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Pancreatitis

The pancreas is very important to a child’s normal growth and development. It sits in the back of the abdomen, behind the stomach and does two things: 1) It produces enzymes, which help with digesting food and 2) it produces hormones such as insulin and glucagon, which help control blood sugar. In the fetus and newborn baby, the pancreas produces almost no pancreatic enzymes (amylase and lipase); then, the child’s pancreas starts making these enzymes, which help in the digestion of food. Production of these enzymes is at full adult levels by age 2 years.

ACUTE PANCREATITIS

Although children can have many of the pancreatic diseases seen in adults, including pancreatitis, other problems are special to children. Table 1 lists the different types of pancreatitis found in childhood, divided into acute (symptoms are new) and chronic (symptoms are old) types.

Acute pancreatitis is where the pancreas becomes inflamed or irritated. This causes belly pain in children varying from mild to severe, ranging from mild inflammation to severe pancreatitis with bleeding and death of the pancreas.

The usual patient with pancreatitis has upper belly pain and tenderness with nausea and vomiting. There also may be back pain and shoulder pain. Approximately 5% to 10% of patients are severely ill with low blood pressure, abnormal blood chemistry tests, severe infection, and total body swelling which may worsen to multiple organ failure.

Because signs and symptoms are often vague, diagnosis may not be made until exploratory surgery or even autopsy. The usual diagnosis before surgery is appendicitis or some other severe infection. The difficulty in making the correct diagnosis is because they usually have other problems besides pancreatitis.

Causes of Pancreatitis

Table 2 lists the different causes of pancreatitis in children, which may be divided into obstructive (drainage from the pancreas is blocked) and nonobstructive (no blockage) causes. Approximately half of the time, the cause of the pancreatitis is never found. Injuries, infections, and drugs are otherwise the most frequent causes. Although there is some controversy about whether corticosteroids cause pancreatitis, approximately 15% of children with severe kidney disease get pancreatitis, and the chance may increase almost three times in children with kidney disease who receive corticosteroids. There is also an increased chance of pancreatitis after kidney transplantation, but some of the same drugs are used in these patients as well. Secondary hyperparathyroidism, a problem of the parathyroid glands, and an increased level of calcium in the blood are also causes. In addition, the pancreas can be infected with viruses in patients on immunosuppressive drugs such as steroids, chemotherapy, or transplant drugs or in children with liver disease who receive corticosteroids.

The acute (symptoms are new) types of pancreatitis that run in families tend to occur over and over again (chronic), eventually producing pancreatic scarring with calcium deposits. Widespread calcium deposits in the pancreas have been found in children with cystic fibrosis, chronic familial pancreatitis, and hyperparathyroidism. Mumps pancreatitis is not common in children today. Pancreatitis has been found in children with other diseases such as collagen vascular disorders, familial aminoaciduria and hyperlipoproteinemia. Additional causes of pancreatitis include infectious mononucleosis, porphyria, and ulcerative colitis.

Obstructive forms of pancreatitis are less common in children than in adults. Obstruction occurs when there is a blockage of the main tube that releases the pancreas enzymes. This can be caused by a parasitic worm, Ascaris lumbricoides. This does cause pancreatitis in children in the United States, but it is much more likely to happen to children in other countries where this intestinal worm is commonly found. In children gallstones and related problems of the tubes that carry bile into the intestine are less common causes of pancreatitis. Birth defects of the pancreas and narrowings of the bile tubes and pancreatic tubes have been found more and more in children with repeat episodes of pancreatitis since doctors started using a new test called endoscopic retrograde cholangiopancreatography (ERCP). ERCP is a test that uses telescopic equipment placed through the patient’s mouth to look at these tubes.

Most children with choledochal cysts have a so-called common channel or tube with a high, abnormal connection between the pancreatic tube and the main bile tube with free flow of pancreatic fluid into the cyst. Although doctors have not shown that bile travels backward into the pancreatic tube, acute pancreatitis occurs in some cases of a choledochal cyst. Pancreatitis is extremely rare after removal of a choledochal cyst. Older children with gallstones may develop pancreatitis, but this is not common. Children with severe infections who develop clogged bile tubes and occasional patients being fed with intravenous nutrition (TPN) may get acute pancreatitis.

Diagnostic Tests

Pancreatitis can be diagnosed early on by blood tests, amylase and lipase levels, which are often several times higher than normal in children with an acute or severe course, although normal in others (Table 3).

Increased levels of amylase in the blood can be seen in children with other medical problems, such as kidney failure, blockage of the main bile tube, inflammation of the main salivary glands, ulcer disease, intestinal blockage, a rupture of the intestine, liver injury, inflammation of the glands that make saliva, ectopic pregnancy (pregnancy outside the uterus), narcotics (pain medicines), and dead intestine. Doctors must make sure the patient does not have one of these problems before the diagnosis of acute pancreatitis can be made. A more detailed study of pancreatic enzymes in the blood (amylase isoenzymes) has been of some benefit in separating acute pancreatitis from other diseases that also may cause high levels of amylase in the blood, such as injury to the main salivary gland. Ultrasound, computed tomography (CT), and magnetic resonance imaging (MRI) have been used to show the size of the pancreas and its main tube, if there are any abnormal cyst-like growths, and the layout of the bile tubes. Ultrasound has been especially helpful in children, and it is one of the best studies for repeated follow-up pictures. CT scans have been useful for the study of children with pancreatic injury and pancreatic tube swelling. ERCP works well in children to show the position of the bile and pancreatic tubes, although its use is not recommended in patients who just started having symptoms of pancreatitis. The procedure itself may cause pancreatitis. Magnetic resonance cholangiopancreatography (MRCP) uses MRI pictures and may be used in place of ERCP sometimes. Children with jaundice (yellow skin and eyes) thought to be a result from pancreatitis may benefit from a nuclear medicine test (hepato-iminodiacetic acid scan).

Treatments Before Surgery May Be Required

Table 4 lists the different treatments for acute pancreatitis, but supportive measures are the main treatment. The first treatment of acute pancreatitis is stopping feeds to decrease the activity of the pancreas. Unless pancreatitis is especially mild, a tube should be placed through the patient’s nose into their stomach for drainage and relief, because most patients have vomiting and absent bowel function. Appropriate IV fluids should be given to replace loss of fluid into the swollen pancreas and to correct fluid problems. A large IV placed in the neck or chest and directed towards the heart usually is needed to give nutrition, IV fluid, and sometimes antibiotics. Pain can be severe, so narcotics (pain medicines) are given. It is probably best to avoid the use of morphine, which causes spasm of the pancreatic connection to the intestine. Antibiotics may or may not be used in patients with acute pancreatitis. Calcium may need to be given.

In severe acute pancreatitis, severe losses of plasma and blood into the body but outside of the bloodstream may require plasma and blood transfusions. Close watch of blood pressure, heart pressures and urine output provide the best guidelines for appropriate replacement. As pancreatitis worsens in severe cases, the need for the patient to breathe more increases while there is a decrease in the patient’s lung function. Extra fluid inside the belly, swelling inside the belly, diaphragm elevation, extra fluid inside the chest but outside the lungs, and belly splinting from pain may combine to produce partial lung collapse and abnormal lung blood flow. This may worsen to severe breathing problems and shock. Placement of the patient on a breathing machine may become necessary. Increased acid in the blood may need to be neutralized with other fluids.

Increased sugar in the blood and decreased calcium in the blood show severe pancreatic inflammation; whereas, elevated levels of two other blood tests: serum lactate dehydrogenase and aspartate aminotransaminase (AST) show pancreas destruction. An elevated blood hemoglobin level shows dehydration in the beginning, but a later decrease means bleeding in the area of the pancreas. High fever usually responds to Tylenol. Steroids are not recommended, but nutrition given through a large IV has been extremely helpful in providing adequate calories to minimize weight loss and loss of strength during the period when the patient may not eat.

Exploratory surgery is not usually done for acute pancreatitis in children when the diagnosis is known. Operation on a severely ill child with unsuspected pancreatitis can lead to increased complications and death. Because pancreatitis is not usually suspected in children, the most common diagnosis before surgery is appendicitis. During the operation, death of fatty tissue and a large amount of fluid inside the belly with high levels of pancreatic enzyme (amylase) usually are found. Complications of pancreatitis, such as pseudocysts and abscesses near the pancreas, often require surgery (Figure 1). These problems usually occur 1 or more weeks after the beginning of pancreatitis, however, and surgery should be planned carefully.

Washing of the belly through a plastic tube placed just below the belly-button has been helpful when children with pancreatitis worsen and go into shock despite supportive therapy. This type of washing generally is continued for 24 to 72 hours, depending on how the patient does and what the drainage fluid looks like. If the drainage fluid contains high levels of pancreatic enzyme or blood or both, washing is continued to dilute the fluid inside the belly. If only a few bacteria are found in the drainage fluid, washing with antibiotics may be continued, but if many bacteria are found, if the drainage fluid continues to be bloody, or if the patient continues to worsen, exploratory surgery is probably needed. Imaging studies, such as ultrasound, CT, or MRCP should be performed first to show any problems that surgery may likely fix.

At planned surgery, the pancreas is examined directly. In patients with severe pancreatitis the pancreas is usually swollen, thickened, and bloody with some areas of gangrene (dead pancreas). Clearly dead tissue is removed. If no specific problems are found that can be repaired, such as blockage of a bile tube or a tumor, the belly is washed with warm salt water to clear waste and dilute the enzyme-containing fluid. Soft drains are placed through the skin near the pancreas, under both sides of the diaphragm and into the pelvis. After surgery, irrigation of the drains is performed at least three times a day to continue to dilute enzymes that may have been activated and to clear waste. This type of washing of the belly has played a major role in saving the lives of children with severe early acute pancreatitis. Placement of a feeding tube through the belly skin into the stomach or intestine has also been useful, but in the beginning, nutrition through IVs is done.

The number of children dying from acute pancreatitis has decreased through the years and is less than that noted in adults. Death from acute pancreatitis usually is from a combination of problems, including infection, bleeding, and shock leading to multiple organ failure. The chance of death from acute pancreatitis in childhood is less than 5%.

Chronic Pancreatitis

Chronic pancreatitis in children has many causes, including birth defects, hereditary, metabolic, inflammatory, and collagen vascular disorders. Most cases have unknown causes. Chronic pancreatitis should be thought of as a possible cause in children with repeat belly pain of unknown cause or in children with jaundice (yellowing of their skin and eyes) and only mild belly pain. Patients with chronic fibrosing pancreatitis usually do not have bile tube or intestinal disease, however, and they are more likely to have a disease that runs in families or to have cystic fibrosis, previous injury, or a combination of problems. Other than repeat belly pain and occasional yellowing of the skin and eyes, the symptoms vary and increase in frequency and severity until pancreatic failure occurs. Then patients have fatty stools which float and weight loss. Diabetes may occur late in a few patients.

Blood tests are often not helpful, but occasionally the secretin/pancreozymin test, which directly stimulates and measures pancreatic enzyme levels, may be helpful. Blood levels of pancreatic enzymes (amylase and lipase) and calcium; fasting blood sugar; and blood fat levels should be done. Tests of the patient’s stool looking at fat, the enzyme trypsin and nitrogen may show poor enzyme function. X-rays of the belly may show calcium deposits, especially in long-standing disease. Ultrasound may show thickening of the pancreas or pancreatic tube swelling in some patients (Figure 2).

ERCP or MRCP may show abnormalities of the pancreatic ducts (tubes), although the “chain-of-lakes” appearance often seen in adults is unusual in children. The doctor must make sure the patient does not have an abnormality of the parathyroid glands, hyperparathyroidism, before the patient is diagnosed with primary chronic pancreatitis.

If the diagnosis of chronic pancreatitis cannot be made by laboratory tests, and this is usually the case, exploratory surgery may be needed. The pancreas is found to be hard and bumpy and big all over. X-rays taken in the operating room are helpful, but if no correctable disease is found, increasing the size of the opening between the pancreas and the intestine should be done. Sometimes, this can be done at the time of ERCP. Careful evaluation should be done for stones or narrowings or both in the pancreas tube; a possible duplication cyst (an extra piece of intestine) in the first part of the small intestine; and possible abnormalities of the pancreas tubes, such as pancreas divisum (see Figure 3).

Familial or hereditary pancreatitis is a disease that runs in families and is caused by an abnormality of a gene on chromosome 7. This abnormality results in the patient’s own enzymes destroying their pancreas, causing acute and chronic pancreatitis. For symptomatic children with uneven pancreatic tube enlargement or stones or both, tube drainage performed by the Puestow method (Figure 4) has worked. Pancreatic tube stenting (placing a plastic or wire tube inside the pancreas tube to keep it open so that it can drain into the intestine) has been used as a treatment for hereditary pancreatitis, especially for narrowing of the bile tubes inside the liver, but most have found that this only works for a short time and that surgery usually is needed. For patients with chronic pancreatitis and normal pancreatic ducts, widening the opening between the pancreas and the intestine has provided long-term relief of symptoms in most patients.

SUGGESTED READING

American Society for Gastrointestinal Endoscopy: The role of ERCP in diseases of the biliary tree and pancreas: Guidelines for clinical application. Gastrointest Endosc 50:1-10, 1999.

This consensus statement concludes that currently ERCP is the best technique for duct visualization and provides the broadest range of therapeutic options.

Article and graphics adapted from O'Neill: Principles of Pediatric Surgery. © 2003, Elsevier.