History
In obtaining the medical history of a patient with portal hypertension, attention should be directed toward determining the cause of the condition and, secondarily, to which complications are present.
Determining the cause of portal hypertension involves obtaining information on the following:
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History of jaundice - Previous jaundice suggests the possibility of a previous acute hepatitis, hepatobiliary disorder, or drug-induced liver disease; recurrence of jaundice suggests the possibility of reactivation, infection with another virus, or the onset of hepatic decompensation
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History of blood transfusions, administration of various blood products, or intravenous drug use - These raise the possibility of infection with hepatitis B and C viruses
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Pruritus
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Family history of hereditary liver disease (eg, hemochromatosis, Wilson disease)
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History of alcohol abuse
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History of high-risk sexual behavior
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History of schistosomiasis in childhood may be obtained from patients who resided in areas where the infection is endemic
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History of other hepatic-related diseases (eg, nonalcoholic steatohepatitis [NASH], autoimmune hepatitis, diabetes mellitus, and hyperlipidemia) - Research suggests that esophageal varices occur in approximately 50% of patients with NASH with severe fibrosis (like patients with other chronic liver disorders, NASH patients with esophagogastric varices need to be followed up carefully)
Despite conflicting studies, the most common causes of gastrointestinal (GI) bleeding are peptic ulcer disease, of which gastric ulcers are usually more common than duodenal ulcers, and a nonspecific mucosal abnormality (21-55%). [22, 23, 24] Bleeding from esophageal varices is responsible for 12-14% of upper GI bleeding; acute gastric erosions/hemorrhagic gastritis, Mallory-Weiss tears, gastric carcinoma, and Dieulafoy lesion, account for less than 10% of cases. [22, 23, 24]
Patient history of risk factors for upper GI bleeding, including the following, should also be assessed:
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Bleeding diathesis
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Use of alcohol or nonsteroidal anti-inflammatory drugs (NSAIDs)
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Documented cirrhosis
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Documented episodes of GI bleeding
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History of recent vigorous retching or emesis before an attack of hematemesis or melena
Symptoms of liver disease include the following:
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Weakness, tiredness, and malaise
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Anorexia
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Sudden and massive bleeding, with or without shock on presentation
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Nausea and vomiting
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Weight loss - This symptom is common with acute and chronic liver disease; it is mainly due to anorexia and reduced food intake and regularly accompanies end-stage liver disease, when a loss of muscle mass and adipose tissue is often a striking feature
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Abdominal discomfort and pain - Usually felt in the right hypochondrium or under the right lower ribs (front, side, or back) and in the epigastrium or the left hypochondrium
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Jaundice or dark urine
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Edema and abdominal swelling
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Pruritus - Usually associated with cholestatic conditions, such as extrahepatic biliary obstruction, primary biliary cirrhosis, sclerosing cholangitis, cholestasis of pregnancy, and benign, recurrent cholestasis
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Spontaneous bleeding and easy bruising
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Symptoms of encephalopathy - These include disturbance of the sleep-wake cycle; deterioration in intellectual function, memory loss, and, finally, an inability to communicate effectively at any level; personality changes; and, possibly, displays of inappropriate or bizarre behavior
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Impotence and sexual dysfunction
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Muscle cramps - Common in patients with cirrhosis
The presence of complications of portal hypertension can be ascertained by determining whether the following are present:
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Hematemesis or melena - May indicate gastroesophageal variceal bleeding or bleeding from portal gastropathy
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Mental status changes - Such as lethargy, increased irritability, and altered sleep patterns; these may indicate the presence of portosystemic encephalopathy
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Increasing abdominal girth - May indicate ascites formation
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Abdominal pain and fever - May indicate spontaneous bacterial peritonitis, although this disease also presents without symptoms
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Hematochezia - May indicate bleeding from portal colopathy
Physical Examination
Check the patient's blood pressure and pulse with the patient in the supine and seated positions.
Signs of portosystemic collateral formation include the following:
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Dilated veins in the anterior abdominal wall - May indicate umbilical epigastric vein shunts
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Venous pattern on the flanks - May indicate portal-parietal peritoneal shunting
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Caput medusae (tortuous paraumbilical collateral veins)
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Rectal hemorrhoids
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Ascites - Shifting dullness and fluid wave (if a significant amount of ascitic fluid is present) [1]
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Paraumbilical hernia
Signs of liver disease include the following:
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Ascites [1] - Abdominal distention due to accumulation of fluid; may be associated with peripheral edema and may involve the abdominal wall and genitalia
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Jaundice
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Spider angiomas
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Gynecomastia in males from failure of liver to metabolize estrogen, resulting in a sex hormone imbalance; loss of pubic hair and axillary hair may also be observed
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Dupuytren contracture
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Muscle wasting
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Palmar erythema and leukonychia - May be present in patients with cirrhosis
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Asterixis ("flapping tremor," "liver flap")
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Testicular atrophy - Common in males with cirrhosis, particularly those with alcoholic liver disease or hemochromatosis
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Splenomegaly
Signs of a hyperdynamic circulatory state include the following:
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Bounding pulses
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Warm, well-perfused extremities
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Arterial hypotension
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Flow murmur over the pericardium
Other signs of portal hypertension and esophageal varices include the following:
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Pallor - May suggest active internal bleeding
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Parotid enlargement - May be related to alcohol abuse and/or malnutrition
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Cyanosis of the tongue, lips, and peripheries - Due to low oxygen saturation
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Dyspnea and tachypnea
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Telangiectasis of the skin, lips, and digits
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Gynecomastia - Results from failure of the liver to metabolize estrogen, resulting in a sex hormone imbalance; loss of pubic and axillary hair may also be observed
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Fetor hepaticus - Occurs in portosystemic encephalopathy of any cause (eg, cirrhosis)
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Small-sized liver
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Venous hums - Continuous noises audible in patients with portal hypertension; may be present as a result of rapid, turbulent flow in collateral veins
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Tarry stool - During the rectal examination, obtain a stool sample for visual inspection; a black, soft, tarry stool on the gloved examining finger suggests upper gastrointestinal bleeding
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Hemorrhoids
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Large esophageal varices with red wale signs seen on endoscopy. Courtesy of Wikimedia Commons.
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Uphill esophageal varices. Barium swallow demonstrates multiple serpiginous filling defects primarily involving the lower one third of the esophagus with striking prominence around the gastroesophageal junction. The patient had cirrhosis secondary to alcohol abuse.
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Barium swallow demonstrating esophageal varices involving the entire length of the esophagus. This appearance may be seen in advanced uphill varices or downhill varices secondary to superior vena cava obstruction at or below the level of the azygous vein.
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Computed tomography scan showing esophageal varices. Note the extensive collateralization within the abdomen adjacent to the spleen as a result of severe portal hypertension.
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Normal venous flow through the portal and systemic circulation. IMC = inferior mesenteric vein; IVC = inferior vena cava; SVC = superior vena cava.
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Redirection of flow through the left gastric vein secondary to portal hypertension or portal venous occlusion. Uphill varices develop in the distal one third of the esophagus. IMC = inferior mesenteric vein; IVC = inferior vena cava; SVC = superior vena cava.
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Portal vein and associated anatomy.
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Power Doppler sonogram through the spleen shows varices at the hilum of an enlarged spleen. The final diagnosis was hepatitis C cirrhosis, hepatocellular carcinoma of the left hepatic lobe (which had ruptured into the peritoneum), and portoarterial fistula (which had developed inside the ruptured tumor, giving rise to severe portal hypertension).
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Duplex spectral Doppler sonogram of the portal vein (same patient as in the previous image) shows a bidirectional flow within the vein. The final diagnosis was hepatitis C cirrhosis, hepatocellular carcinoma of the left hepatic lobe (which had ruptured into the peritoneum), and portoarterial fistula (which had developed inside the ruptured tumor, giving rise to severe portal hypertension).
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Digital subtraction selective common hepatic artery angiogram shows immediate filling of the portal venous radicles in the left lobe of the liver (straight arrow) and early filling of portal vein (curved arrow), suggestive of hepatic arterial-portal vein fistula. The final diagnosis was hepatitis C cirrhosis, hepatocellular carcinoma of the left hepatic lobe (which had ruptured into the peritoneum), and portoarterial fistula (which had developed inside the ruptured tumor, giving rise to severe portal hypertension).
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Delayed venous phase of a selective common hepatic angiogram (same patient as in the previous image) shows the portal vein (P), with filling of the left gastric vein caused by retrograde flow feeding gastric and lower esophageal varices (arrows). Retrograde flow in enlarged umbilical veins also is seen. The final diagnosis was hepatitis C cirrhosis, hepatocellular carcinoma of the left hepatic lobe (which had ruptured into the peritoneum), and portoarterial fistula (which had developed inside the ruptured tumor, giving rise to severe portal hypertension).
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Digital subtraction venous phase of a superior mesenteric artery angiogram (same patient as in the previous 2 images) shows retrograde flow into the left gastric vein (curved arrow) and the inferior mesenteric vein (straight arrow). Note the flow defect of the distal portal vein caused by retrograde flow (open arrowhead). The final diagnosis was hepatitis C cirrhosis, hepatocellular carcinoma of the left hepatic lobe (which had ruptured into the peritoneum), and portoarterial fistula (which had developed inside the ruptured tumor, giving rise to severe portal hypertension).
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This video, captured via esophagoscopy, shows band ligation of esophageal varices. Video courtesy of Dan C Cohen, MD, and Dawn Sears, MD, Division of Gastroenterology, Scott & White Healthcare.