Paeds · gastroenterology-hepatology-and-nutrition
Chronic liver disease, cirrhosis and portal hypertension
Also known as Paediatric cirrhosis · Portal hypertension in children · Oesophageal varices in children · Hepatopulmonary syndrome · Paediatric chronic liver disease
Fellowship guide to chronic liver disease, cirrhosis and portal hypertension in children: the prehepatic, intrahepatic and posthepatic classification, biliary atresia as the paradigmatic paediatric cause, the pathophysiology of the hyperdynamic splanchnic circulation, variceal bleeding prophylaxis and acute management, ascites and spontaneous bacterial peritonitis with the 250 PMN threshold, hepatopulmonary syndrome and portopulmonary hypertension, and the PELD score for transplant listing.
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Target exams
Red flags
- Massive haematemesis or melaena in a child with known or suspected liver disease
- Splenomegaly with thrombocytopenia indicating clinically significant portal hypertension
- Fever, abdominal pain, or altered mental status in a child with ascites suggesting spontaneous bacterial peritonitis
- Dyspnoea, platypnoea, digital clubbing, or cyanosis suggesting hepatopulmonary syndrome
- Rising bilirubin, coagulopathy, or encephalopathy indicating decompensated cirrhosis
- Poor growth and failure to thrive in a child with chronic cholestasis
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- Recognises the presenting features of chronic liver disease and portal hypertension in children including splenomegaly, ascites, and variceal bleeding
- Identifies biliary atresia as the most common paediatric cause of cirrhosis
- Initiates immediate resuscitation of acute variceal bleeding
- Constructs a stepwise management plan for portal hypertension complications including variceal prophylaxis, ascites, and spontaneous bacterial peritonitis
- Interprets the PELD score for transplant listing and prognostication
- Distinguishes hepatopulmonary syndrome from portopulmonary hypertension and manages accordingly
- Differential diagnosis of splenomegaly and upper gastrointestinal bleeding in a child with liver disease
- Diagnostic criteria and management of spontaneous bacterial peritonitis
- Management of acute variceal bleeding in a child
- Takes a focused history and performs the clinical examination of a child with chronic liver disease
- Communicates a complex chronic disease management plan including prophylaxis and transplant assessment
- Counsels a family about liver transplantation and transition planning
- Stage 1: Recognition of splenomegaly, ascites, and signs of chronic liver disease
- Stage 2: Diagnostic workup with endoscopy, imaging, and laboratory assessment of liver synthetic function
- Level 3: Multidisciplinary management of portal hypertension and transplant assessment
- Classification and causes of portal hypertension in children
- Diagnostic criteria for spontaneous bacterial peritonitis and hepatopulmonary syndrome
- Primary prophylaxis of variceal bleeding with beta-blockers versus endoscopic band ligation
- History-taking and examination for chronic liver disease and portal hypertension
- Interpretation of endoscopic and laboratory findings
- Structured explanation of management including transplantation
- NASPGHAN approach to paediatric portal hypertension and variceal management
- PELD score for transplant listing and prognostication
- Recognition and management of hepatopulmonary syndrome
- Recognition of the complications of chronic liver disease in children
- Shared decision-making around prophylactic therapy and transplant
- Acute variceal bleeding recognition and emergency management
- Canadian approach to paediatric portal hypertension management
- Coordinated multidisciplinary care and growth surveillance
- Transition from paediatric to adult hepatology services
Overview & Definition
A child who presents with a massively enlarged spleen, unexplained thrombocytopenia, or an episode of haematemesis may be revealing chronic liver disease that has progressed to portal hypertension long before jaundice or other signs of hepatic failure appear. This is the central clinical challenge: portal hypertension can declare itself through a life-threatening complication while the underlying liver disease has been smouldering silently for years. Understanding the cascade from hepatic fibrosis to the complications of variceal bleeding, ascites, spontaneous bacterial peritonitis, and hepatopulmonary syndrome is essential for every paediatric trainee. [1]
Cirrhosis is the advanced, generally irreversible stage of chronic liver injury in which fibrous septa and regenerative nodules distort the hepatic architecture and disrupt blood flow. Portal hypertension is the pathological rise in pressure within the portal venous system that results from this distortion, and it is the engine driving nearly all the life-threatening complications of chronic liver disease. The pressure gradient that defines it is the hepatic venous pressure gradient, the difference between the wedged hepatic venous pressure and the free hepatic venous pressure. A gradient of 5 mmHg or more defines portal hypertension, while a gradient of 10 mmHg or more defines clinically significant portal hypertension, which is the threshold at which varices form and the risk of bleeding and ascites becomes real. [1]
In children, the causes of cirrhosis and portal hypertension differ fundamentally from those in adults. Biliary atresia is the single most common cause, followed by inherited metabolic diseases, autoimmune hepatitis, cystic fibrosis liver disease, and chronic viral hepatitis. Portal vein thrombosis with cavernous transformation is a uniquely important prehepatic cause in children because it produces severe portal hypertension with well-preserved synthetic liver function. Recognising this distinction is critical because it fundamentally changes the management approach and the prognosis. [9]
References12ShowHide
- [1]de Franchis R, Bosch J, Garcia-Tsao G, Reiberger T, et al Baveno VII - Renewing consensus in portal hypertension. J Hepatol, 2022.PMID 35120736
- [2]Kaplan DE, Ripoll C, Thiele M, Fortune BE, et al AASLD Practice Guidance on risk stratification and management of portal hypertension and varices in cirrhosis. Hepatology, 2024.PMID 37870298
- [3]Cifuentes LI, Gattini D, Torres-Robles R, Gana JC Beta-blockers versus placebo or no intervention for primary prophylaxis of oesophageal variceal bleeding in children with chronic liver disease or portal vein thrombosis. Cochrane Database Syst Rev, 2021.PMID 33498095
- [4]Cifuentes LI, Gattini D, Torres-Robles R, Gana JC Band ligation versus sham or no intervention for primary prophylaxis of oesophageal variceal bleeding in children and adolescents with chronic liver disease or portal vein thrombosis. Cochrane Database Syst Rev, 2021.PMID 33522602
- [5]Gana JC, Cifuentes LI, Gattini D, Torres-Robles R Band ligation versus sclerotherapy for primary prophylaxis of oesophageal variceal bleeding in children with chronic liver disease or portal vein thrombosis. Cochrane Database Syst Rev, 2020.PMID 33164205
- [6]Runyon BA Introduction to the revised American Association for the Study of Liver Diseases Practice Guideline management of adult patients with ascites due to cirrhosis 2012. Hepatology, 2013.PMID 23463403
- [7]Krowka MJ, Fallon MB, Kawut SM, Fuhrmann V, et al International Liver Transplant Society Practice Guidelines: Diagnosis and Management of Hepatopulmonary Syndrome and Portopulmonary Hypertension. Transplantation, 2016.PMID 27326810
- [8]McDiarmid SV, Merion RM, Dykstra DM, Harper AM Use of a pediatric end-stage liver disease score for deceased donor allocation: the United States experience. Indian J Pediatr, 2007.PMID 17476086
- [9]Chardot C, Buet C, Serinet MO, Golmard JL, et al Improving outcomes of biliary atresia: French national series 1986-2009. J Hepatol, 2013.PMID 23402746
- [10]Freeman RB Jr, Wiesner RH, Harper A, McDiarmid SV, et al The new liver allocation system: moving toward evidence-based transplantation policy. Liver Transpl, 2002.PMID 12200791
- [11]Sarin SK, Lahoti D, Saxena SP, Murthy NS, et al Prevalence, classification and natural history of gastric varices: a long-term follow-up study in 568 portal hypertension patients. Hepatology, 1992.PMID 1446890
- [12]Zhou X, Tripathi D, Song T, Shao L, et al Terlipressin for the treatment of acute variceal bleeding: A systematic review and meta-analysis of randomized controlled trials. Medicine (Baltimore), 2018.PMID 30508958