Esophageal varices

Large collateral veins located in the submucosa of the esophagus and stomach, most prominent in the distal esophagus, connecting the portal vein with the superior vena cava. These veins result from chronic high pressure in the portal vein and are particularly prone to rupture with associated gastrointestinal bleeding and often exsanguination and death. Bleeding from varices is the single most common cause of death in cirrhosis of the liver.

System(s) affected: Gastrointestinal, Cardiovascular
Genetics: No known pattern
Incidence/Prevalence in USA: Present in 85% of cases of cirrhosis of the liver. Causes 5-11% of upper gastrointestinal bleeding.
Predominant age: Parallels the ages of cirrhosis with most cases 40-60 years, but can occur at any age
Predominant sex: Male > Female

SIGNS AND SYMPTOMS:

• Intestinal bleeding only symptoms
• Upper GI, 75% of time, painless hematemesis
• Occult GI with anemia 25%
• Abdominal periumbilical collateral circulation present in most
• Signs of cirrhosis
• Large, hard liver
• Splenomegaly
• Ascites

CAUSES:

• Cirrhosis accounts for > 90% of cases. Alcoholic and hepatitis C most common causes of cirrhosis, but hemochromatosis, hepatitis B, nonalcoholic steatonecrosis, biliary cirrhosis, autoimmune cirrhosis account for some.
• Extrahepatic portal vein occlusion from umbilical vein infection, trauma, chronic pancreatitis, thrombotic conditions, polycythemia cause a few
• Noncirrhotic portal hypertension common in patients from Asian continents
• Malignant invasion of liver sinusoids or portal vein. Seen in lymphoma, leukemia, hepatocellular carcinoma, pancreatic carcinoma.
• Metabolic diseases altering liver sinusoids - amyloid, Gaucher's disease, fatty liver
• Budd-Chiari syndrome, veno-occlusive disease due to senecio, thrombotic conditions

RISK FACTORS:

• Cirrhosis of the liver
• Inherited thrombotic conditions such as anti-thrombin III, substance S or R deficiencies
• Prolonged use of estrogen-progesterone birth control pills

DIAGNOSIS

DIFFERENTIAL DIAGNOSIS:

• Upper GI bleeding
• Pulmonary bleeding; hemoptysis
• Peptic ulcer disease
• Gastric malignancy
• Lower GI bleeding
• Hemorrhoids
• Colon malignancy
• Colonic polyp
• Diverticulitis

LABORATORY:

Reflects only the anemia of bleeding, or the abnormalities related to the cirrhosis or other cause

Drugs that may alter lab results: N/A
Disorders that may alter lab results: N/A

PATHOLOGICAL FINDINGS:

Extensive collateral circulation in the mediastinum and in the abdomen in addition to large vessels in the submucosa of the esophagus. When bleeding occurs, these large veins explode into the submucosa of esophagus and rupture in turn into the lumen.

SPECIAL TESTS:

N/A

IMAGING:

• Esophagram following barium swallow with adherent barium demonstrates very advanced varices, but is insensitive to small ones. Is not used when bleeding present for it precludes possible urgent angiography.
• MRI demonstrates large vascular channels intra-abdominally, and in the mediastinum. Demonstrates patency of the intrahepatic portal vein and splenic vein if this is required.
• Doppler sonography demonstrates patency, diameter, and flow in portal vein, and splenic vein, and large collaterals intra-abdominally
• Venous phase celiac arteriography demonstrates portal vein and its collaterals

DIAGNOSTIC PROCEDURES:

• Esophagoscopy as part of EGD endoscopy can identify and treat. Large, protruding, lumenal veins in the distal 1/3 of the esophagus are diagnostic. If recent bleeding, they may be seen to be bleeding in 5%. Useful when active bleeding is present, to identify early varices, and to follow course of treatment.

Endoscopic Findings
Size of varices
A. Small B. Medium C. Large
Number of columns of varices
A. 1-2 B. 2-3 C. >3
Red wale markings
A. None B. Mild C. Severe
Cherry red spots
A. None B. Mild C. Severe

Grading for BleedingRisk

Grade	Findings				Risk
	1	2	3	4
1	A	A	A	A	Rare
2	B	A	A	A	Unlikely
3	C	B	A	B	Possible
4	C	C	C	C	Likely

• Doppler sonography to demonstrate patency of
• portal and splenic veins
• porta-caval shunts
• Venous phase angiography
• Diagnose hepatic vein occlusion
• Endoscopic ultrasound particularly sensitive to gastric varices

TREATMENT

APPROPRIATE HEALTH CARE:

Inpatient for acute bleeding

GENERAL MEASURES:

• As related to cirrhosis
• Hospital management of bleeding varices
• Appropriate resuscitation and maintenance of blood volume
• Urgent upper endoscopy for diagnosis and treatment. Injections of somatostatin or octreotide to control bleeding permit endoscopic treatment of varices.
• Variceal ligation or sclerosant injection for bleeding varices
• Repeat ligation or sclerosant injection if bleeding recurs
• If ligation or sclerosant injection fails to stop bleeding or cannot be accomplished, consider TIPS (transjugular intrahepatic portacaval shunt)
• Management of non-bleeding varices
• If ligation or sclerotherapy started, complete the sequence at intervals of 1-4 weeks. 4-6 treatments usually required to eradicate varices.
• If no bleeding has occurred, and varices are rated grade 2 or more severe, by endoscopy, treat with propranolol - 10 mg q 12h initially titrated up each few days until pulse rate slowed by 25%, average dose 80 mg bid. Remain on this dose for life or until transplant or some form of portacaval shunt.
• Gastric varices
• Do not respond to ligation or sclerotherapy. Beta blockers or TIPS only effective measures.

SURGICAL MEASURES:

Consider when General Measures impractical or fail:
• Portocaval shunt
• Esophageal transection
• Liver transplantation

ACTIVITY:

No restrictions

DIET:

Appropriate to cirrhosis or other conditions present

PATIENT EDUCATION:

• Appropriate to cirrhosis
• National Digestive Information Clearinghouse, 2 Information Way, Bethesda, MD 20892 or American Liver Foundation, 1425 Pompton Way, Cedar Grove, NJ 07009

MEDICATIONS

DRUG(S) OF CHOICE:

• For varices grade 2 or worse: propranolol 80 mg bid
• Increase until pulse rate decreased by 25% from basal
• Other nonspecific beta blockers probably effective. Nadolol proven effective.
• During banding or sclerotherapy: proton pump blocker such as lansoprazole 30 mg q d for one month
• During bleeding: antibiotic prophylaxis for spontaneous peritonitis. Norfloxacin 400 mg q12h for 7 days.

Contraindications: Severe asthma with beta blockers
Precautions: Symptomatic hypotension
Significant possible interactions: N/A

ALTERNATIVE DRUGS:

N/A

FOLLOW UP

PATIENT MONITORING:

• Varix ligation or sclerotherapy, repeated every 1-4 weeks until varices eradicated
• If varices grade 1 or 2 on endoscopy (do not hemorrhage), repeat endoscopy each year. If eradicated, repeat endoscopy each 2 years.
• If TIPS or other portacaval shunt, repeat endoscopy only if clinically bleeding
• If TIPS present, followup as recommended by radiologist, usually Doppler sonogram each 6 months

PREVENTION/AVOIDANCE:

• Endoscope esophagus each 2 years in cirrhosis
• If grade 3, propranolol, 40-120 mg bd
• If grade4, prophylactic endoscopic ligation

POSSIBLE COMPLICATIONS:

• Bleeding. Gastric or other uncommon varices may occur following successful eradication of esophageal varices.
• Educate patient to plan of action if bleeding occurs, particularly if traveling

EXPECTED COURSE AND PROGNOSIS:

• Bleeding diminished and survival prolonged
• Recurrent bleeding is an indication for transplantation listing
• In progressive worsening, grade changes are one grade in 2 years

MISCELLANEOUS

ASSOCIATED CONDITIONS:

• Infections associated with underlying cirrhosis: e.g., influenza and pneumococcal
• Gastric varices often occur after eradication
• Portal hypertensive gastropathy can also bleed. Recognized by endoscopy, and responds to beta blockade and TIPS.
• Collateral circulation may occur with thrombosis of the superior or inferior vena cava
• Hemorrhoids

AGE-RELATED FACTORS:

Pediatric: N/A
Geriatric: N/A
Others: Can occur in all age groups

PREGNANCY:

N/A

SYNONYMS:

N/A

ICD-9-CM:

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