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Background : The definitive treatment of distal vaginal atresia is surgery, but menstrual suppression is often helpful for initial management. Case : A 13-year-old presented with primary amenorrhea and progressive abdominal pain. She was diagnosed with distal vaginal atresia and started on hormonal suppression. She then re-presented with heavy vaginal bleeding and follow-up imaging revealed spontaneous perforation had occurred. There was now evidence of a tract leading from the obstructed vaginal bulge to the introitus. Vaginoplasty was complicated by the tortuosity of the tract. Under ultrasound guidance, a pull-through vaginoplasty was performed. Summary and Conclusion : Spontaneous perforation of an atretic vagina is rare, but in such cases, urgent vaginoplasty is indicated to prevent infection. Despite the presence of a spontaneous tract leading to the obstruction, vaginoplasty can be complex, and intra-operative ultrasound may be beneficial.

OBJECTIVE To assess the degree to which heavy menstrual bleeding is associated with depression, independent of hormonal contraception. STUDY DESIGN We performed a retrospective cohort study of 1168 female adolescents 9-18 years old presenting to general pediatricians for heavy menstrual bleeding or well visits. Depression was the primary outcome and defined as a diagnosis in the health record. Univariable and multivariable regression models were fit to the data to identify factors associated with depression diagnosis. RESULTS In total, 581 adolescents with heavy menstrual bleeding and 587 without heavy menstrual bleeding were included. Depression diagnoses occurred with greater frequency in youth with heavy menstrual bleeding compared with those without heavy menstrual bleeding (50.9% vs 24.2% P

To determine the frequency of persistence of vulvar lichen sclerosus (LS) through the pubertal transition and assess if the symptomatology and exam findings differ by menarchal status at onset of symptoms DESIGN: A retrospective cohort study SETTING: Academic tertiary care hospital PARTICIPANTS: Females aged 21 years or younger with a diagnosis of vulvar LS INTERVENTION: None MAIN OUTCOME MEASURES: Menarchal status at symptom onset, presenting symptoms, exam findings, persistence after menarche RESULTS AND CONCLUSIONS: Of the 196 patients who met criteria, 141 were premenarchal and 55 postmenarchal. Of these 55, 36 had postmenarchal symptom onset, and the others had premenarchal symptom onset or LS diagnosis. Over the data review period, 26 patients were followed through the pubertal transition, and 10 (38.5%) had continued symptoms of LS. The premenarchal group (n = 141) was significantly more likely than the symptom-onset postmenarchal group (n = 36) to present with vulvar itching (70.2% vs 52.8%; P = .048), vulvar bleeding (26.2% vs 5.6%; P = .008), and bowel symptoms (16.3% vs 0%; P = .009). The premenarchal group was significantly more likely on exam to have subepithelial hemorrhages (24.8% vs 5.6%; P = .01). The postmenarchal group had more clitoral adhesions (25.0% vs 4.3%; P.0001) and loss of labia minora (47.2% vs 2.1%; P.0001). Thirteen postmenarchal patients presented with dyspareunia. This study suggests that premenarchal LS can persist after menarche in about 40% of adolescents and can initially develop in postmenarchal adolescents. Initial symptoms and exam findings differ on the basis of menarchal status. Continued surveillance is recommended.

To characterize patterns of cervical cancer screening among adolescents ages 14-20 years before and after the 2009 American College of Obstetrics and Gynecology recommendations.Retrospective cohort study.National Ambulatory Medical Care Survey 2005-2016 data.Female adolescents and young women ages 14-26 years.Multivariable logistic regression models identified independent predictors of unindicated cervical cancer screening at office-based visits among adolescents ages 14-20 years.Cervical cancer screening in women ages 14-20 and 21-26 years in 2 time periods: 2005-2008 and 2009-2016.Between 2005 and 2016, 11,768 office visits were among adolescents ages 14-20 years. Overall, the cervical cancer screening rate for young women ages 14-20 years was 4.0%, which decreased from 4.5% to 0.4% (P = .008) during the study period. Adolescents who received cervical cancer screening during an office visit were older (18-20 years: 24.1% vs 14-17 years: 8.2%; P.001), had a preventive care visit (preventive care: 79.7% vs other visit types: 20.3%; P.001), and saw an obstetrician/gynecologist (obstetrician/gynecologist: 74.81% vs other specialties: 25.1%; P.001). After adjusting for age, year, period, insurance status, region, and provider type, screening for cervical cancer was associated with living in the Southern region of the United States (adjusted odds ratio, 1.88; 95% confidence interval, 1.09-3.25; P = .02) and public insurance (adjusted odds ratio, 0.47, 95% confidence interval, 0.34-0.64; P.001).Despite recommendations, cervical cancer screening continued to occur in the adolescent population-especially older adolescents-creating unnecessary costs and potential harms. These findings show a slow uptake of guidelines nationally and the need for initiatives that encourage implementation of performance measures for providers, including ongoing provider and patient education.

Background Epithelial ovarian cancers are a rare subset of the less than 1% of ovarian cancers diagnosed in children. This case highlights considerations when caring for these patients. Case Evaluation of a 12-year-old postmenarchal girl who presented with suprapubic pain revealed a solid/cystic pelvic mass involving bilateral adnexa and elevated Cancer Antigen 125 (CA-125) level. Diagnostic laparoscopy pathology confirmed low-grade papillary serous ovarian carcinoma. Treatment involved surgical tumor debulking, hysterectomy, bilateral salpingo-oophorectomy, and omentectomy; adjuvant chemotherapy with no residual disease, and normalization of Cancer Antigen 125 (CA-125) level; and an aromatase inhibitor for maintenance. Summary and Conclusion In children with adult-type gynecologic cancers necessitating treatments including surgical sterilization and hormone-modulating therapy, psychological support and developmentally informed collaboration between adult and pediatric services is essential. Clinical decisions for long-term bone and sexual health present opportunities for future research.

Study Objective To compare clinical characteristics, treatment histories, and microbiology of premenarchal girls who presented to a pediatric gynecology specialty clinic with short-duration and chronic vulvar symptoms. Design Retrospective cohort study. Setting Pediatric and adolescent gynecology clinic at a tertiary care children's hospital. Participants One hundred eighty-two premenarchal patients ages 2-14 years who presented to a pediatric gynecology specialty clinic with vulvar complaints and who were evaluated with a yeast and/or bacterial culture. Interventions None. Main Outcome Measures Chronic and short-duration vulvar symptoms, microbiology, and diagnosis. Results Patients with chronic symptoms were more likely to present with itching (59/102 (57.8%) vs 34/80 (42.5%); P = .04), redness or rash (53/102 (52.0%) vs 22/80 (27.5%); P = .0009), and discomfort (59/102 (57.8%) vs 30/80 (37.5%); P = .006), compared with patients with short-duration symptoms. Overall, 44.5% of patients had a history of antifungal treatment, with a greater proportion of patients with chronic symptoms having received antifungal treatment compared with those with short-duration symptoms (53/102 (52.0%) vs 28/80 (35.0%); P = .02). Despite a history of antifungal treatment in nearly half of the patients, Candida albicans was isolated in only 3/144 (2.1%) yeast cultures. Bacterial vulvar cultures were positive in 75/159 (47.2%), and there was no difference among the symptom duration groups (38/71 (53.5%) vs 37/88 (42.1%); P = .15). Conclusion Vulvovaginitis is a common gynecological diagnosis among premenarchal girls with short-duration and chronic vulvar symptoms. Regardless of symptom duration, yeast cultures are rarely positive. Antifungal treatment should be avoided in toilet-trained prepubertal girls.

Iron deficiency is extremely common in adolescents with heavy menstrual bleeding (HMB) presenting to the emergency department; however, patients are rarely screened for this. The objective of this study was to evaluate screening for iron deficiency in adolescents presenting to the emergency department for HMB.This is a secondary analysis of a single-center, cross-sectional observational study using retrospective chart review. The study subjects are adolescents ages 11 to 19 years with International Classification of Diseases, Ninth Revision, Clinical Modification/International Classification of Diseases, Tenth Revision, Clinical Modification, codes for HMB who presented to the emergency department at a national tertiary care hospital from 2006 to 2018. Pregnant adolescents with HMB were excluded. Chart abstraction for demographic data, symptoms, laboratory tests, treatments, and outcomes was performed. The main outcome measure was the number of adolescents who had an iron evaluation in the emergency department and were discharged on oral iron.Of the 258 nonpregnant adolescents who sought care for HMB in the emergency department, 225 (87.2%) were evaluated with serum hemoglobin testing. Ninety-four (41.7%) of those tested were anemic. Only 23 of the 258 patients (8.9%) had iron studies (serum ferritin) performed; 18 of 23 (78.3%) had iron deficiency and 21 of 23 (92.3%) were anemic. Subjects presenting with fatigue, headache, or palpitations were more likely to have iron studies performed than those without these symptoms (all P0.01). Thirty-two of the 258 subjects (12.4%) were discharged on oral iron therapy, which included only 15 of the 18 subjects (83.3%) with iron deficiency determined by ferritin testing.Adolescents presenting to the emergency department with HMB are at significant risk of iron deficiency but are not being screened or treated, which may have significant consequences.

Background There is a high reported rate of sexual dysfunction among women with spina bifida, but little is known about the etiology of this or how sexual satisfaction could be improved. Aim To identify, through the words of women with spina bifida, perceived causes of diminished sexual satisfaction and recommendations to optimize partnered sexual encounters. Methods In this qualitative study, we conducted semi-structured individual interviews with 22 women with spina bifida (median age 26.5 years, range 16–52 years) who have had a romantic partner. Using Grounded Theory, interviews were independently coded by 3 reviewers. Disagreements were resolved by consensus. Outcomes We identified overlapping themes of issues women experienced during sexual intimacy and strategies they learned to improve sexual encounters. Results 7 salient themes emerged from the data: (i) fear of rejection with resulting difficulty setting boundaries and the risk of coercion; (ii) conflict between spontaneity and self-care in sexual encounters; (iii) worry about incontinence during sex; (iv) trial and error in learning optimal sexual positions; (v) decreased genital sensation; (vi) safety considerations; and (vii) sharing advice with other women with spina bifida. Clinical Implications As sexual satisfaction is influenced by physical features as well as psychological, interpersonal, and sociocultural factors, optimizing sexual satisfaction of women with spina bifida is best managed with a holistic approach utilizing a biopsychosocial model. Strengths & Limitations The sample included women with a diverse range of functional impairments. Women were forthright with their comments and thematic saturation was reached. Recruitment was primarily from a single Midwestern institution, which may have limited sampled perspectives. Conclusion While women with spina bifida encounter challenges during sexual encounters, strategies focused on improving communication with partners and addressing specific physical considerations can potentially enhance their sexual experiences.

BACKGROUND: Women with physical disability (WWPD) experience more sexual dysfunction, are typically less sexually active, and engage in fewer intimate relationships than women without physical disability. While patient reported outcome measures (PROMs) can help researchers and providers meet the needs of this population, current measures fail to reflect the relevant experiences of WWPD. The purposes of this study were to 1) understand the experiences of WWPD related to sexual wellness, (2) identify the gaps in the current Patient-Reported Outcomes Measurement Information System Sexual Function and Satisfaction (PROMIS SexFS), and (3) develop a conceptual framework for the measurement of sexual wellbeing on which a new, supplemental measure will be built. METHODS: Women with physical disability (n = 59) were recruited from an online health registry through a large, academic medical center, and took part in semi-structured focus groups and interviews exploring experiences with sexuality, intimate relationships, sexual function, and sexual and reproductive healthcare. Interviews were transcribed and coded using an inductive approach to thematic analysis. RESULTS: Participants expressed that their sexual wellbeing extends beyond simply physical function and includes aspects of their environment, society, and intrapersonal and interpersonal factors. From themes that emerged from the qualitative data, we developed a conceptual framework of sexual wellbeing, which includes sexual health and sexual self-efficacy. The framework is comprised of five constructs that impact sexual health and sexual self-efficacy: physical factors, intrapersonal factors, environmental factors, relationships and partner opportunity, and stigma. CONCLUSION: The conceptual framework can be used to develop PROM items relevant to the experiences of WWPD, facilitate conversations between providers and patients, and identify areas to target for sexual wellness interventions.

Management of contraception in adolescent females with hormone-related venous thromboembolism (VTE) is challenging. We examined the characteristics of this patient population and outcomes, including recurrent VTE, heavy menstrual bleeding, and pregnancy.We performed a single-institution retrospective cohort study of adolescents with a new diagnosis of VTE and concurrent use of estrogen- and/or progestin-containing medication (N = 89). We collected data on additional risk factors for thrombosis, management of hormone therapy, and anticoagulation. We compared outcomes between patients with prescribed contraception within the year after their VTE diagnosis (n = 52) with those without (n = 37).At least one additional risk factor for thrombosis was identified in 92% of patients, and 73% had two or more. The most common additional thrombosis risk factors were obesity (35%), family history (33%), and recent immobility (33%). Ninety-seven percent of patients were receiving combined hormonal medications, and 42% of patients had their medication stopped and not replaced with an alternative. Heavy menstrual bleeding was reported while on anticoagulation in 46% of patients with a documented menstrual history. Recurrent VTE occurred in 9.0% of patients. The group without prescribed contraception had a significantly higher rate of pregnancy in the two years after VTE diagnosis (18% vs. 1.9%, p = .04). Two pregnancies occurred while on warfarin.Early assessment of contraceptive needs and menstrual bleeding symptoms are needed after diagnosis of hormone-related VTE in adolescent females. Access to contraceptive methods associated with low thrombosis risk is important for the prevention of unplanned pregnancy in this patient population.

Background Hereditary hemochromatosis typically presents in adulthood with organ damage secondary to iron overload. In women, menstrual periods are a protective mechanism allowing for monthly loss of iron stores. Case We report the case of a female adolescent whose family history, clinical presentation, and laboratory investigation revealed a diagnosis of hereditary hemochromatosis and von Willebrand disease. For control of heavy menstrual bleeding, menstrual suppression was started with a subsequent increase of her ferritin levels. Summary and Conclusion No significant data exist regarding the management of women with hereditary hemochromatosis who require menstrual suppression. This case highlights the difficulty in balancing the need for hormonal menstrual suppression with its effect on treatment choices, monitoring, and managing iron levels.

Despite the well-established embryological relationship in the development of renal and Müllerian structures, no clear guidelines exist regarding screening for Müllerian anomalies (MA) in the setting of a renal anomaly (RA). Delayed diagnosis of MA can have significant reproductive consequences.To investigate the prevalence of coexisting MA in patients with congenital RA.This is a retrospective cohort study of females age 12-35 years with a diagnosis of RA, identified by diagnosis codes, who were followed for care between 2013 and 2020. Data were collected on demographics, medical history, clinical presentation, and imaging studies. Descriptive statistics were used to summarize the data. This study was IRB approved.A total of 465 patients were included in this study, of whom 326 patients (70.3%) had a pelvic evaluation during the study period. Of these 326 patients, 125 (38.3%) were found to have coexistent MA. About one-third of patients who underwent pelvic evaluation due to pain were found to have MA. For 69.6% (87/125) of patients with MA, the RA was diagnosed prior to the MA. The average age at time of RA diagnosis was 6.4 ± 8.8 years and the average age of MA diagnosis was 16.4 ± 6.9 years. Forty-eight (38.4%) patients had obstructive anomalies. Of the Müllerian obstructions, 93.8% were treated with urgent surgery and the remainder started on hormonal suppression. The prevalence of MA was dependent on the RA diagnosis (Figure). Of patients with a solitary kidney, 67.1% were diagnosed with MA. For other parenchymal RA, the prevalence of MA was 20-23%. In patients with solitary kidney, uterus didelphys was the most common MA (52.1%). Thirty percent of patients with a solitary kidney were diagnosed with an obstructive MA.In this study, 38% of patients with RA who underwent a pelvic evaluation were found to also have MA. Our study shows the strongest association between MA and solitary kidney, but also emphasizes a significant risk with other RA. Almost 40% of patients with diagnosed MA were found to have an obstruction that required urgent treatment. Delays in diagnosis and treatment of Müllerian obstructions can be detrimental for future reproductive health, due to risk of chronic pain, infertility, infection, and endometriosis. Given the high prevalence of MA in patients with RA, especially those with congenital solitary kidney, routine screening with pelvic ultrasound should be performed around the age of expected menarche.

Introduction Women with spina bifida are sexually active, but most never discuss this topic with providers. Aim To determine what women with spina bifida understand about their sexual health, how they learned about it, what questions they have, and their experiences with their sexuality. Methods For this qualitative study, women with spina bifida ages 16 and older without marked developmental delay were individually interviewed. 25 women with spina bifida participated (mean age 27.1 years, range 16–52). Interviews were independently coded for themes by 3 reviewers, using Grounded Theory, with disagreements resolved by consensus. Main Outcome Measures We identified overlapping themes regarding the women’s perception and experience of their sexuality and sexual health education. Results 17 of the 25 (68%) participants had been or were currently sexually active. 5 themes emerged regarding their understanding of their sexuality and their sexual experiences: (i) being perceived as asexual, (ii) sources for sex education, (iii) need for spina bifida–specific sex education, (iv) impact of spina bifida–specific features on sexual encounters, and (v) perceived relationship between low sexual self-confidence and risk for sexual assault. Clinical Implications Women with spina bifida are sexual beings, but they are perceived as asexual by providers, which prevents them from getting adequate sexual health education and leaves them with misconceptions and unanswered questions, as well as vulnerable to sexual abuse. Strength & Limitations The strengths of this study include the diversity of women interviewed, including their age, severity of disability, and experiences with their sexuality, as well as the ability to reach thematic saturation. The limitation of this study is that most women received treatment at a single Midwestern tertiary referral center in the United States. Conclusion Including sexual health discussions in the usual care of women with spina bifida is critical to enhancing their sexual confidence and experience and preventing sexual abuse.

People with cloacal malformation and 46,XX cloacal exstrophy are at risk of developing Müllerian outflow tract obstruction (OTO). Management of OTO requires expertise of many medical and surgical specialties. The primary presenting symptom associated with OTO is cyclical and later continuous pain and can be initially quelled with hormonal suppression as a temporizing measure to allow for patient maturation. The decision for timing and method of definitive treatment to establish a patent outflow tract that can also be used for penetrative sexual activity and potential fertility is a complicated one and incredibly variable based on patient age alone. To understand the management approach to OTO, we put forth five phases with associated recommendations: (1) caregiver and patient education and evaluation before obstruction; (2) presentation, diagnosis, and symptom temporization; (3) readiness assessment; (4) peri-procedural management; (5) long-term surveillance. This review will emphasize the importance of interdisciplinary team management of the complex shared medical, surgical, and psychological decision making required to successfully guide developing patients with outflow obstruction secondary to cloacal malformations and cloacal exstrophy through adolescence.

To characterize the patient population with cognitive or physical impairments that presents for anticipatory guidance of puberty, evaluate caregiver concerns with respect to puberty, and describe chosen management strategies and outcomes following menarche.Retrospective cohort study SETTING: Academic tertiary care women and children's hospital PARTICIPANTS: Eligible female patients with special needs up to age 26 years presenting for anticipatory guidance from 2009 to 2018 MAIN OUTCOME MEASURES: Primary outcomes included characterization of patients presenting for anticipatory guidance and their reasons for menstrual management. Secondary outcomes were satisfaction with menstrual management and bleeding patterns.A total of 61 patients presented for anticipatory guidance of puberty, on average 13.5 months prior to menarche. Compared to the overall adolescent population with special needs who presented for gynecologic care, patients who had autism spectrum disorder (ASD), were nonverbal, or had attention-deficit/hyperactivity disorder (ADD/ADHD) were more likely to present for a pre-menarchal visit to discuss anticipated pubertal development (P.001, P = .009, and P = .04, respectively). More than half of families described potential behavioral changes as their main concern. The majority of post-menarchal patients (80%) desired hormonal management of menses, including 30% of patients who had placement of a levonorgestrel intrauterine device. In all, 96% of patients were satisfied with their final menstrual bleeding pattern; 50% achieved amenorrhea or light spotting.This study describes the important role of pre-menarchal reproductive counseling for girls with disabilities. Anticipation of puberty causes great anxiety in families and patients, especially those with ASD, ADD/ADHD, and non-verbal status. Providers should consider initiating these conversations early in pubertal development.

Müllerian agenesis, or Mayer-Rokitansky-Küster-Hauser syndrome, occurs in 1 in 4500 to 5000 individuals assigned female sex at birth. Pelvic floor symptoms among individuals with Mayer-Rokitansky-Küster-Hauser syndrome have not been well studied, and it is unknown how vaginal lengthening treatments affect these symptoms.This study aimed to assess urinary, prolapse, and bowel symptoms in individuals with Mayer-Rokitansky-Küster-Hauser syndrome and to determine whether symptoms vary by vaginal lengthening treatment.We conducted a cross-sectional study in 2019 using an online survey distributed by the Beautiful You MRKH Foundation via social media to individuals with Mayer-Rokitansky-Küster-Hauser syndrome. Demographics, age at and timing of diagnosis, information about vaginal lengthening treatment, urinary symptoms (Michigan Incontinence Symptom Index), prolapse symptoms (Pelvic Organ Prolapse Distress Inventory short-form version), and bowel symptoms (Bristol Stool Form Scale) were obtained. The inclusion criteria included self-reported diagnosis of müllerian agenesis and female sex. Respondents with a history of renal transplant or dialysis, completion of85% of the survey, and non-English survey responses were excluded. Descriptive analyses were used to describe the sample population. Logistic regression, Kruskal-Wallis, and Fisher exact tests were used to compare the prevalence of pelvic floor symptoms and vaginal lengthening treatments. Associations between age and genitourinary symptoms were investigated with Spearman correlations.Of 808 respondents, 615 met the inclusion criteria, representing 40 countries. 81% of respondents identified as white. The median age of the participants was 29 years (interquartile range, 24-36), with a median age at diagnosis of 16 years (interquartile range, 15-17). Among the 614 respondents, 331 (54%) had vaginal lengthening treatment, 130 of whom (39%) had undergone surgical vaginal lengthening. Of individuals with Mayer-Rokitansky-Küster-Hauser syndrome, 428 of 614 (70%) reported having had one or more urinary symptoms, and 339 of 428 (79%) reported being bothered by these symptoms. Urinary symptoms included urinary incontinence (210 of 614 [34%]), urinary frequency (245 of 614 [40%]), urinary urgency (248 of 614 [40%]), pain with urination (97 of 614 [16%]), and recurrent urinary tract infections (177 of 614 [29%]). Prolapse symptoms included lower abdominal pressure (248 of 612 [41%]), pelvic heaviness or dullness (177 of 610 [29%]), and vaginal bulge (68 of 609 [11%]). In addition, constipation was reported by 153 of 611 respondents (25%), and anal incontinence was reported by 153 of 608 (25%) respondents. Beside recent urinary incontinence (P=.003) and anal incontinence (P.001), the prevalence of pelvic floor symptoms (P.05) did not differ significantly between those with and without vaginal lengthening. Among those with surgical vaginal lengthening, symptomatic vaginal bulge was highest in individuals who underwent a bowel vaginoplasty procedure.Urinary, prolapse, and bowel symptoms are common among individuals with Mayer-Rokitansky-Küster-Hauser syndrome and should be evaluated in this population. Overall, compared with no vaginal lengthening treatment, having vaginal lengthening treatment is not associated with substantial differences in the prevalence of pelvic floor symptoms, with the exception of recent urinary incontinence and anal incontinence. Our data suggested that bowel vaginoplasty may be associated with greater symptoms of vaginal bulge. More robust studies are needed to determine the impact of various vaginal lengthening treatments on pelvic floor symptoms.

To characterize the population of adolescents and young women with special needs presenting for gynecologic care, describe usage patterns of hormonal suppression methods, and evaluate outcomes of menstrual management.This retrospective cohort study included females with special needs up to age 26 years presenting for menstrual management from 2009 to 2018. Demographic, social, and medical histories were collected to investigate effects on bleeding pattern and satisfaction with menstrual management.Of 262 patients who presented for menstrual complaints, final methods of treatment included combined hormonal contraceptives (30.9%), oral progestins (19.8%), depot medroxyprogesterone acetate (8.0%), etonogestrel implant (1.9%), and levonorgestrel intrauterine device (16.8%). Eighty-five percent of patients were satisfied with their final bleeding pattern. Patients with amenorrhea or light regular periods were more likely to be satisfied than patients with heavy or irregular bleeding (p.001). Satisfied patients tried an average of 1.4 methods, compared to 1.8 methods tried by the unsatisfied group (p = .042). By the end of the study, 26.0% were amenorrheic and 12.8% had only light spotting. Satisfaction rates were similar with each method, including 88.4% with use of combined hormonal contraceptives, 82.5% with oral progestins, 93.3% with depot medroxyprogesterone acetate, 100% with etonogestrel implant, and 83.9% with levonorgestrel intrauterine device.Amenorrhea or light regular bleeding led to satisfaction in most patients. No hormonal method was superior. When counseling families and patients who present for menstrual suppression, emphasis should be placed on goals of treatment and expectations for outcomes, as light regular periods may be as acceptable as amenorrhea.

Background Ovarian torsion can occur in Van Wyk Grumbach syndrome, a disorder characterized by severe primary hypothyroidism and ovarian enlargement. To date, all documented cases of torsion in this setting describe oophorectomy, which has significant hormonal and fertility implications. Case A 9-year-old pubertal girl presented to the emergency room with abdominal pain. Magnetic resonance imaging demonstrated bilateral, multi-cystic ovaries. Operative laparoscopy confirmed unilateral adnexal torsion, and detorsion without oophorectomy was accomplished. Postoperative laboratory tests revealed severe primary hypothyroidism. Ovarian size was reduced with hormone replacement therapy. Summary and Conclusion This case demonstrates that prompt interdisciplinary intervention and awareness of severe hypothyroidism as a cause of ovarian torsion related to enlarged, multi-cystic ovaries may reduce the rate of oophorectomy, allowing preservation of pediatric patients’ future fertility, and reducing morbidity postoperatively through prompt, long-term thyroid supplementation.

Objective To provide urologists with a practical guide for how to provide sexual health counseling to girls and women with spina bifida. Methods The recommendations and research of several sources were synthesized to create this guidance, including clinical guidance from the Spina Bifida Association and American College of Obstetricians, the current literature on the sexual health of girls and women with spina bifida, and the multidisciplinary experience of the authors. Results Sexual health education should be viewed by urologists as a continuous discussion, starting in early childhood and gradually building through adolescence. Developing a plan for when and how to bring it up, utilizing parents as educational partners, identifying who will provide the detailed one-on-one counseling if not the primary urologist, establishing a referral network for specialized care (eg, adolescent gynecologist, physical therapist, or sex therapist), becoming familiar with how spina bifida impacts sexual health, and being prepared for challenges are key to providing these girls and women with competent sexual health education. Urologists should also screen for abuse at each visit and be familiar with reporting and resources for when abuse is identified. Conclusion This guidance can serve to direct urologists in providing competent sexual health education to girls and women with spina bifida. This will ensure these girls and women receive the basic education they need, and that they can be referred to appropriate sexual health experts as indicated.

Background: Barriers in the built environment, enduring stereotypes and biases, and limited disability competency of health care providers compromise access to and quality of reproductive health care for women with physical disabilities. One way to improve our understanding of critical factors that drive reproductive health inequity and its impact on access to care is to use patient-reported outcome measures (PROMs) that capture relevant and meaningful information about experience. In this study, we developed a conceptual framework as the foundation for relevant and clinically meaningful patient-reported outcome measures targeting the interface of disability and reproductive health. Materials and Methods: We conducted semistructured focus groups and interviews to assess women's experiences around their reproductive health and contextual factors related to disability. We used deductive and inductive qualitative coding approaches to develop the conceptual framework. Results: Eighty-one women between the ages of 16 and 50 with a self-reported physical disability, defined by an impairment of mobility, participated in 13 focus groups (N = 64) and 17 individual interviews. Five major themes characterized the conceptual framework that emerged—knowledge about reproductive health, communication about reproductive health, relationships, the reproductive health care environment, and self-advocacy/identity—all of which had some relationship with five major reproductive health issues—pregnancy and labor/delivery, periods and menstrual management, contraception, sexuality and sexual functioning, and pelvic examinations. Conclusions: This conceptual framework will serve as a foundation for PROM and guide intervention development to reduce reproductive health inequity and improve reproductive health outcomes of women with physical disabilities.

Study Objective To assess the treatment patterns and efficacy of hormonal (HM) and non-hormonal (NHM) management of heavy menstrual bleeding (HMB) in young women with inherited platelet function disorders (IPFD). Design /Setting/Participants: A retrospective chart review was performed of outpatient treatment of HMB in females age 9-25 years old diagnosed with IPFDs referred to gynecology and/or hematology at a tertiary care hospital between 2006 and 2018. Intervention Study sample was identified using billing codes for IPFD. HM and NHM treatments and outcomes over a one- to two-year period were collected. Initial treatment (IT) was defined as the first treatment prescribed after referral. Descriptive statistics, Pearson Chi Square, and T-tests were used for analysis. Main Outcome Measure Treatment failure defined as a change in treatment method due to continued bleeding. Results Thirty-four girls met inclusion criteria. Following their initial visit, 56% were treated with HM, 35% with NHM, 6% with a combination, and 3% (n=1) were untreated. Fifty-six percent failed IT and subsequently required a mean of two additional treatments during follow-up. Of the 34 included, six remained uncontrolled despite numerous treatment changes and two due to non-compliance. Once control was achieved (n=26), 27% of patients were on combined oral contraceptives (n=7) and 23% on desmopressin acetate (n=6). Conclusions HMB in girls with IPFD can be difficult to control despite ongoing follow-up and treatment changes. While the most effective treatment for HMB in young women with IPFDs was not identified, these findings will help providers and patients with setting expectations. Prospective studies are needed to develop recommendations on best practices.

Rationale and Objectives Women with pelvic pain or abnormal uterine bleeding may undergo diagnostic imaging. This study evaluates patient experience in transvaginal ultrasound (TVUS) and magnetic resonance imaging (MRI) and explores correlations between preference and symptom severity. Materials and Methods Institutional review board approval was obtained for this Health Insurance Portability and Accountability Act-compliant prospective study. Fifty premenopausal women with pelvic symptoms evaluated by recent TVUS and MRI and without history of gynecologic cancer or hysterectomy were included. A phone questionnaire used validated survey instruments including Uterine Fibroid Symptoms Quality of Life index, Testing Morbidities Index, and Wait Trade Off for TVUS and MRI examinations. Results Using Wait Trade Off, patients preferred TVUS over MRI (3.58 vs 2.80 weeks, 95% confidence interval [CI] −1.63, 0.12; P = .08). Summary test utility of Testing Morbidities Index for MRI was worse than for TVUS (81.64 vs 87.42, 95%CI 0.41, 11.15; P = .03). Patients reported greater embarrassment during TVUS than during MRI (P Conclusion Women with pelvic symptoms had a slight but significant preference for TVUS over MRI. Identifying specific distressing aspects of each test and patient factors contributing to negative perceptions can direct improvement in both test environment and patient preparation. Improved patient experience may increase imaging value.

To assess pediatric resident training in diagnosing and managing prepubertal gynecologic conditions.Voluntary 32-question survey e-mailed to participants.E-mail contact through the American Academy of Pediatrics listserv.Seven thousand seventy-five US pediatrics and combined internal medicine-pediatric residents.Descriptive analysis including χResidents' training experiences and comfort, confidence, and knowledge in evaluating pediatric gynecologic concerns.In the 866 of 7075 (12%) completed surveys, a greater proportion of residents reported they were "very" or "extremely" comfortable talking to parents about general pediatric topics compared with gynecologic topics (88.5% vs 30.4%; P .001). Similarly, they reported being "very" or "extremely" confident diagnosing general pediatric conditions compared with prepubertal gynecologic conditions (87.6% vs 32.8%; P .001). These differences were also observed according to residency year (comfort: first year, 10.2% vs third/fourth year, 39.9%; P .001; confidence: first year, 22.5% vs third/fourth year, 37.6%; P .001). Residents learned about vulvovaginal concerns from attendings in clinic (79.8%), residency-specific didactics (34.7%), and conferences, meetings, and workshops (24.1%). Confidence examining, diagnosing, and treating vulvovaginitis was associated with participation in any learning activity and exposure to more than 5 patients with this concern. Additional education or training in prepubertal vulvovaginal conditions was requested by 97% of residents.Our findings suggest that pediatric residents are lacking in comfort, confidence, and knowledge of prepubertal vulvovaginal conditions, especially compared with general pediatric topics. Although this improves during training, it remains low, and more education is indicated and desired by residents.

The incidence of thyroid disease in adolescents with heavy menstrual bleeding is unknown. A retrospective cross-sectional study of 427 adolescents presenting with heavy menstrual bleeding found 0.23% (95% CI 0%-0.7%) had thyroid disease, lower than that expected in the general population. Thyroid testing should only be considered when other symptomatology is present.

Background We present an adolescent with multiple vulvar angiokeratomas within a background of lichen sclerosus. Case A 13-year-old girl presented with vulvar pruritus and wart-like vulvar lesions. Four lesions were resected because of discomfort and uncertainty of the diagnosis. Pathology revealed angiokeratomas with chronic inflammation suggestive of lichen sclerosus. Postoperatively, pruritus continued in the largest excised lesion, which was associated with lichen sclerosus, and symptoms were treated successfully with topical steroids. Summary and Conclusion Vulvar angiokeratomas are asymptomatic red papular lesions and are rare in the female adolescent population. In this case, the pathology revealed the rare co-occurrence of angiokeratomas and lichen sclerosus. Biopsies of vulvar vascular lesions in symptomatic adolescents are recommended. Vulvar angiokeratomas might manifest rare genetic disease in otherwise asymptomatic female patients and warrant further follow-up.

Background Despite the well-established embryological relationship in the development of renal and Mullerian structures, no guidelines exist regarding screening for Mullerian anomalies (MA) in the setting of a renal anomaly (RA). Delayed diagnosis of MA can have significant reproductive consequences. The primary outcome of this study is to evaluate the prevalence and types of MA associated with various RA. Methods This is a retrospective cohort study of females age 12-35 years with a diagnosis of RA, identified by ICD-10 codes, who were followed for care between 2013-2020. Data were collected on demographics, medical history, imaging studies, and clinical presentation. MA were characterized using the American Society for Reproductive Medicine classification system. Descriptive statistics were used to summarize the data. This study was IRB approved. Results Of 490 patients identified, 365 met inclusion criteria and 264 had pelvic imaging performed. In patients with ARM, 77% had MA. Excluding patients with ARM, MA were found in 67% of patients with unilateral renal agenesis, 25% with horseshoe or fused kidneys, 25% with pelvic or ectopic kidneys, and 23% with renal hypoplasia or dysplasia. Overall 129/264 (49%) were diagnosed with MA, including 18% Mullerian agenesis, 18% unicornuate uterus, 47% uterine didelphys, 12% bicornuate uterus, and 6% septate uterus. Fifty-two patients had Mullerian obstruction at the time of diagnosis, including 36 with obstructed hemivagina and ipsilateral renal agenesis. Forty-eight underwent urgent surgery, including 2 with pyocolpos, and 4 were treated with initial hormonal suppression. The average age of RA diagnosis was 4 years, compared to 18 years for MA (range 0-36 years for both). In 3 patients, MA was diagnosed prior to RA, and in 21 cases, both anomaly diagnoses were made simultaneously. The most common imaging modality performed was pelvic ultrasound (58%), often followed by magnetic resonance imaging (32%). Imaging was most commonly performed for abdominal pain or dysmenorrhea. In 15% of cases, pelvic ultrasounds were performed for obstetric reasons. Only 5% of patients had routine screening to evaluate pelvic anatomy; a quarter of whom were pre-pubertal. Conclusions Almost half of patients with congenital RA are eventually diagnosed with MA, of whom 40% have an obstructive anomaly. Screening for MA in patients with RA near the time of menarche can prevent delays in diagnosis that could have significant implications on reproductive health and quality of life.

Background There are many reasons for choosing the etonogestrel subdermal implant (hereafter “implant”). Limited data exists on outcomes of implant use in adolescents, and specifically, on the management of abnormal uterine bleeding (AUB). The goals of this study include characterize the purpose of implant placement in adolescents, describe rates and management of side effects, and identify reasons for early discontinuation of the implant. Methods This is a retrospective cohort study of females less than 18 years who had the implant placed from 2013-2018, identified using Current Procedural Terminology codes. Data were collected on contraceptive history, bleeding profiles, side effects, and outcomes. Descriptive statistics, analysis of variance tests, Fisher's exact test, and chi-square test data are reported. This study was IRB approved. Results A total of 272 patients were included in this study. The average age of menarche was 12 years and average age at insertion was 16 years. The majority of patients (90%) were nulliparous, 65% had been sexually active prior to implant placement, and 84% desired the implant for contraception. Of 241 patients who had at least one follow up visit, 187 reported AUB. On average, adolescents reported AUB starting 2.7 months after placement, which they managed expectantly for 10.3 months prior to desiring removal or trial of medication to resolve AUB. Of the 187 patients who reported AUB, 59% had no documented discussion of options for management and 24% had only one option offered. Trials of different medications for the treatment of AUB had varying effects on bleeding patterns (Table 1). Average time of removal for those with AUB was 22.1 months after implant placement, similar to 21.5 months for those without AUB. Most patients (76%) had the implant in place for at least 12 months. Adolescents with frequent or prolonged bleeding were more likely to have the implant removed prior to 12 months than those with other bleeding patterns (p=0.001). Patients complaining of weight gain and mood issues were also more likely to have early removal (p Conclusions Adolescents mostly chose the implant for contraceptive efficacy and, although AUB was common, three-quarters chose to continue for at least 12 months. Prolonged or heavy bleeding, mood issues, and perceived weight gain were associated with earlier removal. Several options exist to treat implant-related AUB, and improved use of these medications may make bleeding patterns more tolerable. Additional studies are needed to optimize treatment of AUB and address other bothersome side effects.

Background Vulvar lichen sclerosus is a benign dermatologic condition marked by inflammation and epithelial thinning. It is thought to mostly occur in low estrogen states, though a few studies have shown that symptoms may persist following puberty. The purpose of this study is to characterize a cohort of pediatric and adolescent females with lichen sclerosus and compare age of symptom onset, documented symptoms and duration, and exam findings. Methods This is an IRB exempt retrospective cohort study of females aged 0-21 years seen between 2000 and 2020 with the diagnosis of lichen sclerosus by ICD-9 or 10 code. Study variables included age at symptom onset, age at diagnosis, presenting symptoms, exam findings, and presence of symptoms before and after menarche. Groups were compared using the chi square test of independence and Fisher's exact test. Data were analyzed using SPSS Version 27. Results One hundred patients met inclusion criteria. Forty patients noted presence of symptoms following menarche; 26 (65%) reported first symptom onset after age 13, while 14 (35%) had symptoms that started in childhood and persisted after menarche. Eleven patients with childhood onset of symptoms had no documented report of symptoms following menarche. Of the remaining 49 patients, 25 are still under age 13 and 24 were lost to follow-up. After excluding the 7 patients with reported childhood onset of symptoms that were not seen until adolescence, the patients with onset of symptoms at age 13 or older (n=26) were less likely to present with vaginal bleeding (3.8% vs 25.4%, p=0.02), dysuria (15.4% vs 32.8%, p=0.09), and constipation or painful defecation (0% vs 14.9%, p=0.05) than the younger cohort (n=67). Vulvar itching (61.5% vs 71.6%) and pain (38.5% vs 44.8%) were similar in both groups. Seven patients (26.9%), ranging in age from 17-21 years, presented with dyspareunia. On initial physical exam, the patients with symptom onset at age 13 or older were more likely to have clitoral adhesions (19.2% vs 3%, p=0.02), loss of labia minora (38.5% vs 3.0%, p Conclusions This study suggests that lichen sclerosus can present or persist after menarche in at least 40% of patients. It can cause architectural vulvar changes that interfere with sexual function, which may be the presenting complaint. It is important for providers to consider lichen sclerosus as a new diagnosis in both pre and postmenarchal patients as well as monitor for progression of disease throughout the pubertal transition and adolescence.

Müllerian anomalies include a spectrum of anatomic variants of the genital tract arising from abnormal embryologic development, ranging from incomplete resorption of uterine septa to complete vaginal agenesis. Nonobstructive anomalies are often recognized later than obstructive anomalies, because women do not present with pain. However, nonobstructive anomalies frequently have a negative impact on sexual and reproductive health, and careful evaluation and management of these disorders is warranted. This review focuses on the surgical management of nonobstructive Müllerian anomalies.

Objective To investigate the provision of care for pediatric and adolescent patients by gynecologic oncologists. Methods The present prospective cross-sectional study enrolled attending physicians and fellows specializing in gynecologic oncology from the USA during December 2012 and January 2013. A 33-question survey collecting demographic data and details of participant opinions on existing practices was distributed to potential respondents. Survey responses were aggregated and descriptive analyses were performed. Results The survey was distributed to 1252 physicians and 178 (14.2%) surveys were returned; 105 (59.0%) participants thought that the care of patients aged younger than 21 years should be included in their practice. Only 7 (3.9%) participants had received formal training in caring for pediatric patients and young adults; however, 85 (47.8%) felt this should be incorporated into formal fellowship training. Multidisciplinary teams were reported to be the best method for caring pediatric patients by 160 (88.9%) participants but only 42 (23.6%) participants reported that multidisciplinary teams were involved in pediatric gynecologic oncology care at their institutions. Conclusion Gynecologic oncologists wanted to be involved in pediatric care and open dialogue between specialists could help in the provision of optimal, longitudinal care to these patients. Further, the incorporation of formal pediatric patient-focused training into gynecologic oncology fellowship programs should be considered. This article is protected by copyright. All rights reserved.

Investigation of disorders of sex development (DSD) has resulted in the discovery of multiple sex-determining genes. MAP3K1 encodes a signal transduction regulator in the sex determination pathway and is emerging as one of the more common genes responsible for 46,XY DSD presenting as complete or partial gonadal dysgenesis. Clinical assessment, endocrine evaluation, and genetic analysis were performed in six individuals from four unrelated families with 46,XY DSD. All six individuals were found to have likely pathogenic MAP3K1 variants. Three of these individuals presented with complete gonadal dysgenesis, characterized by bilateral streak gonads with typical internal and external female genitalia, while the other three presented with partial gonadal dysgenesis, characterized by incomplete testicular development, resulting in clitoral hypertrophy with otherwise typical female external genitalia. Testing for MAP3K1 variants should be considered in patients with 46,XY complete or partial gonadal dysgenesis, particularly in families with multiple members affected with 46,XY DSD. Identification of a MAP3K1 variant should prompt an evaluation for DSD in female siblings of the proband.

To characterize pediatricians' knowledge, attitudes, and self-efficacy around contraception.Cross-sectional survey.United States.National sample of pediatricians.Assessment of behaviors of providing contraception.Reproductive health practice score.Two hundred twenty-three usable surveys were received, from 163 contraceptive prescribers and 60 nonprescribers. The mean reproductive health practice score was 43.1 (SD, 8.2; total possible score, 84). Prescribers differed in their mean reproductive health score (46.0; SD, 7.0) from nonprescribers (34.0; SD, 4.5; P .001). Prescribers vs nonprescribers differed in their attitude and efficacy in providing contraception. More prescribers believed it was their responsibility to ask about patients' need for birth control, were confident in their ability to prescribe contraception options, and provided contraception to minors despite parental disapproval. Neither group was confident in their ability to place intrauterine devices or believed that the literature supports intrauterine device placement in adolescents. Only efficacy was related to prescribing contraception in a multivariate regression analysis (odds ratio, 1.7; P .001).In this study, we showed that most pediatricians are contraception prescribers but the overall reproductive health score was low for prescribers and nonprescribers. The odds of prescribing contraception increased with higher self-efficacy scores rather than knowledge alone. Many prescribers and nonprescribers would not prescribe birth control if parents disapproved and do not believe it is their responsibility to assess patients' need for birth control. In addition very few pediatricians have training in long-acting reversible contraception, despite being the recommended method for adolescents.

Healthy sexual development is important for adolescents with and without disabilities, yet the topic of sexuality is often ignored in the disabled population. Adolescents with mild or moderate degrees of disability have rates of sexual activity and reproductive health needs comparable to their typically developing peers. Their need for support, risk reduction, and education in sexual health may exceed that of their peers. The medical provider may support healthy sexual development through education, anticipatory guidance, menstrual and contraceptive management, and by expanding the notion of sexuality to include a broader conceptualization of sexual behavior and expression.

Study Objective To explore trends over time in the utilization of the Emergency Department (ED) for adolescents with abnormal uterine bleeding (AUB). Design Retrospective cross-sectional study. Setting Nationwide Emergency Department Sample database (Healthcare Cost and Utilization Project, Agency for Healthcare Research and Quality, Rockville, MD). Participants Subset of adolescents aged 10-19 years from overall cohort of women aged 10-49 years. Interventions All ED visits from 2010-2014 for which International Classification of Diseases, ninth revision codes for AUB were listed as the principal diagnosis were compared. Health insurance coverage, demographic factors, hospital characteristics, comorbid anemia, and comorbid bleeding disorders were explored. Main Outcome Measures Number of visits, ED disposition, blood transfusions, and billing for pelvic ultrasound. Results There were an estimated 262,939 ED visits nationally for women aged 10-49 years for AUB in 2014. Of these, 12.7% (33,511/262,939) were from adolescents aged 10-19 years. The number of adolescent visits with an AUB diagnosis declined by 11% from 2010-2014 (37,642 vs 33,511; P = .026), while visits by patients aged 20-49 did not change significantly (215,309 vs 229,428; P = .19). The number of adolescent visits for which Medicaid was the primary payer increased slightly, from 17,283 to 18,785, from 2010 to 2014 (P = .152), while the number using self-pay decreased from 8769 (23.4%) in 2010 to 5146 (15.4%) in 2014 (P Conclusion ED visits among adolescents with AUB decreased from 2010 to 2014. There was a sharp reduction in the number of visits funded by self-pay and a slight increase in visits funded by Medicaid.

Background The composition of the human vaginal microbiota is related to many aspects of health from infection susceptibility to preterm birth. Factors that influence human vaginal microbiota composition, including its source, are not well understood. Objective The goal of this study was to determine if vaginal microbiota transmission from mother to daughter at birth influences the human vaginal microbiota composition in adolescence. Study Design Weekly vaginal swab samples from 13 adolescents and their mothers were collected for up to 4 weeks. After DNA was isolated from the swabs, the V4 region of the bacterial 16S rRNA genes were amplified, sequenced and analyzed. We calculated distances between the bacterial communities in different samples to investigate the relationship between the vaginal microbiota of the mother/daughter pairs and the daughter’s birth mode. We also cultivated Lactobacillus crispatus from the mother and daughter of 1 pair. To investigate the possibility of direct transmission and persistence of one member of the vaginal microbiota, we isolated DNA from the L. crispatus isolates and compared their genomes with each other and other publicly available L. crispatus genome sequences. Results The vaginal microbiotas of mother/daughter pairs were more similar to each other if the daughter was born by vaginal delivery rather than by C-section. Additionally, genome sequences from an important member of the vaginal microbiota, L. crispatus, isolated from one mother/daughter pair in which the daughter was born by vaginal delivery, were highly similar. Conclusion Both community-level analysis and isolate genome sequence analysis are consistent with birth-mode dependent transmission and persistence of at least some members of the vaginal microbiota.