Management of spontaneous liver hemorrhage in vascular Ehlers Danlos syndrome: A case report

Vascular Ehlers Danlos syndrome (vEDS) is a heritable connective tissue disorder characterized by vascular, solid organ, and hollow viscus fragility. Herein we report a patient with vEDS who presented with a large spontaneous subcapsular liver hemorrhage. This case highlights the challenges associated with managing vEDS patients and discusses approaches to optimize their care.

A 33-year-old woman presented to the emergency department with acute onset abdominal pain. Computed Tomography Angiogram (CTA) revealed a large subcapsular hemorrhage with active extravasation. Interventional Radiology (IR) performed a hepatic artery angiogram which revealed no blush thus embolization was not performed. The closure device pulled through the arterial wall and an expanding groin hematoma was noted despite manual compression. The patient was brought emergently to the Operating room (OR) for left groin exploration where the arterial injury was identified and repaired. Her post-operative course was complicated by pulmonary embolism (PE) and a left groin infection requiring return to the OR.

In the rarely reported cases of hepatic hemorrhage with vEDS, the patients have more commonly presented with multiorgan involvement or died during their hospital admission. These patients have extremely friable tissues and pose additional risk of complications for any invasive procedure.

The case described is the second reported instance of a patient with vEDS who survived their acute hospital course after presenting with a spontaneous hepatic hemorrhage. Key management considerations include preservation of the liver capsule to allow for spontaneous hematoma resorption and cutdown access in the OR for angiography.