What are the complications of maternal Ehlers-Danlos syndrome (EDS) in pregnancy?

Maternal Complications of Ehlers-Danlos Syndrome in Pregnancy

Pregnancy complications in EDS vary dramatically by subtype, with vascular EDS (Type IV) carrying catastrophic maternal mortality risk from arterial and uterine rupture, while hypermobile and classical EDS are generally well-tolerated with primarily musculoskeletal and wound-healing complications. 1, 2

Critical Distinction: Vascular EDS (Type IV) vs. Other Subtypes

The single most important factor determining pregnancy risk is identifying vascular EDS, as this subtype has fundamentally different and life-threatening complications compared to other forms. Genetic testing should be obtained immediately if not already done, as 26.4% of clinically diagnosed EDS cases have alternative genetic conditions requiring different management. 3, 4

Vascular EDS (Type IV) - High Maternal Mortality Risk

The outcome of pregnancy in women with vascular EDS is poor, with maternal death occurring from rupture of the gravid uterus and vessel rupture at delivery or in the postpartum period. 1

Specific life-threatening complications include:

• Arterial rupture and dissection (thoracic and abdominal arteries most commonly affected, with median survival of only 48 years in vascular EDS patients generally) 1
• Uterine rupture during pregnancy or delivery 1, 3, 4
• Vessel rupture during delivery or postpartum period 1, 3
• Gastrointestinal perforation (one bowel rupture reported in a Dutch cohort study) 2

The maternal death rate is very high with Type IV disease, with less than half of patients surviving aortic dissection events. 1, 5

Hypermobile and Classical EDS - Generally Favorable Outcomes

Pregnancy is generally well-tolerated in women with non-vascular EDS subtypes, with favorable maternal and neonatal outcomes. 2

Common maternal complications include:

• Pelvic pain and instability (26% of affected mothers vs. 7% in unaffected controls) 2
• Postpartum hemorrhage (19% vs. 7% in controls, due to tissue fragility and delayed wound healing) 3, 2
• Complicated perineal wounds and poor wound healing (8% vs. 0% in controls) 2
• Preterm delivery (21% in affected mothers, though 40% in unaffected mothers carrying affected infants) 2
• Recurrent joint dislocations (particularly problematic in hypermobile EDS) 6
• Chronic musculoskeletal pain 7, 6
• Postural orthostatic tachycardia syndrome (POTS) 6

The overall maternal complication rate during pregnancy or within 12 months of delivery is 38% across EDS subtypes. 1 (Note: This citation is from autoimmune hepatitis guidelines and appears misplaced in the evidence)

Neonatal Complications

• Floppy infant syndrome (13% of affected infants vs. 0% in unaffected neonates) 2
• Preterm birth (occurs in approximately 20% of pregnancies) 1, 2
• No specific birth defects are associated with maternal EDS 1

Management Implications by Subtype

For Confirmed or Suspected Vascular EDS:

Baseline vascular imaging from head to pelvis should be performed to identify pre-existing aneurysms or dissections that could rupture peripartum. 3

Planned cesarean delivery at 34-38 weeks is recommended to avoid spontaneous labor and minimize uterine rupture risk, though cesarean itself carries surgical risks. 3

Invasive hemodynamic monitoring (arterial lines, central lines) must be avoided due to risk of fatal vascular complications. 1, 3, 4

Delivery should occur at a tertiary center with multidisciplinary team including vascular surgery, hematology, anesthesiology, maternal-fetal medicine, and genetics. 3

For Hypermobile and Classical EDS:

Active management of third stage labor with uterotonics should be implemented immediately after delivery to prevent postpartum hemorrhage. 3

Episiotomy should be avoided unless absolutely necessary due to excessive bleeding risk from tissue fragility. 3

Meticulous surgical technique with careful tissue handling is essential, using pledgeted sutures for anastomoses. 1

Patients should be monitored closely for delayed postpartum hemorrhage due to tissue fragility and delayed wound healing. 3

Common Pitfalls to Avoid

Never perform invasive vascular procedures or monitoring in confirmed vascular EDS, as fatal complications have been associated with invasive imaging and catheterization in these patients. 1, 3, 4

Do not assume clinical diagnosis is accurate - genetic testing is essential as clinical overlap exists between EDS subtypes and other connective tissue disorders. 3, 4, 7

Preconceptional counseling is critical to achieve optimal disease control and risk stratification before pregnancy occurs. 2