Left-Sided Portal Hypertension Caused by an Accessory Spleen

Abstract

Purpose: Left-sided portal hypertension (LSPH) is a localized form of portal hypertension that usually occurs as a result of isolated obstruction of the splenic vein. The incidence of LSPH has increased over the past three decades due to increased awareness of this entity. Most cases are secondary to splenic vein thrombosis. Nonetheless, we are reporting a very unusual cause for LSPH. Case: A 55 year old female presented to the Emergency room with three days history of hematemsis, melena and weakness. She has remote past history of alcohol abuse, however she denied any similar episodes in the past. On presentation she was hypotensive and tachycardic, otherwise the physical examination was essentially unremarkable. In particular, there was no stigmata of chronic liver disease. Her initial hemoglobin was 5.4 (g/dL) and she had normal platelet count. Her liver function tests and INR were within the reference ranges. She was resuscitated with crystalloids and packed red blood cells transfusion. The initial gastroscopy showed isolated gastric varices with stigmata of recent hemorrhage. An ultrasound of her abdomen with doppler showed no hepatic masses, patent hepatic vasculature and splenic vein. A computed tomography of her abdomen showed normal liver and pancreas, patent splenic, hepatic, portal veins without evidence of hepatic steatosis. However, it showed a 6.5 × 5.9 cm rounded mass in the left upper quadrant anterior to the pancreatic tale, most consistent with an accessory spleen. The hepatobiliary surgery team was consulted and the patient underwent laparoscopic resection of the accessory spleen with ligation of the short gastric veins. The patient had an uneventful postoperative course without recurrence of bleeding. A repeat gastroscopy in six months showed disappearance of the gastric varices. Conclusion: Most cases of LSPH are related to splenic vein thrombosis in the context of chronic pancreatitis. However, in this case the LSPH was due to the presence of an accessory spleen. The venous drainage of the accessory spleen into the short gastric veins is probably the reason for development of the varices. To our knowledge, no similar cases have been reported in the literature.Figure: CT abdomen showing the accessory spleen.