What are the pregnancy risks for a patient with Ehlers Danlos Syndrome (EDS) type 5?

Pregnancy Risks in Ehlers-Danlos Syndrome Type 5

EDS Type 5 (Classical EDS) carries moderate pregnancy risks including premature delivery, postpartum hemorrhage, and wound complications, but is significantly safer than vascular EDS (Type IV), which carries extreme maternal mortality risk from arterial or uterine rupture. 1, 2

Critical Risk Stratification by EDS Subtype

The most important first step is confirming the exact EDS subtype, as pregnancy risks vary dramatically:

• Vascular EDS (Type IV): Pregnancy carries extreme risk with maternal mortality rates approaching 25% from uterine or arterial rupture during pregnancy or delivery 1, 3, 4
• Classical EDS (Type 5): Generally favorable outcomes with manageable complications 2
• Hypermobile EDS: Most common type with good maternal-fetal outcomes but increased musculoskeletal symptoms 5

Maternal Risks in Classical EDS (Type 5)

Tissue Fragility Complications

• Postpartum hemorrhage should be anticipated due to tissue fragility and impaired wound healing 1, 6
• Wound dehiscence risk is elevated, requiring extended suture retention periods and prolonged antibiotic prophylaxis until suture removal 1
• Perineal tears may be more extensive and heal more slowly 2
• Pelvic organ prolapse (bladder and uterine) can occur postpartum due to connective tissue laxity 6

Musculoskeletal Complications

• Joint dislocations increase in frequency during pregnancy due to hormonal effects on already lax ligaments 5
• Chronic pain typically worsens, particularly in the second and third trimesters 7
• Pelvic girdle pain is common and may be severe 5

Cardiovascular Monitoring

• While arterial rupture is primarily a vascular EDS concern, baseline vascular imaging should still be considered to exclude undiagnosed vascular involvement 8
• Annual surveillance is not typically required for classical EDS unless vascular abnormalities are identified 8

Fetal and Neonatal Risks

• Premature delivery occurs in approximately 20% of pregnancies, often related to premature rupture of membranes from tissue fragility 6, 2
• No specific birth defects are associated with classical EDS 2
• Autosomal dominant inheritance means 50% risk of transmission to offspring, warranting genetic counseling 1

Pain Management During Pregnancy

First and Second Trimesters

• Nonselective NSAIDs are conditionally recommended for mechanical musculoskeletal pain 7
• NSAIDs are preferred over COX-2 inhibitors due to limited safety data on the latter 7

Third Trimester

• NSAIDs are absolutely contraindicated due to risk of premature ductus arteriosus closure 7
• Low-dose prednisone (≤10 mg daily) may be considered for inflammatory pain, though typically reserved for rheumatic rather than mechanical pain 7
• Opioids should be avoided for chronic pain management as they do not address underlying pathophysiology and carry significant risks 7

Non-Pharmacological Approaches

• Compression garments for joint support are recommended 7
• Physical therapy and joint protection strategies 5
• Brain-gut behavioral therapies for anxiety and psychological distress 7, 8

Delivery Planning

Mode of Delivery

• Vaginal delivery is generally safe in classical EDS, unlike vascular EDS where cesarean is typically performed 1
• Cesarean delivery should be reserved for standard obstetric indications 2
• If cesarean is performed, meticulous surgical technique with careful tissue handling is essential 8

Anesthetic Considerations

• Regional anesthesia is generally safe but requires awareness of joint hypermobility for positioning 2
• Tissue fragility may complicate invasive procedures 6

Delivery Room Preparation

• Active management of third stage to minimize postpartum hemorrhage risk 1
• Have uterotonics readily available 6
• Prepare for potential extensive perineal repair needs 2

Postpartum Management

• Extended wound monitoring for dehiscence, with sutures left in place longer than usual 1
• Prolonged antibiotic prophylaxis until suture removal if surgical delivery 1
• Monitor for pelvic organ prolapse development 6
• Increased joint instability may persist for months postpartum due to hormonal effects 5

Common Pitfalls to Avoid

• Do not assume all EDS types carry equal pregnancy risk - vascular EDS (Type IV) is life-threatening while classical and hypermobile types are generally manageable 1, 3
• Do not use NSAIDs in third trimester regardless of pain severity 7
• Do not rely solely on clinical diagnosis - genetic testing should confirm subtype to guide management 1, 8
• Do not perform invasive vascular procedures without careful consideration of tissue fragility 1, 8