PT  - JOURNAL ARTICLE
AU  - Catherine Gooch
AU  - Caitlin Wright
AU  - Katherine Nelson
AU  - Nathaniel Robin
TI  - Inherited cause of in utero digital malformations
AID  - 10.1136/bcr-2019-232020
DP  - 2020 Mar 01
TA  - BMJ Case Reports
PG  - e232020
VI  - 13
IP  - 3
4099  - http://casereports.bmj.com/content/13/3/e232020.short
4100  - http://casereports.bmj.com/content/13/3/e232020.full
SO  - BMJ Case Reports2020 Mar 01; 13
AB  - Amniotic band sequence (ABS) is common birth defect of incompletely understood origin. Here we describe a case of ABS in a child with paternally inherited Ehlers-Danlos syndrome, vascular type (vEDS). This is the third reported instance of ABS associated with paternally inherited vEDS in the medical literature. The two main theories of ABS formation are the extrinsic and intrinsic. The extrinsic theory states that placental tears form fibrous cords that wrap around the fetus; the intrinsic states that poor vascularisation in the fetus leads to necrosis of distal extremities. We believe this case supports extrinsic theory as it shows that as an amnion weakened by vEDS in fetal components is associated with ABS.