Isolated visceral arteriopathies of the celiac and hepatic artery are rare. We present a case of a Caucasian man who presented with abdominal pain and was found to have a spontaneous celiac artery dissection. Genetic analysis revealed a mutation consistent with Ehlers-Danlos syndrome type IV. The patient died 2 months later from a spontaneous rupture of his hepatic artery.
CASE REPORTA 31-year-old man with fragile, easily bruisable skin, abnormal (atrophic) scarring, joint hypermobility, and extensive varicosities of the legs presented with sudden onset of left upper quadrant abdominal pain. His blood pressure was 146/75 mm Hg and heart rate, 114 beats/ min. The epigastric region was tender and the left upper quadrant of his abdomen was soft. His joints were hypermobile, his skin over the chest was translucent, and severe varicose veins were present on his lower torso and lower extremities. A complete blood count, basic metabolic panel, liver function tests, activated partial thromboplastin time, prothrombin time/international normalized ratio, and amylase and lipase levels were within normal limits (Table). An electrocardiogram showed only sinus tachycardia, and an echocardiogram was normal. Computed tomography (CT) of the abdomen revealed high-density fluid around the pancreas and spleen. A serologic workup for a vasculitis was negative.Due to a normal amylase and lipase level and persistent abdominal pain, a CT angiogram of the thorax and abdomen was performed and revealed a celiac artery dissection with associated thrombus extending into the splenic artery (Figure). Th e spleen was infarcted. Th e patient was managed with anticoagulation by continuous heparin infusion and metoprolol. He responded well to conservative management and was discharged home. Th ere was a clinical suspicion of Ehlers-Danlos syndrome (EDS) type IV. Th e patient's paternal grandmother also had extensive varicosities. Molecular studies revealed a mutation in the COL3A1 gene, which is consistent with the diagnosis of EDS type IV.Approximately 2 months after hospital discharge, the patient complained once again of sudden onset of severe abdominal pain. As he and his wife were on their way to the car to drive to the emergency department, he collapsed and died. Autopsy revealed a ruptured hepatic artery aneurysm with 2.5 to 3 L of fresh blood in the peritoneal cavity. Th eir infant son did not have the COL3A1 mutation.
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