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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.

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.

Study Objective We investigated risk factors and common causes of tubo-ovarian abscess (TOA) in non−sexually active females in order to aid in earlier diagnosis, treatment, and improved outcomes. Design This is a retrospective observational case series of all non−sexually active females younger than age 25 years who were diagnosed with TOA. Review of the existing literature was also performed. Setting Academic tertiary care children's hospital. Participants Ten patients meeting study inclusion criteria were identified for the study, and 33 other patients were identified in the literature. Results Average age at time of diagnosis was 14 years. Average body mass index was 24 kg/m2. Most presented with abdominal pain, often associated with fevers, nausea, vomiting, and diarrhea. Seven of 10 patients were treated surgically with pelvic washout (4 primarily and 3 after failing empiric antibiotic therapy). Most frequently, anaerobic gut flora were isolated on culture. All patients received broad-spectrum intravenous antibiotics, and were then discharged on a course of doxycycline and metronidazole or clindamycin. Three patients required additional admissions and multiple rounds of antibiotics due to persistent symptoms. The average length of stay was 3 days for patients treated with antibiotics only and 6 days for patients requiring surgical intervention. Six patients had complete resolution of symptoms and improvement on ultrasound within 2-4 weeks. The remainder were lost to follow-up. Conclusion These cases, in conjunction with previous case reports, emphasize the importance of considering TOA in patients with concerning imaging or examination findings despite lack of sexual activity. Given the large proportion of cases attributable to anaerobic gut flora, treatment with antibiotics with adequate anaerobic coverage is recommended. Surgical drainage is not always necessary, but is often needed for diagnostic purposes or in patients not clinically improving with conservative measures.

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.

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.

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.