Case Report An Unusual Presentation of Type II Abernethy Malformation Jingbo Lu, Zhiqi Lin, Hao Liu, and Zhengjun Liu, Guangzhou City, Guangdong Province, China

Abernethy malformation is a rare anomaly of the splanchnic venous system. We report a case of an unusual portosystemic shunt via a dilated inferior mesenteric vein. A 20-year-old woman was referred to our hospital with complains of nonspecific abdominal pain for almost 3 years and hematochezia since 15 months old. Computed tomography and further transhepatic splenoportography revealed a hypoplastic portal vein and a giant inferior mesenteric vein, via which part of the portal venous blood drained into the inferior vena cava. The patient underwent a surgical ligation of the portocaval shunt and recovered well. We believe that this is the first case of type II Abernethy malformation presenting as a portosystemic shunt via the giant inferior mesenteric vein.

Abernethy malformation, also known as congenital extrahepatic portosystemic shunt (CEPS), is a rare anomaly of the splanchnic venous system in which portal blood is diverted into systemic veins by various kinds of CEPSs bypassing the liver. In 1793, Abernethy1 first described this congenital anomaly after a postmortem examination of a 10month-old girl. Until the end of 2011, there have only been 54 reported cases of the malformation.2 With tremendous advances in diagnostic medical imaging, CEPS has been recognized more frequently in recent years. Based on the patterns of these shunts, Morgan and Superina3 classified CEPS into 2 types. Type I is characterized by a portal vein that joins the inferior vena cava (IVC) in an endto-side fashion, leading to a complete diversion of portal venous blood into the IVC. Type II malformation consists of a side-to-side connection between

Vascular Surgery Department, Nanfang Hospital, Southern Medical University, Guangzhou City, Guangdong Province, China. Correspondence to: Zhengjun Liu, MD, Vascular Surgery Department, Nanfang Hospital, Southern Medical University, No. 1838, Guangzhou Avenue North, Baiyun District, Guangzhou City, Guangdong Province 510515, China; E-mail: [email protected] Ann Vasc Surg 2014; -: 1–4 http://dx.doi.org/10.1016/j.avsg.2014.01.017 Ó 2014 Elsevier Inc. All rights reserved. Manuscript received: July 20, 2013; manuscript accepted: January 17, 2014; published online: ---.

the portal vein and the IVC, so that there is still partial venous blood supplying the liver via a hypoplastic portal vein. Type I malformation may be further subclassified into Ia and Ib, defined as the superior mesenteric and splenic veins draining into the IVC separately (Ia) or from a common trunk (Ib). In this report, we present a patient diagnosed with an unusual pattern of type II Abernethy malformation. To the best of our knowledge, this is the first reported case of type II Abernethy malformation presenting as a portosystemic shunt via the giant inferior mesenteric vein.

CASE REPORT A 20-year-old woman was referred to our hospital with complains of nonspecific abdominal pain for almost 3 years. The pain was intermittent, irrelevant to eating, and mainly in the left lower quadrant of the abdomen, not associated with fever or vomiting. Since 15 months old, she suffered hematochezia until presentation. She had no history of umbilical sepsis but suffered jaundice in her perinatal period. The patient had no history suggestive of hepatic encephalopathy or hepatopulmonary syndrome (HPS). Physical examination presented no sign of icterus, no clubbing, cyanosis or, edema of arms or legs. She had a soft abdomen without organomegaly. No clinical ascites was noted. Routine blood examination revealed a hemoglobin level of 3.3 g/dL but leukocyte and platelets were within normal limits. Other laboratory 1

2 Case Report

Fig. 1. (A) Contrast-enhanced preoperative CT revealed a hypoplastic portal vein (black solid arrow) and hepatic and splenic cysts (black dotted arrows). (B) Contrastenhanced postoperative CT at 3-month follow-up

tests on presentation, such as liver profile, prothrombin time, serologic examinations of viral hepatitis, a-fetoprotein concentration, and serum ammonia, were all normal. Fibercolonscopy from the referring hospital showed severe internal hemorrhoids, and abdominal ultrasonography showed an anomalous portal vein. Computed tomography (CT) with contrast after admission revealed a normal-sized liver and a slightly enlarged spleen. In the caudate lobe and right lobe of the liver, there was nodular lesion, with both diameters

An unusual presentation of type II Abernethy malformation.

Abernethy malformation is a rare anomaly of the splanchnic venous system. We report a case of an unusual portosystemic shunt via a dilated inferior me...
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