Management of Spontaneous Liver Hematoma in Ehlers-Danlos Syndrome Type IV: A Case Report.

Introduction: Liver hematoma is an uncommon feature of Ehlers-Danlos syndrome (EDS) type IV. The limited literature that exists to guide management does not establish a standard of care.

Case Presentation: A 26-year-old man presented with acute abdominal pain caused by a large, spontaneous liver hematoma. Invasive prophylactic arterial embolization was done twice, but surgical evacuation was not offered because of concern for poor healing and brittle vasculature, later diagnosed as symptoms of the patient's EDS type IV. During hospitalization the patient died of spontaneous intracerebral and intra-abdominal hemorrhaging.

Conclusion: This case illustrates a nonsurgical management option for spontaneous liver hematoma in a patient with EDS type IV. An interdisciplinary team should help guide care, including consideration of invasive procedures such as arterial embolization and surgery. Patient and family education, genetic testing, and timely medical record documentation may reduce the morbidity and mortality of patients with this syndrome.