Cancer of the extrahepatic bile ducts

Case histories of 47 patients with histologically confirmed carcinoma of extrahepatic bile ducts presenting to M. D. Anderson Hospital and Tumor Institute over a 30-year period were studied. The disease was more common in males during the sixth and seventh decades of life. A third of the patients had history of cholelithiasis. Four patients had prior history of chronic ulcerative colitis and one patient had congenital choledochal cyst prior to development of cancer of the bile duct. Jaundice was the earliest and the most common presenting sign. The tumor was located in the common bile duct more often than in the hepatic ducts. The overall median survival was eight months with two-year and five-year survival rates of 15% and 3%, respectively. The respective values for patients who had resectable tumors were 21.7 months, 57% and 20%. Ascending cholangitis was a frequent complication in patients treated with dilation and intubation and, overall, hepatic failure and systemic infection were more frequent causes of death than distant metastases.     

Congenital hepatic fibrosis with saccular dilatation of intrahepatic bile ducts and infantile polycystic kidneys

A case report is presented of a child who died in the neonatal period and who had the sonographic findings of multiple, tubular, fluid-filled spaces in the liver characteristic of Caroli's disease. There was an echodense renal cortex and pyramids. Necropsy findings were consistent with Caroli's disease but the liver also showed evidence of congenital hepatic fibrosis and there was infantile type polycystic disease of the kidneys.     

Inflammatory myofibroblastic tumour of the extrahepatic bile ducts: an unusual cause of obstructive jaundice in children.

Inflammatory myofibroblastic tumour is a rare entity in children with few reported series. The extrahepatic bile duct is an unusual location for this tumour. The authors report here the case of an 8-year-old girl presenting with obstructive jaundice due to inflammatory myofibroblastic tumour of the extrahepatic bile ducts with differential diagnosis of obstructive jaundice in children.     

Congenital dilatations and stenoses of the bile ducts in children (author's transl)

The congenital choledochus cyst is an extremely rare disease pattern. The author presents two own cases of boys aged 4 1/2 years in whom the disease was manifested as a fusiform dilatation of the cholodochus and of the other bile ducts on the one hand, and by a cyst in the second case. Both cases were characterised by an additional annular stenosis of the cholodochus immediately before the junction of the pancreatic duct. The article discusses the most essential criteria in respect of clinic, diagnosis and therapy, as well as the etiological aspects.     

Transient dilatation of intra- and extra-hepatic bile ducts. A case

A case of transient dilatation of intra and extrahepatic bile ducts in the course of an infectious disease is described in a three-year-old girl. The ultrasonic bile duct abnormalities disappeared after three months. The mechanism involved remains uncertain; the hypothesis of a transient inflammatory obstruction is evoked.     

Ultrasonographic detection of spontaneous perforation of the extrahepatic bile ducts in infancy

Diagnosis of spontaneous perforation of the bile ducts in infancy depends on the awareness of this rare condition. The purpose of this article is to report a new case of extra hepatic bile duct perforation, review the literature, and describe in detail the ultrasonographic findings.     

Transient dilatation of the intra- and extra-hepatic bile ducts. A new aspect of bile duct involvement in scarlet fever

An extra- and intra-hepatic bile duct dilatation has been observed in a child in the course of scarlet fever. Manifestations of cholestasis, cytolysis and inflammation were present. Pruritus disappeared within 2 months, biological abnormalities within 3 months and ultrasonic bile duct abnormalities between 3 and 6 months, with a follow-up of 9 months. This case report suggests a relationship between a transitory obstruction, possibly toxic in origin, of the bile ducts and the scarlet fever.     

Congenital bile duct dilatation: review of 35 cases

Thirty-five patients with congenital bile duct dilatation or choledochal cyst were seen in the General Hospital, Kuala Lumpur, during a 10-year period from 1981 to 1990. The highest incidence occured in the group less than 1 year of age. Jaundice was the commonest symptom, occuring in 69% of patients. In most it was mild and intermittent, but a small group (29%) had persistent jaundice and acholic stools. These patients were all less than 1 year of age and represented a poor-prognosis group (30% mortality). Ultrasonography was the most useful diagnostic procedure, having an accuracy of 94%.The treatment of choice was total excision of the cyst and Roux-en-Y hepaticojejunostomy. Internal drainage procedures are not recommended because of the high incidence of cholangitis and the potential for malignancy occuring in the remnant cyst later in life. Operative mortality and morbidity were uncommon, and a good prognosis can be expected on long-term follow-up. Correspondence to: H. K. Goon     

Value of percutaneous blind liver biopsy in preoperative diagnosis of atresia of the extrahepatic bile ducts

During a period of 7 years the case histories of 60 infants with direct hyperbilirubinaemia were prospectively evaluated. At presentation the majority of infants had an age below 1 month. By close clinical follow-up of all infants including the observation of acholic stools (21 infants), quantitative estimations of lipoprotein X during cholestyramine therapy (17 infants), cholescintigraphy (20 infants) and percutaneous liver biopsy (17 infants) the efficiency of the preoperative diagnostic work-up reached 96.6%. Liver biopsies were carried out selectively in only 29.3% of all infants (47.6% in infants with acholic stools and 17.9% in infants with normal stools); their efficiency in the differential diagnosis of extrahepatic biliary atresia was 88.2%.