Type IV Ehlers-Danlos Syndrome: A Surgical Emergency? A Case of Massive Retroperitoneal Hemorrhage

Stephen G Chun1, 2, , *, Patrick Pedro1, , Mihae Yu1, Danny M Takanishi1
1 Department of General Surgery, John A. Burns School of Medicine, University of Hawaii at Manoa, Honolulu, HI
2 Cancer Research Center of Hawaii, University of Hawaii at Manoa, Honolulu, HI

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© Chun et al.; Licensee Bentham Open.

open-access license: This is an open access article licensed under the terms of the Creative Commons Attribution Non-Commercial License ( which permits unrestricted, non-commercial use, distribution and reproduction in any medium, provided the work is properly cited.

* Address correspondence to this author at the Department of Surgery, John A. Burns School of Medicine University of Hawaii at Manoa 1356 Lusitana Street, University Tower 6th Floor Honolulu, HI 96813; Tel: 214-645-8525; Fax: 214-645-8526; E-mail:
These authors contributed equally to the production of this manuscript.


Retroperitoneal hemorrhagic bleeding is a known manifestation of Type-IV Ehlers-Danlos Syndrome that is caused by loss-of-function mutations of the pro-alpha-1 chains of type III pro-collagen (COL3A1) resulting in vascular fragility. A number of previous reports describe futile surgical intervention for retroperitoneal bleeding in Type-IV Ehlers-Danlos Syndrome with high post-operative mortality, although the rarity of retroperitoneal bleeding associated with Type-IV Ehlers-Danlos Syndrome precludes an evidence-based approach to clinical management. We report a 23-year-old male with history of Type-IV Ehlers-Danlos Syndrome who presented with severe abdominal pain and tachycardia following an episode of vomiting. Further work-up of his abdominal pain revealed massive retroperitoneal bleeding by CT-scan of the abdomen. Given numerous cases of catastrophic injury caused by surgical intervention in Type-IV Ehlers-Danlos Syndrome, the patient was treated non-operatively, and the patient made a full recovery. This case suggests that even in cases of large retroperitoneal hemorrhages associated with Ehlers-Danlos Syndrome, it may not truly represent a surgical emergency.

Keywords: Type-IV Ehlers-Danlos Syndrome, retroperitoneal hemorrhage, conservative management, non-operative, COL3A1, connective tissue disorder..