Portal Hypertension
Portal Hypertension: Excerpt from The 5-Minute Pediatric Consult
Rose C. Graham-Maar, MD, MSCE
Portal Hypertension - BASICS
Portal Hypertension - description
- Definition: Elevation of portal BP >5–10 mm Hg
- A major cause of morbidity and mortality in children with chronic liver disease
Portal Hypertension - pathophysiology
- An increase in portal resistance and increased portal blood flow are the main pathogenetic factors initiating the process of portal hypertension:
- Other factors including hyperdynamic circulation, expanded intravascular volume, systemic arteriolar vasodilatation, decreased splanchnic arteriolar tone, and humoral factors (i.e., nitric oxide) contribute to increased portal blood flow and pressure.
- Decompression of the high venous pressure through portosystemic collaterals leads to all the major sequelae of portal hypertension:
- Splenomegaly
- Varices (esophageal, gastric)
- Hemorrhoids
- Caput medusa (periumbilical varices)
- Ascites
- Hepatic encephalopathy
- Coagulopathy
Portal Hypertension - DIAGNOSIS
Portal Hypertension - signs & symptoms
Portal Hypertension - history
- History of umbilical catheterization
- History of hepatitis, abdominal trauma, clotting disorder, contraceptive pills, underlying medical problem such as cystic fibrosis, tyrosinemia, Wilson disease
- Ingestion of excessive amounts of vitamin A
- Hematemesis: Upper GI tract bleed from varices may be the 1st sign of longstanding silent liver disease or previously undiagnosed portal vein thrombosis.
Portal Hypertension - physical exam
- Splenomegaly
- Hepatomegaly may or may not be present
- Ascites (distension, fluid wave)
- Hemorrhoids
- Prominent vascular pattern on the abdomen (caput medusa)
- Digital clubbing
- Telangiectasia
- Palmar erythema
- Growth failure
Portal Hypertension - tests
Portal Hypertension - lab
- CBC and smear: Detect hypersplenism, GI tract blood loss, and chronic liver disease
- PT and PTT: Detect coagulation defects
- Liver enzyme and function tests (alanine aminotransferase [ALT], aspartate aminotransferase [AST], albumin, alkaline phosphatase, γ glutamyl transferase [GGT])
- Serologic tests for hepatitis
Portal Hypertension - imaging
- Doppler ultrasound:
- Liver size and echogenicity
- Biliary anatomy
- Spleen size
- Renal cysts
- Presence of ascites
- Vessel diameter
- Direction of blood flow
- Presence of esophageal varices
- Esophagogastroduodenoscopy (EGD): Definitively identifies the presence of esophageal varices and determines if variceal rupture is the cause of GI tract bleeding
- Barium swallow: An insensitive detector of varices
Portal Hypertension - diag proced-surgery
- Liver biopsy: Identify the underlying cause of the portal hypertension
- Hepatic venous wedge pressure gradient correlates and selective angiography are not used in pediatrics because of a lack of well-documented pediatric measurements and lack of a favorable risk–benefit ratio.
Portal Hypertension - differencial diagnosis
- Prehepatic causes:
- Portal vein thrombosis (umbilical vein catheterization, sepsis, dehydration, hypercoagulable state)
- Splenic vein thrombosis
- Intrahepatic causes:
- Hepatocellular disorders: Congenital hepatic fibrosis, αBiliary tract disorders: Extrahepatic biliary atresia, intrahepatic cholestasis syndromes, sclerosing cholangitis, choledochal cyst, cystic fibrosis
- Posthepatic causes:
- Budd–Chiari syndrome: Occlusion of suprahepatic inferior vena cava or hepatic veins by congenital web, tumor, or thrombus
- CHF
- Venoocclusive disease of hepatic venule
Portal Hypertension - TREATMENT
Portal Hypertension - initial stabilization
- Acute management of variceal bleed:
- Vital signs: Remember that hemodynamic instability can be masked by beta-blockers.
- Fluid resuscitation: Large bore IVs, give crystalloid initially, then RBC transfusion with goal of hemoglobin 10 g/dL
- Nasogastric tube placement: Lavage with room temperature saline until clear; leave tube in place for evaluation and removal of continued or recurrent bleeding
- Correction of coagulopathy: Parenteral vitamin K, fresh frozen plasma, platelet transfusion if platelets <50,000/μL
- IV antibiotics: Acute variceal hemorrhage increases the risk of spontaneous bacterial peritonitis in the setting of ascites.
- Pharmacotherapy:
- Octreotide (somatostatin analogue) decreases splanchnic blood flow via its inhibition of intestinal vasoactive peptide secretion.
- Vasopressin decreases splanchnic blood flow via its vasoconstriction effects, but its use is limited owing to a poor side effect profile
- Nitroglycerin, a venodilator, has been used in conjunction to decrease the side effects.
- Lactulose to prevent hepatic encephalopathy in patients with cirrhosis
- Endoscopy (after stabilization): Document source of hemorrhage (variceal rupture or other, such as gastric ulcer), sclerotherapy, ligation therapy (band or clip).
- Direct tamponade: Sengstaken–Blakemore tube for severe uncontrollable hemorrhage, but high rate of complications
- Surgical intervention: Portosystemic shunt, esophageal devascularization and/or transection, TIPS (transjugular intrahepatic portosystemic shunt), liver transplantation
- The site of bleeding needs to be identified and managed appropriately: Not all GI bleeding in a patient with portal hypertension is an upper GI tract source (i.e., hemorrhoids); nasogastric lavage will help to determine if the problem is from the upper tract.
- Be careful not to overestimate the hemoglobin because equilibration may not have taken place at the time of presentation with an acute bleed.
Portal Hypertension - general measures
Chronic management of varices:
- Surveillance endoscopy and primary prophylaxis in pediatric patients with portal hypertension who have not had a first variceal bleed are controversial and not yet recommended.
- Long-term management of patients with portal hypertension who have had a variceal bleed depends on the underlying cause of the portal hypertension and may include beta-blockers, prophylactic endoscopic sclerotherapy or ligation, portosystemic shunts, and liver transplantation.
Portal Hypertension - diet
Sodium restriction when ascites is present
Portal Hypertension - special therapy
- Endoscopic sclerotherapy: Reduces rebleeding episodes and long-term mortality when initiated after the 1st bleeding episode; it is unclear whether it will prevent occurrence of a first bleed.
- Endoscopic ligation therapy with bands or clips: Alternative methods that may be as effective as and carry fewer complications than sclerotherapy; limited pediatric data are favorable.
Portal Hypertension - medication
- Beta-blockade: Nonselective beta-blockers, such as propranolol, have been shown to be effective in preventing both initial and recurrent variceal bleeds in adults and may improve long-term survival in adult patients with esophageal varices.
- They function to lower portal blood flow and thus portal pressure by both βPropranolol, specifically, may also decrease collateral circulation.
- Beta-blockers are rarely used in patients younger than adolescence for fear of a lack of adaptive cardiovascular response in the event of a hemorrhage, and they cannot be used in patients with asthma or diabetes.
- Diuretic therapy (spironolactone) when ascites present
Portal Hypertension - surgery
- Portosystemic shunt:
- Not commonly used in pediatric patients
- May be helpful in the setting of prehepatic causes of portal hypertension
- Does not improve long-term survival in patients with intrahepatic disease
- Its complications may include thrombosis and worsening of hepatic encephalopathy.
- TIPS procedure may be a more effective bridge to liver transplantation in pediatric patients with progressive liver disease and recurrent variceal bleeds.
- Liver transplantation:
- The current approach at most institutions is liver transplantation for those patients with life-threatening bleeds not amenable to beta-blockade or endoscopic therapies.
Portal Hypertension - FOLLOW UP
- Most patients are followed closely for hepatic decompensation.
- Growth failure, recurrent life-threatening bleeds not controllable with prophylactic intervention, and poor quality of life are indications for liver transplantation.
Portal Hypertension - prognosis
- The disease course and prognosis depend on the underlying cause.
- Variceal bleeding associated with prehepatic causes of portal hypertension such as portal vein thrombosis typically becomes less problematic as the child ages; these patients will most likely not require a shunt and may be easily managed with endoscopic therapy.
- Patients with congenital hepatic fibrosis also do very well, because the underlying disease is not progressive and bleeding may be easily managed with endoscopic therapy.
- Progressive liver disease has a worse prognosis and often requires liver transplantation.
Portal Hypertension - complications
- Hemorrhage from varices hematemesis, hematochezia, melena
- Hypersplenism
- Malabsorption owing to congestion of the intestinal mucosa
- Abnormal sodium retention
- Ascites: Presence of ascites increases risk of spontaneous bacterial peritonitis.
- Hepatorenal syndrome
- Hepatopulmonary syndrome (intrapulmonary right-to-left shunting) leads to hypoxemia, shortness of breath, exercise intolerance, and digital clubbing.
- Pulmonary hypertension can be a life-threatening complication of portal hypertension.
Portal Hypertension - bibliography
- Molleston JP. Variceal bleeding in children. J Pediatr Gastroenterol Nutr. 2003;37:538–545.
- Ryckman FC, Alonso MH. Causes and management of portal hypertension in the pediatric population. Clin Liver Dis. 2001;5:789–818.
- Shashidhar H, et al. Propranolol in prevention of portal hypertensive hemorrhage in children: A pilot study. J Pediatr Gastroenterol Nutr. 1999;29:12–17.
Portal Hypertension - CODES
Portal Hypertension - icd9
572.3 Portal hypertension
Portal Hypertension - FAQ
- Q: What is my child’s long-term prognosis?
- A: The disease course and prognosis depend on the underlying cause. Variceal bleeding associated with prehepatic causes of portal hypertension such as portal vein thrombosis typically becomes less problematic as the child ages and may be easily managed with endoscopic therapy.
- Q: Are there any medications I should avoid?
- A: Avoid aspirin and NSAIDs–containing products.
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Book Source Details
- Book Title: The 5-Minute Pediatric Consult
- Author(s): M. William Schwartz MD; et al.
- Year of Publication: 2008
- Copyright Details: The 5-Minute Pediatric Consult, Copyright © 2008 Lippincott Williams & Wilkins.
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- "The 10-Minute Diagnosis Manual: Symptoms and Signs in the Time-Limited Encounter" (2000)
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- "The Diagnostic Approach to Symptoms and Signs in Pediatrics" (2006)
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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
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More About This Book:
Title: The 5-Minute Pediatric Consult
Authors: M. William Schwartz MD; et al.
Publisher: Lippincott Williams & Wilkins
Copyright: 2008
ISBN: 0-7817-7577-9
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