Fluid tends to accumulate in the abdomen, the spleen may enlarge, and severe bleeding may occur in the esophagus.
The skin and whites of the eyes may turn yellow, and the abdomen may enlarge.
Doctors base the diagnosis on symptoms and results of Doppler ultrasonography.
If possible, the cause is corrected or eliminated, and symptoms are treated.
(See also Overview of Blood Vessel Disorders of the Liver Overview of Blood Vessel Disorders of the Liver The liver receives the oxygen and nutrients it needs in blood that comes from two large blood vessels: Portal vein Hepatic artery The portal vein provides about two thirds of the blood. This... read more .)
Sinusoidal obstruction syndrome is similar to Budd-Chiari syndrome Budd-Chiari Syndrome Budd-Chiari syndrome is caused by blood clots that completely or partially block blood flow from the liver. The blockage may occur anywhere from the small and large veins that carry blood from... read more except that blood flow is blocked only in very small blood vessels in the liver rather than in larger ones in the liver or in blood vessels outside the liver. That is, blockages do not affect the large hepatic veins and the inferior vena cava (the large vein that carries blood from the lower parts of the body, including the liver, to the heart).
Sinusoidal obstruction syndrome may occur at any age.
Because flow out of the liver is blocked, blood backs up in the liver. This backup (congestion) then reduces the amount of blood entering the liver. Liver cells are damaged because they do not get enough blood (ischemia). The congestion causes the liver to become engorged and enlarged. The congestion also causes increased pressure in the portal vein (portal hypertension Portal Hypertension Portal hypertension is abnormally high blood pressure in the portal vein (the large vein that brings blood from the intestine to the liver) and its branches. Cirrhosis (scarring that distorts... read more ). Portal hypertension can result in dilated, twisted (varicose) veins in the esophagus (esophageal varices Gastrointestinal Bleeding Bleeding may occur anywhere along the digestive (gastrointestinal or GI) tract, from the mouth to the anus. Blood may be easily seen by the naked eye (overt), or blood may be present in amounts... read more ). The elevated pressure in the portal vein and the liver congestion lead to fluid accumulating in the abdomen—called ascites Ascites Ascites is the accumulation of protein-containing (ascitic) fluid within the abdomen. Many disorders can cause ascites, but the most common is high blood pressure in the veins that bring blood... read more . The spleen also tends to enlarge.
Such congestion reduces blood flow into the liver. The resulting liver damage leads eventually to severe scarring (cirrhosis Cirrhosis of the Liver Cirrhosis is the widespread distortion of the liver's internal structure that occurs when a large amount of normal liver tissue is permanently replaced with nonfunctioning scar tissue. The scar... read more ).
Blood Supply of the Liver
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Causes of Sinusoidal Obstruction Syndrome
Common causes include the following:
Ingestion of pyrrolizidine alkaloids, which are found in crotalaria and senecio plants (used in Jamaica to make herbal tea) and in other herbs, such as comfrey (see Medicinal herbs and the liver Medicinal herbs and the liver Many drugs can affect the way the liver functions, damage the liver, or do both. (See also Drugs and the Liver.) Some drugs, such as statins (used to treat high cholesterol), can increase the... read more )
Use of certain drugs that occasionally have toxic effects on the liver, including cyclophosphamide and azathioprine (used to suppress the immune system)
Radiation therapy (used to suppress the immune system before bone marrow or stem cell transplantation Stem Cell Transplantation Stem cell transplantation is the removal of stem cells (undifferentiated cells) from a healthy person and their injection into someone who has a serious blood disorder. (See also Overview of... read more )
A reaction after bone marrow or stem cell transplantation (graft-versus-host disease)
In graft-versus-host disease Graft-versus-host disease Transfusions are given to increase the blood's ability to carry oxygen, restore the amount of blood in the body (blood volume), and correct clotting problems. Transfusions are usually safe,... read more , white blood cells in the transplanted tissue attack the recipient’s tissues. This reaction tends to occur about 3 weeks after transplantation.
Symptoms of Sinusoidal Obstruction Syndrome
Symptoms may begin suddenly. The liver enlarges and becomes tender. The abdomen may swell because of fluid accumulating there. The skin and the whites of the eyes may become yellow—a condition called jaundice Jaundice in Adults In jaundice, the skin and whites of the eyes look yellow. Jaundice occurs when there is too much bilirubin (a yellow pigment) in the blood—a condition called hyperbilirubinemia. (See also Overview... read more .
Varicose veins in the esophagus may rupture and bleed, sometimes profusely, causing people to vomit blood and often go into shock Shock Shock is a life-threatening condition in which blood flow to the organs is low, decreasing delivery of oxygen and thus causing organ damage and sometimes death. Blood pressure is usually low... read more . The blood may pass through the digestive tract, making stools black, tarry, and foul-smelling (called melena). When bleeding is severe, shock ensues. A few people develop liver failure Liver Failure Liver failure is severe deterioration in liver function. Liver failure is caused by a disorder or substance that damages the liver. Most people have jaundice (yellow skin and eyes), feel tired... read more with deterioration of brain function (hepatic encephalopathy Hepatic Encephalopathy Hepatic encephalopathy is deterioration of brain function that occurs in people with severe liver disease because toxic substances normally removed by the liver build up in the blood and reach... read more ), resulting in confusion and coma.
Other people develop cirrhosis Cirrhosis of the Liver Cirrhosis is the widespread distortion of the liver's internal structure that occurs when a large amount of normal liver tissue is permanently replaced with nonfunctioning scar tissue. The scar... read more over time, usually over months, depending on the cause and repeated exposure to toxic agents.
Diagnosis of Sinusoidal Obstruction Syndrome
A doctor's evaluation
Liver blood tests and blood-clotting tests
Ultrasonography or invasive tests
Doctors suspect sinusoidal obstruction syndrome based on symptoms or blood test results that suggest liver dysfunction, particularly if people have ingested substances or have conditions that may cause the disease (particularly people who have had a bone marrow or stem cell transplant). Blood tests, if they have not already been done, are done to determine how well the liver is functioning and whether it is damaged (liver tests Liver Blood Tests Liver tests are blood tests that represent a noninvasive way to screen for the presence of liver disease (for example, hepatitis in donated blood) and to measure the severity and progress of... read more ) and to evaluate blood clotting.
Doppler ultrasonography Doppler ultrasonography Ultrasonography uses high-frequency sound (ultrasound) waves to produce images of internal organs and other tissues. A device called a transducer converts electrical current into sound waves... read more often confirms the diagnosis. Occasionally, invasive tests are necessary. These tests include liver biopsy or measurement of blood pressure in the hepatic veins and portal vein. To measure blood pressure in these veins, doctors insert a catheter into a vein in the neck (jugular vein) and thread it to the hepatic veins. A liver biopsy Biopsy of the Liver Doctors can obtain a sample of liver tissue during exploratory surgery, but more often they obtain a sample by inserting a hollow needle through the person's skin and into the liver. This type... read more can be done at the same time.
Prognosis of Sinusoidal Obstruction Syndrome
The prognosis depends on how extensive the damage is and whether the condition causing it reoccurs or continues—for example, when people continue to drink senecio tea.
Overall, about one fourth of people with sinusoidal obstruction syndrome die of liver failure Liver Failure Liver failure is severe deterioration in liver function. Liver failure is caused by a disorder or substance that damages the liver. Most people have jaundice (yellow skin and eyes), feel tired... read more .
When the cause is graft-versus-host disease Graft-versus-host disease Transfusions are given to increase the blood's ability to carry oxygen, restore the amount of blood in the body (blood volume), and correct clotting problems. Transfusions are usually safe,... read more after bone marrow or stem cell transplantation Stem Cell Transplantation Stem cell transplantation is the removal of stem cells (undifferentiated cells) from a healthy person and their injection into someone who has a serious blood disorder. (See also Overview of... read more , sinusoidal obstruction syndrome often resolves on its own within a few weeks. Increasing the dose of drugs used to suppress the immune system can also cause graft-versus-host disease to resolve. However, some of these people die of severe liver failure.
If the cause is an ingested substance, stopping its use helps prevent further liver damage.
Treatment of Sinusoidal Obstruction Syndrome
Treatment of cause
Treatment of problems resulting from blocked vessels
There is no specific treatment for the blockage. If possible, the cause should be eliminated or treated. For example, if people are consuming a substance (such as an herbal tea) or taking a drug that can damage the liver, it should be stopped.
Ursodeoxycholic acid helps prevent sinusoidal obstruction syndrome from developing after bone marrow or stem cell transplantation Stem Cell Transplantation Stem cell transplantation is the removal of stem cells (undifferentiated cells) from a healthy person and their injection into someone who has a serious blood disorder. (See also Overview of... read more . Sinusoidal obstruction syndrome due to graft-versus host disease may be treated by increasing the dose of drugs used to suppress the immune system or defibrotide sodium.
Problems resulting from the blocked vessels are treated. For example, a low-salt (low-sodium) diet and diuretics help keep fluid from accumulating in the abdomen.
If portal hypertension Portal Hypertension Portal hypertension is abnormally high blood pressure in the portal vein (the large vein that brings blood from the intestine to the liver) and its branches. Cirrhosis (scarring that distorts... read more develops, doctors sometimes try to create an alternate route for blood flow using a procedure called transjugular intrahepatic portal-systemic shunting (TIPS) Portosystemic shunting Portal hypertension is abnormally high blood pressure in the portal vein (the large vein that brings blood from the intestine to the liver) and its branches. Cirrhosis (scarring that distorts... read more . However, it is unclear whether the procedure is effective.
Drugs Mentioned In This Article
Generic Name | Select Brand Names |
---|---|
cyclophosphamide |
Cyclophosphamide, Cytoxan, Neosar |
azathioprine |
Azasan, Imuran |
defibrotide |
Defitelio |