at birth but typically virilize at puberty to a variable degree. Historically, most individuals with 5-alpha reductase deficiency were raised females. However, reports that over half of patients who underwent a virilizing puberty adopted an adult male gender identity have challenged this practice. Consensus guidelines on assignment of sex of rearing at birth are equivocal or favor male assignment in the most virilized cases. While a male sex of rearing assignment may avoid lifelong hormonal therapy and/or allow the potential for fertility, female sex assignment may be more consistent with external anatomy in the most severely undervirilized cases. Herein, we describe five patients with 46,XY DSD due 5-alpha-reductase type 2 deficiency, all with a severe phenotype. An inter-disciplinary DSD medical team at one of two academic centers evaluated each patient. This case series illustrates the complicated decision-making process of assignment of sex of rearing at birth in 5-alpha reductase type 2 deficiency and the challenges that arise when the interests of the child, parental wishes, recommendations of the medical team, and state law collide.

Integration of child life services in the delivery of multi-disciplinary differences in Sexual Development (DSD) and Congenital Adrenal Hyperplasia (CAH) care.

INTRODUCTION: Multiple studies have demonstrated the benefit of incorporating certified child life specialist (CCLS) services in various aspects of pediatric care. Although the significance of psychosocial support of patients with Disorders of Sexual Development (DSD) and Congenital Adrenal Hyperplasia (CAH) is increasingly recognized, the involvement of CCLS services into the DSD and CAH multidisciplinary care model has yet to be described. OBJECTIVE: To evaluate the feasibility, acceptability, and patient and family experience of routinely incorporating CCLS services into the multidisciplinary DSD and CAH care model. STUDY DESIGN: As part of a quality improvement initiative, CCLS services were routinely incorporated in the multidisciplinary DSD and CAH clinics at our institution. Encounters for patients seen in clinic between July 2018 through October 2019 were reviewed for demographic information, DSD diagnosis classification, CCLS documentation, and whether an exam under anesthesia (EUA) was required due to an incomplete clinical exam. CCLS documentation was reviewed for assessments, interventions, whether patients tolerated their physical exams, time of CCLS services, and additional CCLS support beyond the physical exam. All patients were limited to one physical exam per clinic visit. RESULTS: Out of the 45 encounters with CCLS involvement, 42 (93.3%) exams were well-tolerated. CCLS assessments considered patient development, communication considerations, temperament, medical stressors, coping preferences, and patient preferences for activities and distractions. Interventions included preparing patients for their physical exams, encouragement before and during exams, addressing patient stressors, distractions and coping mechanisms, and advocating for the patient. No patients required an EUA. DISCUSSION: The CCLS aimed to provide families with a sense of control during clinic visits and teach them to advocate for themselves. The CCLS helped prepare and distract patients for their clinic visit and addressed the sensitive nature of the physical exam by focusing on the emotional and development needs of patients. CCLS contributions to a positive patient experience are consistent with multiple studies demonstrating the benefit of CCLS services for pediatric care. This quality improvement initiative ultimately helped to create a positive experience for patients and families. CONCLUSION: This study demonstrates the feasibility, acceptability, and positive impact of CCLS services in the delivery of patient and family-centered care for patients with DSD and CAH as part of the multidisciplinary team model.

Experiences and attitudes of young adults with congenital bowel and bladder conditions.

INTRODUCTION: Young adults with complex congenital bowel and bladder anomalies are a vulnerable population at risk for poor health outcomes. Their experiences with the healthcare system and attitudes towards their health are understudied. OBJECTIVE: Our objective was to describe how young adults with congenital bladder and bowel conditions perceive their current healthcare in the domains of bladder and bowel management, reproductive health, and transition from pediatric to adult care. STUDY DESIGN: At a camp for children with chronic bowel and bladder conditions, we offered a 50-question survey to the 62 adult chaperones who themselves had chronic bowel and bladder conditions. Of the 51 chaperones who completed the survey (a response rate of 82%), 30 reported a congenital condition and were included. RESULTS: The cohort of 30 respondents had a median age of 23 years and almost half of the subjects (46%) reported not having transitioned into adult care. Most reported bowel (81%) and bladder (73%) management satisfaction despite high rates of stool accidents (85%), urinary accidents (46%), and recurrent urinary tract infections (70%). The majority of respondents (90%) expressed interest in having a reproductive health provider as part of their healthcare team. The median ages of the first conversation regarding transition to adult care and feeling confident in managing self-healthcare were 18 and 14 years, respectively. Most (85%) reported feeling confident in navigating the medical system. DISCUSSION: In this cohort of young adults who reported confidence with self-care and navigating the medical system, the proportion who had successfully transitioned into to adult care was low. These data highlight the need for improved transitional care and the importance of patient-provider and provider-provider communication throughout the transition process. CONCLUSION: These data highlight the need to understand the experience of each individual patient in order to provide care that aligns with their goals.

Patient-reported outcomes in adult females with bladder exstrophy: A study of long-term sexual, reproductive and urinary outcomes using social media.

INTRODUCTION: Limited data exist on patient-reported outcomes in adults with bladder exstrophy (BE). We partnered with the Association for the Bladder Exstrophy Community (A-BE-C) using social media to survey adult females with BE. The aim of the study was to assess long-term patient-reported sexual, reproductive and continence outcomes. MATERIALS AND METHODS: Between December 3, 2018 and January 18, 2019, A-BE-C promoted an anonymous survey of adult females with BE on social media. The survey included the Female Sexual Function Index (FSFI) and International Consultation on Incontinence Questionnaire - Short Form (ICIQ-SF) in addition to questions on demographics, reproductive and gynecological outcomes. RESULTS: A total of 130 women with a median age of 30 years (IQR 26, 41) completed the survey. The majority of women were born in the United States (N = 86, 66.2%). Women reported a median of 10 (IQR 5, 17) surgeries performed for their condition. The mean ICIQ-SF score was 6.2 ± 6.2 (moderate). Only 19.2% (N = 25) reported volitionally voiding and the majority reported intermittent catheterization through a catheterizeable channel or the urethra (Summary Table). The mean FSFI score was (20.1 ± 9.0), indicating risk for sexual dysfunction (FSFI < 26.55). Of the respondents, 28.5% (N = 37) were treated for uterine prolapse, 56.9% (N = 74) required surgery in order to use tampons or have penetrative intercourse, and over half (55.4%) reported some degree of dissatisfaction with the appearance of their external genitalia. Forty-seven women (36.2%) reported pregnancies, and of these 32 (68.1%) reported complications with pregnancy. Outcomes of 100 pregnancies included miscarriage/abortion (41.0%), preterm vaginal (3.0%), preterm cesarean section (19.0%), term vaginal (2.0%), and term cesarean section (35.0%). Seven (15.2%) women identified having children with a medical diagnosis, none of which included BE. Infertility was reported in 17.3% (22/127) women. Women identified sexual function, fertility, and body image as areas that need future research. CONCLUSION: This is the largest study of patient-reported outcomes in females with BE achieved through partnership with an advocacy group and social media. We found that continence and sexual function were both impaired in adult women with BE. Respondents reported poor obstetric and gynecological outcomes including infertility, uterine prolapse, vaginal stenosis, and need for multiple surgeries. BE surgeons, through multi-institutional collaboration, should continue to reassess surgical techniques at initial repair to better address these functional issues. These results should be a part of counseling parents of newborns with BE.

Bits and Pieces: A Crowd-Sourced Series of 54 Cases of Fractured Hormonal Implants.

STUDY OBJECTIVE: The fracture of hormonal implants, including Implanon, Nexplanon (both from Merck & Co, Inc), and histrelin acetate is rare. Our aim was to describe patient demographic characteristics, mechanisms, and consequences of fractured implants by surveying physicians' experience via listservs and social media. DESIGN, SETTING, PARTICIPANTS, INTERVENTIONS, AND MAIN OUTCOME MEASURES: We developed a Research Electronic Data Capture survey for physicians regarding their experience with implant fracture, including patient characteristics, mechanism of fracture, changes in menstrual bleeding patterns, time from insertion to fracture, and time from fracture to seeking care. We distributed the survey to providers in listservs for the North American Society for Pediatric and Adolescent Gynecology, the Family Planning Fellowship, the Ryan Program, and the Facebook Physician Moms Group and Facebook OB-GYN Mom Group. We performed descriptive analyses. RESULTS: We received 42 survey responses, representing 54 discrete implant fractures of which 70% (n=14) were Nexplanon, 26% (n=38) were Implanon, and 4% (n=2) were histrelin acetate. Mechanisms of implant fracture included patient manipulation (23%, n=12), unintentional trauma (11%, n=6), interpersonal violence (8%, n=4), lifting/carrying (6%, n=3), fracture with removal (6%, n=3), and unknown (47%, n=25). Bleeding pattern was not altered in 78% (n=42) of cases. Time interval between placement and fracture was less than 2 years for 63% (n=34) of cases. Thirty-nine percent (n=21) of patients presented for care more than 1 month from the time of fracture. CONCLUSION: Patients should be counseled about potential for hormonal implant fracture, advised against excessive manipulation of implants, and counseled to present for care immediately upon noticing an implant fracture. Surveying physicians through listervs and social media is an effective strategy to increase the reporting of rare complications and events.

Use of 3D reconstruction cloacagrams and 3D printing in cloacal malformations.

INTRODUCTION: Cloacal anomalies are complex to manage, and the anatomy affects prognosis and management. Assessment historically includes examination under anesthesia, and genitography is often performed, but these do not consistently capture three-dimensional (3D) detail or spatial relationships of the anatomic structures. Three-dimensional reconstruction cloacagrams can provide a high level of detail including channel measurements and the level of the cloaca (<3 cm vs. >3 cm), which typically determines the approach for surgical reconstruction and can impact long-term prognosis. Yet this imaging modality has not yet been directly compared with intra-operative or endoscopic findings. OBJECTIVES: Our objective was to compare 3D reconstruction cloacagrams with endoscopic and intraoperative findings, as well as to describe the use of 3D printing to create models for surgical planning and education. STUDY DESIGN: An IRB-approved retrospective review of all cloaca patients seen by our multi-disciplinary program from 2014 to 2016 was performed. All patients underwent examination under anesthesia, endoscopy, 3D reconstruction cloacagram, and subsequent reconstructive surgery at a later date. Patient characteristics, intraoperative details, and measurements from endoscopy and cloacagram were reviewed and compared. One of the 3D cloacagrams was reformatted for 3D printing to create a model for surgical planning. RESULTS: Four patients were included for review, with the Figure illustrating 3D cloacagram results. Measurements of common channel length and urethral length were similar between modalities, particularly with confirming the level of cloaca. No patient experienced any complications or adverse effects from cloacagram or endoscopy. A model was successfully created from cloacagram images with the use of 3D printing technology. DISCUSSION: Accurate preoperative assessment for cloacal anomalies is important for counseling and surgical planning. Three-dimensional cloacagrams have been shown to yield a high level of anatomic detail. Here, cloacagram measurements are shown to correlate well with endoscopic and intraoperative findings with regards to level of cloaca and Müllerian development. Measurement discrepancies may be due to technical variation indicating a need for further evaluation. The translation of the cloacagram images into a 3D printed model demonstrates potential applications of these models for pre-operative planning and education of both families and trainees. CONCLUSIONS: In our series, 3D reconstruction cloacagrams yielded accurate measurements of urethral length and level of cloaca common channel and urethral length, similar to those found on endoscopy. Three-dimensional models can be printed from using cloacagram images, and may be useful for surgical planning and education.

Management of Large Ovarian Neoplasms in Pediatric and Adolescent Females.

The overwhelming majority of ovarian cysts in pediatric and adolescent girls are physiologic; however, large simple and complex ovarian lesions often require surgical intervention due to the increased risk of neoplasia. In this review article, we discuss the preoperative evaluation and intraoperative management of large ovarian neoplasms. We review the current literature regarding long term ovarian function and fertility, rates of recurrence and residual disease, and novel surgical approaches. Managing large ovarian neoplasms in the pediatric and adolescent population requires careful preoperative and intraoperative care to optimally resect neoplasia while maximizing fertility and minimizing pain.

Long acting reversible contraception in adolescents with cardiovascular conditions.

STUDY OBJECTIVE: The purpose of this case series was to evaluate the efficacy and complication rate with use of long-acting reversible contraceptive (LARC) methods in adolescents with cardiovascular conditions. DESIGN AND PARTICIPANTS: We performed a retrospective chart review of adolescents age 22 or younger with congenital cardiac anomalies or major cardiovascular conditions who had a LARC device placed at Seattle Children's Hospital or University of Washington Medical Center between January 1, 2007 and March 1, 2012. MAIN OUTCOME MEASURES: Patient characteristics, medical conditions, indications, and complications including perforation, pregnancy, expulsion, and pelvic infection were collected and analyzed. RESULTS: Thirty adolescents with congenital cardiac anomalies or other cardiovascular conditions had a LARC device inserted during the study period. Mean age at placement was 17.7 years (range 12-22). Twelve patients (40%) had reported a previous pregnancy and 12 (40%) had never been sexually active at time of placement. Common primary and secondary indications were desired contraception (22 patients, 73%), desired menstrual suppression (15 subjects, 50%) and treatment of heavy menstrual bleeding (8 subjects, 27%). Twenty-nine patients had a structural cardiac anomaly. There were 27 levonorgestrel-releasing intrauterine devices (IUD), 1 copper IUD, and 3 etonogestrel implants placed. There were 2 confirmed IUD expulsions and 1 removal due to persistent irregular bleeding and cramping. There were no cases of pelvic inflammatory disease or pregnancies with LARC methods in place. Twenty-seven (90%) patients were continuing LARC at last follow-up. CONCLUSIONS: In this small case series of adolescents with cardiovascular conditions, LARC devices were effective at pregnancy prevention without serious complications.

Teratomas and ovarian lesions in children.

Ovarian pathology in children is common and the pathology can be quite diverse. The most common benign ovarian tumor in childhood is a teratoma. In this article, we discuss the origin of these germ cell tumors followed by a complete discussion of ovarian pathology.

Unverifiable and erroneous publications reported by obstetrics and gynecology residency applicants.

OBJECTIVE: To estimate the rate of erroneous and unverifiable publications in applications for an obstetrics and gynecology residency and to determine whether there were associated characteristics that could assist in predicting which applicants are more likely to erroneously cite their publications. METHODS: This was a review of the Electronic Residency Application Service applications submitted to the University of Washington obstetrics and gynecology residency for the 2008 and 2009 matches. Publications reported to be peer-reviewed articles and abstracts were searched by querying PubMed, Google, and journal archives (first tier), topic-specific databases (second tier), and by e-mailing journal editors (third tier). Errors were categorized as minor, major, and unverified. RESULTS: Five-hundred forty-six (58%) of 937 applicants listed a total of 2,251 publication entries. Three-hundred fifty-three applicants (37.7%) listed 1,000 peer-reviewed journal articles and abstracts, of which 751 were reported as published and 249 as submitted or accepted. Seven-hundred seventy (77.0%) publications were found by a first-tier search, 51 (5.1%) were found by a second-tier search, 23 (2.3%) were found by a third-tier search, and 156 (15.6%) were unverified. Of the 353 applicants listing peer-reviewed articles or abstracts, 25.5% (90 of 353) committed major errors, 12.5% (44 of 353) committed minor errors, and 24.1% (85 of 353) had articles or abstracts that were unverified. CONCLUSION: Most applicants reported their publications accurately or with minor errors; however, a concerning number of applicants had major errors in their citations or reported articles that could not be found, despite extensive searching. Reported major and unverified publication errors are common and should cause concern for our specialty, medical schools, and our entire medical profession.