11. Use of the Levonorgestrel Intrauterine System in an Adolescent with Type IV Vascular Ehlers-Danlos Syndrome and Heavy Menstrual Bleeding

Ehlers-Danlos Syndrome (EDS) is a connective tissue disorder resulting in altered collagen synthesis. Heavy menstrual bleeding (HMB) and dysmenorrhea are common among adolescents with EDS. The levonorgestrel intrauterine system (LNG-IUS) is an effective treatment option for HMB in the general population, however its use in patients with vascular EDS has historically been avoided due to the perceived risk of spontaneous uterine rupture with IUD use in these patients.

A 16-year-old female with vascular EDS presented with concerns for HMB. She reported menarche at age 13, with cycles every 4-6 weeks, lasting seven days in duration. She reported changing soaked pads every 3-4 hours and regularly passing dime-sized clots, with a total pictorial blood loss assessment chart score of 254. She was known to have a glycine substitution in her COL3A1 gene—a vascular EDS subtype, associated with aortic and viscus rupture, particularly with surgical interventions. After thorough discussion, she desired to have a LNG-IUS placed for menstrual management. The procedure was recommended under ultrasound guidance and with sedation to optimize the chance of successful and safe placement, given concerns the family had regarding uterine rupture with IUS placement, as reported to them by previous providers. Intraoperatively, she was noted to have an anteverted, anteflexed uterus which sounded to 7 cm. A tenaculum was used to grasp the cervix and the LNG-IUS was deployed at the fundus under transabdominal ultrasound guidance. Hemostasis of the cervix was achieved after applying brief pressure and no complications occurred. At six-week follow-up, she reported moderate vaginal bleeding and cramping for one week following LNG-IUS placement. She noted two days of light bleeding without cramping with her subsequent menstrual cycle and was overall very satisfied with the LNG-IUS. At six-month follow up, she reported only occasional spotting with her IUD and significant improvement in her energy.

Many individuals with EDS experience heavy menstrual bleeding and dysmenorrhea. The LNG-IUS has been underutilized in this population, especially for those with vascular EDS, due to theorized concerns for uterine perforation and significant bleeding. Previous publications have recommended using extreme caution with LNG-IUSs in patients with vascular EDS given a lack of evidence supporting their use. Our case demonstrates use of the LNG-IUS can be a safe and effective option for HMB in this population. Furthermore, risk of complications may be mitigated by optimizing successful placement with adequate pain control and ultrasound guidance.