Gynecologic Management of Pediatric and Adolescent Patients with Ehlers-Danlos Syndrome
Abstract
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…
How strong is the evidence?
Evidence Hub reads this as a cohort study, done in people. Its evidence level is 3 of 5 (Moderate · trial / cohort / secondary analysis). Trials without a large randomized design, cohorts and secondary analyses give moderate evidence. Reported size: 156 participants or records.
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