Diagnosis

Esophageal varices

Etiology

Cause [zey]

• Portal hypertension:

  • Prehepatic:

    • Portal vein obstruction

    • Massive splenomegaly with increased splenic vein blood flow

  • Posthepatic:

    • Severe right-sided heart failure

    • Constrictive pericarditis

    • Hepatic vein obstruction (Budd-Chiari syndrome)

  • Intrahepatic:

    • Cirrhosis (most common cause)

• Schistosomiasis

• Fatty change

• Nodular regenerative hyperplasia affecting portal microcirculation

• Diffuse fibrosing granulomatous disease (sarcoidosis)

• Wilson disease

• Alpha-1 antitrypsin deficiency

• Primary biliary cirrhosis

• Tuberculosis

• Constrictive pericarditis

Pathophysiology [zey]

1. Normal portal pressure: 5-10 mmHg

2. The portal venous system has no valves --> resistance at any level between the splanchnic vessels and the heart results in retrograde flow and elevated pressure

3. Liver Cirrhosis --> portal hypertension --> collaterals connect the systemic circulation to the portal venous system --> congested submucosal venous plexus with tortuous dilated veins in the distal esophagus

4. Increasing size of varices --> increase in variceal-wall tension --> rupture

5. Mortality rate from variceal bleeding: 20% when patients are treated in hospital

Causes for cirrhosis in children

1. Intrahepatic:

   • Cholestatic disease:

     • Biliary atresia

     • Progressive familial intrahepatic cholestasis

     • Primary sclerosis cholangitis

     • Cystic fibrosis-related liver disease

     • Intestinal failure-associated liver disease

   • Hepatocellular disease:

     • Autoimmune hepatititis

     • Chronic hepatitis B

     • Chronic hepatitis C

     • Alpha-antitrypsin deficiency

     • Nonalcoholic fatty liver disease

   • Fibrotic disease:

     • Congenital hepatic fibrosis

     • Caroli disease

2. Prehepatic:

   • Portal vein occlusion:

     • Portal vein thrombosis

     • Tumor infiltration (hpatoblastoma, HCC, focal nodular hyperplasia)

   • Nodular regenerative hyperplasia:

     • Drug (6-thioguanine, Azathioprine)

     • Turner syndrome

   • Portal venopathy/portal sclerosis:

     • Schistosomiasis

     • HIV

     • Cystic fibrosis

3. Posthepatic:

   • Hepatic vein occlusion:

     • Budd-Chiari syndrome

     • Inferior vena cava obstruction

     • Cognitive heart failure

     • Veno-occlusive disease

Complications [zey]

• Aspiration

• Multiorgan failure

• Encephalopathy

• Perforation of the esophagus

• Death

Epidemiology

Incidence per 100.000 [wfr][1pu][eta]

Approach

• Blood test

• Esophagogastroduodenoscopy

• Ultrasound abdomen (signs of portal hypertension)

Treatment

1. Volume resuscitation: crystalloid or blood (hemoglobin goal of 7-9 g/dl) [zey]

2. Medication therapy:

   • Octreotide IV 50 µg/hr

   • Antibiotics IV

   • PPI IV (portal hypertensive gastropathy and/or gastric ulcerations may be present)

   • Terlipressin: 2 mg q4h IV

   • Erythromycin 250 mg IV

   • Beta blockers (nitrates are an alternative)

3. Endoscopy once the patient is hemodynamically stable:

   • Band ligation

   • Sclerotherapy

4. Surgical Management:

   • Interventional radiology

     • Portal vein stent placement

     • Transjugular Intrahepatic Portosystemic Shunts (TIPS)

   • Surgical intervention:

     • Portal vein venoplasty

     • Shunt surgery: Meso Rex bypass shunt if extrahepatic portal venous obstruction and preserved liver architecture

     • Portosystemic shunt: Distal splenorenal shunts

     • Liver transplantation

     • Splenectomy

Differential diagnoses

Budd-Chiari syndrome, Chronic pancreatitis, Gastric cancer, Gastroesophageal reflux disease, Gastrointestinal hemorrhage, Heart failure, Hemochromatosis, Hypertensive gastropathy, Mallory-Weiss tear, Portal vein thrombosis, Primary biliary cirrhosis, Primary sclerosing cholangitis, Sarcoidosis, Schistosomiasis, Ulcer disease, Viral hepatitis, Wilson's disease