Basics
Description
- Definition: elevation of portal blood pressure greater than 10 mm Hg
- May be pre-, intra-, or posthepatic in origin
- A major cause of morbidity and mortality in children with chronic liver disease
Pathophysiology
- An increase in portal resistance and increased portal blood flow are the main pathogenic factors initiating the process of portal hypertension:
- Other factors contribute to increased portal blood flow and pressure including hyperdynamic circulation, expanded intravascular volume, systemic arteriolar vasodilatation, decreased splanchnic arteriolar tone, and humoral factors (i.e., nitric oxide).
- Decompression of the high venous pressure through portosystemic collaterals leads to all the major sequelae of portal hypertension:
- Splenomegaly
- Varices (esophageal, gastric)/GI bleeding
- Hemorrhoids
- Caput medusa (periumbilical varices)
- Ascites
- Hepatic encephalopathy
- Hepatopulmonary syndrome
Diagnosis
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 or melena: Upper GI tract bleed from varices may be the 1st sign of long-standing silent liver disease or previously undiagnosed portal vein thrombosis.
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
Diagnostic Tests & Interpretation
Lab
- CBC and smear: detect hypersplenism, GI tract blood loss, and chronic liver disease
- PT/INR and PTT: detect coagulation defects
- Hepatic function panel includes liver enzymes (alanine aminotransferase [ALT], aspartate aminotransferase [AST]), albumin (measure of hepatic function), alkaline phosphatase, and ³-glutamyl transferase (GGT; may be elevated with cholestasis and bile duct injury).
- Additional laboratory testing to determine the cause of underlying liver disease, depending on clinical scenario (see chapter on "Cirrhosis " for more details)
Imaging
- Abdominal ultrasound with Doppler to evaluate
- 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) to identify the presence of esophageal varices. EGD is also useful for determining if variceal rupture is the cause of GI tract bleeding.
Diagnostic Procedures/Other
- 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.
Differential Diagnosis
- Prehepatic causes:
- Portal vein thrombosis with cavernous transformation (increased risk with umbilical vein catheterization, sepsis, dehydration, hypercoagulable state)
- Splenic vein thrombosis
- Intrahepatic causes:
- Hepatocellular disorders: viral hepatitis,α1-antitrypsin deficiency, chronic hepatitis, autoimmune hepatitis, Wilson disease, glycogen storage disease, tyrosinemia, schistosomiasis, peliosis hepatitis, vitamin A toxicity
- Biliary tract disorders: extrahepatic biliary atresia, ductal plate malformation/congenital hepatic fibrosis, intrahepatic cholestasis syndromes, primary 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
- Congestive heart failure
- Veno-occlusive disease of hepatic venule
Treatment
Medication
- ²-Blockade: Nonselective ²-blockers, such as propranolol, have been shown to be effective in preventing both initial and recurrent variceal bleeds in adults. Data are limited on use of ²-blockers in children with portal hypertension to prevent primary or secondary variceal bleeds. Use in children for this indication is empiric and mainly based on adult data:
- Mechanisms include lowering portal blood flow and thus portal pressure by both ²2-blockade, which increases splanchnic tone, and ²1-blockade, which decreases cardiac output.
- Propranolol, specifically, may also decrease collateral circulation.
- ²-Blocker effect on decreasing cardiac output may blunt an adaptive cardiovascular response (elevated heart rate) in the event of a hemorrhage; these medications should not be used in patients with asthma or diabetes.
- Owing to lack of sufficient prospective data, routine use of ²-blockers in children for primary or secondary prevention of variceal bleeding cannot be recommended.
- Diuretic therapy (spironolactone +/ ’ chlorothiazide) when ascites is present
Additional Treatment
General Measures
Chronic management of varices:
- Surveillance endoscopy and primary prophylaxis in pediatric patients with portal hypertension who have not had a 1st variceal bleed are controversial; however, they may be recommended in select patients.
- Long-term care of patients with portal hypertension who have had a variceal bleed depends on the underlying cause of the portal hypertension and may include secondary prophylactic endoscopic band ligation or sclerotherapy, portosystemic shunts, and liver transplantation.
Additional Therapies
- Endoscopic sclerotherapy: reduces rebleeding episodes and long-term mortality when initiated after 1st bleed; unclear whether effective primary prophylaxis; in general, supplanted by endoscopic band ligation, except for small children
- Endoscopic band ligation is preferred, as it carries fewer complications compared with sclerotherapy. Endoscopic band ligation is not feasible in small patients.
Surgery/Other Procedures
- Portosystemic shunts
- May be helpful in the setting of prehepatic causes of portal hypertension to reduce portal pressure. The Rex shunt (mesenteric-left portal bypass) has been used successfully in the setting of cavernous transformation of the portal vein.
- Does not improve long-term survival in patients with intrahepatic disease
- Complications may include thrombosis and worsening of hepatic encephalopathy.
- Transjugular intrahepatic portosystemic shunt (TIPS) procedure may be an effective bridge to liver transplantation in pediatric patients with progressive liver disease and recurrent variceal bleeds.
- Data in pediatric patients are limited.
- Liver transplantation
- The current approach at most institutions is liver transplantation for those patients with life-threatening bleeds not amenable to ²-blockade or endoscopic therapies.
Inpatient Considerations
Initial Stabilization
- Acute management of variceal bleed
- Vital signs: Remember that hemodynamic instability can be masked by ²-blockers.
- Fluid resuscitation: two large-bore IV catheters or intraosseous needles, give crystalloid initially, then RBC transfusion with goal hemoglobin of around 10 g/dL
- Nasogastric tube placement: Lavage with room temperature saline or sterile water 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/ ΌL
- IV antibiotics: Acute variceal hemorrhage increases the risk of spontaneous bacterial peritonitis in the setting of ascites.
- IV proton pump inhibitor or histamine receptor antagonist to decrease risk of bleeding from ulcers or erosions
- Pharmacotherapy to control active bleeding
- Octreotide (somatostatin analog) decreases splanchnic blood flow via its inhibition of intestinal vasoactive peptide secretion. In turn, portal blood pressure is decreased. Somatostatin can be used but has a shorter half-life compared with octreotide.
- Vasopressin decreases splanchnic blood flow via its vasoconstriction effects, but its use is limited owing to systemic vasoconstriction and a poor side effect profile. Nitroglycerin, a venodilator, has been used in conjunction to decrease side effects. This combination is not preferred.
- Lactulose to prevent hepatic encephalopathy in patients with cirrhosis
- Endoscopy (after stabilization) to determine source of hemorrhage (variceal rupture or other, such as gastric ulcer) and perform therapeutic procedures such as sclerotherapy or band ligation for varices or electrocautery or clip placement for ulcer
- Direct tamponade: Sengstaken-Blakemore tube for severe uncontrollable hemorrhage, but high rate of complications
- Interventional radiology: variceal embolization, TIPS
- Surgical intervention: portosystemic shunt, esophageal devascularization, and/or transection; in general, last resort
Alert
- 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.
Ongoing Care
Follow-up Recommendations
- Patients are followed closely for hepatic decompensation.
- Growth failure, recurrent life-threatening bleeds not controllable with prophylactic intervention, refractory ascites, and poor quality of life are indications for liver transplantation.
Diet
Sodium restriction when ascites is present
Prognosis
- The disease course and prognosis depend on the underlying cause.
- Acute variceal bleeding is associated with a 6-week mortality of up to 30% in adults. The mortality rate in children is much lower.
- 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 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.
Complications
- Hemorrhage from varices may present as hematemesis, hematochezia, or melena.
- Hypersplenism
- Malabsorption due 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.
Additional Reading
- El-hamid NA, Taylor RM, Marinello D, et al. Aetiology and management of extrahepatic portal vein obstruction in children: King 's College Hospital experience. J Pediatr Gastroenterol Nutr. 2008;47(5):630 " 634. [View Abstract]
- Gugig R, Rosenthal P. Management of portal hypertension in children. World J Gastroenterol. 2012;18(11):1176 " 1184. [View Abstract]
- Ling SC. Advances in the evaluation and management of children with portal hypertension. Semin Liver Dis. 2012;32(4):288 " 297. [View Abstract]
- Schneider BL, Abel B, Haber B, et al. Cross-sectional multi-center analysis of portal hypertension in 163 children and young adults with biliary atresia. J Pediatr Gastroenterol Nutr. 2012;55(5):567 " 573. [View Abstract]
Codes
ICD09
- 572.3 Portal hypertension
- 572.2 Hepatic encephalopathy
- 573.5 Hepatopulmonary syndrome
- 456.8 Varices of other sites
- 456.1 Esophageal varices without mention of bleeding
ICD10
- K76.6 Portal hypertension
- K72.90 Hepatic failure, unspecified without coma
- K76.81 Hepatopulmonary syndrome
- I86.8 Varicose veins of other specified sites
- I85.11 Secondary esophageal varices with bleeding
SNOMED
- 34742003 Portal hypertension (disorder)
- 13920009 Hepatic encephalopathy (disorder)
- 371067004 hepatopulmonary syndrome (disorder)
- 402844009 Abdominal varicosities (disorder)
- 195474004 Esophageal varices associated with another disorder
FAQ
- Q: What is my child 's long-term prognosis?
- A: Disease prognosis of portal hypertension depends 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 managed with endoscopic therapy.
- Q: Are there any medications I should avoid?
- A: Avoid aspirin and NSAID-containing products such as ibuprofen.