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Recurrent pyogenic cholangitis
A specific type of cholangitis occurs in patients of Asiatic or Oriental origin, affecting predominantly the intrahepatic bile ducts, which contain soft stones and strictures (see also Sections 41.5 171 and 41.6 172). The left hepatic system is more frequently affected than is the right, and liver abscesses are a common complication. Both sexes are affected equally, and the condition presents at a younger age than Western cholelithiasis. The gallbladder is only inflamed in about one-fifth of patients, and it rarely contains stones.
Pathology Infection of small biliary radicles by bowel organisms, probably from an episode of gastroenteritis, is thought to be the cause. The disease is more common in malnourished people and in some populations there is an association with infection by Clonorchis sinensis and Ascaris lumbricoides. Bacterial enzymes split soluble conjugated bilirubin, forming bilirubinate sludge. Strictures of the ducts are also a constant feature, but it is uncertain whether the stones or the strictures appear first.
The primary pathology is in the bile ducts, and the liver is involved secondarily. In the acute stage the liver is oedematous and there is inflammation around the portal tracts and thrombophlebitis of the portal veins. After recurrent attacks the bile ducts become thickened and stenosed, surrounded by fibrous tissue and a chronic inflammatory infiltrate. Secondary changes develop in the liver.
Diagnosis A clinical diagnosis is easy to make in the right context. There are typical symptoms of recurrent cholangitis in a young patient of Asian or Oriental origin and signs of chronic hepatic infection. Viral hepatitis is the principal differential diagnosis. Ultrasound and ERCP are the main diagnostic investigations required, but blood culture and examination of the stools for parasites are also important.
Treatment Treatment of the acute stage is directed at controlling the infection with intravenous fluids and antibiotics. Surgery is avoided unless the patient's condition deteriorates because of septicaemia from severe obstruction or generalized peritonitis from pancreatitis, rupture, or an empyema of the gallbladder, or rupture of a distended hepatic duct on the surface of the liver. Acute operations are directed at draining the biliary tree with a t-tube through a choledochotomy after clearing the duct of as many stones as possible.
Elective surgery is intended to remove the stones from the biliary tract and to relieve any strictures that are present. This is difficult and tedious surgery, because the stones are very soft and do not wash away easily. On occasion some form of limited hepatic resection may be the best form of treatment; this is particularly useful if only the left hepatic system is diseased. Most surgeons also remove the gallbladder. As the name implies the disease tends to recur with time although the ultimate prognosis is very unpredictable. When complications develop the outlook is poor.
Biliary hydatid disease The liver is the most common site for a hydatid cyst in man (see also Section 41.8 173). Such cysts grow slowly in size and about two-thirds of patients present with simple hepatomegaly. Of the remainder one in eight are found by accident and a similar number present with biliary colic and transient jaundice due to rupture of the cyst into the biliary tree. Attention is mostly directed towards treatment of the primary cyst, which includes treatment with drugs which kill the parasite. Imaging of the biliary system is important. If hydatid elements are present in the ducts they must be removed through a choledochotomy at the same time as removal of the main cyst. Choledochoscopy before closure is useful to ensure that the duct is clear, and the choledochotomy is then closed over a t-tube. Sometimes it is wise to perform a transduodenal sphincteroplasty to ensure free drainage of any residual hydatid material into the bowel. Nowadays it might be easier to do this endoscopically soon after removal of the cyst. Occasionally, a biliary fistula persists after removal of hydatid cyst: ERCP and sphincterotomy with removal of any daughter cysts or hydatid debris from the bile ducts should allow the fistula to close.
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