Abstract

INTRODUCTION: Budd-Chiari syndrome (BCS) is defined as hepatic venous outflow tract obstruction. Chronic BCS eventually leads to post-sinusoidal hypertension resulting in the symptoms of the disease. There have been case reports of BCS leading to intra-abdominal bleeding from ectopic varices, however, these are cases of venous bleeding. Here, we report a unique case of a Chinese woman with BCS presenting with bleeding from arterial aneurysms. CASE DESCRIPTION/METHODS: A 61-year-old woman with BCS requiring multiple prior porto-systemic shunting procedures was brought by EMS for coffee ground emesis. At the ED, she was stuporous and developed bloody emesis and hypotension. She was intubated and transferred to the ICU. Exam was remarkable for abdominal distention (AD) with a fluid wave. Labs showed leukocytosis; prolonged coags, elevated troponins, lactate, LDH and decreased fibrinogen and haptoglobin. FAST showed intra-abdominal free fluid. EGD showed grade II varices in the esophagus that were banded. However, the patient continued to deteriorate with increasing AD. CT-AP revealed a cirrhotic liver with occluded left and middle hepatic veins and extravasation of contrast near the greater curvature of the stomach into a LUQ hematoma (Figure 1). IR embolized the left gastric artery and identified aneurysms along a branch of the SMA (Figure 2) in addition to an aneurysm at the celiac artery origin (Figure 3). On the 2nd post-op day, the patient again developed hypotension with AD. Attempted embolization of the SMA branch was unsuccessful. On the 5th post-op day, the family agreed to withdrawal of life support and the patient was extubated and vasopressors were stopped. The patient passed away in a few hours. DISCUSSION: Chronic BCS presents with complications of cirrhosis; i.e., ascites and rupture of esophageal varices, like in our patient. On CTA, both the hepatic veins and part of the IVC were occluded. In fact, 31% of BCS patients have outflow occlusion at both these locations. However, in our patient, CTA revealed aneurysms in the celiac artery and distal portions of an SMA branch along with left gastric artery bleeding. Multiple arterial aneurysms have been associated with disorders such as PAN, Behcet's, Ehler-Danlos etc. By comparison, there have not been many reports of multiple idiopathic arterial aneurysms in adults. Several of these possible causes were unable to be worked up for due to the acute presentation, although our patient’s history was not typical for these diseases.

Full Text
Published version (Free)

Talk to us

Join us for a 30 min session where you can share your feedback and ask us any queries you have

Schedule a call