Understanding and managing hepatic disease, portal hypertension and stomal varices

Abstract

Scarring (cirrhosis) from advanced liver diseases can block normal hepatic circulation of blood through the liver, causing increased pressure in the venous portal system (portal hypertension). This can result in pressurised veins (varices), usually in the gastro-oesophageal region, which are liable to bleed. Variceal bleeding is dangerous, leading to haemodynamic destabilisation and death, and thus requires emergency intervention. Treatments include medications and endoscopic suturing, sclerotherapy, ligation or embolisation. Transjugular intrahepatic portosystemic shunt (TIPS) is a high-risk option that can relieve the underlying portal hypertension. People with a stoma and cirrhosis may develop peristomal varices (caput medusae), which are at risk of copious bleeding. These require immediate treatment, followed by the stoma care nurse reconsidering the stoma management routine and appliance use to reduce the risk of rebleeding.

Maddie White, Colorectal Nursing Team Leader, Queen Elizabeth Hospital Birmingham maddie.white@uhb.nhs.uk

Key words

„ Hepatic disease

„ Liver cirrhosis

„ Portal hypertension

„ Peristomal varices (caput medusae)

„ Variceal bleeding

This article has been subject to double-blind peer review

Accepted 31 January 2023

Patients with liver disease often develop certain comorbid conditions that require specialist management. Among the most common and serious comorbidities of cirrhosis are portal hypertension and variceal bleeding. To be able to anticipate, recognise and manage these conditions, specialist nurses should have a full understanding of the underlying functions of the hepatic portal system and how it interacts with gastrointestinal function, as well as how liver damage can cause pathophysiological disruption to this system.

Liver function and hepatic circulation

The liver is an organ that sits in the right upper quadrant of the abdomen, above the gallbladder and pancreas. These three organs form the pancreato-hepatobiliary system, which interacts with the vascular system and gastrointestinal tract to support and regulate many biochemical processes essential to a balanced physiological performance state, including regulation of most chemical levels in the blood and absorption of nutrients from digested food (Table 1). The liver comprises two main lobes, each with eight segments, which in turn comprise around 1000 lobules. Around 60% of the liver’s cells are hepatocytes, which perform the liver’s many functions. Hepatocytes excrete a product called bile, a mixture of water, bile acids and bile pigments that helps carry away waste products from the liver. Each day, 500–1000ml of bile is transported via a system of ducts, through the common hepatic duct, to the gallbladder and duodenum (the first part of the small intestine). As blood passes through the liver, nutrients are absorbed; hormones and digestive enzymes are produced and regulated; and harmful substances are broken down, detoxified or removed. This process also allows drugs to be metabolised into forms that are easier for the body to use. By-products from these processes are excreted into the bile or blood. Blood byproducts are filtered out by the kidneys and leave the body in the form of urine. Bile byproducts enter the intestine and leave the body in the form of faeces.

The liver is highly vascularised, receiving 25% of the total cardiac output during each cardiac cycle (Harkins and Ahmad, 2022). Uniquely among human organs, blood enters the liver via two separate routes. Around 25% of the liver’s blood supply is oxygenated blood from the aorta, which enters the liver via the hepatic artery. The remaining 75% enters the liver via the portal venous system—comprising the coeliac and superior and inferior mesenteric veins, feeding into the hepatic portal vein— and comprises nutrient-rich blood from the gastrointestinal tract, as well as blood from the gallbladder, pancreas and spleen. Once depleted of oxygen or nutrients, this blood then drains through the hepatic veins out of the liver and back into systemic circulation (Figure 1).

Liver disease and cirhossis

Liver function can be impaired by a variety of conditions. These include viral infections, such as hepatitis B and C, and genetic conditions, such as haemochromatosis, Wilson’s disease and alpha 1-antitrypsin deficiency. Lifestyle factors are the definitive features of many common liver diseases, such as alcohol-related liver disease (ARLD), non-alcoholic fatty liver disease (NAFLD) and non-alcoholic steatohepatitis (NASH) (Hecht, 2018). For example, NASH affects 2–6% of the global population (Henry et al, 2022), and its prevalence is rising alongside that of obesity, diabetes and insulin resistance (Boregowda et al, 2019). NASH is the second most common cause of cirrhosis among patients waiting for a liver transplant.

These different liver diseases typically progress through fibrosis (scarring) to cirrhosis (extensive, irreversible scarring). Cirrhosis disrupts the architecture and function of the liver (Garrido and Djouder, 2021) and is associated with increased risk of cancers such as hepatocellular carcinoma and intrahepatic cholangiocarcinoma (Williams, 2006). Cirrhosis is characterised by formation of generative nodules surrounded by fibrotic tissue, and the global burden of cirrhosis is predicted to rise in the next few decades (Ginès et al, 2021).

Cirrhosis is also associated with prolonged cholestasis and certain vascular causes, including Budd-Chiari syndrome and veno-occlusive disease (Ginès et al, 2021).

Table 1. Functions of the liver

Production and synthesis

• Production of bile, for breaking down fats

• Synthesis of lipids (cholesterol and specific proteins)

• Synthesis of major blood plasma proteins and clotting, albumin, fibrinogens and globulins, for haemostasis

• Production of glucose, for homeostasis (gluconeogenesis)

• Production of hormones and enzymes, for homeostasis

• Production of immune factors, for resisting infections

Conversion and processing

• Conversion of amino acids, lipids and carbohydrates into glucose, which is required for energy (gluconeogenesis)

• Conversion of excess glucose into glycogen, which can later be converted back to glucose for energy (glycogenolysis)

• Processing of haemoglobin into iron, which is essential for red blood cell function

• Conversion of poisonous ammonia into urea, a product of protein metabolism that is excreted in the urine

Regulation

• Metabolism of carbohydrates, lipids and proteins, for energy

• Regulation of blood clotting (haemostasis)

• Regulation of hormones, including sex hormones

Storage

• Storage of iron, for red blood cell production

• Storage of vitamins A, D and B12, for nutrition

• Storage of glycogen, for nutrition

• Storage of macrophages (Kupffer cells), for combatting infections

Excretion

• Clearance of bilirubin, cholesterol, hormones, bacteria and drugs, which prevents toxic accumulation of these substances, converting them into an inactive form or more water-soluble for excretion via kidneys

Portal hypertension and variceal bleeding

Patients with cirrhosis often develop portal hypertension, a clinical syndrome defined as a pathological increase in the pressure within the portal venous system (Sauerbruch et al, 2018). Portal hypertension is diagnosed when the portal venous pressure gradient between the hepatic portal vein and inferior vena cava exceeds 5mmHg (Berzigotti et al, 2013; Bari and Garcia-Tsao, 2012; Boregowda et al, 2019).

Portal hypertension is a complex, multifactorial process, involving both blood retention in the portal system (Cichoz-Lach et al, 2008) and increased vascular resistance preventing portal blood flow to the systemic system (Boregowda et al, 2019), as well as increased portal inflow caused by splanchnic vasodilatation (Gunarathne et al, 2020).

When comorbid with cirrhosis, portal hypertension is often a direct result of the blood flow through the liver being impeded or blocked by hepatocytes that have been structurally distorted by scarring of the liver tissues (Tsochatzis et al, 2014). However, portal hypertension can also result from any condition that interferes with blood flow or vascular resistance in the portal venous system, such as blood clots (thrombosis), vascular disorders or neoplastic processes (Khanna and Sarin, 2014).

Up to 90% of people with cirrhosis will eventually develop large swollen veins in the abdomen, known as varices (Figure 2). Varices appear where blood, blocked from its usual route through the liver, is instead forced to flow through other channels, causing the pressure gradient to rise above 10mmHg and these portosystemic collateral veins to dilate (Gunarathne et al,

2020). Varices present a major risk of rupture and bleeding. Variceal bleeding can rapidly destabilise haemostasis and lead to death, and thus this lifethreatening event represents the most significant potential complication of portal hypertension. Varices typically arise in the gastro-oesophageal region, but rarely they can also occur at other sites along the gastrointestinal tract, such as the duodenum, jejunum, ileum, colon, rectum or biliary tree. These ectopic varices account for 1–5% of variceal bleeds in patients with intrahepatic portal hypertension and 20–30% of those with extrahepatic portal hypertension (Sarin and Kumar, 2012). Ectopic varices may be comparatively difficult to access and manage (Sharma and Misra, 2005).

Treatment for portal hypertension and variceal bleeding

The risk of variceal bleeding can be minimised by reducing the portal vein pressure and thus the size of the varices. Ideally, this involves reversing the factors of portal hypertension, for example by treating any associated liver disease (Bari and Garcia-Tsao, 2012; Boregowda et al, 2019).

Portal vein pressure can also be managed with beta blockers, such as propranolol. Meanwhile, the progression cirrhosis may be slowed by prescribing statins, due to their anti-inflammatory effects (Sauerbruch et al, 2018).

Oesophageal and gastric varices should undergo endoscopic evaluation and be categorised to determine the risk of bleeding and the most appropriate treatment. Patients with varices will also require support and information, and clinicians should ensure they have a clear understanding of how to access suitable clinical services should a bleed develop.

Variceal bleeding usually requires emergency hospital admission, especially if the bleeding is so severe that the patient is haemodynamically unstable. Once admitted, the patient should undergo immediate assessment to identify the bleeding vessels using an ultrasound scan, doppler scan and/or computed tomography (CT) angiogram. Then, the first treatment priority is to stop the bleeding and restore haemodynamic stability. Intravenous fluids and a blood transfusion may be required. Oesophageal variceal bleeding can be temporarily controlled using a Sengstaken-Blakemore tube (Figure 3), which is often stored in the ward fridges as an emergency measure (Weatherspoon, 2018).

There are a variety of treatment modalities that can stop variceal bleeding and minimise the risk of rebleeding, and a combination of these may be required. The treatment choice will depend on the severity of the bleed and the physiological function of the liver. Therefore, the patient will also require a full assessment, including full blood count and specific blood tests to measure renal and liver function, including clotting factors. Patients who have presented previously and are well-known to the liver team should have established records monitoring their hepatic function, enabling the team to determine whether their liver health has deteriorated.

Less severe variceal bleeding may be medically controlled with pharmacologic agents. Less aggressive endoscopic interventions include suturing or injection (sclerotherapy) of the bleeding vessel (Figure 4). Other ways to stop the bleeding include endoscopic placement of elastic bands (ligation) and formation of artificial blood clots (embolisation).

If these interventions are unsuccessful, patients may undergo placement of a transjugular intrahepatic portosystemic shunt (TIPS), an artificial channel that allows blood to flow from the portal vein to the hepatic vein, bypassing the liver (Berzigotti and García-Pagán, 2008). TIPS placement is relatively high-risk, and direct procedure-related morbidity rates are as high as 20% (Gaba et al, 2011). These morbidities include bleeding, infection and damage to the veins or bile ducts, resulting in continuous heart or kidney failure or accelerated liver failure, potentially causing prolonged hospitalisation or death (Yin et al, 2022). However, in a life-threatening situation, TIPS may be the patient’s only option, and it is indicated in: high-risk varices; acute variceal bleeding that cannot be successfully controlled medically or endoscopically; recurrent or refractory ascites intolerant to medical management; and patients with portal hypertension requiring abdominal surgery to reduce risk of intraabdominal bleeding.

Peristomal varices (caput medusae)

In people with a surgical ostomy, portal hypertension can lead to ectopic varices and variceal bleeding at the stoma site.

Peristomal variceal bleeding should be distinguished from other forms of stomal bleeding. Minor stomal bleeding is common in all settings, because the healthy bowel mucosa used to form a stoma has a voluminous blood supply and so is liable to bleed with even slight friction. This is usually a result of trauma, although it can result from peristomal skin irritation or stomal ulceration (Kabeer et al, 2007). It usually presents as bright red blood visible on tissues used to wipe the surface of the stoma. Minor intermittent stomal bleeding should be managed conservatively and can usually be stopped by applying pressure to the surface or using a wet cold compress. Alternatively, minor bleeding around a stoma may be the result of granulomas. These otherwise harmless papules bleed readily, but this usually stops on its own. If not, the granulomas may require treatment following clinical guidelines from the Association of Stoma Care Nurses UK (2016).

In contrast, peristomal varices can be easily identified as prominent subcutaneous veins with an engorged and pressurised appearance and a bluish tinge (Figure 5). They are known as caput medusae, Latin for ‘Medusa’s head’, after their resemblance to the snake-like hair tendrils of the Medusa (Perrin et al, 2023).

Peristomal varices develop when the flow from the high-pressure portal venous system decompresses into lower pressure systemic veins of the abdominal wall via the mucocutaneous venous network around the stoma site, defined as portosystemic collateralisation between the portal system of the stoma and the systemic venous system of peristomal skin (Krishnamurty et al, 2017). These varices are common in patients with intrahepatic portal hypertension secondary to primary sclerosing cholangitis and those with ileostomies after proctocolectomy for inflammatory bowel disease associated with primary sclerosing cholangitis (Akhter and Haskal, 2012). This phenomenon can occur in up to 50% of patients (Kwok et al, 2012; Romano et al, 2019).

Peristomal variceal bleeding

These vessels are often fragile and can bleed easily. The risk of stomal bleeding in this setting has been estimated at around 27% (Kwok et al, 2012; Romano et al, 2019). This bleeding usually comes from the mucocutaneous junction, and is typically spontaneous, vigorous and copious. The blood may fill the stoma appliance and start to leak out and drip down the abdomen (Coleman, 2020). Stomal variceal bleeding can be catastrophic, with unrelenting torrential blood loss causing the patient to become haemodynamically unstable. Patients require immediate attention and should attend the emergency for intervention.

The assessment and management of peristomal variceal bleeding should be similar to that of other forms of variceal bleeding, as described above. However, the peristomal rather than gastrointestinal location of the varices allows for local topical treatments to try to stem the bleeding, including application of pressure, epinephrinesoaked gauze and cauterisation. Sclerotherapy, suturing and percutaneous embolisation may be deployed without an endoscope. It is vital that the stoma appliance is removed and a thorough inspection is undertaken to identify the exact bleeding point. Once stabilised, the patient’s stoma should be reviewed by a senior member of the medical staff and treatment instigated to fully stop the bleeding and avoid recurrence. Ultimately, the patient may still require TIPS placement to control the underlying portal hypertension and so reduce the risk of further bleeding.

The specialist stoma care nurse (SCN) should review the patient’s stoma management routine and consider whether their appliance is contributing to the problem. For example, an appliance with a convex baseplate may be placing extra pressure around the stoma. If so, the SCN can recommend trying a more suitable appliance or supporting products to avoid exacerbating pressure on the varices and lower the risk of further variceal bleeding. The SCN should also offer regular contact to monitor risk and progress.

Conclusions

CPD reflective questions

„ What processes can cause the development of varices in people with advanced liver disease?

„ What interventions are available to treat variceal bleeding?

„ How can peristomal varices (caput medusae) be identified and distinguished from other forms of stomal bleeding?

StatPearls Publishing; 2022

Hecht M. Caput medusae. 2018. www.healthline.com/health/ caput-medusae (accessed 9 February 2023)

GN

Managing portal hypertension and variceal bleeding in liver cirrhosis, including stomal varices, requires specialist knowledge and intervention. Nurses in hepatology, endoscopy and stoma care should have an understanding of the relevant anatomical, physiological and pathophysiological processes, as well as the available options for treatment and management. Patients diagnosed with liver disease should be supported by a team of specialists to ensure that they are able to live as full a life as possible until the underlying aetiology can be resolved.

Declaration of interest None

Akhter NM, Haskal ZJ. Diagnosis and management of ectopic varices. Gastrointest Intervent. 2012; 1(1):3–10. https://doi. org/10.1016/j.gii.2012.08.001

Association of Stoma Care Nurses UK. National Clinical Guidelines. 2016. https://ascnuk.com/_userfiles/pages/files/ national_guidelines.pdf (accessed 2 February 2023)

Bari K, Garcia-Tsao G. Treatment of portal hypertension. World J Gastroenterol. 2012; 18(11):1166–1175. https://doi. org/10.3748/wjg.v18.i11.1166

Berzigotti A, García-Pagán JC. Prevention of recurrent variceal bleeding. Dig Liver Dis. 2008; 40(5):337–42. https://doi. org/10.1016/j.dld.2007.12.003

Berzigotti A, Seijo S, Reverter E, Bosch J. Assessing portal hypertension in liver diseases. Expert Rev Gastroenterol Hepatol. 2013; 7(2):141–155. https://doi.org/10.1586/ egh.12.83

Boregowda U, Umapathy C, Halim N et al. Update on the management of gastrointestinal varices. World J Gastrointest Pharmacol Ther 2019; 10(1):1–21. https://doi.org/10.4292/ wjgpt.v10.i1.1

Cichoz-Lach H, Celinski K, Słomka M, Kasztelan-Szczerbinska B. Pathophysiology of portal hypertension. J Physiol Pharmacol. 2008; 59 Suppl 2:231–238

Coleman L. Stomal Bleeding: Assessment, management and a case study of caput medusae. Gastrointestinal Nursing. 2020; 18(8):16–20. https://doi.org/10.12968/gasn.2020.18.8.16

Gaba RC, Khiatani VL, Knuttinen MG et al. Comprehensive review of TIPS technical complications and how to avoid them. Am J Roentgenol. 2011; 196(3):675–85. https://doi. org/10.2214/AJR.10.4819

Garrido A, Djouder N. Cirrhosis: a questioned risk factor for hepatocellular carcinoma. Trend Cancer. 2021; 7(1):29–36. https://doi.org/10.1016/j.trecan.2020.08.005

Ginès P, Krag A, Abraldes JG et al. Liver cirrhosis. Lancet. 2021; 398(10308):1359–76. https://doi.org/10.1016/S01406736(21)01374-X

Gunarathne LS, Rajapaksha H, Shackel N et al. Cirrhotic portal hypertension: from pathophysiology to novel therapeutics. World J Gastroenterol. 2020; 26(40):6111–6140. https://doi. org/10.3748/wjg.v26.i40.6111

Harkins JM, Ahmad B. Anatomy, abdomen and pelvis, portal venous system (hepatic portal system). Treasure Island (FL):

Henry L, Paik J, Younossi ZM. Review article: the epidemiologic burden of non‐alcoholic fatty liver disease across the world. Aliment Pharmacol Ther. 2022; 56(6):942–956. https://doi.org/10.1111/apt.17158

Kabeer MA, Jackson L, Widdison AL et al. Stomal varices: a rare cause of stomal hemorrhage (a report of three cases). Ostomy Wound Manage. 2007; 53(8):20–26

Khanna R, Sarin SK. Non-cirrhotic portal hypertension –Diagnosis and management. J Hepatol. 2014; 60(2):421–441. https://doi.org/10.1016/j.jhep.2013.08.013

Krishnamurty DM, Blatnik J, Mutch M. Stoma complications. Clin Colon Rectal Surg. 2017; 30(3):193–200. https://doi. org/10.1055/s-0037-1598160

Kwok ACH, Wang F, Maher R et al. The role of minimally invasive percutaneous embolisation technique in the management of bleeding stomal varices. J Gastrointest Surg. 2013; 17(7):1327–30. https://doi.org/10.1007/ s11605-013-2180-y

Perrin A, White M, McPhail J, Baker M. Managing difficult stomas. Springer eBooks. 2023:207–237. https://doi. org/10.1007/978-3-031-07799-9_12

Romano J, Welden CV, Orr J et al. Case series regarding parastomal variceal bleeding: presentation and management. Ann Hepatol. 2019; 18(1):250–257. https:// doi.org/10.5604/01.3001.0012.7934

Sarin SK, Kumar CKN. Ectopic varices: ectopic varices. Clin Liv Dis. 2012; 1(5):168–172. https://doi.org/10.1002/cld.95

Sauerbruch T, Schierwagen R, Trebicka J. Managing portal hypertension in patients with liver cirrhosis. F1000 Res. 2018; 7:533. https://doi.org/10.12688/ f1000research.13943.1

Sharma D, Misra SP. Review article: ectopic varices in portal hypertension. Ind J Surg. 2005; 67(5)

Tsochatzis EA, Bosch J, Burroughs AK. Liver cirrhosis. Lancet. 2014; 383(9930):1749–1761. https://doi.org/10.1016/ S0140-6736(14)60121-5

Weatherspoon D. Sengstaken-Blakemore tube. 2018. www.healthline.com/health/sengstaken-blakemore-tube (accessed 9 February 2023)

Williams R. Global challenges in liver disease. Hepatology. 2006; 44(3):521–526. https://doi.org/10.1002/hep.21347

Yin X, Gu L, Zhang M et al. Covered TIPS procedurerelated major complications: incidence, management and outcome from a single center. Front Med. 2022; 9:834106. https://doi.org/10.3389/fmed.2022.834106