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Biliary injury (bile duct injury) is the traumatic damage of the bile ducts. It is most commonly an iatrogenic complication of cholecystectomy — surgical removal of gall bladder, but can also be caused by other operations or by major trauma. The risk of biliary injury is more during laparoscopic cholecystectomy than during open cholecystectomy. Biliary injury may lead to several complications and may even cause death if not diagnosed in time and managed properly. Ideally biliary injury should be managed at a center with facilities and expertise in endoscopy, radiology and surgery.
A person with cholangitis may complain of abdominal pain (particularly in the right upper quadrant of the abdomen), fever, rigors (uncontrollable shaking) and a feeling of uneasiness (malaise). Some may report jaundice (yellow discoloration of the skin and the whites of the eyes).
Physical examination findings typically include jaundice and right upper quadrant tenderness. Charcot's triad is a set of three common findings in cholangitis: abdominal pain, jaundice, and fever. This was assumed in the past to be present in 50–70% of cases, although more recently the frequency has been reported as 15–20%. Reynolds' pentad includes the findings of Charcot's triad with the presence of septic shock and mental confusion. This combination of symptoms indicates worsening of the condition and the development of sepsis, and is seen less commonly still.
In the elderly, the presentation may be atypical; they may directly collapse due to sepsis without first showing typical features. Those with an indwelling stent in the bile duct (see below) may not develop jaundice.
Gallstones may be asymptomatic, even for years. These gallstones are called "silent stones" and do not require treatment. The size and number of gallstones present does not appear to influence whether people are symptomatic or asymptomatic. A characteristic symptom of gallstones is a gallstone attack, in which a person may experience colicky pain in the upper-right side of the abdomen, often accompanied by nausea and vomiting, that steadily increases for approximately 30 minutes to several hours. A person may also experience referred pain between the shoulder blades or below the right shoulder. These symptoms may resemble those of a "kidney stone attack". Often, attacks occur after a particularly fatty meal and almost always happen at night, and after drinking.
In addition to pain, nausea, and vomiting, a person may experience a fever. If the stones block the duct and cause bilirubin to leak into the bloodstream and surrounding tissue, there may also be jaundice and itching. This can also lead to confusion. If this is the case, the liver enzymes are likely to be raised.
Pain is the most common presenting symptom. It is usually described as sharp right upper quadrant pain that radiates to the right shoulder, or less commonly, behind the breastbone. Nausea and vomiting can be associated with biliary colic. Individuals may also present with pain that is induced following a fatty meal and the symptom of indigestion. The pain often lasts longer than 30 minutes, up to a few hours.
Patients usually have normal vital signs with biliary colic, whereas patients with cholecystitis are usually febrile and more ill appearing. Lab studies that should be ordered include a complete blood count, liver function tests and lipase. In biliary colic, lab findings are usually within normal limits. Alanine aminotransferase and aspartate transaminase are usually suggestive of liver disease whereas elevation of bilirubin and alkaline phosphatase suggests common bile duct obstruction. Pancreatitis should be considered if the lipase value is elevated; gallstone disease is the major cause of pancreatitis.
Most people with gallstones do not have symptoms. When a gallstone lodges in the cystic duct, they experience biliary colic. Biliary colic is abdominal pain in the right upper quadrant or epigastric region. It is episodic, occurs after eating greasy or fatty foods, and leads to nausea and/or vomiting. People who suffer from cholecystitis most commonly have symptoms of biliary colic before developing cholecystitis. The pain becomes more severe and constant in cholecystitis. Nausea is common and vomiting occurs in 75% of people with cholecystitis. In addition to abdominal pain, right shoulder pain can be present.
On physical examination, fever is common. A gallbladder with cholecystitis is almost always tender to touch. Because of the inflammation, its size can be felt from the outside of the body in 25–50% of people with cholecystitis. Pain with deep inspiration leading to termination of the breath while pressing on the right upper quadrant of the abdomen usually causes pain (Murphy's sign). Murphy's sign is sensitive, but not specific for cholecystitis. Yellowing of the skin (jaundice) may occur but is often mild. Severe jaundice suggests another cause of symptoms such as choledocholithiasis. People who are old, have diabetes, chronic illness, or who are immunocompromised may have vague symptoms that may not include fever or localized tenderness.
Gallstone disease refers to the condition where gallstones are either in the gallbladder or common bile duct. The presence of stones in the gallbladder is referred to as cholelithiasis, from the Greek "chol"- (bile) + "lith"- (stone) + -"iasis" (process). If gallstones migrate into the ducts of the biliary tract, the condition is referred to as choledocholithiasis, from the Greek "chol"- (bile) + "docho"- (duct) + "lith"- (stone) + "iasis"- (process). Choledocholithiasis is frequently associated with obstruction of the biliary tree, which in turn can lead to acute ascending cholangitis, from the Greek: "chol"- (bile) + "ang"- (vessel) + "itis"- (inflammation), a serious infection of the bile ducts. Gallstones within the ampulla of Vater can obstruct the exocrine system of the pancreas, which in turn can result in pancreatitis.
Ascending cholangitis, also known as acute cholangitis or simply cholangitis, is an infection of the bile duct (cholangitis), usually caused by bacteria ascending from its junction with the duodenum (first part of the small intestine). It tends to occur if the bile duct is already partially obstructed by gallstones.
Cholangitis can be life-threatening, and is regarded as a medical emergency. Characteristic symptoms include yellow discoloration of the skin or whites of the eyes, fever, abdominal pain, and in severe cases, low blood pressure and confusion. Initial treatment is with intravenous fluids and antibiotics, but there is often an underlying problem (such as gallstones or narrowing in the bile duct) for which further tests and treatments may be necessary, usually in the form of endoscopy to relieve obstruction of the bile duct. The word is from Greek "chol"-, bile + "ang"-, vessel + -"itis", inflammation.
A number of complications may occur from cholecystitis if not detected early or properly treated. Signs of complications include high fever, shock and jaundice. Complications include the following:
- Gangrene
- Gallbladder rupture
- Empyema
- Fistula formation and gallstone ileus
- Rokitansky-Aschoff sinuses
Biliary dyskinesia is a disorder of some component of biliary part of the digestive system in which bile physically can not move normally in the proper direction through the tubular biliary tract. It most commonly involves abnormal biliary tract peristalsis muscular coordination within the gallbladder in response to dietary stimulation of that organ to squirt the liquid bile through the common bile duct into the duodenum. Ineffective peristaltic contraction of that structure produces postprandial (after meals) right upper abdominal pain (cholecystodynia) and almost no other problem. When the dyskinesia is localized at the biliary outlet into the duodenum just as increased tonus of that outlet sphincter of Oddi, the backed-up bile can cause pancreatic injury with abdominal pain more toward the upper left side. In general, biliary dyskinesia is the disturbance in the coordination of peristaltic contraction of the biliary ducts, and/or reduction in the speed of emptying of the biliary tree into the duodenum.
A biliary fistula is a type of fistula in which bile flows along an abnormal connection from the bile ducts into nearby hollow structure. Types of biliary fistula include:
- bilioenteric fistula: abnormal connection to small bowel, usually duodenum.
- thoracobiliary fistula: abnormal connection to pleural space or bronchus (rare).
These may be contrasted to a bile leak, in which bile escapes the bile ducts through a perforation or faulty surgical anastomosis into the abdominal cavity. Damage to a bile duct may result in a leak, which may eventually become a biliary fistula.
A biliary fistula often occurs in be suspected in a person who has recently undergone a surgical procedure, Pain may occur if the leaked bile is also infected, which can subsequently lead to biliary peritonitis.
Extensive ascites may accumulate, especially in the setting of sterile bile leakage, which is often asymptomatic in nature.
The presence of gallstones can lead to inflammation of the gall bladder (cholecystitis) or the biliary tree (cholangitis) or acute inflammation of the pancreas (pancreatitis). Rarely, a gallstone can become impacted in the ileocecal valve that joins the caecum and the ileum, causing gallstone ileus (mechanical ileus).
Complications from delayed surgery include pancreatitis, empyema, and perforation of the gallbladder, cholecystitis, cholangitis, and obstructive jaundice.
Biliary pain in the absence of gallstones, known as postcholecystectomy syndrome, may severely impact the patient's quality of life, even in the absence of disease progression.
Diagnosis may or may not be determined by an ultrasound, but most likely the disease and other biliary diseases of the liver, gallbladder, and bile duct are found by what is most commonly referred to as a hepatobiliary or HIDA scan. This type of imaging is known as cholescintigraphy.
Cholescintigraphy or hepatobiliary scintigraphy is scintigraphy of the hepatobiliary tract, including the gallbladder and bile ducts. The image produced by this type of medical imaging, called a cholescintigram, is also known by other names depending on which radiotracer is used, such as HIDA scan, PIPIDA scan, DISIDA scan, or BrIDA scan. Cholescintigraphic scanning is a nuclear medicine procedure to evaluate the health and function of the gallbladder and biliary system. A radioactive tracer is injected through any accessible vein and then allowed to circulate to the liver (which takes one hour), after which you are given another tracer which acts as an already digested meal (CCK) to see how fast it takes your gallbladder to fill up (which takes an additional 32 minutes), where it is excreted into the bile ducts and stored by the gallbladder until released into the duodenum.
Most patients have symptoms in the first year of life. It is rare for symptoms to be undetected until adulthood, and usually adults have associated complications. The classic triad of intermittent abdominal pain, jaundice, and a right upper quadrant abdominal mass is found only in minority of patients.
In infants, choledochal cysts usually lead to obstruction of the bile ducts and retention of bile. This leads to jaundice and an enlarged liver. If the obstruction is not relieved, permanent damage may occur to the liver - scarring and cirrhosis - with the signs of portal hypertension (obstruction to the flow of blood through the liver) and ascites (fluid accumulation in the abdomen). There is an increased risk of cancer in the wall of the cyst.
In older individuals, choledochal cysts are more likely to cause abdominal pain and intermittent episodes of jaundice and occasionally cholangitis (inflammation within the bile ducts caused by the spread of bacteria from the intestine into the bile ducts). Pancreatitis also may occur. The cause of these complications may be related to either abnormal flow of bile within the ducts or the presence of gallstones
Biliary sludge may cause complications such as biliary colic, acute cholecystitis, acute cholangitis, and acute pancreatitis.
The first symptoms typically include fever, intermittent abdominal pain, and hepatomegaly. Occasionally, jaundice occurs.
Morbidity is common and is caused by complications of cholangitis, sepsis, choledocholithiasis, and cholangiocarcinoma. These morbid conditions often prompt the diagnosis. Portal hypertension may be present, resulting in other conditions including splenomegaly, hematemesis, and melena. These problems can severely affect the patient's quality of life. In a 10-year period between 1995 and 2005, only 10 patients were surgically treated for Caroli disease, with an average patient age of 45.8 years.
After reviewing 46 cases of Caroli disease before 1990, 21.7% of the cases were the result of an intraheptic cyst or nonobstructive biliary tree dilation, 34.7% were linked with congenital hepatic fibrosis, 13% were isolated choledochal cystic dilation, and the remaining 24.6% had a combination of all three.
Signs and symptoms of pancreatic pseudocyst include abdominal discomfort and indigestion.
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.
Biliary sludge is typically diagnosed by CT scan or transabdominal ultrasonography. Endoscopic ultrasonography is another more sensitive option. However, the gold standard is considered to be direct microscopy of aspirated gallbladder bile. This method is much more sensitive, although it is less practical.
They were classified into 5 types by Todani in 1977.
Classification was based on site of the cyst or dilatation. Type I to IV has been subtyped.
- Type I: Most common variety (80-90%) involving saccular or fusiform dilatation of a portion or entire common bile duct (CBD) with normal intrahepatic duct.
- Type II: Isolated diverticulum protruding from the CBD.
- Type III or Choledochocele: Arise from dilatation of duodenal portion of CBD or where pancreatic duct meets.
- Type IVa: Characterized by multiple dilatations of the intrahepatic and extrahepatic biliary tree.
- Type IVb: Multiple dilatations involving only the extrahepatic bile ducts.
- Type V: Cystic dilatation of intrahepatic biliary ducts without extrahepatic duct disease. The presence of multiple saccular or cystic dilations of the intrahepatic ducts is known as Caroli's disease.
- Type VI: An isolated cyst of the cystic duct is an extremely rare lesion. Only single case reports are documented in the literature. The most accepted classification system of biliary cysts, the Todani classification, does not include this lesion. Cholecystectomy with cystic duct ligation near the common bile duct is curative.
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.
Biliary microlithiasis refers to the creation of small gallstones less than 3mm in diameter in the biliary duct or gallbladder.
It has been suggested as a cause of postcholecystectomy syndrome, or PCS, the symptoms of which include:
- Upset stomach, nausea, and vomiting.
- Gas, bloating, and diarrhea.
- Persistent pain in the upper right abdomen.
Primary sclerosing cholangitis is typically classified into three subgroups based on whether the small and/or large bile ducts are affected. The subgroups of PSC include the following:
- Classic PSC
- Small-duct PSC
- PSC associated with autoimmune hepatitis
Suppurative cholangitis, liver abscess, empyema of the gallbladder, acute pancreatitis, thrombophlebitis of hepatic or portal veins, and septicemia are acute complications of the disease, to which patients may succumb during the acute attacks.
Chronically, complications include cholangiocarcinoma and intraductal papillary neoplasm.
Nearly half of people with PSC do not have symptoms and are often incidentally discovered to have PSC due to abnormal liver function tests, but a substantial proportion will have debilitating signs and symptoms of the disease. Signs and symptoms of PSC may include severe itching and non-specific fatigue. Yellowing of the skin and white portion of the eyes may also be seen. Enlargement of the liver and spleen are seen in approximately 40% of affected individuals. Abdominal pain affects about 20% of people with PSC.
Multiple episodes of life-threatening acute cholangitis (infection within the bile ducts) can be seen due to impaired drainage of the bile ducts, which increases the risk of infection.
- Dark urine due to excess conjugated bilirubin, which is water-soluble and excreted by the kidneys (i.e. choluria)
- Malabsorption, especially of fat, and steatorrhea (fatty stool), due to an inadequate amount of bile reaching the small intestine, leading to decreased levels of the fat-soluble vitamins, A, D, E and K.
- Portal hypertension, a complication of cirrhosis, which can manifest with esophageal and parastomal varices as well as hepatic encephalopathy (mental status alteration/disturbance caused by liver dysfunction and shunting of blood away from the scarred liver; such that ammonia detoxification is reduced with concomitant encephalopathy).