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Bilal Hameed, Uma Mahadevan, and Kay Washington, Section Editors
An Unusual Cause of Hematochezia Q2
Xiang-Yang Wang,* Zhi-Yuan Chen,* and Yu-Ming Yang Department of Gastroenterology, Hunan Provincial People’s Hospital, Hunan Normal University, Changsha, Hunan, China
Question: A 21-year-old man with a 3-year history of hematochezia presented to the gastroenterology department with drop bright red blood after defecation, which was aggravated after eating spicy foods. He denied any history of hepatitis. On physical examination, the patient had an anemic appearance. Laboratory tests showed severe anemia (hemoglobin, 2.8 g/dL), but normal coagulation function, serum ammonia, and liver function. Colonoscopy showed internal hemorrhoids with no other abnormalities. Contrast-enhanced computed tomography revealed large varicose dilatation of the splenic and superior mesenteric veins, pronounced dilatation of the inferior mesenteric vein (Figure A, arrow) and dilatation of the pelvic rectal venous plexus (Figure B, arrows). However, the intrahepatic portal veins and liver size were normal. The patient was diagnosed with hematochezia owing to internal hemorrhoids. What is the cause of internal hemorrhoids? See the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI.
*Authors share co-first authorsip. Q1
Conflicts of interest The authors disclose no conflicts. © 2017 by the AGA Institute 0016-5085/$36.00 http://dx.doi.org/10.1053/j.gastro.2017.01.039
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Answer to: Image 2: Abernethy Malformation Type II
Indirect portovenography showed that a large proportion of blood from the splenic and superior mesenteric veins flowed toward a shunt traveling through the inferior mesenteric vein (Figure C), the pelvic and rectal venous plexuses, and the iliac vein. The final diagnosis was Abernethy malformation type II. Abernethy malformation is the congenital absence of the portal vein, resulting in blood flow bypassing the liver to drain directly into the systemic veins, such as the inferior vena cava. This malformation was first reported in 1793.1 There are 2 types: type I, an end-to-side shunt (total absence of intrahepatic portal flow), and type II, a side-to-side shunt (with partially preserved portal flow).2 Complications of Abernethy malformations include hepatic encephalopathy, hepatic tumors, hepatopulmonary syndrome, and portopulmonary syndrome. Liver transplantation is the option for type I malformation with no intrahepatic portal vein; there is no standard treatment for a type II malformation. The most common treatment is either surgical or percutaneous transcatheter coil placement to occlude the shunts.3 Hemorrhoids are a rare complication of a type II malformation. In our case, hemorrhoids were formed owing to portal hypertension resulting in collateral circulation. To decrease vascular pressure and prevent the establishment of other collateral circulations, such as esophagogastric varices, the patient underwent inferior vena cava to inferior mesenteric vein bypass (Figure D), rather than discontinuation of the portocaval shunt. The patient recovered postoperatively, exhibiting no hematochezia. At follow-up, his routine blood test, blood ammonia level, and liver function were normal.
References 1. 2. 3.
Abernethy J. Account of two instances of uncommon formation in the viscera of the human body. Trans R Soc 1793; 83:59–66. Morgan G, Superina R. Congenital absence of the portal vein: two cases and a proposed classification system for portasystemic vascular anomalies. J Pediatr Surg 1994;29:1239–1241. Bernard O, Franchi-Abella S, Branchereau S, et al. Congenital portosystemic shunts in children: recognition, evaluation, and management. Semin Liver Dis 2012;32:273–287.
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