From the Guidelines
Individuals with Hypermobile Ehlers-Danlos Syndrome (hEDS) are at an increased risk during pregnancy due to potential complications such as tissue fragility, vascular events, and wound complications. Although the provided study 1 specifically discusses vascular Ehlers-Danlos syndrome, the principles of tissue fragility and potential for complications during labor and delivery can be applied to hEDS as well. Pregnancy in women with hEDS can exacerbate existing symptoms and introduce additional complications, including increased joint instability and pain, premature labor, rapid deliveries, postpartum hemorrhage, and pelvic floor dysfunction.
Key Considerations
- Tissue fragility complicates labor and delivery, posing risks for vascular events and wound complications, as noted in the context of vascular Ehlers-Danlos syndrome 1.
- Management of hEDS during pregnancy typically involves multidisciplinary care, including maternal-fetal medicine specialists, geneticists, cardiologists, and pain specialists.
- Physical therapy before and during pregnancy can help maintain joint stability, which is crucial for individuals with hEDS.
- Close monitoring throughout pregnancy is essential, with particular attention to blood pressure, cervical length, and fetal growth, to minimize risks and manage symptoms effectively.
Clinical Implications
Given the potential risks, a personalized care plan addressing the specific manifestations of hEDS is crucial for minimizing risks and managing symptoms effectively. This approach should be based on the most recent and highest quality evidence available, such as the 2022 ACC/AHA guideline for the diagnosis and management of aortic disease 1, while considering the unique aspects of hEDS. By prioritizing morbidity, mortality, and quality of life, healthcare providers can offer tailored guidance and support to individuals with hEDS throughout their pregnancy.
From the Research
Risks Associated with Hypermobile Ehlers-Danlos Syndrome (hEDS) in Pregnancy
- Individuals with hEDS may experience complications during pregnancy, including recurrent joint dislocations and history of surgical joint stabilization procedures, secondary autonomic pain, and postural orthostatic tachycardia syndrome 2.
- Pregnancy outcome in patients with hEDS is poorly documented, but cases of preterm premature rupture of membranes have been reported, highlighting the risk of severe prematurity 3.
- Patients with hEDS can have an increased risk for pregnancy complications, including prematurity due to cervical incompetence and premature rupture of membranes 3.
Management and Outcomes
- Increased awareness of hEDS and a multi-disciplinary approach to management can result in good outcomes for both mother and baby 2.
- Early diagnosis of EDS and subtype characterization can aid in pre-pregnancy counseling, planning of antenatal care, risk assessment of obstetric and neonatal complications, and influence both obstetric and anesthetic management 4.
- Obstetrical management should be individualized, as the variation in EDS manifestations can result in moderate to severe effects on life expectancy for some types 5.
- Cesarean section may be deemed the most appropriate delivery method in patients with hEDS due to possible complications, including risk of joint dislocation and pain morbidity 6.
Key Considerations
- Pregnant women with hEDS generally have a favorable outcome, but those with vascular EDS are more likely to suffer from severe maternal complications 4.
- Collaboration between the obstetrician and the medical geneticist is recommended in the obstetrical management of patients with hEDS 3.
- Standardized obstetric management guidelines are lacking due to the spectrum of clinical manifestation and overlap between phenotypes 6.