STUDY OBJECTIVE Ehlers-Danlos syndrome (EDS) is a connective tissue disorder resulting in abnormal collagen synthesis leading to skin, joint, ligament, blood vessel, and organ abnormalities. Studies in adult women show an association with heavy menstrual bleeding, dysmenorrhea, and pelvic organ prolapse. We aimed to evaluate gynecologic complaints in pediatric and adolescent patients with EDS and their management by pediatric and adolescent gynecology (PAG) Providers. DESIGN, SETTING, PARTICIPANTS, INTERVENTIONS, AND MAIN OUTCOME MEASURES: Charts of female individuals less than 21 years of age with an International Classification of Diseases (ICD) Ninth or Tenth revision diagnosis of EDS who were evaluated between July 1, 2007, and July 31, 2017, were reviewed for menstrual history, gynecologic complaints, and interventions prescribed. RESULTS A total of 156 patients met inclusion criteria, and 26 (16.7%) were seen by PAG providers. The mean age was 14.5 ± 1.9 years. Fifteen (57.7%) reported dysmenorrhea, 13 (50%) complained of heavy menstrual bleeding (HMB), 10 (38.5%) reported irregular menses, and 7 (26.9%) sought contraception. Concurrent medical problems were reviewed, as this affected hormone choice. The cohort was stratified into 2 groups: patients whose menstrual cycles were well controlled on a single method (group A), and patients who tried multiple medications (group B). Progesterone-only pills were most commonly used in froup A. Eleven (73%) patients in group B tried depot medroxyprogesterone acetate (DMPA), but ultimately a levonorgestrel intrauterine device (IUD) was the most popular final choice of treatment and was used by 4 (27%) patients. CONCLUSION Few adolescents with EDS are referred to PAG providers despite the prevalence of gynecologic complaints and potential for obstetric and gynecologic complications. In this population, early entry to gynecologic care would be beneficial.

中文翻译：

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Ehlers-Danlos综合征的儿童和青少年患者的妇科管理。

研究目的Ehlers-Danlos综合征（EDS）是一种结缔组织病，可导致胶原蛋白合成异常，从而导致皮肤，关节，韧带，血管和器官异常。对成年女性的研究表明，与月经大量出血，痛经和盆腔器官脱垂有关。我们的目标是评估儿童和青少年EDS患者的妇科主诉及其由儿童和青少年妇科（PAG）提供者的管理。设计，地点，参与者，干预措施和主要观察指标：在2007年7月1日至7月之间评估了21岁以下，具有国际疾病分类（ICD）EDS的第九或第十次修订版诊断的女性个体图表。回顾了2017年3月31日的月经史，妇科主诉和规定的干预措施。结果共有156例患者符合入选标准，PAG提供者观察到26例（16.7％）。平均年龄为14.5±1.9岁。报告痛经的有15（57.7％），有严重月经出血（HMB）的有13（50％），月经不调的有10（38.5％），有避孕的报告有7（26.9％）。并发医疗问题进行了审查，因为这会影响激素的选择。队列分为两组：月经周期在单一方法下得到很好控制的患者（A组）和尝试多种药物治疗的患者（B组）。仅孕激素药丸最常用于治疗A组。B组中有11名（73％）患者尝试使用醋酸甲羟孕酮（DMPA），但最终左炔诺孕酮宫内节育器（IUD）是最受欢迎的最终治疗方法， 4名（27％）患者。结论尽管有妇科疾病的患病率以及产科和妇科并发症的可能性，但很少有EDS的青少年转诊至PAG提供者。在这种人群中，尽早进入妇科保健将是有益的。