Curr Hepatology Rep (2017) 16:406–415 https://doi.org/10.1007/s11901-017-0382-6 PORTAL HYPERTENSION (E TSOCHATZIS AND J ABRALDES, SECTION EDITORS) The Management of Bleeding from Anorectal Varices Marcus Robertson1,2 & Alexandra Ines Thompson1 & Peter Clive Hayes1 Published online: 7 November 2017 # The Author(s) 2017. This article is an open access publication Abstract alternative first-line treatments; all methods offer a technically Purpose of Review The purpose of this review is to summa- simple and efficacious method of achieving haemostasis, and rize available strategies for the diagnosis and management of local expertise will determine which procedure is employed. bleeding anorectal varices. Recent Findings Interventional radiological procedures, in- Keywords Anorectal varices . Endoscopy . Cirrhosis . Portal cluding TIPS, BRTO and/or embolization, have been hypertension . Band ligation . Sclerotherapy established as efficacious treatments, particularly in the setting of treatment failure. Summary Anorectal varices are prevalent in patients with por- Introduction tal hypertension. Acute bleeding is uncommon, but can be massive and life-threatening. Anorectal varices should be con- Variceal bleeding is a common and life-threatening manifes- sidered as a differential diagnosis in any patient with cirrhosis tation of portal hypertension and remains an important cause or portal hypertension who presents with lower gastrointesti- of death in patients with cirrhosis [1]. Esophago-gastric vari- nal bleeding. No evidence-based guidelines exist to guide the ces are by far the most common cause of acute variceal bleed- management of bleeding anorectal varices, which typically ing (AVB), the management of which is well-established and requires a multidisciplinary team of endoscopists, evidence-based. Ectopic varices are defined as dilated hepatologists, surgeons and interventional radiologists. portosystemic collateral veins occurring anywhere in the gas- Administration of prophylactic antibiotics and vasoactive trointestinal tract other than the esophago-gastric region and therapy is recommended based on efficacy in bleeding oe- include duodenal, jejunal, ileal, stomal, colonic and anorectal sophageal varices. Urgent endoscopy should be performed varices [2]. Ectopic varices constitute 2–5% of all variceal in all patients. Endoscopic sclerotherapy has the greatest body bleeds but are the cause of bleeding in 20–30% of patients of evidence and should be considered the first-line therapy; with extrahepatic portal hypertension [3–5]. Although rare, efficacy and safety may be increased if this is performed with acute ectopic variceal bleeding can be massive and life-threat- endoscopic ultrasound. Endoscopic banding or obturation are ening. The diagnosis and management of bleeding ectopic varices remains challenging, and their anatomical diversity Marcus Robertson and Alexandra Ines Thompson joint first-author makes development of standardized guidelines extremely difficult. This article is part of the Topical Collection on Portal Hypertension Anorectal varices represent portal-systemic collaterals * Peter Clive Hayes manifesting as discrete, dilated submucosal veins, extending [email protected] proximal to the dentate line and into the rectum [6]. Bleeding rectal varices typically present as hematochezia; an acute 1 Department of Hepatology, Royal Infirmary of Edinburgh, bleed from anorectal varices is rare but may result in life- Edinburgh, UK threatening haemorrhage. This review aims to summarize 2 Department of Gastroenterology, Monash Health, available strategies for the diagnosis and management of Melbourne, Australia bleeding anorectal varices. Curr Hepatology Rep (2017) 16:406–415 407 Pathophysiology, Aetiology and Prevalence endoscopic treatment of esophageal varices [23]. There is con- of Anorectal Varices flicting evidence regarding the occurrence of rectal varices following treatment of esophageal varices. Some studies have Portal hypertension is a well-recognized and common mani- suggested that injection sclerotherapy or band ligation of festation of chronic liver disease. In portal hypertension, a esophageal varices may influence the natural history of ectop- combination of increased splanchnic blood flow and ic varices. In the case of anorectal varices, this is postulated to intrahepatic resistance to portal blood flow leads to a patho- be due to development of collateral vessels in the inferior logical increase in portal pressures, which results in the devel- mesenteric venous system following obliteration of supplying opment of portosystemic collaterals, the most clinically sig- vessels (such as the left gastric, posterior gastric or short gas- nificant of which are gastroesophageal varices [7]. The hepatic tric veins) which ultimately leads to the formation of rectal venous pressure gradient (HVPG) is a useful clinical measure varices [11, 23, 24 ]. The correlation between treatment of of portal pressures and is defined as the gradient between the esophageal or gastric varices and subsequent formation of wedged hepatic venous pressure and the free hepatic venous anorectal varices has not been conclusively demonstrated. pressure [8–10]. The HVPG can be used to risk stratify pa- Despite the high prevalence of anorectal varices, massive tients with portal hypertension, although in practice it is not haemorrhage remains an uncommon event and studies report frequently performed due to the invasiveness of the procedure. a frequency ranging from 0.5 to 3.6% [25]. The prevalence of The normal HVPG ranges between 1 and 5 mmHg and a haemorrhage from anorectal varices is significantly increased portal pressure gradient of ≥ 12 mmHg is well established as in larger varices and in patients with high-risk stigmata such as the baseline elevated pressure above which variceal develop- a positive “red colour” sign [26]. There is no evidence to ment and bleeding may occur. suggest that the incidence of bleeding is increased with the Anorectal varices represent porto-systemic collateral ves- presence of oesophageal variceal bleeding, a history of previ- sels that constitute a pathway for portal venous blood flow ous treatment of oesophageal varices or the aetiology of portal between the superior rectal veins of the inferior mesenteric hypertension [6, 26]. system and the middle inferior rectal veins of the iliac system [2, 11, 12]. They manifest as dilated and engorged submucosal veins in the rectum [6]. Anorectal varices most commonly Clinical Presentation, Diagnosis and Evaluation result from portal hypertension secondary to cirrhosis [13]. of Anorectal Varices A variety of conditions that result in non-cirrhotic portal hy- pertension are also associated with the development of Bleeding anorectal varices most commonly presents as anorectal varices [14], including mesenteric [15–17]orsplen- hematochezia (bleeding per rectum), which is typically acute ic vein obstruction [18] from carcinoid syndrome or pancrea- or chronic recurrent. The diagnosis of anorectal varices is titis respectively, along with cavernous malformation of the usually made at endoscopy (Fig. 1); however, endoscopic ul- portal vein. In addition, systemic conditions such as conges- trasound (EUS) and imaging have also been employed. tive heart failure or congenital vascular anomalies have been postulated as potential causes of anorectal varices [6]. Endoscopy The incidence of anorectal varices varies widely between studies, ranging from 38 to 95% [19, 20]. In patients with Diagnosis of anorectal variceal bleeding is primarily achieved established liver cirrhosis, prevalence ranges from 38 to at endoscopy (either flexible sigmoidoscopy or colonoscopy) 92% [19, 21]. Multiple studies have suggested a higher prev- [25], which remains the gold-standard investigation. alence of anorectal varices in patients with non-cirrhotic portal Anorectal varices typically appear as blue-tinted, serpentine, hypertension or extra-hepatic portal vein obstruction (63– sub-mucosal varicose veins located near the anus, which al- 95%) and varices were significantly larger in this cohort [19, ways cross the dentate line to extend cranially and can also 20]; no identifiable causes have been found to explain these extend into the rectum [6, 11, 22]. Diagnosis can be difficult in differences [6]. In addition, Hosking et al. demonstrated in a the setting of massive gastrointestinal bleeding and a high- prospective trial that the incidence of anorectal varices may be index of suspicion is vital whenever a patient with known related to duration of portal hypertension, with a 19% inci- portal hypertension presents with rectal bleeding. Patients dence in patients with early cirrhosis, increasing to 59% in with anorectal varices may also have other lower gastrointes- patients with a long duration of portal hypertension [22]. tinal sources of bleeding, and thus, a colonoscopy may be A large study by Watabane et al. found that anorectal var- required to accurately locate the bleeding point. ices were the most common site of ectopic varices, constitut- A critical task at endoscopy is to differentiate between ing 45% of the cohort. Furthermore, patients with rectal vari- anorectal varices and haemorrhoids, which can be difficult in ces were frequently noted to have a history of esophageal the setting of active bleeding. Unlike anorectal varices, the varices (94.8%) and the majority (87%) had received prevalence of haemorrhoids is not increased in patients with 408 Curr Hepatology Rep (2017) 16:406–415