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

Abstract

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.

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