What Is Portal Hypertension?
Portal hypertension is a condition in which there is elevated pressure in the venous portal system. The portal vein is one of the major veins in the liver. The most common cause of portal hypertension is liver cirrhosis, but it occurs due to noncirrhotic reasons too.
What Is Noncirrhotic Portal Hypertension?
Noncirrhotic portal hypertension includes a group of conditions characterized by increased portal pressure without cirrhosis. This group of disorders affects the vascular system of the liver. They are divided on the basis of anatomy into pre-hepatic, hepatic (pre-sinusoidal, sinusoidal, and post-sinusoidal), and post-hepatic.
What Are the Causes of Noncirrhotic Portal Hypertension?
The causes of noncirrhotic portal hypertension can be divided into
1. Presinusoidal Causes:
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Porto-Sinusoidal Vascular Disease (PSVD) - Porto-sinusoidal vascular disease is a vascular liver disease characterized by portal hypertension when liver cirrhosis or other liver diseases are not there. .
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Portal Vein Obstruction (Neoplastic and Non-Neoplastic) - It is a complication of several metabolic and autoimmune diseases.
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Polycystic Disease - It is a condition characterized by fluid-filled cysts within the liver.
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Schistosomiasis - It is a disease caused by a parasitic worm.
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Arteriovenous Fistulas - It is an irregular connection between the artery and the vein.
2. Sinusoidal Causes:
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Amyloidosis - It is a rare disease in which there is an accumulation of a protein called amyloid.
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Viral Hepatitis - It is an infection resulting in liver inflammation and damage.
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Non-Alcoholic Steatohepatitis - It is the inflammation of the liver caused by excess fat in it.
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Gaucher’s Disease - It is the disease characterized by the build-up of certain fatty substances in the liver and spleen.
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Drug-Induced - The liver disease caused by drugs.
3. Post-sinusoidal Causes:
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Budd-Chiari Syndrome - It is characterized by the narrowing or blocking of the hepatic veins by a clot.
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Hypervitaminosis A - This is an excess amount of Vitamin A in the body by excess intake than recommended by international standards.
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Epithelioid Hemangioendothelioma - It is a rare cancer that develops from the cells that form the blood vessel.
4. Other Causes:
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Medications and Toxins - Several other medicines and toxins can cause, like Azathioprine, 6-thioguanine, and Arsenic.
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Genetic Disorders - Some congenital disorders like Turner’s syndrome and Adams-Oliver syndrome have also been shown to link to noncirrhotic portal hypertension.
What Are the Symptoms of Portal Hypertension?
The signs and symptoms of portal hypertension are:
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Ascites - It is the fluid accumulation in the abdomen resulting in swelling.
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Edema - Edema of the legs.
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Caput Medusa - It is the visible network of the dilated veins around the navel.
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Encephalopathy- It affects brain and causes confusion and fogginess in thinking.
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Jaundice - Yellowishness of the skin, the white part of the eyes, and nails.
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Gastrointestinal Bleeding - There can be blood in the stools, or one may vomit blood when any large abdominal vessel ruptures due to portal hypertension.
How Is Portal Hypertension Diagnosed?
There are many ways to diagnose portal hypertension, and they are:
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Imaging Studies - An imaging study helps in the diagnosis of portal hypertension. Also, they are noninvasive, and the doctors can see the detailed image of the portal venous system. The most preferred test is the duplex Doppler ultrasound. These ultrasound sound waves help to see how the blood flows in the portal vein. Through the ultrasound, the doctor will get an idea of the blood vessels and the surrounding organs and also the speed and direction of the blood flow in the portal vein.
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Pressure Measurement Studies - In this test, the radiologist will access one of the veins through the jugular vein to measure the pressure level. This can be done on an outpatient basis.
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Endoscopic Diagnosis - Endoscopy is another way to diagnose varices, which are large vessels associated with portal hypertension. Endoscopy provides a much clearer diagnosis of the varices, and also, the doctor can treat the active bleeding and, thereby, reduce the associated risk. Also, during the procedure, the doctor will be able to see the mucus lining of the gastrointestinal tract, which involves the esophagus, stomach, and duodenum.
For the Endoscopy:
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Anesthesia is given so that the gag reflex is avoided. Painkillers, other medications, and sedatives are also given.
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The patient is asked to lie on the left side, known as the left lateral position.
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The doctor will then insert the endoscope, which is a thin, flexible tube with a light and a camera attached to it, into the esophagus through the mouth and pharynx.
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The doctor can see the inner of the esophagus, stomach, and duodenum.
What Are the Complications of Portal Hypertension?
The complications of portal hypertension, regardless of the causes, are:
1. Ascites - Ascites are a condition in which there is fluid in the abdomen. The symptoms of ascites are:
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Feeling of fullness
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Protruding abdomen.
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Difficulty in breathing when the fluid starts to push the lungs.
2. Varices - Varices refer to the varicose veins that are associated with portal hypertension. The doctor can visualize them during an endoscopy or using other imaging techniques. This is mostly seen in the esophagus and the stomach due to portal hypertension. When there is acute bleeding from the varices in portal hypertension patients, they require immediate medical attention to control and prevent the bleeding.
3. Encephalopathy - Hepatic encephalopathy is an impairment in neuropsychiatric function associated with portal hypertension. Symptoms are usually mild, with subtle changes in behavior, sleep pattern changes, confusion, or slurred speech. However, it can progress to more serious symptoms, including severe lethargy and coma. There is an association between increased levels of ammonia in the body and encephalopathy. Hepatic encephalopathy is diagnosed in the clinic and is usually treated with medications like lactulose, which will decrease the absorption of ammonia in the gastrointestinal tract. Other medicines used are Rifaximin and Neomycin.
How Is Portal Hypertension Treated?
Portal hypertension is treated in the following ways:
1. Medications - Medications are used to lower the pressure in the portal veins and hence prevent them from bleeding. The medications used are beta-blockers and Octreotide.
2. Endoscopic Therapy - There are different ways of endoscopic treatment to treat this condition, and they are:
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Banding: Banding is the preferred method to treat variceal hemorrhage. While doing the endoscopy, the doctors place an elastic ring over the vein, which blocks the blood supply to varix. This treatment method will consist of four to five sessions in order to obliterate the varices.
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Balloon Tamponade: In this procedure, a balloon is inflated in the stomach or esophagus, which will apply pressure to the bleeding veins to control severe bleeding.
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Transjugular Intrahepatic Portal-Systemic Shunt: It is a procedure done to create a connection between the portal vein to the hepatic vein.
3. Liver Transplantation - This is the only treatment that will cure portal hypertension in patients with liver cirrhosis. A healthy liver is placed instead of a cirrhosed liver. This complex surgery takes longer but has the highest success rate than other treatment modalities.
4. Shunting Procedures - It is an alternate method for controlling acute bleeding. There are two methods of shunting, and they are:
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Nonsurgical Transjugular Intrahepatic Portal-Systemic Shunt (TIPSS) - In this, the hepatic vein is accessed through the jugular vein, and a needle is passed through the liver into the portal vein. Then the vein is dilated, and a stent is placed, which keeps the vein open.
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Surgical Shunts - They are done to reduce portal vein pressure andmaintain regular blood flood around the liver.
Conclusion
Noncirrhotic portal hypertension can also occur in patients without any liver cirrhosis. It is a common misconception that portal hypertension results from cirrhosis of the liver. The symptoms of portal hypertension, when observed, consult a doctor. If treatment is not received on time, it can further cause complications that can be life-threatening.
