Trends in dedicated care for females with bleeding disorders within U.S. hemophilia treatment centers.

Graphical representation of increasing percentage of female patients seen at HTCs, percentage of females by diagnosis, number of clinics in existence, and absolute number of female patients seen over a 10-year period (top left then clockwise).

Caregiver goals and satisfaction for menstrual suppression in adolescent females with developmental disabilities: A prospective cohort study.

BACKGROUND: Adolescents with developmental disabilities and their caregivers often seek menstrual management. Caregivers frequently serve as medical decision-makers, and little is known about caregiver goals for menstrual management and satisfaction over time. OBJECTIVE: Assess caregiver reasons for initiating menstrual management in adolescents with disabilities and satisfaction over 12 months. METHODS: Prospective cohort study of caregivers of adolescents with developmental disabilities seeking menstrual management at a pediatric and adolescent gynecology clinic. Data derive from caregiver surveys and adolescents' electronic medical records. RESULTS: Ninety-two caregiver-adolescent pairs enrolled. The mean age of adolescents was 14.4 (±2.6). The most common method started was levonorgestrel intrauterine device (LNG-IUD; 52, 56.5%), followed by oral norethindrone acetate (21, 22.8%). Caregivers cited hygiene concerns (84.8%), behavioral problems (52.2%), and heavy/excessive bleeding (48.9%) as reasons for initiating menstrual suppression. Caregivers who identified hygiene or heavy/excessive bleeding as the most important reason for management were more likely to select LNG-IUD (p = 0.009). Caregivers who cited behavioral/mood or seizure concerns as the most important reason were more likely to choose other methods (p < 0.05). At 12 months, caregiver satisfaction with all methods was high (66.2-86.9 on a 100-point scale). For every additional day of bleeding, satisfaction decreased by 3.7 points (95% CI: 2.3-5.0). CONCLUSIONS: Caregiver satisfaction with all methods is high; however, it negatively correlates with days of bleeding. Caregiver reasons for menstrual suppression influence the method chosen. Management may reflect both patient and caregiver priorities; research is needed to better understand shared decision-making models that promote reproductive autonomy in adolescents with a developmental disability.

Hymenal Anomalies Interfering with Dilation in Women with Mullerian Agenesis: A Case Series.

BACKGROUND: Women with Mullerian agenesis, also known as Mayer-Rokitansky-Küster-Hauser syndrome (MRKH), have aplasia or hypoplasia of the uterus and vagina. Regular use of dilators can successfully create a functional vagina in up to 95% of cases. We present 3 women with Mullerian agenesis who failed dilation therapy due to pain and were subsequently found to have hymenal anomalies. CASES: Patients presented at age 16 or 17 to initiate dilation. Initial attempts were discontinued due to pain. On examination, a septate hymen was identified in 2 patients and a microperforate hymen in 1 patient. All patients underwent hymenectomy and thereafter continued dilation with less discomfort. SUMMARY AND CONCLUSION: These cases illustrate the importance of recognizing and treating hymenal anomalies in women with Mullerian agenesis to prevent pain, leading to unsuccessful dilation.

Use of the Levonorgestrel Intrauterine System to Treat Heavy Menstrual Bleeding in Adolescents and Young Adults with Inherited Bleeding Disorders and Ehlers-Danlos Syndrome.

STUDY OBJECTIVE: To report on the rate of amenorrhea among adolescents and young adults with a bleeding diathesis after insertion of the 52-mg levonorgestrel intrauterine system (LNG-IUS) DESIGN: Retrospective chart review SETTING: Tertiary care, multidisciplinary Gynecology-Hematology clinic or Adolescent Gynecology clinic PARTICIPANTS: The cohort included 35 females aged 12-25 years presenting from January 2010 to January 2020 with heavy menstrual bleeding, 23 with an inherited blood disorder, and 12 with Ehlers-Danlos syndrome INTERVENTIONS: The 52-mg LNG-IUS MAIN OUTCOME MEASURES: Primary outcome was bleeding profile after LNG-IUS insertion. Secondary outcomes included rates of amenorrhea, intrauterine device (IUD) expulsion, IUD discontinuation, and unplanned pregnancy. RESULTS: Mean age at menarche was 11.6 years, with mean age at insertion of 16.9 (range 11-23). Most participants were white (n = 26, 74.3%). Von Willebrand disease was present in 16 patients (45.7%) and Ehlers-Danlos syndrome in 12 (34.3%). Most (91.4%) had tried at least 1 hormonal regimen prior to LNG-IUS. Most participants (81.8%) reported improvement in bleeding, with 60.6% reporting spotting or amenorrhea. LNG-IUS expulsion occurred in 3 participants (9.1%) within the first 21 days, despite hemostatic agents at time of insertion. Mean continuation was 5.08 years (95% CI, 4.24-5.92), with 79% likelihood that participants kept their IUD in place for at least 2.5 years, and some up to 6 years. CONCLUSION: The 52-mg LNG-IUS is an effective treatment option for adolescents and young adults with heavy menstrual bleeding and a bleeding diathesis, with high rates of amenorrhea. Rates of IUD expulsion appeared higher during the first 30 days, but long-term continuation remained high.

Application of a Standard Cross-Specialty Workup for Diagnosis and Metabolic Screening of Obese Adolescents With Polycystic Ovary Syndrome.

PURPOSE: Polycystic ovary syndrome (PCOS) is a common disorder. The used diagnostic criteria and screening for related metabolic disease in adolescent girls with PCOS vary with medical specialty, causing discrepancies in care. We sought to improve and standardize care to guidelines for adolescents with PCOS and obesity. METHODS: This is a quality improvement project in a multispecialty, tertiary care children's hospital. Providers in Adolescent Medicine, Gynecology, Pediatric Endocrinology, and Endocrine-Metabolic clinics participated. Diagnostic testing and metabolic screening data were collected for new patient encounters with PCOS and obesity, with two improvement cycles from December 2012 to March 2017. Providers received education on diagnostics tests and metabolic screening recommendations, and electronic medical record tools were created. The number of diagnostic and metabolic screening tests ordered per cycle and per clinic were analyzed and compared with baseline. RESULTS: The preintervention cycle included 74 encounters-44 in Cycle 1 and 58 in Cycle 2. Diagnostic test completeness improved by Cycle 2 (46%-68%) for all clinics. Screening for metabolic disease only improved in Gynecology (27%-71%). Adolescent Medicine, Endocrinology, and Endocrine-Metabolic used note templates, and Adolescent Medicine and Gynecology used order sets. Note templates were associated with more diagnostic tests ordered in Endocrinology (30%-88%; p = .002). CONCLUSIONS: Implementation of quality improvement measures improved the number of diagnostic and metabolic screening tests performed in new patient encounters for PCOS, although the most effective strategy varied by clinic type and electronic medical record habits. Similar efforts should be implemented to standardize care at other institutions.

Association of a Pediatric Gynecology eLearning Module With Resident Knowledge and Clinical Skills: A Randomized Controlled Trial.

OBJECTIVE: To assess whether a pediatric and adolescent gynecology electronic learning (eLearning) module improves knowledge and clinical performance among obstetrics and gynecology residents. METHODS: We conducted a multi-institutional, single-blinded, randomized controlled trial across four university programs; three had pediatric and adolescent gynecology rotations, and two had pediatric and adolescent gynecology fellowship-trained faculty. Applying permutated block randomization, residents were randomized to no intervention or completion of a validated eLearning module on prepubertal bleeding. All residents subsequently completed a pediatric and adolescent gynecology-related knowledge assessment that queried understanding of prepubertal bleeding and an objective structured clinical examination that assessed history collection, performance of a prepubertal genital examination, vaginal culture, and vaginoscopy for a pediatric patient. Objective structured clinical examinations were videotaped and reviewed by two faculty, blinded to randomization group; interrater reliability score was 97%. We calculated descriptive frequencies and compared randomization groups using χ analyses and Fisher exact tests for categorical variables, and median tests for continuous variables; a value of P<.05 was considered significant. RESULTS: From July 2018 to June 2019, we invited 115 residents to participate; 97 (83%) completed both objective structured clinical examination and follow-up knowledge assessments. Most were female (91%) and the majority reported limited pediatric and adolescent gynecology didactic or clinical experience, with 36% reporting prior didactics on prepubertal vaginal bleeding and 33% reporting prior exposure to the prepubertal genital examination. Forty-five participants (46%) were randomized to the module and groups were similar across training levels. Residents assigned to the module scored significantly higher on the knowledge assessment (4/5 vs 2/5, P<.001) and objective structured clinical examination (13/16 vs 7/16, P<.001) and were more likely to avoid a speculum in the examination of a pediatric patient (95.6% vs 57.7%, P<.001). CONCLUSION: Our pediatric and adolescent gynecology eLearning module resulted in improved short-term resident knowledge and simulated clinical skills among obstetrics and gynecology residents. Applying this learning technique in other programs may help address deficiencies in pediatric and adolescent gynecology education and training.

Resident Education Curriculum in Pediatric and Adolescent Gynecology: The Short Curriculum 2.0.

The degree of exposure to pediatric and adolescent gynecology (PAG) varies across residency programs in obstetrics and gynecology and pediatrics. Nevertheless, these programs are responsible for training residents and providing opportunities within their programs to fulfill PAG learning objectives. To that end, the North American Society for Pediatric and Adolescent Gynecology has taken a leadership role in PAG resident education by creating and systematically updating the Short Curriculum. This curriculum outlines specific learning objectives that are central to PAG education and lists essential resources for learners' reference. This updated curriculum replaces the previous 2014 publication with added content, resources, and updated references. Additionally, attention to the needs of learners in pediatrics and adolescent medicine is given greater emphasis in this revised North American Society for Pediatric and Adolescent Gynecology Short Curriculum 2.0.

Improving Resident Knowledge in Pediatric and Adolescent Gynecology: An Evaluation of the North American Society for Pediatric and Adolescent Gynecology Short Curriculum.

STUDY OBJECTIVE: Resident education in pediatric and adolescent gynecology (PAG) is challenging. It encompasses patients from neonates to young adults with different disorders involving multiple subspecialties. Residents have inadequate exposure to PAG topics and report lack of knowledge in this area. The objective of this study was to determine if the North American Society for Pediatric and Adolescent Gynecology (NASPAG) Short Curriculum improves self-reported knowledge in PAG among obstetrics and gynecology (ObGyn), family medicine, and pediatric residents. DESIGN, SETTING, PARTICIPANTS, INTERVENTIONS, AND MAIN OUTCOME MEASURES: Participants were 47 US ObGyn, family medicine, and pediatric residency training programs across a 4-month study window, from September to December 2016. The NASPAG Short Curriculum was distributed to them with a request to complete a retrospective pre- and post-test survey. Primary outcome measure was improvement in self-perceived knowledge after exposure to the curriculum. RESULTS: Forty-eight programs responded to the study comprising a total of 1130 residents. One program was excluded because of logistical barriers to the distribution of study incentive. In total, 1080 residents were invited and 103 chose to participate (10% response rate); 68 residents completed all survey questions to be included in the final analysis. After completing the curriculum, self-reported knowledge improved in all 10 learning objectives, across all 3 specialties (47% [32/68] to 82% [56/68]; P < .01). Pre-test knowledge correlated with previous clinical exposure to PAG patients, but did not correlate with year of residency training, type of residency, or previous PAG lectures. CONCLUSION: Significant deficiencies exist regarding self-reported knowledge of core PAG topics among ObGyn, family medicine, and pediatric residents. Use of the NASPAG Short Curriculum improves self-reported knowledge in PAG trainees across all 3 specialties.

Creation and Dissemination of a Multispecialty Graduate Medical Education Curriculum in Pediatric and Adolescent Gynecology: The North American Society for Pediatric and Adolescent Gynecology Resident Education Committee Experiences.

STUDY OBJECTIVE: The goal was to develop a multispecialty committee to address deficiencies in pediatric and adolescent gynecology (PAG) resident education through curricular development under the auspices of the North American Society for Pediatric and Adolescent Gynecology. DESIGN, SETTING, PARTICIPANTS, INTERVENTIONS, AND MAIN OUTCOME MEASURES: A multispecialty North American committee was organized to develop short as well as long curricula in PAG through a combination of conference calls and face-to-face meetings. Content was guided by objectives of national accrediting organizations. The curricula used print as well as interactive electronic resources. RESULTS: After publication of the short and long curricula, a dissemination strategy was developed to present the information at national meetings. A curricular study was performed after introduction of the curriculum to evaluate its efficacy. Long-term plans for further curricular components and expansion of educational tools are ongoing. CONCLUSION: We gathered a diverse multispecialty group of doctors to collaborate on a unified educational goal. This committee developed and disseminated resident PAG curricula using a variety of learning tools. This curricular development and implementation can occur with a minimal financial burden.

Clinical Predictors of Isolated Tubal Torsion: A Case Series.

STUDY OBJECTIVE: A rare cause of adolescent abdominal pain includes isolated tubal torsion (ITT). Presentation is nonspecific and few studies have investigated ITT in adolescents. Our study objective was to describe the presentation and management of ITT in a large case series. DESIGN: Retrospective observational case series. SETTING: Tertiary care children's hospital. PARTICIPANTS: Participants were female and aged 3-21 years, presenting to Children's Hospital Colorado and diagnosed with ITT between January 2004 and August 2015. INTERVENTIONS AND MAIN OUTCOME MEASURES: Clinical presentation, physical exam, laboratory findings, surgical diagnosis, and treatment provided. RESULTS: A total of 19 cases were included. Average age was 13.3 (range, 11-18) years. In patients with unilateral abdominal pain (n = 16), there was 100% correlation with side of adnexal pathology. Ultrasound examination in 14 of 18 cases (78%) noted abnormal findings ipsilateral to the ITT. Most cases were managed with laparoscopy (84%; n = 16 of 19) and detorsion with or without cystectomy (74%; n = 14 of 19). Salpingectomy was more common with prolonged pain greater than 24 hours (relative risk 5.6, 95% confidence interval, 0.7-39.0). The most common intraoperative finding was a paratubal cyst (74%; n = 14 of 19). When Doppler flow was performed, it was present in 88% (n = 16 of 18) of the affected adnexa. ITT was more common on the left side (68%; n = 13 of 19). CONCLUSION: The high occurrence of paratubal cysts might suggest pathologic predisposition for ITT. Providers should maintain a high index of suspicion for ITT, particularly if associated with a paratubal cyst. Classic examination findings of surgical abdomen, leukocytosis, fever, and absence of Doppler flow are infrequently present. Laparoscopy and detorsion are appropriate treatments for managing ITT.

Treatment of Acute Abnormal Uterine Bleeding in Adolescents: What Are Providers Doing in Various Specialties?

STUDY OBJECTIVE: The purpose of this study was to assess whether variability exists in the management of acute abnormal uterine bleeding (AUB) in adolescents between pediatric Emergency Department (ED) physicians, pediatric gynecologists, and adolescent medicine specialists. DESIGN: Retrospective chart review. SETTING: Tertiary care medical center ED. PARTICIPANTS, INTERVENTIONS, AND MAIN OUTCOME MEASURES: We included girls aged 9-22 years who presented from July 2008 to June 2014 with the complaint of acute AUB. Patients were identified using the International Classification of Diseases, ninth revision codes for heavy menstrual bleeding, AUB, and irregular menses. Exclusion criteria included pregnancy and current use of hormonal therapy. One hundred fifty patients were included. RESULTS: Among those evaluated, 61% (n = 92) were prescribed hormonal medication to stop their bleeding by providers from the ED, Adolescent Medicine, or Pediatric Gynecology. ED physicians prescribed mostly single-dose and multidose taper combined oral contraceptive pills (85%; n = 24), compared with Adolescent Medicine (54%, n = 7), and Gynecology (28%, n = 13). Pediatric gynecologists were more likely than ED physicians to treat patients with norethindrone acetate, either alone or in combination with a single dose combined oral contraceptive pill (61%, n = 33 vs 7%, n = 2; P < .001). CONCLUSION: Variations in treatment strategies for adolescents who present with acute AUB exist among pediatric specialties, which reflects a lack of standardized care for adolescents. Prospective evaluation of the shortest interval to cessation of bleeding, side effects, and patient satisfaction are valuable next steps.

An Atypical Presentation of Vaginal Agenesis.

BACKGROUND: Vaginal agenesis is rare and generally presents with primary amenorrhea and cyclic abdominal pain. We describe a case in which the diagnosis was delayed due to lack of initial pelvic examination and atypical findings on imaging. CASE: A 13-year-old girl with a known renal anomaly presented to the emergency department with primary amenorrhea and cyclic abdominal pain. She declined a pelvic examination and had normal laboratory testing and pelvic magnetic resonance imaging results. At 16 months later, she presented again and was diagnosed with vaginal agenesis and a large endometrioma. SUMMARY AND CONCLUSION: This case illustrates the importance of the physical examination in the evaluation of primary amenorrhea. Further, it demonstrates that hematometra may not be present on imaging. Here, an endometrioma was the only abnormality noted on magnetic resonance imaging after 18 months of retrograde menstruation.

Resident education curriculum in pediatric and adolescent gynecology: the short curriculum.

The degree of exposure to Pediatric and Adolescent Gynecology (PAG) varies across academic programs in Obstetrics and Gynecology, Pediatrics, and Adolescent Medicine. Nevertheless, these programs are responsible to train residents and provide opportunities within their training programs to fulfill PAG learning objectives. To that end, North American Society for Pediatric and Adolescent Gynecology has taken a leadership role in PAG resident education by disseminating the Short Curriculum with specific learning objectives and list of essential resources where key concepts in PAG can be covered.