From the Guidelines
Cervical cerclage in patients with Ehlers-Danlos syndrome should be approached with caution due to the risks associated with tissue fragility and potential complications during labor and delivery. Considering the connective tissue abnormalities inherent to Ehlers-Danlos syndrome, careful evaluation of the benefits and risks of cerclage placement is necessary. According to the 2022 ACC/AHA guideline for the diagnosis and management of aortic disease 1, shared decision-making is essential, and complications may occur after vaginal or cesarean deliveries, highlighting the need for a personalized approach.
Key considerations for cervical cerclage in Ehlers-Danlos syndrome patients include:
- Tissue fragility, which complicates labor and delivery and poses risks for vascular events and wound complications 1
- The potential for increased risk of cervical insufficiency and preterm birth, particularly in hypermobility and vascular types of Ehlers-Danlos syndrome
- The need for an experienced surgeon familiar with Ehlers-Danlos syndrome to perform the procedure, ideally between 12-14 weeks gestation
- The use of non-absorbable suture material, such as Mersilene tape, and careful consideration of anesthesia options, with regional anesthesia often preferred but requiring careful administration due to potential resistance to local anesthetics in Ehlers-Danlos syndrome patients.
A multidisciplinary approach involving maternal-fetal medicine specialists, geneticists, and anesthesiologists is crucial for optimal outcomes, and patients should be counseled about activity restrictions, signs of preterm labor, and the potential need for cesarean delivery depending on their specific Ehlers-Danlos syndrome subtype and obstetric history. The decision to perform cervical cerclage should be made on a case-by-case basis, taking into account the individual patient's risk factors and medical history, and considering the potential benefits and risks of the procedure, as well as the need for close monitoring for complications.
From the Research
Considerations for Cervical Cerclage in Ehlers-Danlos Syndrome
- The decision to perform a cervical cerclage in patients with Ehlers-Danlos syndrome should be made on a case-by-case basis, taking into account the individual's specific condition and pregnancy history 2.
- Cervical cerclage may be considered in patients with Ehlers-Danlos syndrome who are at risk of cervical insufficiency or preterm birth, as it may help to prevent these complications 3.
- The Shirodkar and McDonald techniques are two common methods of cerclage, and the choice of technique may depend on the individual patient's needs and circumstances 4, 3.
- Patients with Ehlers-Danlos syndrome may be at increased risk of complications during pregnancy, including preterm birth and fetal growth restriction, and close monitoring and management may be necessary to minimize these risks 2.
- The use of cerclage in patients with Ehlers-Danlos syndrome should be carefully considered, as it may not always be effective in preventing preterm birth or other complications, and may be associated with its own risks and complications 5.
Risks and Complications
- Patients with Ehlers-Danlos syndrome who undergo cervical cerclage may be at risk of complications such as premature rupture of membranes, chorioamnionitis, and maternal complications such as cervicovaginal fistula and deep vein thrombophlebitis 5.
- The risk of complications may be higher in patients with Ehlers-Danlos syndrome due to their underlying condition, and close monitoring and management may be necessary to minimize these risks 2.
Indications for Cerclage
- Cervical cerclage may be indicated in patients with Ehlers-Danlos syndrome who have a history of preterm birth or cervical insufficiency, or who are at risk of these complications due to their underlying condition 3.
- The decision to perform a cerclage should be based on individual patient factors, including their medical history, pregnancy history, and current pregnancy status 4, 3.