Perinatology.  2017 Dec;28(4):166-170. 10.14734/PN.2017.28.4.166.

The Vascular Type Ehlers-Danlos Syndrome Diagnosed after Delivery

• Kim SM
• Kang YD

• KMID: 2409126
• DOI: http://doi.org/10.14734/PN.2017.28.4.166

Abstract

Vascular Ehlers-Danlos sydrome (vEDS) is a life-threatening autosomal dominant inherited disorder of connective tissue characterized by arterial aneurysm, dissection and rupture, bowel rupture, and rupture of the gravid uterus. vEDS results from mutation of COL3A1, which encodes the chains of type III collagen, a major protein in vessel walls and hollow organs. A 32-year-old primigravida had aneurysmal rupture in right posterior popliteal artery 9 days after induced vaginal delivery. The reason of labor induction was preterm premature rupture of membrane. She was diagnosed with vEDS by sequence analysis of COL3A1 mutation. A multidisciplinary team is required to provide tailored counseling and education about vEDS. Also, a long term follow up is need for individuals with vEDS.

Keyword

COL3A1; Ehlers-Danlos syndrome; Type IV; Vascular type; Postpartum; Rupture

MeSH Terms

Adult
Aneurysm
Collagen Type III
Connective Tissue
Counseling
Education
Ehlers-Danlos Syndrome*
Follow-Up Studies
Humans
Membranes
Popliteal Artery
Postpartum Period
Rupture
Sequence Analysis
Uterus
Collagen Type III

Figure

• 

  Fig. 1 Computed tomography venography revealed aneurysmal rupture of right posterior tibial artery (arrow) with hematoma formation (arrowheads) without deep vein thrombosis.

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  Fig. 2 Axial scan of contrast-enhanced computed tomography (CT) angiography (A) showed aneurysmal dilatation of right proximal posterior tibial artery (arrow) with adjacent hematoma (arrowheads). CT angiography with three-dimensional reconstruction scan (B) also revealed aneurysmal dilatation of right proximal posterior tibial artery (arrow).

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  Fig. 3 Computed tomography (CT) angiography with three-dimensional reconstruction scan (A) showed luminal irregularity in common iliac artery, external iliac artery (arrowheads). Axial scan of contrast-enhanced CT angiography (B) also showed luminal irregularity of celiac trunk (arrow).

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