Ehlers-Danlos syndrome (EDS) is a group of connective tissue disorders of altered collagen synthesis. People with vascular EDS are at increased risk for vascular and hollow viscous rupture. Heavy menstrual bleeding (HMB) is common among adolescents with EDS. The levonorgestrel intrauterine device (LNG-IUD) is an effective treatment option for HMB; however, its use in patients with vascular EDS has historically been avoided due to perceived risk of uterine rupture. This is the first known case report on use of the LNG-IUD in an adolescent with vascular EDS.
A 16-year-old female with vascular EDS and HMB underwent placement of the LNG-IUD. Placement of the device was performed in the operating room under ultrasound guidance. At the 6-month follow-up, the patient reported significant improvement in bleeding and high satisfaction. No complications were identified at the time of placement or follow-up.
Summary and Conclusion
LNG-IUD may be a safe and effective option for menstrual management in individuals with vascular EDS.
Abbreviations:LNG-IUD (levonorgestrel intrauterine device), IUD (intrauterine device), HMB (heavy menstrual bleeding), EDS (Ehlers-Danlos Syndrome)
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- Ehlers-Danlos syndrome: insights on obstetric aspects.Obstet Gynecol Surv. 2007; 62: 51-57
- Gynecologic management of pediatric and adolescent patients with Ehlers-Danlos syndrome.J Pediatr Adolesc Gynecol. 2020; 33: 291-295
- Gynecologic symptoms and the influence on reproductive life in 386 women with hypermobility type Ehlers-Danlos syndrome: a cohort study.Orphanet J Rare Dis. 2016; 11: 124
- Obstetric and gynecologic challenges in women with Ehlers-Danlos syndrome.Obstet Gynecol. 2014; 123: 506-513
- Hypermobility syndromes in heavy menstrual bleeding.in: Srivaths LV Hematology in the Adolescent Female. Springer International Publishing, Springer Nature Switzerland2020: 89-98
- The type of variants at the COL3A1 gene associates with the phenotype and severity of vascular Ehlers-Danlos syndrome.Eur J Hum Genet. 2015; 23: 1657-1664
- Effectiveness and tolerability of levonorgestrel intrauterine system in adolescents.J Obstet Gynaecol. 2010; 30: 489-491
- Use of the levonorgestrel intrauterine system to treat heavy menstrual bleeding in adolescents and young adults with inherited bleeding disorders and Ehlers-Danlos syndrome.J Pediatr Adolesc Gynecol. 2022; 35 (147-152.e1)
- Pregnancy and the Ehlers–Danlos syndrome: a retrospective study in a Dutch population.Acta Obstet Gynecol Scand. 2002; 81: 293-300
- The levonorgestrel-releasing intrauterine system in heavy menstrual bleeding: a benefit-risk review.Drugs. 2012; 72: 193-215
Published online: February 20, 2023
Accepted: February 3, 2023
Received in revised form: January 30, 2023
Received: November 28, 2022
Publication stageIn Press Journal Pre-Proof
Conflict of Interest Statement: The authors declare that they have no competing interests.
Consent for publication: The patient and family agreed in writing to the use of information pertaining to this case, including all images, for educational purposes.
© 2023 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.