Ectopic varices are defined as dilated portosystemic collateral veins located in unusual sites other than the gastro-esophageal region 1 and constitute 1% to 5% of all variceal bleeds in patients with intrahepatic portal hypertension and 20% to 30% of those with extrahepatic portal hyperten-sion. 2,3 Ectopic varices represent a clinical challenge because they are difficult to localize, and at present, there are no clear guidelines on the management of ectopic varices due to the diversity of presentation and absence of randomized controlled trials. Recommendations for management are based on various case series and case reports and on individual experience based on local expertise. Hence, algorithms are needed for the treatment and stepwise management of patients with ectopic varices. Etiology and Prevalence of Ectopic Varices There is considerable heterogeneity in the epidemiology of ectopic varices because of the modality of detection, etiology of portal hypertension, and significant interobserver variability. 4,5 In a study by Norton et al., 3 out of 169 patients with variceal bleeding due to ectopic varices, 17% occurred in the duodenum, 17% occurred in the jejunum or ileum, 14% occurred in the colon, 8% occurred in the rectum, 9% occurred in the peritoneum, and a few were located in other rare sites (e.g., the vagina and the ovaries). The authors also found that duodenal varices were common in patients with extrahepatic portal thrombosis on upper gastrointestinal (GI) endoscopy, and 26% of patients with peristomal varices had bled during their study. In another large study of 173 patients, Watanabe et al. 6 found that the mean age of patients with ectopic varices was 62.3 years; 32.9% of patients had duodenal varices, 4% had varices in the jeju-num, 1.2% had varices in the ileum, and 3.5% had varices in the colon, and contrary to the previous study, 44.5% of patients had rectal varices and only 5.8% had peristomal varices. Another study by Stephan and Miething 7 showed that the prevalence of duodenal varices was 40% in patients with cirrhosis undergoing angiography. Misra et al. 8 showed an 18% prevalence of ileal varices in patients with cirrhosis undergoing ileocolonoscopy. Colonic varices have been reported in 3.4% of patients with intrahepatic portal hyper-tension, 9,10 and anorectal varices have been reported in 10%
CITATION STYLE
Sarin, S. K., & Kumar, C. K. N. (2012). Ectopic varices. Clinical Liver Disease, 1(5), 168–172. https://doi.org/10.1002/cld.95
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