X-Rays

Angiography:

Is occasionally needed to evaluate the blood vessels of the liver. If a clot in the vessels leading to or leaving from the liver is suspected, specific x-rays showing these blood vessels may be needed. A dye can be injected into the artery supplying the liver by putting a long tube or catheter into the artery in the groin and threading it up into the liver. Follow up images of these pictures or arteriograms can demonstrate the vein leading to the liver (the portal vein). Other techniques can be used to put dye into the portal vein directly. If an abnormality of the hepatic vein, the vein that leaves the liver, is suspected, dye can be injected through a vein in the neck into the inferior vena cava, a large vessel which takes blood back to the heart. Through this vessel, the hepatic vein can be reached and pressures can be measured. This test is performed by an interventional radiologist, with the patient under sedation or anesthesia.

CT Scan or CAT Scan or Computerized Tomography:

Can be used to get a more accurate picture of the liver. This x-ray is usually done with the patient fasted for several hours. The patient is often asked to drink dye, and dye is injected into the veins as well. This x-ray can give an accurate picture of the liver and of the blood vessels that lead to and flow from it, as well as the bile ducts. This study may be used to demonstrate gallstones, tumors, fatty changes in the liver, etc.

Hepatobiliary Scan:

Is a nuclear medicine study (sometimes also called a DISIDA scan or HIDA scan) that can be used to assess bile flow. In this test a harmless radioactive substance is injected into the child's vein. This substance is accumulated in the liver and then secreted into the bile which accumulates in the gallbladder. When the gallbladder squeezes, the dye should go through the bile ducts and appear in the intestine. If liver function is poor, the dye may not accumulate well in the liver. If bile flow is obstructed (if the bile ducts did not form as in biliary atresia, or if the bile ducts are blocked by a stone when the patient has gallstones) then bile flow from the liver to the intestine is not seen in this study. Sometimes, to make sure that the radiologist has given the patient every chance to demonstrate flow, a follow-up x-ray is done at 24 hours. It is important to know that not every child who has "no excretion" on the hepatobiliary scan actually has a blockage to bile ducts. Poor liver function can make the liver appear as if bile flow is blocked.

MRCP or Magnetic Resonance Cholangiopancreatography:

May be used to get a better picture of the bile ducts. In this new technology, an MRI scan of the abdomen is done with the patient fasted and lying very still (often sedated). Special computer techniques allow for pictures of the bile ducts and pancreatic ducts to be taken from these images. These pictures may be used to look for blockage or narrowing of the ducts that leave the liver.

Ultrasound:

Ultrasound is carried out in the x-ray department on a child who has not eaten in several hours. A lubricant jelly is placed on the child's abdomen and a smooth wand that looks much like a microphone is touched to the patient's skin and moved about. This test is not painful. A black and white picture representing sound waves going through the liver is created. This test can look at the tissue of the liver to see if it is too fatty, too dense or irregular. It may identify a spot such as an abscess, cyst or tumor. This test can also evaluate bile ducts to see if the bile ducts are swollen or dilated (this might suggest a blockage downstream). The ultrasound may also show a stone that is stuck in a bile duct. Ultrasound can also be done with a test called Doppler which looks at the flow of blood in the vessels of the liver. Dopplers may be able to identify blockages of arteries or veins that go to the liver or veins that leave the liver. Ultrasound can also assess for the presence or absence of the gallbladder and can look at other organs in the abdomen such as the pancreas, kidneys, etc.

PROCEDURES:

Endoscopy:

Endoscopy is a procedure which permits the gastroenterologist to look inside the patient's intestine, take pictures, take samples, and at times perform therapy. Endoscopy is carried out with a long fiberoptic tube about the diameter of your little finger. This long tube is inserted into the esophagus through the mouth while the patient is under deep sedation or general anesthesia. The lining of the esophagus can be examined. If the esophagus is inflamed (esophagitis), the esophagus will look thick and sore. In a patient with chronic liver disease with portal hypertension (high pressures in the portal vein leading to the liver), large veins called varices can be seen sticking out of the walls of the esophagus. At times these may break open and cause the child to throw up blood. The scope can examine the stomach and look for ulcers, prominent veins from portal hypertension (portal hypertensive gastropathy) and even Helicobacter pylori infection. The small intestine can also be examined either for abnormalities of the lining or for abnormal blood vessels.

The endoscope can sometimes be used to correct problems. If there is a bleeding ulcer in the stomach, the scope can be used to burn the blood vessel that is causing the bleeding; the scope can also be used to inject epinephrine into the bleeding ulcer. If the child is bleeding from varices (large veins) in the esophagus, two therapies can be undertaken. Esophageal banding is a procedure where a rubber band is placed through the scope around the varix (remember that the varix is a large vein in the esophagus). The rubber band chokes off the blood supply to the varix making it shrink down and clot off. Alternatively, sclerotherapy can be carried out. In sclerotherapy, an irritating substance is injected into the varix blood vessel. This will make it scar down and also stop/prevent bleeding. Esophageal banding or sclerotherapy can be complicated by causing bleeding, causing ulceration or even causing narrowing of the esophagus due to scar tissue. These complications are not common. Even less common are reactions to the process in other organ systems such as heart or lungs. The patient requires careful monitoring during and after endoscopic management of esophageal varices. Several sessions of band ligation or sclerotherapy separated by a few weeks are usually necessary to eliminate the varices.

ERCP (Endoscopic Retrograde Cholangiopancreatography):

Endoscopic retrograde cholangiopancreatography or ERCP may be ordered when the doctor is concerned that there may be a disease affecting the bile ducts or pancreas. This test is performed with a special fiberoptic instrument called an endoscope. While the child is deeply sedated or under general anesthesia, the scope is advanced through mouth into the esophagus, stomach, and into the small intestine. In the small intestine, the ampulla of Vater (which is a little opening where juices from the liver and pancreas spill into the intestine to help digest food) is identified. A tiny catheter is advanced from the scope into the bile duct. Dye is then injected into the bile duct which fills the entire branching network of bile ducts that is present in the liver. If there is a narrowing or irregularity to the ducts, it can be seen on the x-ray pictures that are taken during the ERCP. If a stone is stuck in the bile duct, the endoscopist may be able to use balloons and baskets to pull the stone out. This sometimes requires cutting to enlarge the ampulla of Vater. This cutting is called a "sphincterotomy".

ERCP is commonly performed by adult GI endoscopists, with assistance from their pediatric colleagues. This is because the procedure is not needed often in children, whereas adult endoscopists build up much experience doing this procedure. Sometimes a special tiny infant scope must be borrowed from the manufacturer and may take several days to acquire. Antibiotics are typically given at the time of the ERCP to prevent infection of the bile ducts when the dye is squirted in. Like in any procedure, there is a small risk of reaction to anesthetic/sedation, of making a hole in the intestine, and of bleeding. The most important risk of ERCP is the fact that pancreatitis, inflammation of the pancreas causes abdominal pain and vomiting, can develop in as many as 5% of patients who undergo the procedure, with severe pancreatitis developing in 0.5%. Because of this risk, patients are sometimes observed overnight after undergoing ERCP.

Liver Biopsy:

A liver biopsy may be recommended to further evaluate the child's liver disease. During this procedure, a small fragment of the liver is removed, preserved, stained, and looked at under the microscope. This sample of the liver may provide further information to explain the patient's symptoms or blood test results. There are several ways to do a liver biopsy. The first and most common way is called a "percutaneous liver biopsy". This means that the biopsy is done with a needle inserted through the skin of the ribcage. This kind of biopsy, which can be done by a gastroenterologist or a surgeon, does not require a large incision or stitches. Liver biopsy can also, under certain circumstances, be done through a vein or through a surgical incision (see below).

Preparation for the Biopsy:

Your child will be asked not to eat or drink for several hours before the biopsy. This is done to make sure the stomach is empty so the child will not throw up and choke. Laboratory studies are done before the biopsy to make sure the clotting times (PT/PTT) and platelets are normal enough to help prevent bleeding from the biopsy. Your child should not receive aspirin or ibuprofen on the days before the procedure.

Sedation:

In adults, percutaneous liver biopsy is done without sedation. A local anesthetic (numbing medicine) is just injected into the skin where the biopsy will take place. Young children usually require sedation or anesthesia. Your doctor may choose to sedate the child with intravenous medicines that result in "conscious sedation". This means that the child is awake and breathing on his own but is not aware and will not remember the experience. Commonly, Demerol or Fentanyl (narcotic drugs), and Versed (a Valium-like drug that decreases recall of the procedure), are used. The child is carefully monitored during such a procedure. At some institutions the liver biopsy is done under general anesthesia. That means an anesthesiologist puts the child completely to sleep. Depending on the kind of anesthetic used, this may involve the child briefly having a breathing tube during the procedure.

Percutaneous Liver Biopsy:

Once the child is sedated or asleep, an area in the lower right ribcage over the liver is selected. Under special circumstances, ultrasound may be used to pick the right spot. The area is sterilized with some iodine and alcohol and a tiny nick is made in the skin over one of the ribs. The needle is then inserted into the liver, the sample is obtained, and the needle is withdrawn. This may be done more than once if multiple samples are needed. The fragment of liver removed is very small, about the size of the inside of a needle.

Like any procedure, liver biopsy has its risks. There is always a risk associated with sedating or anesthetizing anyone. These risks include allergic reactions to medication, breathing difficulty and physical injury. The biopsy itself can result in significant bleeding, since there are many blood vessels in the liver. The chance of bleeding enough to require a blood transfusion is about 1 to 2% in children. Life-threatening bleeding can occur in less than 1 in 1,000 cases. Injury to the lung, gallbladder or intestine can also occur, and could even require surgery. A few patients have some pain after the biopsy. Since most problems occur within the first few hours after the biopsy, children are observed closely those first few hours, laying on their right side. At some institutions they are kept overnight for observation, and in others they are discharged after a few hours if the heart rate and blood pressure are stable and a repeat blood count is good. You should call your doctor if excessive pain, breathing difficulty, pallor or dizziness occur.

Transjugular Liver Biopsy:

When abnormal blood clotting or low platelet counts make standard biopsy unsafe, Interventional Radiology can biopsy "from the inside" with a large IV placed in a neck vein. During this procedure, done with the patient sedated or asleep, a very long tube with a needle goes through the neck vein, into the large inferior vena cava, into a liver vein. The liver is biopsied through that internal vein so that any bleeding can be confined. The procedure is typically done under anesthesia.

Open Liver Biopsy:

An "open liver biopsy" is done when the risk of bleeding is very high. It may be planned when blood clotting or platelets are abnormal, making percutaneous biopsy unsafe. Open biopsy is also done when a large piece of liver is needed for special tests. Sometimes, when another surgery is already taking place, an open biopsy is done while the abdomen is open. This procedure, done under general anesthetic, requires that a surgeon make a 1 or 2 inch cut in the skin, clip off a piece of the liver, and either stitch or burn the edge so that it does not bleed. An open biopsy is also done under general anesthesia.

Tests on the Liver Biopsy:

A number of tests can be done on the tissue obtained during a liver biopsy depending on the child's underlying condition. Most commonly, standard stains including "H & E" and other stains are done so that the liver can be looked at under the microscope. There, inflammation (immune cells from the body may be attacking the liver) can be seen. Abnormalities in the liver cells themselves can also be seen. Bile ducts can be looked at and evaluated, as can blood vessels. Special stains can show how much scar tissue is present in the liver. Sometimes these tests can give a lot of information about a possible diagnosis. Other times, however, the results are simply descriptive: they will describe what the pathologist saw without telling you exactly what your child has. In some specialized situations, the liver biopsy is sent for electron microscopy. This special microscope does a real "close-up" on the liver cells looking for rare and specific metabolic or infectious problems. Special stains can also be done on a liver biopsy if a disease is suspected which involves storage of copper, iron, fat, or other substances. If an infection is suspected, a tiny piece of the biopsy can be cultured for viruses, bacteria or fungus. These infections are very rare, and many hepatitis viruses cannot be cultured easily; CMV virus, which is especially important in transplant patients, can be cultured this way. Lastly, a piece of liver can be frozen immediately in very cold temperatures. Later, special metabolic tests can be done on these samples if the activity of a certain enzyme or chemical needs to be assessed. Frozen tissue will preserve these specimens and allow these specialized tests to be done. This is especially important for some metabolic diseases where there are abnormalities in the body's chemistry.

After the Biopsy:

It may take from 1 to several weeks for results of the biopsy to be available, depending on which tests are ordered.

Paracentesis:

Some patients with liver disease have a large amount of fluid called "ascites" in their abdomen outside the organs. A procedure called paracentesis can be used to obtain some fluid, either to check for infection or to make the patient more comfortable. A small needle is inserted into the abdominal wall and fluid is pulled out.

PTC: Percutaneous Transhepatic Cholangiogram:

Sometimes, physicians need a detailed look at the bile ducts in a patient in whom ERCP is not possible (see above). Some babies are too small for ERCP to be practical. Some patients have had previous surgery on their bile ducts, which would make them difficult to reach through a scope of this sort. When another approach to taking pictures of the bile ducts is needed, PTC is sometimes arranged. This procedure is carried out by an interventional radiologist, usually under general anesthetic or deep sedation. A small needle is introduced into the liver, typically between the ribs, and advanced into the liver until it enters a bile duct. Dye is then injected allowing the x-rays to take a picture of the bile ducts and their branches. If a narrowing or leak is noticed, the area can be balloon-dilated (opened up) or a long tube (stent) can be placed through the skin into the liver through the abnormal area and out into the intestine. While this procedure does not run the risk of causing pancreatitis like ERCP does, it has the potential to cause serious bleeding or injury to other organs. Children are observed for several hours or overnight after a PTC.

TIPS (Transjugular Portosystemic Shunt):

TIPS is used to treat complications of portal hypertension. Portal hypertension occurs when blood flow from the intestine, flowing into the portal vein, cannot get into the liver because the liver is scarred or cirrhotic. The blood backs up into veins in the esophagus called varices which can break open and lead to massive bleeding. The TIPS is a shunt which can relieve this backup of pressure. With the patient sedated, an interventional radiologist inserts a long catheter into the neck vein and advances it into the liver. There, he uses special equipment to make a shunt connection between blood entering and leaving the liver so blood does not back up. Unfortunately, these shunts often clot off over time; they have to be checked by ultrasound and often repaired. While they are very effective at stopping bleeding that results from portal hypertension, they can lead to worsening of encephalopathy (confusion that can come with portal hypertension) or of liver function. Patients usually do well but are monitored closely.

[Keywords: Gastroenterology, Angiography, liver, arteriograms, portal vein, hepatic vein, CT Scan, CAT Scan, Computerized Tomography, bile ducts, gallstones, tumors, Hepatobiliary Scan, DISIDA scan, HIDA scan, gallbladder, MRCP, Magnetic Resonance Cholangiopancreatography, pancreatic ducts, Ultrasound, abscess, cyst, tumor, Endoscopy, pancreas, kidney, intestine, portal hypertension, portal hypertensive gastropathy, Helicobacter pylori infection, Esophageal banding, sclerotherapy, ERCP, Endoscopic Retrograde Cholangiopancreatography, sphincterotomy, ampulla of Vater, pancreatitis, liver biopsy, Transjugular Liver Biopsy, Paracentesis, ascites, PTC, Percutaneous Transhepatic Cholangiogram, TIPS, Transjugular Portosystemic Shunt, portal hypertension.]

Disclaimer: The information provided is not intended to replace the advice of a medical professional. If you have medical concerns, seek the guidance of a medical professional. Consult your physician about any medications, supplements or treatments you are considering, and when seeking treatment, disclose all medications you are taking or treatments you are receiving. Riley Hospital for Children, University Pediatric Associates and IU School of Medicine disclaim any liability for the decisions you make based on this information.

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