Esophageal Varices Disease

BASICS

DESCRIPTION

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 are the most common causes of cirrhosis.
- Other causes include hemochromatosis, hepatitis B, nonalcoholic steatonecrosis (NASH), biliary cirrhosis, and autoimmune cirrhosis.
Extrahepatic portal vein occlusion (blockage outside the liver) can be caused by umbilical vein infection, trauma, chronic pancreatitis, thrombotic conditions, and polycythemia. [Image of the hepatic portal vein and its tributaries]
Noncirrhotic portal hypertension is common in patients from Asian continents.
Malignant invasion of liver sinusoids or portal vein, seen in lymphoma, leukemia, hepatocellular carcinoma, and pancreatic carcinoma.
Metabolic diseases altering liver sinusoids: amyloid, Gaucher's disease, fatty liver.
Budd-Chiari syndrome (blockage of the hepatic veins) and 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

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

1. Size of varices
A. Small B. Medium C. Large

2. Number of columns of varices
A. 1-2 B. 2-3 C. >3

3. Red wale markings
A. None B. Mild C. Severe

4. Cherry red spots
A. None B. Mild C. Severe

Grading for Bleeding Risk

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

FOLLOW UP

PREVENTION/AVOIDANCE

- Endoscope esophagus each 2 years in cirrhosis
  - If grade 3, propranolol, 40-120 mg bd
  - If grade 4, 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 (of esophageal varices)
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

OTHER NOTES

N/A

ABBREVIATIONS

N/A

Clinical Investigations

ROLE OF HOMOEOPATHY

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