EHLERS–DANLOS SYNDROME AND PREGNANCY: CURRENT STATE OF KNOWLEDGE, RISKS AND OBSTETRIC COMPLICATIONS

Анотація

Ehlers–Danlos syndrome (EDS) is a heterogeneous group of inherited connective tissue disorders caused by abnormalities in its structure and functional properties, which may significantly affect the course of pregnancy, childbirth, and the postpartum period. Clinical manifestations and the degree of risk of obstetric complications depend on the specific EDS subtype, necessitating an individualized approach to the management of affected patients. This article presents the current state of knowledge and summarizes the scientific literature on the main clinical subtypes of EDS, their manifestations during pregnancy, and their potential impact on maternal and perinatal outcomes. The major obstetric risks are reviewed, including preterm birth, preterm prelabor rupture of membranes, postpartum hemorrhage, traumatic soft tissue injuries, impaired wound healing, as well as pain and joint hypermobility, which may complicate pregnancy. Particular attention is paid to the vascular subtype of EDS, which is associated with an increased risk of arterial rupture and dissection, uterine rupture, and other potentially life-threatening complications. Current approaches to preconception counseling, genetic diagnosis, individual risk assessment, antenatal surveillance, and delivery planning are analyzed, taking into account the clinical subtype of the disorder and the patient's obstetric history. The importance of timely diagnosis, interdisciplinary collaboration among obstetricians and gynecologists, geneticists, and other relevant specialists, as well as the early detection of potentially dangerous conditions, is emphasized. It is concluded that optimizing the management of pregnant women with EDS requires an individualized approach, further accumulation of clinical data, and high-quality research to clarify maternal and perinatal risks and improve evidence-based recommendations for pregnancy management and mode of delivery.

References

Haem, T., Benson, B., Dernoncourt, A., Gondry, J., Schmidt, J., & Foulon, A. (2024). Vascular Ehlers-Danlos syndrome and pregnancy: A systematic review. BJOG: An International Journal of Obstetrics & Gynaecology, 131(12), 1620–1629. https://doi.org/10.1111/1471-0528.17893

Pezaro, S., Brock, I., Buckley, M., et al. (2024). Management of childbearing with hypermobile Ehlers-Danlos syndrome and hypermobility spectrum disorders: A scoping review and expert co-creation of evidence-based clinical guidelines. PLOS ONE, 19(5), Article e0302401. https://doi.org/10.1371/journal.pone.0302401

Alrifai, N., Alhuneafat, L., Jabri, A., et al. (2023). Pregnancy and fetal outcomes in patients with Ehlers-Danlos syndrome: A nationally representative analysis. Current Problems in Cardiology, 48(7), Article 101634. https://doi.org/10.1016/j.cpcardiol.2023.101634

Ravi, U., & Daley, S. F. (2026, June 17). Ehlers-Danlos syndromes. In StatPearls. StatPearls Publishing.

Tadakawa, M., Yamaguchi, T., Tomita, H., Mochii, N., Hamada, H., Tsubata, M., Kosho, T., Aoki, Y., & Saito, M. (2026). Fatal aortic rupture at term pregnancy caused by vascular Ehlers-Danlos syndrome diagnosed by postmortem genetic testing using formalin-fixed, paraffin-embedded tissue. Journal of Obstetrics and Gynaecology Research, 52(5), Article e70299. https://doi.org/10.1111/jog.70299

Malfait, F., Francomano, C., Byers, P., et al. (2017). The 2017 international classification of the Ehlers-Danlos syndromes. American Journal of Medical Genetics Part C: Seminars in Medical Genetics, 175(1), 8–26. https://doi.org/10.1002/ajmg.c.31552

Zschocke, J., Demirdas, S., & van Dijk, F. S. (2024). Genetic diagnosis of the Ehlers-Danlos syndromes. Medizinische Genetik, 36(4), 235–245. https://doi.org/10.1515/medgen-2024-2061

Hakim, A. (2024, February 22). Hypermobile Ehlers-Danlos syndrome. In GeneReviews®. University of Washington, Seattle. https://www.ncbi.nlm.nih.gov/books/NBK1279/

Kang, J., Hanif, M., Mirza, E., & Jaleel, S. (2020). Ehlers-Danlos syndrome in pregnancy: A review. European Journal of Obstetrics & Gynecology and Reproductive Biology, 255, 118–123. https://doi.org/10.1016/j.ejogrb.2020.10.033

Almheiri, S., ElMubarak, A., & Fadlalsaed, O. (2026). Pregnancy outcomes in women with vascular Ehlers-Danlos syndrome: A systematic review of maternal and fetal complications. Cureus, 18(8), Article e114336. https://doi.org/10.7759/cureus.114336

van den Bersselaar, L. M., van de Laar, I. M. B. H., Baars, M. J. H., Baas, A., et al. (2026). Pregnancy and delivery outcomes in vascular Ehlers-Danlos syndrome: A retrospective multicentre cohort study. BJOG: An International Journal of Obstetrics & Gynaecology, 133, 463–470. https://doi.org/10.1111/1471-0528.18142

De Backer, J., Haugaa, K. H., Hasselberg, N. E., et al. (2025). 2025 ESC guidelines for the management of cardiovascular disease and pregnancy. European Heart Journal, 46(43), 4462–4568. https://doi.org/10.1093/eurheartj/ehaf193

Benistan, K., & Guichou, G. (2026). Les syndromes d’Ehlers-Danlos: Diagnostic et parcours de soins de ces maladies complexes et multisystémiques [Ehlers-Danlos syndromes: Diagnosis and care pathway of these complex and multisystemic diseases]. La Revue de Médecine Interne, 47(1), 32–39. https://doi.org/10.1016/j.revmed.2025.08.002

Ogawa, S., Mariya, T., Fujibe, Y., et al. (2022). Twin pregnancy with untyped Ehlers-Danlos syndrome requiring prompt genetic testing: A case report. Case Reports in Women’s Health, 33, Article e00384. https://doi.org/10.1016/j.crwh.2022.e00384