Hepatic Arterial Pumps

Indicated for patients with metastatic colon cancer, hepatic arterial pumps deliver chemotherapy to the liver through a catheter placed in the hepatic artery. The catheter is typically inserted via laparoscopic or open surgery and a pump, which delivers the chemotherapy, is implanted subcutaneously. The pump is generally filled with chemotherapy once a month.

Percutaneous Ethanol Injection Therapy (PEIT)

Another option for patients who are not surgical candidates, PEIT involves the injection of alcohol into the tumor, causing immediate dehydration of the cytoplasm with consequent coagulation, necrosis and fibrous reaction. PEIT results in complete ablation in up to 75% of selected patients with hepatocellular carcinoma.

Pancreaticoduodenectomy (Whipple Procedure)

A pancreaticoduodenectomy, also known as a Whipple procedure, involves the removal of the pancreas head due to a tumor in the pancreas or bile duct, or pancreatitis.

If a tumor exists in the head of the pancreas, it is usually necessary to remove the pancreas head, duodenum, gallbladder and a portion of the bile duct (Figure 1).
Sometimes, part of the stomach
is also removed.

The end of a patient's bile duct and the remaining pancreas are then connected to the small bowel (Figure 2) to ensure flow of bile and enzymes into the intestines.

Distal Pancreatectomy (laparoscopic or open)
Indicated for tumors in the body and tail of the pancreas, a distal pancreatectomy involves the removal of cystic neoplasms either laparoscopically or with open surgery. With both laparoscopic and open distal pancreatectomy procedures, surgeons attempt to preserve the spleen.

Drainage Procedures
With chronic pancreatitis, a dilated pancreatic duct usually reflects obstruction. Procedures to improve ductal drainage include:

-Longitudinal Pancreaticojejunostomy (Puestow Procedure): The pancreatic duct is opened from the tail to the head of the pancreas and attached to the small bowel.

-Distal Pancreaticojejunostomy (Du Val Procedure): The pancreas is divided transversely at the neck, and the body and tail are drained via attachment to the small bowel.

-Sphincteroplasty: When endoscopic sphincterotomy is unsuccessful, surgical sphincteroplasty may be required of the minor or major papilla.

Pancreas Transplantation
A pancreas transplant is indicated for patients with insulin-dependent (Type 1) diabetes.

Biliary Tumors and Injuries

Biliary surgery is most frequently performed for stones, strictures and tumors. Among the treatment options available at California Pacific include:

Resection of primary biliary neoplasms (cholangiocarcinoma
Treatment of bile duct cancer usually requires removal of the bile duct and possibly portions of the liver, gallbladder, pancreas and small intestine. After resecting the neoplasms -- either through open surgery or laparoscopically -- the surgeon reconnects the bile ducts to the small intestine for proper biliary drainage (see illustration).

Biliary Drainage Procedures
Biliary drainage procedures are performed when the bile duct becomes blocked, narrowed or injured. During surgery, continuity of the biliary tree is usually re-established via a hepaticojejunostomy.

Conditions and diseases of the hepatobiliary system.

The hepatobiliary system refers to the liver, gall bladder and bile ducts – organs that are involved with the production, storage, transport and release of bile, a secretion that prepared fats for further digestion.

There are numerous conditions that can harm the hepatobiliary system, some of which are life-threatening and ultimately, require surgery and/or liver transplantation. Liver damage can occur from a variety of sources: infections with viruses (hepatitis A, B, and C viruses), exposure to toxic drugs or chemicals, excessive use of alcohol, genetic disorders , diabetes, heart failure, cancer and shock. Some of the signs and symptoms of liver disease include:

• jaundice (yellowing of the skin and eye)
• pruritus (itching)
• dark, tea-colored urine
• weight loss
• muscle wasting
• ascites (swelling of the abdomen with fluid)
• fatigue
• easy bruising and bleeding (bleeding gums or frequent nosebleeds)
• vomiting blood
• blood in the stool (bright red blood or black, tar-like stool)
• mental confusion

In many cases, the liver is able to repair itself; in others, a variety of treatments may be effective. However, if liver damage is severe, the organ may not recover, resulting in liver failure which is life threatening. Once this happens, the patient may need a new liver.

Cirrhosis

Cirrhosis describes a condition in which damaged liver cells are replaced by scar tissue. Over time, the build-up of scar tissue becomes so great that it impacts blood flow through the liver, destroying more liver cells and ultimately leading to greater and greater loss of liver function.

Almost any liver disease can result in cirrhosis, but excessive use of alcohol remains the most common cause.

Hepatitis

Inflammation of the liver is called hepatitis. Inflammation (or soreness) of the liver can be traced to many different causes, including viral infections, alcohol, fat accumulation in the liver, an incorrectly functioning immune system, exposure to chemicals and other toxins, and certain drugs.

Hepatitis C

The hepatitis C virus (formerly referred to as non-A, non-B hepatitis) is by far the leading indication for liver transplantation in the United States, accounting for between 30% and 50% of liver transplants, depending on the state where the patient lives. According to the American Liver Foundation, more than 4 million people in the U.S. have hepatitis C, but only about 30 percent who have the virus are aware that they do. The hepatitis C virus kills between 10,000 and 12,000 Americans each year, but the future holds even grimmer prospects. According to some estimates, about 10 million Americans will have hepatitis C within the next couple of decades.

Hepatitis C progresses very slowly, often over the course of 10-20 years, and does much of its damage without symptoms to indicate something might be wrong until liver failure sets in. When a person with hepatitis C does have symptoms, they typically are those associated with a damaged liver. The damage to the liver eventually takes the form of cirrhosis in about 25% of patients, in which healthy liver cells are injured and scar tissue forms in their place. Over time, cirrhosis impairs the liver’s ability to perform critical functions and reduces the amount of blood that flows through the vital organ. Alcohol use increases the progress of liver disease in Hepatitis C; further, the risk of liver cancer is increased in patients with the infection.

The hepatitis C virus, discovered in 1989, is transmitted via blood, so anyone who had a blood transfusion before 1992 or has used intravenous drugs could be at risk. It’s possible for an infected mother to pass along the virus to her child at birth. Researchers aren’t sure whether hepatitis C can be transmitted sexually. If so, it’s a rare occurrence, but people who’ve had multiple partners are encouraged to be tested for the virus. Certain groups of people have higher rates of hepatitis C than the general population, such as military veterans (especially those who served in Vietnam); prisoners; hemophiliacs; the homeless; and people with HIV.

Hepatitis B

The hepatitis B virus was once a major public health threat in the United States?and it still is in some parts of the world, such as Asia and the Pacific. While the hepatitis B vaccine has become a valuable tool against the virus, those people who currently are infected or are not vaccinated and contract hepatitis B can face serious health problems, including liver cancer, the need for a liver transplant or even death. In the United States, 1.25 million people have chronic hepatitis B, and there are 5,000 to 6,000 hepatitis B-related deaths each year. Fortunately, the hepatitis B virus, which is transmitted via blood and bodily fluids, causes chronic infection in less than five percent of the cases.

Hepatitis A

The hepatitis A virus is an acute disease that typically is transmitted via contaminated water or food and resolves without becoming a chronic disorder in almost all cases. It is relatively uncommon in the United States, but international travelers (particularly those headed for developing countries or places with poor sanitation systems) are encouraged to be vaccinated against the hepatitis A virus.

Acute Fulminant Viral Hepatitis: This describes a sudden and catastrophic loss of liver function due to infection from any of the three previously described viruses. Fortunately, only a small percentage of patients (less than 1 percent) that have viral hepatitis develop this variety. The patient deteriorates rapidly from being healthy one day to possibly being comatose in 7-10 days. In addition to causing rapidly progressive liver failure there may be kidney failure, blood-clotting disorders, brain damage and coma.

Toxic hepatitis: Rarely, medications intended to help patients can cause the liver to become suddenly inflamed, causing toxic hepatitis. For that reason, acetaminophen (Tylenol™), isoniazid (a drug used to treat tuberculosis), and many anti-convulsants should not be taken by people with liver disease. Even with a healthy person, an excessive amount of acetaminophen could cause toxic hepatitis. In an occupational setting, toxic hepatitis can occur when workers are exposed to certain chemicals in a confined space and are not wearing or properly using respiratory protective gear.

Autoimmune Hepatitis: This is a condition in which a person’s immune system starts attacking their own organs, as if they did not belong to that individual. Autoimmune hepatitis primarily affects women and starts as early as adolescence. As the name suggests, autoimmune hepatitis can cause inflammation of the liver and other symptoms common to liver disease?such as jaundice and itching?as well as some associated with autoimmune disorders, namely, aching joints. Fatigue, common to both, is a frequently reported symptom. Lab tests show the presence of increased gamma globulin and smooth muscle antibodies in the blood.

Many people with autoimmune hepatitis respond well to prednisone treatment, which can relieve symptoms and cause elevated liver enzyme levels to return to normal. Autoimmune hepatitis is a condition that needs monitoring over the long term, as it can lead to cirrhosis and ultimately, the need for a liver transplant.

Alcoholic Liver Disease

Heavy drinking over a long period of time can take its toll on the liver by causing cirrhosis. Before the onset of cirrhosis the liver sometimes may accumulate an excessive amount of fat (known as fatty liver disease), which interferes with its proper functioning. Moreover, alcohol consumption can accelerate the rate at which other conditions, such as hepatitis C, damage the organ. According to the Scientific Registry of Transplant Recipients, alcoholic liver disease was the third leading indication for liver transplantation in 2003 (26 percent of the transplant recipients in this category also were infected with hepatitis C).

If alcoholic liver disease is identified in an early stage and if the person stops drinking, it is possible that his or her condition could improve. However, these are two major “ifs,” considering the often-silent nature of liver disease and the difficulty of breaking an addiction. Sometimes, the liver damage is advanced, and a liver transplant is needed.

Patients with alcoholic liver disease must meet the following criteria to be considered for liver transplantation at University Hospital: (1) abstinence from alcohol use; (2) ongoing participation in an alcohol treatment program or support group; and (3) presence of an adequate psychosocial support system. These patients must enter a formal contract with the transplant team outlining the parameters of abstinence from alcohol use.

Liver Tumors

Tumors found in the liver can be benign (non-cancerous) or malignant (cancerous). Some of the benign tumors can be left untreated, but sometimes they must be removed. Primary liver cancer (cancer that originates in the liver) is not as common in the United States as it is in other parts of the world, but there are still about 16,000 cases diagnosed in America each year. Most of the time, liver cancer begins in another organ and spreads to the liver. These tumors are called secondary liver tumors or metastases. Liver cancer usually is not diagnosed until it has reached an advanced stage, when removing a tumor surgically is less often a possible treatment choice.

Malignant (Cancerous) Liver Tumors

Primary Liver Cancer or Hepatoma (Hepatocellular Cancer-HCC). While HCC is relatively rare in the United States, it is becoming more common as an unwanted consequence of the hepatitis C epidemic. About 80 percent of the time, HCC occurs in people who have cirrhosis, which can be the result of chronic hepatitis B or C, alpha-1-antitrypsin deficiency, or alcoholic or nonalcoholic fatty liver disease.

HCC is difficult to treat, especially when the patient also has cirrhosis. When the tumor is small or in only one lobe of the liver, surgeons may be able to remove it. This procedure is known as liver resection. If the liver cirrhosis is advanced, liver resection carries the risk of complete failure. In such instances liver transplantation is the only option left for a possible cure.

If either of the above surgeries is not an option, radiofrequency ablation or chemoembolization, two minimally invasive procedures, may be considered to shrink the size of the tumor (see Treatments for a complete description of these procedures).

Secondary Liver Cancer or Liver metastases: Cancer that begins in another part of the body and then spreads to the liver is by far the most common form of liver cancer. The usual cancers that spread to the liver originate in the colon (large intestine), breast and lung. Usually liver metastases do not cause symptoms in their early stages and when symptoms such as jaundice or pain do occur, it is often too late. That’s why it is important that patients with treated cancers have regular follow ups to discover any reoccurrence or spread of their tumors to the liver or other organs.

If there is only a single tumor or a few confined to one side of the liver, the best treatment is to remove them by liver resection. Sometimes, even when tumors are present in both sides of the liver (as long as they are not very large and too many), patients will benefit from a liver resection. If resection is not feasible then the best course of treatment is chemotherapy. Unlike in HCC or hepatoma, liver transplantation does not benefit patients with liver metastases, except in patients with metastases of a special type called neuroendocrine tumors. Metastatic neuroendocrine tumors (such as carcinoid tumors) originate in the pancreas or small bowel and then travel to the liver. Patients typically experience severe abdominal pain, nausea, and diarrhea. When chemotherapy or cryosurgery–which freezes the tumors as a method of destroying them–are ineffective, liver transplantation may be indicated.

Benign (Non-Cancerous) Liver Tumors

Hepatic Hemangioma: A hemangioma is a cluster of tiny blood vessels that form a non-cancerous tumor. A hepatic hemangioma is a common, benign tumor found in the liver, occurring more frequently in women than in men. They are often found when patients undergo ultrasound or CT scans for other problems. Most of the time, hepatic hemangiomas do not cause any problems and do not require treatment. When they do, it often is because of their size and/or their proximity to other organs. Large hepatic hemangiomas can cause pain or enlargement of the liver, and in rare cases, they can rupture. In those instances when the tumor is causing problems or thought to be in a troublesome location, surgical removal is indicated. In most instances surgeons are able to peel out the hemangioma without removing much of the normal liver.

Hepatic Adenoma: A hepatic adenoma is an uncommon, benign tumor that on rare occasion becomes a malignant hepatocellular carcinoma. Adenomas usually do not have any symptoms but are more likely to rupture than any of the liver tumors and therefore patients with these tumors need to seek medical advice. The long-term use of oral contraceptives is associated with the development of adenomas, and sometimes if a woman stops using that form of birth control, the adenoma may shrink. If the tumors do not shrink quickly surgical removal is indicated.

Focal Nodular Hyperplasia (FNH)

With this type of benign tumor, a nodule (often containing central scar tissue) grows in the liver. An FNH tumor usually does not present any symptoms, but if it is unusually large or causing pain, surgical removal often is recommended. This is because in rare instances these tumors can rupture; removing the ones that are large and most likely to cause problems can be prudent. They are found more frequently in women than in men, and it’s thought that hormones “feed” FNH tumors

Other Liver Diseases

Alpha 1-Antitrypsin (AAT) Deficiency. AAT (also known as alpha1 proteinase inhibitor) is a protein that is made primarily in the liver. AAT belongs to a group of proteins which help prevent certain white blood cell enzymes (proteinases) from going beyond their regular infection-fighting functions to attack healthy body tissue. When the body doesn’t make enough AAT, the lungs often don’t work as efficiently (patients tend to develop emphysema) and the liver may be adversely affected, leading to cirrhosis. AAT deficiency is an inherited condition with two defective genes passed along–one by each parent. This rare deficiency affects fewer than 100,000 Americans.

Familial Amyloidosis Polyneuropathy

This inherited disease, which occurs most often in people of Swedish and Portuguese descent, is a mutation of the transthyretin (TTR) molecule. Instead of the TTR protein being formed, an incorrect protein - amyloid - is produced. With this condition, amyloids are found throughout the body, destroying nerves and interfering with other organs. Neurological symptoms, low blood pressure, and muscle wasting are common manifestations of the disease, which can run a course of several years but has no cure. Because TTR is made in the liver, liver transplantation has been used as treatment.

Mushroom poisoning

Eating wild mushrooms can make a person severely ill, or in the worst-case scenario, cause a painful death. One particular type of mushroom, the Amanita phalloides, causes about 90 percent of all mushroom eating-related deaths. Mushroom poisoning typically involves a latent period of as long as 24 hours, when no symptoms are felt, followed by extreme gastrointestinal upset; damage to several organs, including the liver; heart failure; seizures; and coma.

The mushrooms’ toxins can have an especially severe effect on the liver, attacking the organ’s cells and shutting down some of its key functions. When the liver begins to fail but there is medical reason to believe the patient could survive the poisoning, that person may be a candidate for liver transplantation.

Fatty liver and Non-Alcoholic Steatohepatitis

In this condition an excessive amount of fat accumulates in the liver due to a variety of reasons. The most common reasons are excess weight, diabetes, excessive use of alcohol and hepatitis C. Because of the rapid rise in obesity in America, including among children, the number of individuals with fatty liver is rapidly rising. Most patients with fatty liver related to obesity or diabetes retain normal liver function and do not experience any problems during their lifetime. However, in some patients the excess fat causes inflammation in the liver known as Nonalcoholic steatohepatitis or NASH, as the condition sometimes is called. The damage is similar to that which occurs with alcoholic liver disease, but with NASH, the patients have consumed little or no alcohol. Like other liver diseases, NASH is typically “silent” in its early stage, and the first indication of the disease often comes from a routine blood test. Elevated liver enzymes indicate to a physician that something is irregular, leading to more tests and elimination of other conditions. It takes a liver biopsy to confirm a diagnosis of NASH; if there is only fat in the liver, but no inflammation or damage, that’s indicative of a simple fatty liver.

There is currently no treatment for NASH, but studies indicate that by losing weight, getting more exercise, and eating a healthy diet, patients can stop or even reverse the damage caused by NASH.

Polycystic liver disease

This is a congenital (individuals are born with this problem) condition, which may not be recognizable until adulthood. When large cysts form in the liver, the condition is known as polycystic liver disease. It’s common for patients with polycystic liver disease to also have cysts in their kidneys, or polycystic kidney disease. When polycystic liver disease is severe, the liver looks like a sponge. Polycystic liver disease rarely causes liver failure, despite the presence of hundreds of cysts. The most common symptoms are enlargement of the abdomen and pain, which sometimes require treatment. Treatment consists of either aspiration under x-ray guidance or “unroofing” by creating an opening in the cyst wall for drainage, a procedure known as fenestration. The relief from aspiration is less durable than with fenestration. Rarely, the severity of the pain calls for liver transplantation.

Wilson’s disease: Normally, the liver helps the body rid itself of copper. In people with Wilson’s disease, the gene responsible for this process is defective, and copper builds up in the liver, the brain, and other organs. Over time, too much copper in the liver can lead to symptoms such as jaundice and swelling of the abdomen and, untreated, lead to acute liver failure, a need for liver transplantation, or death. Many patients, when diagnosed early on, respond well to the drugs that help remove copper from the bloodstream. They must take the medicine for the rest of their lives. There is an acute form of Wilson’s disease in which the copper overload is swift and the organ damage is rapid. In these cases with liver failure, liver transplantation is life-saving.

Bilary & Pancreatic Conditions

Biliary Injury

Laparoscopic removal of the gall bladder has many benefits, but one of its possible complications is injury to the bile ducts. In as much as 1 percent of these procedures, there is unintentional trauma to the bile ducts, which may cause bile to leak or the bile ducts to become narrow (bile duct stricture). There are other causes of bile duct stricture, such as cancer, but people who have persistent pain or discomfort 10-14 days after laparoscopic gall bladder surgery might have had a bile duct injury during the procedure. Some patients have fever or develop jaundice related to the injury, as well.

Bile duct repair involves surgery that can be relatively simple or complex; if infection has occurred, that must be treated, also. The earlier a bile duct injury is detected and repaired, the better. Over time, an untreated injured duct will not drain properly, leading to a build-up of bile in the liver.

Primary sclerosing cholangitis (PSC). This slow, progressive disease affects the bile ducts both inside and outside the liver. Inflammation and scarring cause the ducts to narrow, and bile accumulates in the liver. Many times, patients also have inflammatory bowel disease. Patients with PSC have an increased risk of developing cancer in the bile ducts. More common in men than in women, PSC carries symptoms of itching, jaundice, fatigue and bouts of fever. Sometimes blocked bile ducts can be opened through surgery or other procedures, improving the bile flow. When the ducts remain affected, liver failure may occur after several years. Liver transplantation is the only apparent cure.

Primary biliary cirrhosis (PBC) is caused by chronic inflammation of the bile ducts inside the liver, which progressively destroy the bile ducts. It is an autoimmune condition of unknown cause that leads to cirrhosis. PBC has a long and protracted course of progression. Some medicines are available for PBC in the early stages, but when cirrhosis has set in and symptoms of liver failure begin to show liver transplantation is the best treatment option.

Cholangiocarcinoma

Cancer of the bile ducts, or cholangiocarcinoma, is slow-growing, but also not usually diagnosed until it is in an advanced stage. The malignant tumors block the bile ducts, which causes jaundice. The best possible treatment is to remove the tumor-containing bile ducts. If the tumor is situated in the bile ducts as they come out of the liver, removal of a portion of the liver along with the bile duct may be required to remove the tumor. Even if the tumors cannot be removed, there are endoscopic and surgical techniques that can unblock the obstructions. When surgery is not possible, radiation either alone or with chemotherapy is a course of treatment.

Pancreatic Cancer

Cancer of the pancreas, the fourth leading cause of cancer death in America, is typically aggressive, hard to treat and metastasizes (spread) to other organs, including the liver. In relatively few cases, a complex surgery known as the Whipple procedure is performed to remove the cancer. Most patients are not candidates for the surgery and receive a combination of chemotherapy and radiation. Unfortunately, the prognosis for patients at this stage of the disease is very poor.

Pancreatic cancer can be very painful, so while there might not be a cure in sight, there are procedures that can help to keep the patient comfortable. For example, the bile ducts of patients with pancreatic cancer can become blocked, leading to jaundice and related lack of appetite and itching. By stent placement, gastroenterologists can open the blocked bile ducts and relieve the symptoms of jaundice. To ease the pain of pancreatic cancer, a celiac nerve block can be used to inject certain agents, such as alcohol or a steroid, into the identified nerve or nerves. The numbness brings pain relief that narcotic medications cannot match.

Pancreatic Cancer

What is pancreatic cancer?

Pancreatic cancer is the fourth most common cancer in men and women in the US, according to the American Cancer Society. The majority of pancreatic cancer cases occur in people 50 years of age or older.

There are several types of pancreatic cancers, including:

• adenocarcinoma of the pancreas - the most common type of pancreatic cancer; occurs in the lining of the pancreatic duct.
• cystadenocarcinoma - a rare pancreatic cancer.

Some noncancerous tumors in the pancreas include:

• insulinoma - a rare pancreatic tumors that secretes insulin, the hormone that lowers glucose levels in the blood.
• gastrinoma - a tumor which secretes above average levels of gastrin, a hormone which stimulates the stomach to secrete acids and enzymes. Gastrinoma can cause peptic ulcers.
• glucagonoma - a tumor that secretes glucagon, a hormone which raises levels of glucose in the blood, leading to a rash.

What are the symptoms of pancreatic cancer?

The following are the other most common symptoms of pancreatic cancer. However, each individual may experience symptoms differently. Symptoms may include:

• pain in the upper abdomen
• poor appetite
• weight loss
• jaundice
• indigestion
• nausea
• vomiting
• diarrhea
• fatigue

The symptoms of pancreatic cancer may resemble other conditions or medical problems. Consult a physician for diagnosis.

How is pancreatic cancer diagnosed?

In addition to a complete medical history and medical examination, diagnostic procedures for pancreatic cancer may include:

• ultrasound - a diagnostic technique which uses high-frequency sound waves to create an image of the internal organs.
• computerized tomography (CT or CAT scan) - a non-invasive procedure that takes cross-sectional images of the brain or other internal organs; to detect any abnormalities that may not show up on an ordinary x-ray.
• magnetic resonance imaging (MRI) - a non-invasive procedure that produces two-dimensional views of an internal organ or structure, especially the brain and spinal cord.
• endoscopic retrograde cholangiopancreatography (ERCP) - this procedure involves inserting an endoscope (viewing tube) through the stomach and into the small intestine. A special dye injected during this procedures shows the ducts in the biliary system.
• biopsy of the pancreas
• special blood tests

Treatment for pancreatic cancer:

Treatment depends on the extent of the cancer and type of cancer. Specific treatment will be determined by your physician.

Treatment may include:

• surgery to remove the tumor or entire pancreas and/or the small intestine
• radiation therapy - high-dose x-rays used to kill cancer cells
• chemotherapy - drugs used to kill cancer cells
• pain medication
• oral enzyme preparations
• insulin treatment

The Pancreas

Anatomy of the pancreas:

The pancreas is an elongated, tapered organ located across the back of the abdomen, behind the stomach. The right side of the organ (called the head) is the widest part of the organ and lies in the curve of the duodenum. The tapered left side extends slightly upward (called the body of the pancreas) and ends near the spleen (called the tail).

The pancreas is made up of two types of tissue:

• exocrine tissue
  The exocrine tissue secretes digestive enzymes. These enzymes are secreted into a network of ducts that join the main pancreatic duct, which runs the length of the pancreas.

• endocrine tissue
  The endocrine tissue, which consists of the islets of Langerhans, secretes hormones into the bloodstream.

Functions of the pancreas:

The pancreas has digestive and hormonal functions:

• The enzymes secreted by the exocrine tissue in the pancreas help break down carbohydrates, fats, proteins, and acids in the duodenum. These enzymes travel down the pancreatic duct into the bile duct in an inactive form. When they enter the duodenum, they are activated. The exocrine tissue also secretes a bicarbonate to neutralize stomach acid in the duodenum (the first section of the small intestine).

• The hormones secreted by the endocrine tissue in the pancreas are insulin and glucagon (which regulate the level of glucose in the blood), and somatostatin (which prevents the release of the other two hormones).

The Pancreas

Anatomy of the pancreas:

The pancreas is an elongated, tapered organ located across the back of the abdomen, behind the stomach. The right side of the organ (called the head) is the widest part of the organ and lies in the curve of the duodenum. The tapered left side extends slightly upward (called the body of the pancreas) and ends near the spleen (called the tail).

The pancreas is made up of two types of tissue:

• exocrine tissue
  The exocrine tissue secretes digestive enzymes. These enzymes are secreted into a network of ducts that join the main pancreatic duct, which runs the length of the pancreas.

• endocrine tissue
  The endocrine tissue, which consists of the islets of Langerhans, secretes hormones into the bloodstream.

Functions of the pancreas:

The pancreas has digestive and hormonal functions:

• The enzymes secreted by the exocrine tissue in the pancreas help break down carbohydrates, fats, proteins, and acids in the duodenum. These enzymes travel down the pancreatic duct into the bile duct in an inactive form. When they enter the duodenum, they are activated. The exocrine tissue also secretes a bicarbonate to neutralize stomach acid in the duodenum (the first section of the small intestine).

• The hormones secreted by the endocrine tissue in the pancreas are insulin and glucagon (which regulate the level of glucose in the blood), and somatostatin (which prevents the release of the other two hormones).

Splenomegaly (Enlarged spleen)

What is splenomegaly?

Splenomegaly is an enlargement of the spleen beyond its normal size. The spleen is an organ involved in the production and maintenance of red blood cells, the production of certain circulating white blood cells, and is a part of the lymph system and the immune system. Because of its wide variety of functions, the spleen may be affected by many conditions involving the blood or lymph system, and by infection, cancers, liver disease, and parasites.

What are the causes?

1. Infections
   
   
   
   • Viral infections
     
   • Parasitic infections
     
   • Bacterial infections
   
   
2. Diseases involving the liver
   
   
   
   • Cirrhosis (portal vein obstruction, portal hypertension)
     
   • Sclerosing cholangitis
     
   • Wilson’s disease
     
   • Biliary atresia
     
   • Cystic fibrosis
   
   
3. Haemolytic anaemias
   
   
   
   • Thalassaemia
     
   • Haemoglobinopathies
     
   • Haemolytic anaemia due to G6PD deficiency
     
   • Immune haemolytic anaemia
   
   
4. Cancer
   
   
   
   • Leukaemia
     
   • Lymphoma
     
   • Hodgkin’s disease
   
   
5. Other causes
   
   
   
   • Sarcoidosis

What is the diagnosis?

Splenomegaly is usually detected during a physical examination by a health care provider. A detailed examination of the abdomen will probably be performed. Diagnostic tests may be required, including:

1. Blood tests such as a CBC
   
2. Tests for suspected causes
   
3. Abdominal film or CT scan

How to take care at home?

Rupture of the enlarged spleen is particularly possible in infectious mononucleosis and several other causes of splenomegaly. Appropriate limitation of activity, including avoiding contact sports, will help prevent trauma that might cause the spleen to rupture. Care will be required for the specific condition causing the splenomegaly. Follow the instructions given by the doctor regarding appropriate care

Gall bladder cancer

What is gall bladder cancer?

Gallbladder is a small, pear-shaped organ on the right side of the abdomen, just beneath the liver. Its main function is to store bile; a fluid that helps digest fats and carries cholesterol and toxins from the body. Cancers of the gallbladder and bile duct (the gallbladder and bile duct together are called the biliary tract) seldom produce symptoms in the early stages. In fact, early gallbladder cancer is often discovered only when the gallbladder is removed as a treatment for gallstones. Otherwise, biliary tract cancers are often quite advanced by the time they are diagnosed.

What are the symptoms?

Gallbladder cancer rarely produces signs and symptoms in the early stages. When symptoms do appear, they often resemble those of other, more common, gallbladder problems, such as gallstones or infection.

The signs and symptoms include:

• Abdominal pain
  
• Nausea and vomiting
  
• Jaundice results from high blood levels of bilirubin. Normally, bilirubin is metabolised in the liver and eliminated through the bile ducts. But a blocked bile duct can cause bilirubin to accumulate in the blood.
  
• Loss of appetite or weight

Jaundice is the most common initial sign of bile duct cancer (cholangiocarcinoma). Other signs and symptoms include:

• Enlarged gallbladder
  
• Intense itching (pruritus)
  
• Brown coloured urine
  
• Loss of appetite and weight
  
• White or clay-coloured stools
  
• Fever

How is the diagnosis made?

Many gallbladder cancers are discovered when a pathologist examines a gallbladder that has been removed for symptoms of gallstones. But many gallbladder and most bile duct cancers are found only after related signs and symptoms appear. The doctor conducts a physical exam and the following tests:

1. Blood tests: This is to check for elevated levels of bilirubin or the enzyme alkaline phosphatase.
   
   
2. Ultrasound: Ultrasound is usually one of the first tests done in the evaluation of patients with jaundice and is especially good at diagnosing the presence of gallstones and obstructed bile ducts. It can also show the presence and extent of tumours.
   
   
3. CT scan: This helps determine if cancerous cells have spread to the common bile duct, lymph nodes or the liver.
   
   
4. MRI: This test uses a powerful magnetic field and radio waves to create images and can help determine whether the flow of bile is blocked or a tumour has invaded the liver.
   
   
5. Endoscopic retrograde cholangiopancreatiography (ERCP): This test is most sensitive for detecting an obstruction of the bile ducts and its cause, and can also be used in preparation for surgery. ERCP can also allow a biopsy to be performed, confirming a diagnosis.
   
   
6. Laparoscopy: This is often used to confirm how far the cancer has spread.
   
   
7. Biopsy: In this procedure, a small sample of tissue is removed and examined for cancerous cells under a microscope. It is the only way to make a definitive diagnosis of cancer.

What is the treatment?

Treatment for biliary tract cancers depends on the type and stage of cancer as well as on the patient’s age and overall health. The goal of treatment is to completely eliminate the cancer. Unfortunately, this is often not possible and the focus may need to be changed to treating the signs and symptoms the cancer is causing or preventing the tumour from causing more harm.

• For gallbladder cancer: Surgical removal of the gallbladder usually offers the best hope for people with gallbladder cancer. If the tumour is very small and has not spread to the deeper layers of the gallbladder tissue, the surgeon may perform a simple cholecystectomy, which removes only the gallbladder. If the cancer is more advanced, some liver tissue and nearby lymph nodes are removed along with the gallbladder.
  
  
• For bile duct cancer: Surgery usually offers the best chance for people with bile duct cancer. But the type of surgery varies depending on the location of the cancer and how extensive it is. For example, removing the affected part of the liver treats tumours that are within the liver. Tumours that develop where the hepatic duct leaves the liver (perihilar tumours) are usually treated by surgically removing a portion of the bile duct, the gallbladder, and parts of the liver, pancreas and the small intestine.

What are the complications?

A tumour that blocks the bile duct can cause pain, jaundice, nausea and vomiting. Tumours located where the pancreatic duct enters the small intestine may block the small intestine, preventing normal passage of food. Tumours may make it difficult to digest and absorb nutrients from the food by blocking the flow of the pancreatic enzymes. The most serious complication of biliary tract cancer is metastasis. Since biliary tract cancers are rarely discovered in the early stages, they often tend to spread to the other organs around the gall bladder, including liver, stomach, pancreas and intestines or to the nearby lymph nodes.

Congenital Liver Defects

What are congenital liver defects?

Defects of the liver at birth usually affect the bile ducts. Though rare, some congenital liver defects include the following:

• biliary atresia - a condition in which the bile ducts are absent or have developed abnormally.

• choledochal cyst - a malformation of the hepatic duct that can obstruct flow of bile in infants.

What are the indicators that a congenital liver defect may be present?

Congenital liver defects that affect the flow of bile share some common symptoms. The following are the most common symptoms of congenital liver defect. However, each individual may experience symptoms differently. Symptoms may include:

• jaundice - yellowing of the skin and eyes.
• dark urine
• pale stool

The symptoms of congenital liver defects may resemble other medical conditions or problems. Always consult your child's physician for a diagnosis.

How are congenital liver defects diagnosed?

Congenital liver defects that affect the flow of bile are usually diagnosed at birth or shortly afterward. In addition to a complete medical history and physical examination, diagnostic procedures for a congenital liver defect may include the following:

• laboratory tests
  
  
• liver function tests - a series of special blood tests that can determine if the liver is functioning properly.
  
  
• liver biopsy - a procedure in which tissue samples from the liver are removed (with a needle or during surgery) from the body for examination under a microscope.

Treatment for congenital liver defects:

Specific treatment for congenital liver defects will be determined by your child's physician based on:

• your child's age, overall health, and medical history
• extent of the disease
• your child's tolerance for specific medications, procedures, or therapies
• expectations for the course of the disease
• your opinion or preference

Treatment may include surgery to reconstruct or bypass the bile ducts. Sometimes, a liver transplant may be necessary.

Diagnostic Procedures

How is a liver, biliary, or pancreatic disorder diagnosed?

In order to reach a diagnosis for liver, biliary, or pancreatic disorders, a thorough and accurate medical history will be taken by your physician, noting the symptoms you have experienced and any other pertinent information. A physical examination is also done to help assess the problem more completely.

The most commonly performed diagnostic procedures include the following:

• cholecystography (Also called oral cholecystography or gallbladder series.) - a series of x-rays are taken of the gallbladder after a special contrast dye is swallowed, making it possible to detect gallstones, cholecystitis, and other abnormalities.
• computed tomography scan (CT or CAT scan) - a diagnostic imaging procedure using a combination of x-rays and computer technology to produce cross-sectional images (often called slices), both horizontally and vertically, of the body. A CT scan shows detailed images of any part of the body, including the bones, muscles, fat, and organs. CT scans are more detailed than general x-rays.
• endoscopic retrograde cholangiopancreatography (ERCP) - a procedure that allows the physician to diagnose and treat problems in the liver, gallbladder, bile ducts, and pancreas. The procedure combines x-ray and the use of an endoscope - a long, flexible, lighted tube. The scope is guided through the patient's mouth and throat, then through the esophagus, stomach, and duodenum. The physician can examine the inside of these organs and detect any abnormalities. A tube is then passed through the scope, and a dye is injected which will allow the internal organs to appear on an x-ray.
• esophagogastroduodenoscopy (Also called EGD or upper endoscopy.) - a procedure that allows the physician to examine the inside of the esophagus, stomach, and duodenum. A thin, flexible, lighted tube, called an endoscope, is guided into the mouth and throat, then into the esophagus, stomach, and duodenum. The endoscope allows the physician to view the inside of this area of the body, as well as to insert instruments through a scope for the removal of a sample of tissue for biopsy (if necessary).

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• hepatobiliary scintigraphy - an imaging technique of the liver, bile ducts, gallbladder, and upper part of the small intestine.
• laparoscopy - use of a viewing tube with a lens or camera (and a light on the end), which is inserted through a small incision in the abdomen to examine the contents of the abdomen and remove tissue samples.
• liver biopsy - a procedure in which tissue samples from the liver are removed (with a needle or during surgery) from the body for examination under a microscope.
• magnetic resonance imaging (MRI) - a diagnostic procedure that uses a combination of large magnets, radiofrequencies, and a computer to produce detailed images of organs and structures within the body.
• percutaneous transhepatic cholangiography (PTC) - a needle is introduced through the skin and into the liver where the dye (contrast) is deposited and the bile duct structures can be viewed by x-ray.
• ultrasound (Also called sonography.) - a diagnostic imaging technique, which uses high-frequency sound waves and a computer to create images of blood vessels, tissues, and organs. Ultrasounds are used to view internal organs of the abdomen such as the liver, spleen, and kidneys and to assess blood flow through various vessels.
• x-ray - a diagnostic test which uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film.

Biliary Cirrhosis / Bile Duct Cancer

What is biliary cirrhosis?

Biliary cirrhosis is a rare form of liver cirrhosis, caused by disease or defects of the bile ducts. Symptoms usually include cholestasis (accumulation of bile in the liver). There are two types of biliary cirrhosis:

• primary biliary cirrhosis - inflammation and destruction of bile ducts in the liver.
• secondary biliary cirrhosis - results from prolonged bile duct obstruction or narrowing or closure of the bile duct.

What is bile duct cancer (cholangiocarcinoma)?

Next to gallstones, cancer is the most common cause of bile duct obstruction. The majority of bile duct cancers develop in the part of the ducts that are outside the liver and are sometimes referred to as extrahepatic tumors. Most bile duct cancers are adenocarcinomas which means they develop from the glandular cells of the bile duct.

What are the symptoms of bile duct cancer?

The following are the most common symptoms of bile duct cancer. However, each individual may experience symptoms differently. Symptoms may include:

• jaundice - yellowing of the skin and eyes.
• abdominal pain
• poor appetite
• weight loss
• itching

The symptoms of bile duct cancer may resemble other medical conditions or problems. Always consult your physician for a diagnosis.

How is bile duct cancer diagnosed?

In addition to a complete medical history and physical examination, diagnostic procedures for bile duct cancer may include the following:

• ultrasound (Also called sonography.) - a diagnostic imaging technique which uses high-frequency sound waves to create an image of the internal organs. Ultrasounds are used to view internal organs of the abdomen such as the liver spleen, and kidneys and to assess blood flow through various vessels.
• computed tomography scan (CT or CAT scan) - a diagnostic imaging procedure using a combination of x-rays and computer technology to produce cross-sectional images (often called slices), both horizontally and vertically, of the body. A CT scan shows detailed images of any part of the body, including the bones, muscles, fat, and organs. CT scans are more detailed than general x-rays.
• cholangiography - x-ray examination of the bile ducts using an intravenous (IV) dye (contrast).
• biopsy - a procedure in which tissue samples are removed (with a needle or during surgery) from the body for examination under a microscope.
• endoscopic retrograde cholangiopancreatography (ERCP) - a procedure that allows the physician to diagnose and treat problems in the liver, gallbladder, bile ducts, and pancreas. The procedure combines x-ray and the use of an endoscope - a long, flexible, lighted tube. The scope is guided through the patient's mouth and throat, then through the esophagus, stomach, and duodenum. The physician can examine the inside of these organs and detect any abnormalities. A tube is then passed through the scope, and a dye is injected which will allow the internal organs to appear on an x-ray.

Treatment for bile duct cancer:

Specific treatment for bile duct cancer will be determined by your physician based on:

• your age, overall health, and medical history
• extent of the disease
• cause of the disease
• your tolerance for specific medications, procedures, or therapies
• expectations for the course of the disease
• your opinion or preference

Treatment may include:

• surgery
  Surgery may be necessary to remove cancerous tissue, as well as nearby noncancerous tissue. Surgery may also be used to relieve blockage of the bile duct. Surgery may also be used to palliate or relieve symptoms.
• external radiation (external beam therapy)
  External radiation is a treatment therapy that precisely sends high levels of radiation directly to the cancer cells. The machine is controlled by the radiation therapist. Since radiation is used to kill cancer cells and to shrink tumors, special shields may be used to protect the tissue surrounding the treatment area. Radiation treatments are painless and usually last a few minutes. Radiation therapy may be given after surgery, to kill small areas of cancer that may not be seen during surgery, or instead of surgery. Radiation may also be used to ease (palliate) symptoms such as pain, bleeding, or blockage.
• chemotherapy
  Chemotherapy is the use of anticancer drugs to treat cancerous cells. In most cases, chemotherapy works by interfering with the cancer cell’s ability to grow or reproduce. Different groups of drugs work in different ways to fight cancer cells. The oncologist will recommend a treatment plan for each individual.

Cholecystitis

What is cholecystitis?

Cholecystitis is an inflammation of the gallbladder wall and nearby abdominal lining. Cholecystitis is usually caused by a gallstone in the cystic duct, the duct that connects the gallbladder to the hepatic duct. Other causes of cholecystitis may include the following:

• bacterial infection in the bile duct system
  The bile duct system is the drainage system that carries bile from the liver and gallbladder into the area of the small intestine called the duodenum.
  
  
• tumor of the pancreas or liver
  
  
• decreased blood supply to the gallbladder
  This can sometimes occur in persons with diabetes.
  
  
• gallbladder "sludge"
  This "sludge" is a thick material that cannot be absorbed by bile in the gallbladder and most commonly occurs in pregnant women or individuals who have experienced a rapid weight loss.

Cholecystitis can occur suddenly or gradually over many years.

What are the symptoms of cholecystitis?

A typical attack of cholecystitis usually lasts two to three days. The following are the most common symptoms of gallstones. However, each individual may experience symptoms differently. Symptoms may include:

• intense and sudden pain in the upper right part of the abdomen
  
  
• recurrent painful attacks for several hours after meals
  
  
• pain (often worse with deep breaths and extends to lower part of right shoulder blade)
  
  
• nausea
  
  
• vomiting
  
  
• rigid abdominal muscles on right side
  
  
• slight fever
  
  
• chills
  
  
• jaundice - yellowing of the skin and eyes.
  
  
• itching (rare)
  
  
• loose, light-colored bowel movements
  
  
• abdominal bloating

The symptoms of cholecystitis may resemble other medical conditions or problems. Always consult your physician for a diagnosis.

How is cholecystitis diagnosed?

In addition to a complete medical history and medical examination, diagnostic procedures for cholecystitis may include the following:

• ultrasound (Also called sonography.) - a diagnostic imaging technique which uses high-frequency sound waves to create an image of the internal organs. Ultrasounds are used to view internal organs of the abdomen such as the liver spleen, and kidneys and to assess blood flow through various vessels.
  
  
• hepatobiliary scintigraphy - an imaging technique of the liver, bile ducts, gallbladder, and upper part of the small intestine.
  
  
• cholangiography - x-ray examination of the bile ducts using an intravenous (IV) dye (contrast).
  
  
• percutaneous transhepatic cholangiography (PTC) - a needle is introduced through the skin and into the liver where the dye (contrast) is deposited and the bile duct structures can be viewed by x-ray.
  
  
• endoscopic retrograde cholangiopancreatography (ERCP) - a procedure that allows the physician to diagnose and treat problems in the liver, gallbladder, bile ducts, and pancreas. The procedure combines x-ray and the use of an endoscope - a long, flexible, lighted tube. The scope is guided through the patient's mouth and throat, then through the esophagus, stomach, and duodenum. The physician can examine the inside of these organs and detect any abnormalities. A tube is then passed through the scope, and a dye is injected which will allow the internal organs to appear on an x-ray.
  
  
• computed tomography scan (CT or CAT scan) - a diagnostic imaging procedure using a combination of x-rays and computer technology to produce cross-sectional images (often called slices), both horizontally and vertically, of the body. A CT scan shows detailed images of any part of the body, including the bones, muscles, fat, and organs. CT scans are more detailed than general x-rays.

Treatment for cholecystitis:

Specific treatment for cholecystitis will be determined by your physician based on:

• your age, overall health, and medical history
• extent of the disease
• your tolerance of specific medicines, procedures, or therapies
• expectations for the course of the disease
• your opinion or preference

Treatment for acute cholecystitis usually involves a hospital stay, to reduce stimulation to the gallbladder. Antibiotics are usually administered to reduce the inflammation and/or fight the infection. Sometimes, the gallbladder is surgically removed.

Other treatment options may include:

• oral dissolution therapy - drugs made from bile acid are used to dissolve the stones
  
  
• medications (to prevent the formation of gallstones)
  
  
• low-fat diet
  
  
• pain management

The overall prognosis for cholecystitis is favorable. In some individuals, complications may arise if other organs are involved. Gallstones can return in the bile duct system after surgical removal of the gallbladder.