pn Perinatology Perinatology 2508-4887 2508-4895 The Korean Society of Perinatology 10.14734/PN.2017.28.04.166 pn-28-166 Case report The Vascular Type Ehlers-Danlos Syndrome Diagnosed after Delivery KimSu-MiMD KangYun-DanMD Department of Obstetrics and Gynecology, Dankook University College of Medicine, Cheonan, Korea Correspondence to Yun-Dan Kang, MD Department Obstetrics and Gynecology, Dankook University College of Medicine, 119 Dandae-ro, Dongnam-gu, Cheonan 31116, Korea Tel: +82-41-550-3792 Fax: +82-41-556-3878 E-mail: yundan76@dankook.ac.kr 012017 19012017 284166 170 01082017 29082017 09092017 Copyright © 2017 The Korean Society of Perinatology 2017 This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

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.

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

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).

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).

Diagnostic Criteria for Vascular Ehlers-Danlos Syndrome (vEDS)7

Major criteria
1. Family history of vEDS with documented causative variant in COLA3A1
2. Arterial rupture at a young age
3. Spontaneous sigmoid colon perforation in the absence of known
diverticular disease or other bowel pathology
4. Uterine rupture during the third trimester in the absence of previous
cesarean section and/or Severe peripartum perineum tears
5. Carotid-cavernous sinus fistula formation in absence of trauma
Minor Criteria
1. Bruising unrelated to identified trauma and/or in unusual sites such as
cheeks and back
2. Thin, translucent skin with increased venous visibility
3. Characteristics facial appearance
4. Spontaneous pneumothorax
5. Acrogeria
6. Talipes equinovarus
7. Congenital hip dislocation
8. Hypermobility of small joints
9. Tendon and muscle rupture
10. Keratoconus
11. Gingival recession and gingival fragility
12. Early onset of varicose veins (under age 30 and nulliparous if female)