Postoperative Bleeding Two Weeks After Longitudinal Vaginal Septum Resection with the LigaSure Device: A Case Report.

BACKGROUND: A longitudinal vaginal septum (LVS) is a rare congenital anomaly often identified during adolescence. Surgical removal is a mainstay in treatment of symptomatic cases; however, there is variation in the techniques used. Little is known about the risk for postoperative complications associated with novel methods. CASES: We present the cases of 2 adolescent females, ages 15 and 22, diagnosed with an LVS who elected to undergo surgical removal. A LigaSure device was used for resection, and both individuals experienced significant postoperative bleeding almost 2 weeks following resection. SUMMARY AND CONCLUSIONS: This report outlines two occurrences of postoperative bleeding after LVS resection, which may suggest inadequate surgical site hemostasis with use of the LigaSure apparatus. Further research on outcomes related to this technique is needed.

An Interdisciplinary Approach to Müllerian Outflow Tract Obstruction Associated with Cloacal Malformation and Cloacal Exstrophy.

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.

Lichen Sclerosus throughout Childhood and Adolescence: Not Only a Premenarchal Disease.

STUDY OBJECTIVE: 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.

Should we screen for Müllerian anomalies following diagnosis of a congenital renal anomaly?

INTRODUCTION: 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. OBJECTIVE: To investigate the prevalence of coexisting MA in patients with congenital RA. STUDY DESIGN: 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. RESULTS: 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. CONCLUSIONS: 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.

Non-Obstetric Traumatic Vulvar Hematomas in Premenarchal and Postmenarchal Girls.

BACKGROUND: Traumatic non-obstetrical hematomas of the vulva are rare, and most reports only involve adult patients. There are no data on presentation, management, and outcomes from either conservative or surgical management in pediatric and adolescent patients. The objective of this project was to compare the etiology, treatment, and outcomes of traumatic vulvar hematomas occurring in premenarchal and postmenarchal young women. METHODS: A retrospective chart review was performed on females aged 0-24 years seen at a tertiary care academic center using ICD 9 and 10 codes for traumatic vulvar hematoma from 2006-2019. Data describing their clinical presentation and course were collected. IRB approval was obtained. RESULTS: Twenty patients, aged 3-23 years (median age of 13.5 years) were identified. All 8 premenarchal patients presented with a straddle injury, whereas only 50% of postmenarchal patients were found to have a straddle injury. Other etiologies among postmenarchal patients included consensual sexual intercourse and recent vulvar surgery. Hematoma diameter ranged from 1-3 cm in premenarchal patients and 0.4-7 cm in postmenarchal patients. Associated perineal lacerations were reported in 50% of the premenarchal girls and 8% of postmenarchal young women. Of the 8 premenarchal patients, 5 were managed conservatively, and 3 were taken to the operating room for repair of perineal lacerations; 1 patient also underwent evacuation of a 3-cm hematoma. Of the 12 postmenarchal patients, 5 had surgical intervention, 2 for pain secondary to large 7-cm hematomas and 3 for suspected vulvar abscesses, which were identified as hematomas after drainage. One patient in each group required a Foley catheter for comfort. Two postmenarchal patients required a second surgery for further wound management. One premenarchal patient with surgical treatment required a follow-up exam under anesthesia. Four patients were admitted for pain and postoperative observation, 1 of whom was premenarchal. Eleven patients were seen for follow-up, and 10 were doing well. One postmenarchal patient in the conservative management group returned to the Emergency Department with continued pain 10 days later. CONCLUSIONS: In this study that examined traumatic vulvar hematomas in premenarchal and postmenarchal young women, the only mechanism of injury in premenarchal girls was straddle injury, and surgical intervention was usually needed only for repair of perineal lacerations, not a primary hematoma. In the postmenarchal patients, surgical intervention was undertaken for larger hematomas and suspected vulvar abscesses. Our study suggests that most hematomas up to 3 cm in premenarchal patients and up to 6 cm in postmenarchal patients can be managed conservatively.

A multidisciplinary approach and consensus statement to establish standards of care for Angelman syndrome.

BACKGROUND: Angelman syndrome (AS) is a rare neurogenetic disorder present in approximately 1/12,000 individuals and characterized by developmental delay, cognitive impairment, motor dysfunction, seizures, gastrointestinal concerns, and abnormal electroencephalographic background. AS is caused by absent expression of the paternally imprinted gene UBE3A in the central nervous system. Disparities in the management of AS are a major problem in preparing for precision therapies and occur even in patients with access to experts and recognized clinics. AS patients receive care based on collective provider experience due to limited evidence-based literature. We present a consensus statement and comprehensive literature review that proposes a standard of care practices for the management of AS at a critical time when therapeutics to alter the natural history of the disease are on the horizon. METHODS: We compiled the key recognized clinical features of AS based on consensus from a team of specialists managing patients with AS. Working groups were established to address each focus area with committees comprised of providers who manage >5 individuals. Committees developed management guidelines for their area of expertise. These were compiled into a final document to provide a framework for standardizing management. Evidence from the medical literature was also comprehensively reviewed. RESULTS: Areas covered by working groups in the consensus document include genetics, developmental medicine, psychology, general health concerns, neurology (including movement disorders), sleep, psychiatry, orthopedics, ophthalmology, communication, early intervention and therapies, and caregiver health. Working groups created frameworks, including flowcharts and tables, to help with quick access for providers. Data from the literature were incorporated to ensure providers had review of experiential versus evidence-based care guidelines. CONCLUSION: Standards of care in the management of AS are keys to ensure optimal care at a critical time when new disease-modifying therapies are emerging. This document is a framework for providers of all familiarity levels.

Distal Vaginal Atresia with Spontaneous Perforation: A Case Report.

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 that 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 intraoperative ultrasound could be beneficial.

Preparing for Puberty in Girls With Special Needs: A Cohort Study of Caregiver Concerns and Patient Outcomes.

STUDY OBJECTIVE: 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. DESIGN: 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. RESULTS: 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. CONCLUSIONS: 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.

Satisfaction With Hormonal Treatment for Menstrual Suppression in Adolescents and Young Women With Disabilities.

PURPOSE: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Considerations in Adolescent Use of the Etonogestrel Subdermal Implant: A Cohort Study.

Objectives: To describe bleeding patterns and other side effects in adolescent implant users and characterize their impact on early discontinuation of the implant. Study Design: This is a retrospective cohort study of female patients under 18 years who had an implant placed from 2013 to 2018. Data were collected on demographics, medical history, and side effects. Results: Of 212 adolescents, the average age at insertion was 16 years and 84% desired placement for contraception. Common side effects included AUB (80%), mood changes (10%), and perceived weight gain (9%). Most (76%) used the implant for at least 12 months. Average time to removal was 22.1 months (SD 13.0 months) and this did not depend on presence of side effects. Twenty-seven percent of teens were able to achieve amenorrhea. Adolescents with frequent or prolonged bleeding were more likely to have implant removal prior to 12 months than those with other bleeding patterns (p = 0.003). Early removal was also more common in girls reporting weight or mood issues than those who did not (p < 0.001 and p = 0.045, respectively). BMI increased in 64% of adolescents. Average percentage change in BMI was 3.2% (0.87 kg/m2). There was no difference in baseline use of any mood-modulating medications in patients who did and did not complain of mood side effects following implant placement (p = 0.801). Conclusion: Characterization of bleeding patterns following implant placement in adolescents have not previously been reported. Prolonged or heavy bleeding, mood issues, and perceived weight gain were associated with earlier removal of the implant. A relatively small number had early removal of the implant due to weight or mood complaints. Therefore, a history of obesity, depression, or other mood disorders should not be a deterrent to implant placement.

Addressing the Needs of Transgender Patients: How Gynecologists Can Partner in Their Care.

IMPORTANCE: With the increase in patients who identify as transgender, it is crucial that gynecologists are culturally and clinically competent in understanding their unique needs. OBJECTIVE: The aim of this study was to identify the key gynecologic issues facing transgender patients and develop an overarching framework of tools needed to address these issues. EVIDENCE ACQUISITION: A review of the existing literature was undertaken to address the key clinical aspects of care. RESULTS: Various aspects of the gynecologic care of transgender patients, including health maintenance and cancer screening examinations, hormone replacement therapy, hysterectomy and salpingo-oophorectomy, and referral and collaboration with the patient's care team, are outlined. CONCLUSIONS AND RELEVANCE: Transgender patients are more likely to be engaged and seek care if their identity and their needs are understood. While many aspects of transgender health care follow standard practices, there are significant and important differences, including gender-affirming therapies. This article aims to give gynecologists the necessary tools to partner in the care of transgender patients.

The Effect of Menstrual Issues on Young Women with Angelman Syndrome.

STUDY OBJECTIVE: To characterize menstrual health issues and their effect in young women with Angelman syndrome (AS). Our secondary objective was to compare them with young women with autism spectrum disorders (ASDs). DESIGN: Cross-sectional convenience sample survey. SETTING: An institutional review board-approved Web-based survey of young female members of the Angelman Syndrome Foundation. PARTICIPANTS: Caregivers of young women with AS, aged 12-25 years. INTERVENTIONS: None. MAIN OUTCOME MEASURES: Symptom characterization and effect of menses on young women with AS. RESULTS: Menstrual and premenstrual symptoms were common among young women with AS, but infrequently caused problems at home or school. Less than half of the subjects used hormones to control their flow. Of those who used hormones, 75% used them continuously. Caregivers were satisfied with their method to control periods. Girls with seizures were more likely to use hormonal methods to control menses than those without seizures. Menstrual-associated morbidity in young women with ASD and AS was fairly similar, but with greater morbidity in the ASD group than in the AS group. However, girls with AS had more problems with menstrual hygiene with almost all of them requiring full assistance for managing hygiene. CONCLUSION: In this group of young women with AS, who have moderate to severe neurodevelopmental disabilities and cannot manage their own hygiene, menstruation is not associated with significant problems. Menstrual management by hormones is used by less than half. When hormonal therapy is used, it is most commonly used continuously to suppress menses.

Occurrence of Gonadoblastoma in Patients with 45,X/46,XY Mosaicism.

STUDY OBJECTIVE: To estimate the overall frequency of gonadal tumors in patients with 45,X/46,XY mosaicism who underwent gonadectomy and to determine whether the degree of external masculinization or the location of gonads were associated with the presence of gonadal tumor. DESIGN: Retrospective study of patients with karyotype of 45,X/46,XY or variant who received care at the study institution between 1995 and 2012. SETTING: University of Michigan Health System (Ann Arbor, Michigan), a tertiary care academic center. PARTICIPANTS: Sixteen patients with karyotype of 45,X/46,XY who underwent gonadectomy. INTERVENTIONS: None. MAIN OUTCOME MEASURE: Presence of pathology-confirmed gonadal tumor. RESULTS: In patients who underwent bilateral gonadectomy, gonadoblastomas were detected in 36.4% (4 of 11), and all were identified in patients with normal female external genitalia (4 of 8 [50.0%]). Abdominal gonads were associated with a nonsignificant increase in rate of gonadal tumor compared with inguinal or scrotal gonads. No malignant tumors were identified. CONCLUSION: The overall rate of gonadoblastoma was higher than previously reported. The high rate of gonadoblastoma in patients with female external genitalia and the lack of gonadal function support continuing the standard of care of practice of prophylactic gonadectomy in this patient population.

Lichen sclerosus in children and adolescents.

PURPOSE OF REVIEW: This review of lichen sclerosus in children and adolescents will discuss the disease and highlight the most recent literature. RECENT FINDINGS: Lichen sclerosus continues to be poorly recognized and misdiagnosed by clinicians. There is growing support for an autoimmune component in the cause of this disease. Recent studies confirm that lichen sclerosus does not resolve after puberty but usually improves. In small case series, topical calcineurin inhibitors are effective as second-line therapy. SUMMARY: Lichen sclerosus is an uncommon, poorly recognized disease in girls and adolescents and is likely to have a chronic course requiring long-term follow-up and treatment. There needs to be increased awareness among providers of this disease as a cause of vulvar itching. Because of the lack of knowledge of the natural course and treatment outcomes, prospective, multicenter studies are needed.