| Choledochal cysts | |
|---|---|
| Other names | Bile duct cyst |
 | |
| Different types of choledochal cysts | |
| Specialty | Medical genetics  |
Choledochal cysts (a.k.a. bile duct cyst) are congenital conditions involving cystic dilatation of bile ducts. [1] They are uncommon in western countries [2] but not as rare in East Asian nations like Japan and China.
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.[ citation needed ]
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.[ citation needed ]
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). Inflammation of the pancreas 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.[ citation needed ]
They were classified into 5 types by Todani in 1977. [3]
Classification was based on site of the cyst or dilatation. Type I to IV has been subtyped.
Choledochal cysts are treated by surgical excision of the cyst with the formation of a roux-en-Y anastomosis hepaticojejunostomy/ choledochojejunostomy to the biliary duct. Future complications include cholangitis and a 2% risk of malignancy, which may develop in any part of the biliary tree. A recent article published in the Journal of Surgery suggested that choledochal cysts could also be treated with single-incision laparoscopic hepaticojejunostomy with comparable results and less scarring. In cases of saccular type of cyst, excision and placement of T-shaped tube is done.[ citation needed ]
Currently, there is no accepted indication for fetal intervention in the management of prenatally suspected choledochal cysts. [6] [7]
A bile duct is any of a number of long tube-like structures that carry bile, and is present in most vertebrates.
Cholecystitis is inflammation of the gallbladder. Symptoms include right upper abdominal pain, pain in the right shoulder, nausea, vomiting, and occasionally fever. Often gallbladder attacks precede acute cholecystitis. The pain lasts longer in cholecystitis than in a typical gallbladder attack. Without appropriate treatment, recurrent episodes of cholecystitis are common. Complications of acute cholecystitis include gallstone pancreatitis, common bile duct stones, or inflammation of the common bile duct.
Caroli disease is a rare inherited disorder characterized by cystic dilatation of the bile ducts within the liver. There are two patterns of Caroli disease: focal or simple Caroli disease consists of abnormally widened bile ducts affecting an isolated portion of liver. The second form is more diffuse, and when associated with portal hypertension and congenital hepatic fibrosis, is often referred to as "Caroli syndrome". The underlying differences between the two types are not well understood. Caroli disease is also associated with liver failure and polycystic kidney disease. The disease affects about one in 1,000,000 people, with more reported cases of Caroli syndrome than of Caroli disease.
Cholecystectomy is the surgical removal of the gallbladder. Cholecystectomy is a common treatment of symptomatic gallstones and other gallbladder conditions. In 2011, cholecystectomy was the eighth most common operating room procedure performed in hospitals in the United States. Cholecystectomy can be performed either laparoscopically, or via an open surgical technique.
Biliary atresia, also known as extrahepatic ductopenia and progressive obliterative cholangiopathy, is a childhood disease of the liver in which one or more bile ducts are abnormally narrow, blocked, or absent. It can be congenital or acquired. It has an incidence of one in 10,000–15,000 live births in the United States, and a prevalence of one in 16,700 in the British Isles. Biliary atresia is most common in East Asia, with a frequency of one in 5,000.
A Klatskin tumor is a cholangiocarcinoma occurring at the confluence of the right and left hepatic bile ducts. The disease was named after Gerald Klatskin, who in 1965 described 15 cases and found some characteristics for this type of cholangiocarcinoma
The common bile duct, sometimes abbreviated as CBD, is a duct in the gastrointestinal tract of organisms that have a gallbladder. It is formed by the confluence of the common hepatic duct and cystic duct and terminates by uniting with pancreatic duct, forming the ampulla of Vater. The flow of bile from the ampulla of Vater into the duodenum is under the control of the sphincter of Oddi.
Mirizzi's syndrome is a rare complication in which a gallstone becomes impacted in the cystic duct or neck of the gallbladder causing compression of the common hepatic duct, resulting in obstruction and jaundice. The obstructive jaundice can be caused by direct extrinsic compression by the stone or from fibrosis caused by chronic cholecystitis (inflammation). A cholecystocholedochal fistula can occur.
Cholangiocarcinoma, also known as bile duct cancer, is a type of cancer that forms in the bile ducts. Symptoms of cholangiocarcinoma may include abdominal pain, yellowish skin, weight loss, generalized itching, and fever. Light colored stool or dark urine may also occur. Other biliary tract cancers include gallbladder cancer and cancer of the ampulla of Vater.
Courvoisier's law states that a painless palpably enlarged gallbladder accompanied with mild jaundice is unlikely to be caused by gallstones. Usually, the term is used to describe the physical examination finding of the right-upper quadrant of the abdomen. This sign implicates possible malignancy of the gallbladder or pancreas and the swelling is unlikely due to gallstones.
Common bile duct stone, also known as choledocholithiasis, is the presence of gallstones in the common bile duct (CBD). This condition can cause jaundice and liver cell damage. Treatments include choledocholithotomy and endoscopic retrograde cholangiopancreatography (ERCP).
Cholestasis is a condition where bile cannot flow from the liver to the duodenum. The two basic distinctions are an obstructive type of cholestasis where there is a mechanical blockage in the duct system that can occur from a gallstone or malignancy, and metabolic types of cholestasis which are disturbances in bile formation that can occur because of genetic defects or acquired as a side effect of many medications. Classification is further divided into acute or chronic and extrahepatic or intrahepatic.
Ascending cholangitis, also known as acute cholangitis or simply cholangitis, is inflammation of the bile duct, usually caused by bacteria ascending from its junction with the duodenum. It tends to occur if the bile duct is already partially obstructed by gallstones.
Neonatal cholestasis refers to elevated levels of conjugated bilirubin identified in newborn infants within the first few months of life. Conjugated hyperbilirubinemia is clinically defined as >20% of total serum bilirubin or conjugated bilirubin concentration greater than 1.0 mg/dL regardless of total serum bilirubin concentration. The differential diagnosis for neonatal cholestasis can vary extensively. However, the underlying disease pathology is caused by improper transport and/or defects in excretion of bile from hepatocytes leading to an accumulation of conjugated bilirubin in the body. Generally, symptoms associated with neonatal cholestasis can vary based on the underlying cause of the disease. However, most infants affected will present with jaundice, scleral icterus, failure to thrive, acholic or pale stools, and dark urine.
The biliary tract refers to the liver, gallbladder and bile ducts, and how they work together to make, store and secrete bile. Bile consists of water, electrolytes, bile acids, cholesterol, phospholipids and conjugated bilirubin. Some components are synthesized by hepatocytes ; the rest are extracted from the blood by the liver.
Percutaneous transhepatic cholangiography, percutaneous hepatic cholangiogram (PTHC) is a radiological technique used to visualize the anatomy of the biliary tract. A contrast medium is injected into a bile duct in the liver, after which X-rays are taken. It allows access to the biliary tree in cases where endoscopic retrograde cholangiopancreatography has been unsuccessful. Initially reported in 1937, the procedure became popular in 1952.
Biloma occurs when there is excess bile in the abdominal cavity. It happens when there is a bile leak. There is an increased chance of a person developing biloma after having a gallbladder removal surgery, which is known as. Laparoscopic Cholecyestctomy's has an incidence of 0.3–2% of having a bile leak occur. Other causes are liver biopsy, abdominal trauma, and, rarely, spontaneous perforation. The formation of Biloma does not occur frequently. Biliary fistulas are also caused by injury to the bile duct and can result in the formation of bile leaks. Biliary fistulas are abnormal communications between organs and the biliary tract. Once diagnosed, they usually require drainage. The term "biloma" was first coined in 1979 by Gould and Patel. They first discover it after they reported a case with extrahepatic bile leakage. The cause of this is trauma to the upper right quadrant of the abdomen. Originally, Biloma was described as an "encapsulated collection" of extrahepatic bile. Biloma is now described as extrabiliary collections of bile that can be either intrahepatic or extrahepatic. The most common cause to Biloma is trauma to the liver. There are other causes such as abdominal surgery, endoscopic surgery and percutaneous catheter drainage. Injury and abdominal trauma can cause damage to the biliary tree. The biliary tree is a system of vessels that direct secreations from the liver, gallbladder, and pancreas through a series of ducts into the duodenum. This can result in a bile leak which is a common cause of the formation of Biloma. It is possible for Biloma to be associated with mortality, though it is not common. Bile leaks occur in about one percent of causes.
Choledochoduodenostomy (CDD) is a surgical procedure to create an anastomosis, a surgical connection, between the common bile duct (CBD) and an alternative portion of the duodenum. In healthy individuals, the CBD meets the pancreatic duct at the ampulla of Vater, which drains via the major duodenal papilla to the second part of duodenum. In cases of benign conditions such as narrowing of the distal CBD or recurrent CBD stones, performing a CDD provides the diseased patient with CBD drainage and decompression. A side-to-side anastomosis is usually performed.
Biliary endoscopic sphincterotomy is a procedure where the sphincter of Oddi and the segment of the common bile duct where it enters the duodenum are cannulated and then cut with a sphincterotome, a device that includes a wire which cuts with an electric current (electrocautery).