Portal hypertension due to jejunal vascular malformation

We report a case of hepatic portal venous hypertension secondary to a small-bowel vascular malformation in a 24-year-old man with anemia. Preoperative diagnostic evaluation included gastrointestinal barium studies and endoscopy (showing esophageal varices), hepatic panangiography, peroral small-bowel biopsy, percutaneous transhepatic portal venography, and manometry. Preoperative injection of methylene blue dye into the jejunal arteries supplying the malformation resulted in easy localization of the lesion at surgery. Direct manometry at the time of segmental jejunal resection yielded a portal pressure of 35 mmHg, with subsequent reduction to 21 mmHg.