A case of image-guided hematometrocolpos drainage requiring tissue plasminogen activator in a pediatric patient.

Hematometrocolpos (HMC) is a rare disorder that occurs when an anatomical anomaly like imperforate hymen causes menstrual blood to be retained in the uterus and vagina. There is no standard of care established for HMC beyond urgent vaginoplasty which requires a demanding post-operative course that may not be suited for all pediatric patients. This is a case report of successful use of image-guided percutaneous drainage of HMC with tissue plasminogen activator (TPA) followed by vaginoplasty in a 13-year-old female with lower vaginal atresia. Additionally, this case explores the role of menstrual suppression and the need for individualized guidelines. It emphasizes the potential of image-guided percutaneous drainage with TPA as a promising, less-invasive treatment option for pediatric HMC as well as the impact on follow-up surgery.

Hemostatic considerations for gender affirming care.

Gender dysphoria or gender incongruence is defined as "persons that are not satisfied with their designated gender" [1]. The awareness and evidence-based treatment options available to this population have grown immensely over the last two decades. Protocols now include an Endocrine Society Clinical Practice Guideline [1] as well as the World Professional Association of Transgender Health Standards of Care (WPATH SOC) [2]. Hematologic manifestations, most notably thrombosis, are one of the most recognized adverse reactions to the hormones used for gender-affirming care. Therefore, hematologists are frequently consulted prior to initiation of hormonal therapy to help guide safe treatment. This review will focus on the scientific evidence related to hemostatic considerations for various gender-affirming therapies and serve as a resource to assist in medical decision-making among providers and patients.

Dermatologic care of patients with differences of sex development.

Background: Differences of sex development (DSD or disorders of sex development) are uncommon congenital conditions, characterized by atypical development of chromosomal, gonadal, or anatomic sex. Objective: Dermatologic care is an important component of the multidisciplinary care needed for individuals with DSD. This article discusses the most common primary dermatologic manifestations of DSD in addition to the cutaneous manifestations of hormonal and surgical therapies in individuals with DSD. Data sources: Published articles including case series and case reports on PubMed. Study selections: Selection was conducted by examining existing literature with a team of multidisciplinary specialists. Methods: Narrative review. Limitations: This article was not conducted as a systematic review. Results: In Klinefelter syndrome, refractory leg ulcers and incontinentia pigmenti have been described. Turner syndrome is associated with lymphatic malformations, halo nevi, dermatitis, and psoriasis. Virilization can be seen in some forms of congenital adrenal hyperplasia, where acne and hirsutism are common. Conclusion: Dermatologists should consider teratogenic risk for treatments of skin conditions in DSD depending on pregnancy potential. Testosterone replacement, commonly used for Klinefelter syndrome, androgen insensitivity syndrome, 5-alpha reductase deficiency, gonadal dysgenesis, or ovotesticular DSD, may cause acne.

Hormonal therapies in females with blood disorders: thrombophilia, thrombosis, hemoglobinopathies, and anemias.

There is widespread use of gonadal steroid hormone therapy for a variety of indications throughout the reproductive and postreproductive lifespan. These therapies may have particular benefits and specific risk among those with blood disorders, including inherited or acquired bleeding disorders, thrombophilia, thrombosis, or anemia. This clinical review is intended to provide a guidance for counseling and management of adolescent and adult biologic females with thrombophilic risk factors and/or thrombosis who require hormonal therapy. In general, synthetic estrogens present in contraceptive products should be avoided in those with a personal or strong family history of thrombosis or thrombophilias. In contrast, natural estrogens present in formulations for climacteric symptom management do not need to be avoided, and vaginal or transdermal formulations are preferred. Likewise, transdermal estradiol is preferred for gender-affirming hormone therapy and requires individualized assessment in those at high risk of thrombosis. Progestogens (either synthetic progestins or naturally occurring progesterone) can be used safely in nearly all patients. There is minimal safety evidence among anticoagulated patients at risk for thrombosis, which requires a patient-specific approach when discussing hormone therapies.

Sexual Assault/Domestic Violence.

Sexual assault and intimate partner violence (IPV) of children, adolescents, and adult women are prevalent in the United States and have long-term physical and mental health, financial, and social effects. Pregnant women and women of color are particularly high-risk populations. Obstetrics and gynecology providers are uniquely situated to assess and treat survivors of IPV and sexual assault. A timely, thorough forensic medical examination, appropriate evaluation, and prophylactic therapy are all vital components in the care of these patients.

Frequency of Sexual Orientation and Gender Identity Documentation After Electronic Medical Record Modification.

STUDY OBJECTIVE: Documentation of sexual orientation (SO) and gender identity (GI) is crucial to identify lesbian, gay, bisexual, and transgender youth and perform meaningful research to improve health disparities in this community. As a result, some electronic medical records (EMRs) have incorporated SO and GI into part of the provider's workflow for documentation. We aimed to evaluate the effect this modification has had on the frequency of SO and GI documentation. DESIGN, SETTING, PARTICIPANTS, INTERVENTIONS, AND MAIN OUTCOME MEASURES: This was a retrospective chart review of patient encounters from an outpatient pediatric and adolescent gynecology clinical practice. The rate of documentation of SO and GI were compared between encounters that took place before the implementation of the EMR modification and those that took place after. Additionally, we examined rates of GI and SO documentation according to visit type and patient race. RESULTS: A statistically significant increase in the frequency of SO and GI documentation after the EMR modification was detected. The documentation rate of SO increased from 10/73 (13.7%) to 32/73 (45.1%) (P < .01) and GI documentation rate went from 1.4% to 46.5% (P < .01) after the EMR changes were implemented. SO or GI was most commonly documented in social history (90%). There were no differences in documentation on the basis of race or type of encounter. CONCLUSION: Including a specific tab for SO and GI in the EMR significantly increased the frequency of SO and GI documentation. Despite this increase, frequency of documentation remained at less than 50%, emphasizing the need for further improvement.

Clinical research with adolescents: barriers and how to overcome them.

PURPOSE OF REVIEW: This review aims to emphasize the importance of including adolescents in research, outline current standards and evaluate barriers that prevent adolescent research. We also hope to provide some guidance and call for much needed additional direction in the field. RECENT FINDINGS: The absolute necessity for including adolescents in research has been an area of focus within the community of adolescent providers and care takers. Recent policy and support from major organizations has emphasized the importance of including this population in research but also pointed out many of the complexities. SUMMARY: Adolescent research is imperative to guiding policy and promoting evidence-based care. Current standards provide both guidance and barriers, yet more research is needed to further guide conduct of ethical research in this population.

Primary Primitive Neuroectodermal Tumor Arising from an Ovarian Mature Cystic Teratoma in a 12-Year-Old Girl: A Case Report.

BACKGROUND: Ovarian mature cystic teratomas (MCTs) rarely transform to primary primitive neuroectodermal tumors. This case report offers evidence that MCTs might have undetected microfoci of malignant neural tumors. CASE: We describe the case of a 12-year-old girl who presented with right-sided abdominal pain and distention. Intraoperative findings revealed a right ovarian MCT. However, pathology showed a 0.5-cm focus of malignant neural tumor within the 11-cm MCT. SUMMARY AND CONCLUSION: This patient will need close follow-up with a multidisciplinary team because the clinical implications of this transformation has yet to be defined.

Labial adhesions and outcomes of office management.

STUDY OBJECTIVE: To evaluate clinical outcomes of labial adhesions (LA) and to examine the association between LA, lichen sclerosus (LS), eczema (ECZ), or asthma. DESIGN: Retrospective study. SETTING: Single pediatric and adolescent gynecology clinic, Houston, Texas. PARTICIPANTS: 50 girls diagnosed with LA from 2006-2011. INTERVENTIONS AND MAIN OUTCOME MEASURES: Resolution, recurrence, single vs multiple treatments, need for surgery, and conditions such as LS, ECZ, and asthma were reviewed. RESULTS: Mean age was 19.6 months (range 0-84 months), and 48% were Caucasian. Most patients were symptomatic (62%) and all 50 patients chose estrogen treatment. The majority (74%) required multiple treatments, as opposed to a single treatment (26%). Patients with multiple treatments were more likely to be severely agglutinated (P = .05) and to need manual separation after failed topical treatment (P = .08). The prevalence of asthma, LS, and ECZ was 9.8%, 7.8%, and 3.9% respectively. There was no association between LS, ECZ, or asthma, and number of treatments. Both asthma (N = 3; 8%), and LS (N = 2; 5%) were present among the severe agglutinated group; however, this difference was not statistically significant (P values .59 and .99). No association with ECZ was seen in either group. CONCLUSION: Severe agglutination tends to be associated with need of multiple treatments and manual separation. A concurrent diagnosis of LS, ECZ, or asthma was not associated with number of treatments but there appears to be a trend towards severity of LA in patients with asthma and LS.

A multicenter survey of contraceptive knowledge among adolescents in North America.

STUDY OBJECTIVE: To assess knowledge about contraceptive efficacy and side effects in an adolescent population seen in Pediatric and Adolescent Gynecology referral centers. DESIGN: This is a multisite cross-sectional survey study. A 23-question survey assessing knowledge of contraception and demographic information was administered. Data analysis was performed using descriptive statistics, simple paired t tests, and chi-square analyses using SAS 9.3. SETTING: Pediatric and Adolescent Gynecology clinics in 4 tertiary care centers. The study was conducted in 3 institutions in the United States and 1 institution in Canada. PARTICIPANTS: A convenience sample of 354 female patients aged 10-24 y seeking reproductive healthcare at participating institutions. INTERVENTIONS: None MAIN OUTCOME MEASURES: The percentage of correct answers to questions assessing general knowledge about contraception, familiarity with different contraceptive methods, and comparison of results between study sites. RESULTS: The mean percentage of correct answers among all participants was 55.8% ± 17%. Younger participants (age 10-13 years) scored significantly lower than their older counterparts (49%, 55%, and 60% respectively, P < .05). There was no correlation between score and ethnicity or location of the participating site. Subjects reporting the internet as a source of information, those who were sexually active, and those familiar with long acting reversible contraceptives scored significantly higher. Of all contraceptive methods, participants were least likely to have heard of etonogestrel implants (18%), rhythm method/natural family planning (28%), and IUDs (32%). CONCLUSION: Adolescents and young adults performed poorly overall demonstrating both the lack of overall knowledge regarding methods of contraception and misinformation about side effects.

Pubic osteomyelitis resulting in rectovaginal fistula in an adolescent female.

BACKGROUND: Pelvic osteomyelitis is a rare but serious condition that has the potential for systemic and muscular complications if left untreated. The diagnosis is difficult due to the relative rarity of the disease, the difficulty of localizing the site of the infection, and the tendency for the presentation to mimic other disease processes. CASE: A 13-year-old female who presented with complaint of severe right lower abdominal pain thought to be related to a hemorrhagic cyst but was found to have pubic osteomyelitis resulting in an abscess and vaginal fistula. CONCLUSION: Although rare, pelvic osteomyelitis should be included in the differential diagnosis of pelvic pain.

Use of microcatheter for intraabdominal survey.

BACKGROUND: Laparoscopy is the most common procedure for minimally invasive intraabdominal surveys. Patients with pulmonary hypertension (PHTN) may need an alternate approach because the systemic absorption of carbon dioxide may lead to hypercapnia, acidemia, and increases in systemic and pulmonary pressures. We report a case of intraabdominal survey using a microcatheter trocar (Check-Flo Introducer) in a patient with a large adnexal mass and PHTN. CASE: An adolescent girl with severe PHTN and multiple cardiac anomalies presented with oligomenorrhea. During the course of workup, an ultrasound revealed an 8-cm simple cyst in the right adnexa, which required removal. The decision was made to proceed with cystectomy. The patient was not a candidate for laparoscopy, and an alternative method was used. The Check-Flo Introducer, a microcatheter trocar; crystalloid fluid infusion; and a 3-mm laparoscopic camera were used to survey the abdominal cavity, revealing a 10-cm simple paratubal cyst that could be safely drained and removed via a minilaparotomy incision. CONCLUSION: The microcatheter trocar is a useful and novel alternative in patients who cannot tolerate increases in intraabdominal pressure.

Platelet function disorders and menorrhagia in adolescents: a review of laboratory diagnosis.

This article provides an overview of the prevalence and pathophysiology of platelet function disorders (PFDs) in adolescents with menorrhagia. In addition, this article reviews the various testing modalities employed for diagnosing PFDs including platelet aggregometry, platelet function analyzer (PFA-100), platelet electron microscopy (EM), flow cytometry, and thromboelastography (TEG), discuss their utility and drawbacks, and allude to the recent recommendations and consensus statements about some of these modalities. Finally, the authors have sketched out a diagnostic algorithm for platelet function testing, which will guide treating physicians to a step-wise approach while evaluating adolescents with menorrhagia for PFDs.

Management of heavy menstrual bleeding in adolescents.

PURPOSE OF REVIEW: Heavy menstrual bleeding (HMB) is an extremely common problem among adolescents. This article reviews the differential diagnosis and clinical presentation. Additionally, we aim to present the most up-to-date guidelines for evaluation and treatment. RECENT FINDINGS: Bleeding disorders are now recognized as a common cause for menorrhagia. The recommended laboratory evaluation has evolved in the last few years. Most forms of hormonal contraception, including the levonorgestrel intra-uterine device, are effective and have been studied in adolescents. SUMMARY: HMB is prevalent in the adolescent population and is associated with serious complications. Laboratory analysis to rule out bleeding disorders should be considered. Medical management, the cornerstone of treatment, has been proven to be safe and effective in this population.

Knowledge of sexually transmitted infections among adolescents in the Houston area presenting for reproductive healthcare at Texas Children's Hospital.

OBJECTIVE: Studies indicate a deficiency in knowledge of sexually transmitted infections (STIs) among adolescents, yet adolescents comprise 25% of the sexually active (SA) population and account for 48% of STIs acquired annually. This survey assesses knowledge of STIs among adolescent females. The goal of this study was to assess knowledge of STIs and how it relates to safe sex behaviors and educational access. DESIGN: A confidential 10-question STI survey was administered to a convenience sample of female adolescents. Data analysis included descriptive statistics, chi-square analysis, and linear regression analysis. SETTING: Texas Children's Hospital Pediatric and Adolescent Gynecology Clinic. INTERVENTIONS: None. PARTICIPANTS: Seventy-five female participants between the ages of 10-21 years. MAIN OUTCOME MEASURES: Age, sexual activity, educational access, preferred methods of risk reduction and questions answered correctly on the STI survey. RESULTS: The mean age of participants was 14.9 ± 2.4 years; mean age of menarche was 10.9 ± 2.9 years. Based on survey responses, all adolescents demonstrated similar knowledge of specific STIs regardless of demographic factors. However, middle and late adolescent groups had increased awareness of STIs. SA participants (36%) were more likely to choose 2 or more methods of risk reduction compared to non-SA participants (P = 0.014). There was no correlation between educational access and preferred methods of risk reduction even though 92% of respondents reported receiving STI education from school, parents, or peers. CONCLUSIONS: Current efforts at STI education are not effective. Different approaches to STI education are necessary to increase knowledge and motivate adolescents to reduce high risk behaviors.

Idiopathic pulmonary hypertension causing acquired von Willebrand disease and menorrhagia.

BACKGROUND: Von Willebrand disease (VWD) maybe inherited or acquired; both etiologies can be associated with heavy menstrual bleeding. Pulmonary arterial hypertension may result in acquired VWD due to the destruction of high molecular weight von Willebrand multimers. We report a case of menorrhagia due to acquired VWD in a patient with idiopathic pulmonary hypertension. CASE: An adolescent female with known idiopathic pulmonary hypertension developed acquired VWD. Her primary disease necessitates the use of platelet inhibitors and intermittent anticoagulation. At menarche she also developed menorrhagia due to acquired VWD. She is currently controlled with stimate and progesterone-only therapy. VWD in a patient with idiopathic pulmonary hypertension causing menorrhagia. Although VWD and menorrhagia are commonly linked, the treatment and disease process in a patient with idiopathic pulmonary arterial hypertension is incredibly complex.

Consequences of routine delivery at 38 weeks for A-2 gestational diabetes.

OBJECTIVE: To report our intrapartum experience with routine delivery at 38 weeks of gestation of A-2 diabetic pregnancies requiring primarily oral hypoglycemic therapy. METHODS: This retrospective study consisted of 143 consecutive women with gestational diabetes not controlled with diet alone (A-2). Each underwent a routine trial of labor at 38 weeks of gestation. The preinduction condition of the cervix, need for oxytocin, and primary cesarean rates were primary endpoints. For comparison, a control group during that same period consisted of 137 consecutive diet-controlled diabetic (A-1) pregnancies with the same eligibility criteria who underwent expectant management at 38 weeks. RESULTS: The study group was more likely to have an unfavorable cervix (75% versus 45%; p < 0.001) and to require oxytocin (76% versus 56%; p < 0.001). Early onset meconium was less common in the study group (3.5% versus 13.1%; p < 0.01). Primary cesarean rates were low and not different between the study and control groups (12.7% versus 11.7%; p < 0.8). The only stillbirth was in the control group and was associated with a tight double nuchal cord encirclement. Mean birth weights and the frequency of birth weights > 4000 g were not different between groups. Shoulder dystocia, low Apgar scores, and admissions to the special care nursery were infrequent in either group. No respiratory difficulties requiring resuscitation or prolonged nursery care were encountered. CONCLUSION: Routine delivery at 38 weeks in an A-2 diabetic population is not associated with additional intrapartum morbidity or a greater need for cesarean delivery.