Isolated inferior mesenteric portal hypertension with giant inferior mesenteric vein and anomalous inferior mesenteric vein insertion
A 4-year-old girl presented with chronic anemia and malena. She was treated with multiple transfusions. The physical examination just revealed palpable spleen. The hemoglobin was 4 gm%, and platelet count, liver function tests were normal. The abdominal Doppler ultrasonography revealed thrombosis of portal vein with collaterals around portal hilum and mild splenomegaly. The thrombus in the portal vein was extending up to the liver hilum.\nThe patient was prepared for the surgery. The abdomen was explored by transverse supraumblical incision. It revealed giant inferior mesenteric vein measuring more than 3 cm with collaterals around the cecum and rectum []. Inferior mesenteric vein was found joining the superior mesenteric vein at confluence with the portal vein. The splenic vein was less than 8 mm, mild splenomegaly, and no perisplenic collaterals. Since consent for internal jugular vein graft for Rex shunt was not available and renal vein was too small, an inferior mesenterico-caval shunt was performed, in addition to splenectomy. The child was relieved of GI bleed postoperatively. No symptoms of encephalopathy and no evidence of overwhelming postsplenectomy sepsis were observed during the follow-up period of eight months.