How do I prepare for postpartum hemorrhage in a patient with possible Ehlers-Danlos syndrome?

Preparing for Postpartum Hemorrhage in Possible Ehlers-Danlos Syndrome

Given that bleeding in EDS results primarily from structural vascular and connective tissue defects rather than platelet dysfunction, preparation for postpartum hemorrhage must focus on minimizing vascular trauma, ensuring immediate access to uterotonics and hemostatic agents, and assembling a multidisciplinary team with blood products immediately available.

Critical Pre-Delivery Preparation

Determine EDS Subtype Risk Profile

• Obtain genetic testing immediately if not already done to distinguish between hypermobile EDS (lower vascular risk) and vascular EDS (life-threatening risk), as 26.4% of clinically diagnosed cases have alternative genetic conditions requiring different management 1
• If vascular EDS is confirmed or suspected (COL3A1 variant), pregnancy carries significant risk of uterine rupture and vessel rupture during delivery 2
• Baseline vascular imaging from head to pelvis should be performed if vascular EDS is suspected to identify pre-existing aneurysms or dissections that could rupture peripartum 2, 3
• Consider phenotype over genotype alone when variants of unknown significance are present—if the patient lacks clinical features of vascular EDS (no spontaneous arterial rupture, no family history of sudden death, no characteristic facial features), management can be less restrictive 4

Assemble Multidisciplinary Team

• Coordinate care with vascular surgery, hematology, anesthesiology, maternal-fetal medicine, and genetics before delivery, as EDS requires specialized perioperative management to prevent complications 2, 5, 6
• Ensure vascular surgery is immediately available for potential vessel rupture or hemorrhage that may require surgical intervention 5
• Have interventional radiology on standby for potential arterial embolization if massive hemorrhage occurs 2

Delivery Planning and Timing

Mode and Timing of Delivery

• For confirmed or suspected vascular EDS, most guidelines recommend planned cesarean delivery at 34-38 weeks to avoid spontaneous labor and minimize risk of uterine rupture, though this carries its own surgical risks 2
• For hypermobile EDS without vascular features, vaginal delivery with instrument assistance can be considered to minimize maternal pushing effort and reduce tissue trauma 4
• Plan delivery at a tertiary center with capability for emergent multidisciplinary mobilization 7
• Schedule delivery when full surgical and blood bank teams are available, avoiding nights and weekends 5

Anesthetic Considerations

• Neuraxial anesthesia (epidural/spinal) can be offered in hypermobile EDS or when vascular EDS is genetically possible but phenotypically absent 4
• For confirmed vascular EDS, general anesthesia may be safer to avoid vascular trauma from needle placement, though this remains controversial 5
• Avoid invasive hemodynamic monitoring (arterial lines, central lines) in vascular EDS due to risk of fatal vascular complications 3, 1

Hemorrhage Prevention Strategy

Active Management of Third Stage

• Implement aggressive active management of third stage of labor with uterotonics (oxytocin) immediately after delivery to enhance uterine contraction and promote placental separation, as this is the primary mechanism to control placental bed bleeding 7
• The hemostatic system is less important than sustained myometrial contractions for controlling placental bed hemorrhage, but tissue trauma bleeding will be exacerbated by structural defects 7

Minimize Tissue Trauma

• Use meticulous surgical technique with careful tissue handling to prevent wound dehiscence and excessive bleeding from tissue fragility 2
• Avoid episiotomy unless absolutely necessary, as perineal trauma can lead to excessive bleeding 7
• If cesarean delivery is performed, use pledgeted sutures for uterine closure to prevent tissue tearing 2

Hemostatic Agent Preparation

• Have desmopressin (DDAVP) and tranexamic acid immediately available, as these agents have shown marked benefit in achieving hemostasis during obstetric hemorrhage in vascular EDS 8
• Desmopressin works by releasing von Willebrand factor and may help compensate for platelet function abnormalities that correlate with bleeding severity in EDS 8
• Tranexamic acid prevents fibrinolysis and should be given early if hemorrhage occurs 8

Immediate Hemorrhage Management Protocol

First-Line Uterotonic Therapy

• Administer oxytocin intravenously as first-line therapy for uterine atony, as this is standard management and anticoagulants do not predispose to atonic bleeding 7
• If oxytocin fails, proceed immediately to carboprost tromethamine (Hemabate) 250 mcg intramuscularly, which is FDA-approved for postpartum hemorrhage due to uterine atony unresponsive to conventional management 9
• Carboprost has resulted in cessation of life-threatening bleeding and avoidance of emergency surgical intervention in high proportion of cases 9
• Use carboprost cautiously in EDS patients with cardiovascular disease or hypertension, as 4% of patients experience increased blood pressure 9

Hemostatic Adjuncts

• Administer tranexamic acid 1 gram IV immediately if hemorrhage exceeds 500 mL or appears ongoing 8
• Consider desmopressin 0.3 mcg/kg IV for hemorrhage that does not respond rapidly to uterotonics, as this has shown marked benefit in vascular EDS obstetric hemorrhage 8
• These agents address the structural vascular defects and any concurrent platelet dysfunction that correlates with bleeding severity 8

Blood Product Strategy

• Have type-specific packed red blood cells, fresh frozen plasma, and platelets immediately available in the delivery room before delivery begins 5
• Activate massive transfusion protocol early if hemorrhage exceeds 1000 mL or continues despite uterotonic therapy 5
• Tissue fragility may cause ongoing bleeding from multiple sites that requires aggressive transfusion support 10

Critical Pitfalls to Avoid

• Never perform invasive vascular procedures or monitoring in confirmed vascular EDS due to risk of fatal complications—this includes arterial lines and central venous catheters 3, 1
• Do not delay administration of hemostatic agents (tranexamic acid, desmopressin) while waiting to see if uterotonics alone will work, as structural defects require additional hemostatic support 8
• Avoid assuming normal hemostasis based on normal platelet counts or coagulation studies, as the primary defect is structural vessel and tissue fragility, not measurable by standard tests 2
• Do not use opioids for postpartum pain management if patient has gastrointestinal manifestations of EDS 3, 1
• Recognize that chorioamnionitis may inhibit uterine response to carboprost, similar to other oxytocic agents, and may require earlier escalation to surgical management 9

Postpartum Surveillance

• Monitor closely for delayed postpartum hemorrhage (24 hours to 6 weeks postpartum), as tissue fragility and delayed wound healing increase this risk 7, 10
• Watch for wound dehiscence, which is common in EDS and may present with secondary hemorrhage 10
• Ensure follow-up imaging if vascular EDS is confirmed, as pregnancy-related vascular changes may persist 2