Management of Ostomy‐Related Varices

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Abbreviations

BRTO

balloon‐occluded retrograde transvenous obliteration

EGO

esophagogastroduodenoscopy

EUS

endoscopic ultrasound

GI

gastrointestinal

Hct

hematocrit

SV

stomal varix

TIPS

transjugular intrahepatic portosystemic shunt

Ostomy‐related varices, also called peristomal, parastomal, or stomal varices (SVs), develop at the enterocutaneous junction of a stoma. These varices represent venous shunting between the portal circulation of the small intestine or colon and the systemic circulation of the abdominal wall (Fig. 1), similar to the pathophysiology of caput medusae. ¹ , ² It is unclear whether these are more likely to develop in a patient who undergoes a bowel resection after developing portal hypertension or in a patient who has a long‐standing ostomy and later develops portal hypertension. The prevalence of SVs is largely reported based on active bleeding events because diagnosis based on visual or endoscopic assessment alone is limited. Bleeding rates range from 27% to 50%, with time to bleeding spanning between 1 month and 23 years after formation of the stoma. ³ , ⁴ , ⁵ In a study of 117 patients with chronic liver disease undergoing colectomy, 27% of patients with a stoma suffered from SV bleeding over a 5‐year period. In the same study, patients who underwent primary anastomosis after colectomy, as opposed to stoma creation, had no perianastomotic bleeding events. ³

FIG 1.

Imaging findings of ostomy‐related varices. (A) Cross‐sectional computed tomography image of SVs. Note the large branch vein, arising from the superior mesenteric vein, feeding into the stoma to create a variceal communication with the small systemic veins of the subcutaneous space. (B) Cross‐sectional computed tomography of SVs with sclerosant filling the variceal bed. (C) SVs under angiographic evaluation. There is a blush of small venous collaterals surrounding the stoma (large arrow) with a single dominant varix noted at the 11 o’clock position (arrowhead). There is an outflow tract through collaterals (small arrow) that fill into the right greater saphenous vein. (D) Filling of SV from a branch of the superior mesenteric vein (arrowhead) with caput appearance of vessels surrounding the stoma (arrow). (A) Reproduced with permission from Journal of Vascular and Interventional Radiology. ¹¹ Copyright 2011, Society of Interventional Radiology. (B, C) Reproduced with permission from Techniques in Gastrointestinal Endoscopy. ¹ Copyright 2017, Elsevier.

Because these variceal complexes form at the enterocutaneous junction they are difficult to diagnose. Unlike other ectopic varices within the gastrointestinal (GI) tract, SVs are not usually visible to the naked eye and due to their shallow location are often not visible under general endoscopic evaluation either. In most cases, SVs go undiagnosed unless they bleed or are captured incidentally through cross‐sectional imaging done for other indications. Identifying SVs can be difficult if not captured during active bleeding, but one series noted bluish skin discoloration, raspberry appearance of the stoma, visibly dilated submucosal veins, and caput medusae of the stoma in up to one‐third of patients presenting with SV bleeding (Fig. 2). Because all of the patients in this study presented with active bleeding, it is unclear whether these findings can or should be used for prediction of SV bleeding. ⁵ However, when SV bleeding is suspected, removing the stoma appliance and evaluating the underlying area for these characteristic signs may help guide diagnosis and management. ⁵

FIG 2.

Cutaneous manifestations of a stoma presenting with variceal bleeding. These are the most common cutaneous findings reported in patients with SV bleeding. Black arrowheads indicate bluish skin discoloration noted with stoma presenting with bleeding; long arrow indicates the stoma itself has a raspberry appearance to it; white arrowheads indicate visibly dilated submucosal veins spreading out from the stoma in a caput medusae pattern. Reproduced with permission from Techniques in Gastrointestinal Endoscopy. ¹ Copyright 2017, Elsevier.

Bleeding from SVs often presents as an innocuous event with patients describing their ostomy appliance acutely filling with red blood or visualizing a constant, pressurized stream of bleeding from a peristomal site. In many cases, patients report the bleeding stops with manual pressure or simply stops on its own, likely related to reduced portal pressure once an adequate volume of blood is lost. Although there is no direct evidence in patients with SV bleeding, initial procedures should mimic those for any suspected variceal bleed. ⁶ This includes placing adequate intravenous access, following a modest transfusion strategy as opposed to an aggressive strategy, use of a vasoactive agent, such as octreotide or terlipressin, and initiation of antibiotics. ⁶ Unless patients have directly witnessed bleeding from a peristomal area, it is important to first ensure that they are not having a brisk upper GI bleed, specifically related to esophageal or gastric variceal bleeding, because this is a more common form of bleeding in patients with portal hypertension and may be more life‐threatening if presenting as bright red blood filling an ostomy appliance. Once endoscopy has ruled out an upper GI bleed, then an imaging assessment of the peristomal area should be completed. This may include cross‐sectional imaging using computed tomography or magnetic resonance imaging with a portal venous phase of contrast, direct portal venography, or simply Doppler ultrasound of the peristomal area. There are limited data to support the use of one imaging modality over the other, but cross‐sectional imaging or direct portal venography are likely best because they also provide other information, such as shunting pathways and presence of portal‐mesenteric thrombosis, and give providers more information regarding vascular access to the varix. Figure 3 represents an algorithm for the diagnosis and management of acute SV bleeding.

FIG 3.

Proposed algorithm for the management of bleeding SVs.

Once peristomal bleeding is diagnosed, therapeutic options are mostly limited to angiographic interventions. Due to their shallow location from the abdominal wall, endoscopic interventions are difficult to perform, although endoscopic ultrasound (EUS)‐guided cyanoacrylate injection has been reported. ⁷ Direct percutaneous interventions using cyanoacrylate have been reported and in the right setting can be successful. ⁸ However, for this to be safe, preprocedure imaging needs to be performed to ensure the outflow tract of the variceal complex can be adequately compressed so that systemic embolization of a cyanoacrylate thrombus does not occur. Transjugular intrahepatic portosystemic shunt (TIPS) has been reported for various kinds of ectopic variceal bleeding, including SV, and has shown relatively good results at stopping the initial bleeding event, but rebleeding rates are up to 40%. In these patients, the average portosystemic gradient was <12 mm Hg, suggesting that SVs will bleed at lower‐than‐expected pressures, and therefore TIPS alone may not be sufficient to prevent rebleeding. ⁹ , ¹⁰ The addition of direct variceal embolization should be considered during TIPS to reduce this risk for rebleeding. Similarly, balloon‐occluded retrograde transvenous obliteration (BRTO) also has been reported and allows direct embolization of the varix while also disrupting the portosystemic shunt. ¹¹ Although it stands to reason that beta‐blockade would work for both primary and secondary prophylaxes for SV bleeding, there are no data to support this currently and so it cannot be recommended.

In conclusion, SVs represent a unique variceal complex created by juxtaposing the mesenteric vasculature of the portal system with the subcutaneous systemic veins. They are likely present in all patients with portal hypertension and stomal formation, but the exact risk for bleeding is currently unknown. Regardless, removing the stomal appliance and assessing for changes of portal hypertension around the stoma are necessary when these patients present with evidence of bleeding. Further studies are needed in the future to better define the prevalence of SVs, their risk for bleeding, and optimal treatment modalities.