Preparing for Postpartum Hemorrhage in Possible Ehlers-Danlos Syndrome

Critical Pre-Delivery Preparation

The American College of Radiology recommends obtaining genetic testing immediately if not already done to distinguish between hypermobile EDS and vascular EDS, as 26.4% of clinically diagnosed cases have alternative genetic conditions requiring different management 1
Pregnancy carries significant risk of uterine rupture and vessel rupture during delivery if vascular EDS is confirmed or suspected, with a COL3A1 variant 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

The American College of Radiology recommends coordinating care with vascular surgery, hematology, anesthesiology, maternal-fetal medicine, and genetics before delivery, as EDS requires specialized perioperative management to prevent complications 2
Interventional radiology should be on standby for potential arterial embolization if massive hemorrhage occurs 2

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
Delivery should be planned at a tertiary center with capability for emergent multidisciplinary mobilization 4

Invasive hemodynamic monitoring, such as arterial lines and central lines, should be avoided in vascular EDS due to risk of fatal vascular complications 1, 3

Aggressive active management of third stage of labor with uterotonics, such as oxytocin, should be implemented immediately after delivery to enhance uterine contraction and promote placental separation 5
Meticulous surgical technique with careful tissue handling should be used to prevent wound dehiscence and excessive bleeding from tissue fragility 2
Episiotomy should be avoided unless absolutely necessary, as perineal trauma can lead to excessive bleeding 5

Oxytocin should be administered intravenously as first-line therapy for uterine atony, as this is standard management and anticoagulants do not predispose to atonic bleeding 5
Never perform invasive vascular procedures or monitoring in confirmed vascular EDS due to risk of fatal complications, including arterial lines and central venous catheters 1, 3

Patients should be monitored closely for delayed postpartum hemorrhage, as tissue fragility and delayed wound healing increase this risk 6
Follow-up imaging should be ensured if vascular EDS is confirmed, as pregnancy-related vascular changes may persist 2