Familial hepatic rupture in vascular Ehlers-Danlos syndrome in pregnancy with atypical thromboses

Jesal Patel; Cai Neville; Raj Kumar; Elisabeth Grey-Davies; Renata Hutt; Fleur S van Dijk; Li Yuan Chan; Edward Walter

doi:10.1177/20542704231215970

Familial hepatic rupture in vascular Ehlers-Danlos syndrome in pregnancy with atypical thromboses

JRSM Open. 2023 Dec 15;14(12):20542704231215970. doi: 10.1177/20542704231215970. eCollection 2023 Dec.

Authors

Jesal Patel¹, Cai Neville², Raj Kumar³, Elisabeth Grey-Davies⁴, Renata Hutt⁵, Fleur S van Dijk⁶, Li Yuan Chan⁴, Edward Walter¹

Affiliations

¹ Department of Intensive Care, Royal Surrey County Hospital, Surrey, UK.
² Department of Rheumatology, Royal Surrey County Hospital, Surrey, UK.
³ Department of General Surgery, Royal Surrey County Hospital, Surrey, UK.
⁴ Department of Haematology, Royal Surrey County Hospital, Surrey, UK.
⁵ Department of Obstetrics and Gynaecology, Royal Surrey County Hospital, Surrey, UK.
⁶ National EDS Service, London North West University NHS Trust, Harrow, UK.

Abstract

This case highlights the importance of genetic testing over fibroblast testing and presents the first published thromboelastometry data in vascular Ehlers-Danlos syndrome.

Keywords: Vascular Ehlers–Danlos syndrome; liver rupture; thromboelastometry; thrombosis.

Publication types

Case Reports