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A majority of individuals born with pancreas divisum will not have symptoms. In some cases, pancreas divisum is only detected during autopsy. A small group of individuals will develop symptoms which commonly include abdominal pain, nausea, vomiting, and acute and chronic pancreatitis.
It is typically associated with abnormal embryological development, however adult cases can develop. It can result from growth of a bifid ventral pancreatic bud around the duodenum, where the parts of the bifid ventral bud fuse with the dorsal bud, forming a pancreatic ring. It can also result if the ventral pancreatic bud fails to fully rotate, so it remains on the right or if the dorsal bud rotates in the wrong direction, such that the duodenum is surrounded by pancreatic tissue. Blockage of the duodenum develops if inflammation (pancreatitis) develops in the annular pancreas.
Early signs of abnormality include polyhydramnios (an excess of amniotic fluid), low birth weight, and feeding intolerance immediately after birth.
Pancreas or Pancreatic divisum is a congenital anomaly in the anatomy of the ducts of the pancreas in which a single pancreatic duct is not formed, but rather remains as two distinct dorsal and ventral ducts.
X-ray computed tomography (CT scan) findings of cysts in the pancreas are common, and often are benign. In a study of 2,832 patients without pancreatic disease, 73 patients (2.6%) had cysts in the pancreas. About 85% of these patients had a single cyst. Cysts ranged in size from 2 to 38 mm (mean, 8.9 mm). There was a strong correlation between the presence of cysts and age. No cysts were identified among patients less than 40 years of age, while 8.7 percent of the patients aged 80 to 89 years had a pancreatic cyst.
Cysts also may be present due to intraductal papillary mucinous neoplasm.
A pancreatic fistula is an abnormal communication between the pancreas and other organs due to leakage of pancreatic secretions from damaged pancreatic ducts. An "external" pancreatic fistula is one that communicates with the skin, and is also known as a pancreaticocutaneous fistula, whereas an internal pancreatic fistula communicates with other internal organs or spaces. Pancreatic fistulas can be caused by pancreatic disease, trauma, or surgery.
Pancreas divisum is a malformation in which the pancreas fails to fuse. It is a rare condition that affects only 6% of the world's population, and of these few, only 1% ever have symptoms that require surgery.
Signs and symptoms of pancreatic pseudocyst include abdominal discomfort and indigestion.
Marked recent weight loss is a major clinical manifestation, and unresponsiveness of the ascites to diuretics is an additional diagnostic clue.
Periampullary cancer is a cancer that forms near the ampulla of Vater, an enlargement of the ducts from the liver and pancreas where they join and enter the small intestine.It consists of:
1. ampullary tumour from ampulla of Vater,
2. cancer of lower common bile duct, and
3. duodenal cancer adjacent to ampulla.
4. carcinoma head of pancreas
It presents with painless jaundice which may have waxing and waning nature because at times the sloughing of the tumor tissue relieves the obstruction partially.
Complication of pancreatic pseudocyst include infection, hemorrhage, obstruction and rupture. For obstruction, it can cause compression in the GI tract from the stomach to colon, compression in urinary system, biliary system, and arteriovenous system.
Pancreatic disorders are often accompanied by weakness and fatigue. The past Medical history may reveal previous disorders of the biliary tract or duodenum, abdominal trauma or surgery, and metabolic disorders such as diabetes mellitus. The medication history should be detailed and specifically include the use of thiazides, furosemide, estrogens, corticosteroids, sulfonamides, and opiates. Note a family history of pancreatic disorders. In the review of systems, obtain a complete description of any pain in the upper abdomen or epigastric area. Symptoms that may be important in relation to pancreatic disorders are pruritus, abdominal pain, dyspnea, nausea, and vomiting. The functional assessment includes data about the patient’s dietary habits and use of alcohol.
Note any restlessness, flushing, or diaphoresis during the examination. Vital signs may disclose low-grade fever, tachypnea, tachycardia, and hypotension. Inspect the skin for jaundice. Assess the abdomen for distention, tenderness, discoloration, and diminished bowel sounds.
Tests and procedures used to diagnose pancreatic disorders include laboratory analyses of blood, urine, stool, and pancreatic fluid, and imaging studies. Specific blood studies used to assess pancreatic function include measurements of serum amylase, lipase, glucose, calcium, and triglyceride levels. Urine amylase and renal amylase clearance tests may also be ordered. Stool specimens may be analyzed for fat content. The secretin stimulation test measures the bicarbonate concentration of pancreatic fluid after secretin is given intravenously to stimulate the production of pancreatic fluid.
Accessory pancreas is a rare condition in which small groups of pancreatic cells are separate from the pancreas. They may occur in the mesentery of the small intestine, the wall of the duodenum, the upper part of the jejunum, or more rarely, in the wall of the stomach, ileum, gallbladder or spleen. The condition was first described by Klob in 1859.
Accessory pancreas is a small cluster of pancreas cells detached from the pancreas and sometimes found in the wall of the stomach or intestines.
Individuals are classified as having a functional gallbladder disorder if the above criteria are met, if the gallbladder is present, and if the testing of liver enzymes, conjugated bilirubin, and pancreatic enzymes (amylase and lipase) are normal.
Functional disorders of the gallbladder, bile duct and pancreas have been defined and classified by the Rome criteria for functional gastrointestinal disorders. The criteria outline three variants of functional disorders of the gallbladder, bile duct and pancreas, termed "functional gallbladder disorder", "functional biliary sphincter of Oddi disorder" and "functional pancreatic sphincter of Oddi disorder". All of the following criteria need to be met for as part of the definition of a functional disorder of the gallbladder:
- the pain must be located in the upper part of the abdomen and/or the right upper quadrant of the abdomen
- episodes of pain must last at least 30 minutes
- the symptoms must be recurrent, and occur at differing intervals
- the pain must incrementally increase to a "steady level"
- the pain must be severe enough the patient's daily activities are affected, or that the patient must attend the emergency department
- the pain must not be relieved by any of bowel movements, change in posture, or antacids; and,
- other structural disorders that could explain the symptoms must be excluded.
Gastrinoma causes the following symptoms:
- Hypergastrinemia
- Ulcers of the duodenum, stomach, and small intestine.
- Severe diarrhea.
- Generalized cancer symptoms.
HP is characterised by attacks of epigastric pain, which are often associated with nausea and vomiting. Symptoms may start shortly after birth but onset varies periodically, with some patients not exhibiting symptoms until adulthood. There is usually progression to chronic pancreatitis with endocrine and exocrine failure and a mortally increased risk of pancreatic cancer. Lifetime risk of cancer has been variously calculated as 35–54% to the age of 75 years and screening for early pancreatic cancer is being offered to HP sufferers on a scientific basis. Some patients may choose to have their pancreas surgically removed to prevent pancreatic cancer from developing in the future.
The epidemiology of HP follows a similar pattern to alcohol-associated chronic pancreatitis, but there are important differences. For example, HP typically has an earlier age of pancreatitis onset; although malabsorption and diabetes mellitus occur at a later stage in the disease progression.
The clinical signs can vary from mild gastrointestinal upset to death, with most dogs presenting with common gastrointestinal signs of upset, such as vomiting, anorexia, painful abdomen, hunched posture, diarrhea, fever, dehydration, and lack of energy, with vomiting being the most common symptom. These signs are not specific just for pancreatitis and may be associated with other gastrointestinal diseases and conditions.
Acute pancreatitis can trigger a build-up of fluid, particularly in abdominal and thoracic (chest) areas, acute renal failure, and cause inflammation in arteries and veins. The inflammation triggers the body's clotting factors, possibly depleting them to the point of spontaneous bleeding. It is this form which can be fatal in animals and in humans.
Chronic pancreatitis can be present even though there are no clinical signs of the disease.
Pancreatitis can result in exocrine pancreatic insufficiency, if the organ's acinar cells are permanently damaged; the pancreatic enzymes then need replacement with pancrelipase or similar products. The damage can also extend into the endocrine portion of the pancreas, resulting in diabetes mellitus. Whether the diabetes is transient (temporary) or permanent depends on the severity of the damage to the endocrine pancreas beta cells.
Hereditary pancreatitis (HP) is an inflammation of the pancreas, attributed to genetic causes. It was first described in 1952 by Comfort and Steinberg but it was not until 1996 that Whitcomb "et al" isolated the first responsible mutation in the trypsinogen gene ("PRSS1") on the long arm of chromosome seven ("7q35").
The term "hereditary pancreatitis" is used when a genetic biomarker is identified, and "familial pancreatitis" otherwise.
Hemosuccus pancreaticus is a rare entity, and estimates of its rate are based on small case series. It is the least frequent cause of upper gastrointestinal bleeding (1/1500) and is most often caused by chronic pancreatitis, pancreatic pseudocysts, or pancreatic tumors. As a result, the diagnosis may easily be overlooked. The usual presentation of hemosuccus is the development of symptoms of upper or lower gastrointestinal bleeding, such as melena (or dark, black tarry stools), maroon stools, or hematochezia, which is frank rectal bleeding. The source of hemorrhage is usually not determined by standard endoscopic techniques, and the symptoms of the condition are usually grouped as a cause of obscure overt gastrointestinal hemorrhage. Over one-half of patients with hemosuccus also develop abdominal pain, usually located in the epigastrium, or uppermost part of the abdomen. The pain is described as being "crescendo-decrescendo" in nature, meaning that it increases and decreases in intensity slowly with time. This is thought to be due to transient blockage of the pancreatic duct from the source of bleeding, or from clots. If the source of the bleeding also involves obstruction of the common bile duct (such as with some tumours of the head of the pancreas), the patient may develop jaundice, or "silver stools", an uncommon finding of acholic stools mixed with blood.
Hemosuccus pancreaticus, also known as pseudohematobilia or Wirsungorrhage is a rare cause of hemorrhage in the gastrointestinal tract. It is caused by a bleeding source in the pancreas, pancreatic duct, or structures adjacent to the pancreas, such as the splenic artery, that bleed into the pancreatic duct, which is connected with the bowel at the duodenum, the first part of the small intestine. Patients with hemosuccus may develop symptoms of gastrointestinal hemorrhage, such as blood in the stools, maroon stools, or melena, which is a dark, tarry stool caused by digestion of red blood cells. They may also develop abdominal pain. It is associated with pancreatitis, pancreatic cancer and aneurysms of the splenic artery. Hemosuccus may be identified with endoscopy (esophagogastroduodenoscopy), where fresh blood may be seen from the pancreatic duct. Alternatively, angiography may be used to inject the celiac axis to determine the blood vessel that is bleeding. This may also be used to treat hemosuccus, as embolization of the end vessel may terminate the hemorrhage. However, a distal pancreatectomy—surgery to removal of the tail of the pancreas—may be required to stop the hemorrhage.
Patients with Zollinger–Ellison syndrome may experience abdominal pain and diarrhea. The diagnosis is also suspected in patients who have severe ulceration of the stomach and small bowel, especially if they fail to respond to treatment.
- Chronic diarrhea, including steatorrhea (fatty stools)
- Pain in the esophagus, especially between and after meals at night
- Nausea
- Wheezing
- Vomiting blood
- Malnourishment
- Loss of appetite
Gastrinomas may occur as single tumors or as multiple small tumors. About one-half to two-thirds of single gastrinomas are malignant tumors that most commonly spread to the liver and to lymph nodes near the pancreas and small bowel.
Nearly 25 percent of patients with gastrinomas have multiple tumors as part of a condition called multiple endocrine neoplasia type 1 (MEN 1). MEN I patients have tumors in their pituitary gland and parathyroid glands, in addition to tumors of the pancreas.
AIP is relatively uncommon and is characterized by the following features:
1. Scleral Icterus (yellow eyes), jaundice (yellow skin) which is usually painless, usually without acute attacks of pancreatitis.
2. Relatively mild symptoms, such as minimal weight loss or nausea.
3. Increased serum levels of gamma globulins, immunoglobulin G (IgG) or IgG4.
4. The presence of serum autoantibodies such as anti-nuclear antibody (ANA), anti-lactoferrin antibody, anti-carbonic anhydrase II antibody, and rheumatoid factor (RF).
5. Contrast-enhanced CT demonstrates a diffusely enlarged (sausage-shaped) pancreas.
6. Diffuse irregular narrowing of the main pancreatic duct, and stenosis of the intrapancreatic bile duct on endoscopic retrograde cholangiopancreatography (ERCP).
7. Rare pancreatic calcification or cyst formation.
8. Marked responsiveness to treatment with corticosteroids.
Two-thirds of patients present with either obstructive painless jaundice or a "mass" in the head of the pancreas mimicking carcinoma. It is mandatory to rule out carcinoma prior to making a diagnosis of AIP.
Loss of Pancreatic enzymes leads to maldigestions and malabsorption which may lead to:
- steatorrhea
- weight loss
- fatigue
- flatulence and abdominal distention (bacterial fermentation of unabsorbed food)
- edema (hypoalbuminemia)
- anemia (Vitamin B12, iron, folate deficiency)
- bleeding disorders (Vitamin K malabsorption)
- Metabolic bone disease (Vitamin D deficiency)
- neurologic manifestation
- hypocalcemia
Zollinger–Ellison syndrome (ZES) is a disease in which tumors cause the stomach to produce too much acid, resulting in peptic ulcers. Symptoms include abdominal pain and diarrhea.
The syndrome is caused by a gastrinoma, a neuroendocrine tumor that secretes a hormone called gastrin. The tumor causes excessive production of gastric acid, which leads to the growth of gastric mucosa and proliferation of parietal and ECL cells.
ZES may occur on its own or as part of an autosomal dominant syndrome called multiple endocrine neoplasia type 1 (MEN 1). The primary tumor is usually located in the pancreas, duodenum or abdominal lymph nodes, but ectopic locations (e.g., heart, ovary, gallbladder, liver, and kidney) have also been described.