Chronic abdominal pain: Budd-Chiari syndrome with converging pathologies

Chronic abdominal pain can be diagnostically challenging, especially when vascular causes like Budd-Chiari Syndrome (BCS) are overlooked. BCS, often linked to prothrombotic conditions such as protein S deficiency, may be further complicated by anatomical anomalies like May-Thurner Syndrome (MTS), leading to significant hepatic congestion and morbidity. This case highlights the need to consider vascular etiologies in patients with hypercoagulable risk factors to ensure timely diagnosis and intervention.

A 21-year-old Caucasian female with a complex history including type 1 diabetes mellitus (DM1), protein S deficiency, and prior pulmonary embolism presented with progressive right upper quadrant abdominal pain, early satiety, bloating, and edema. Imaging and venography revealed BCS due to hepatic venous congestion, as well as MTS with chronic thrombus in the left iliac vein and inferior vena cava. She underwent successful iliac vein stenting, which initially improved her symptoms, but later experienced recurrent pain linked to biliary dyskinesia. Gallbladder ultrasound and hepatobiliary iminodiacetic acid (HIDA) scan revealed wall thickening, sludge, and reduced ejection fraction, leading to laparoscopic cholecystectomy. Her symptoms significantly improved postoperatively, highlighting the role of overlapping vascular and hepatobiliary pathologies in chronic abdominal pain.

This case highlights the importance of evaluating vascular causes in young patients with chronic abdominal symptoms, especially when thrombophilia is present. Early identification and coordinated management of BCS, MTS, and associated complications can prevent serious hepatic consequences. A multidisciplinary, vascular-focused approach was essential in achieving a positive outcome in this diagnostically complex presentation.