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Query: UMLS:C0000737 (abdominal pain)
 31,184 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Caroli's disease is a rare congenital disorder characterized by segmentary saccular dilatation of the intrahepatic bile ducts, which manifests itself clinically by cholangitis and bile stones. We report two cases of Caroli's disease in schoolgirls with clinical signs of recurrent abdominal pain associated with intermittent crises of cholestatic jaundice. Diagnosis was made by retrograde cholangiography in one patient and by intravenous cholangiography in the other.
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PMID:Caroli's disease in childhood: report of two new cases. 664 53

Caroli's disease is characterized by uni- or bilobar cystic enlargement of the intrahepatic biliary tract. Clinical symptoms include recurrent cholangitis, right upper quadrant abdominal pain and jaundice. The diagnosis is usually established preoperatively based on abdominal ultrasound, computertomography and ERCP. Eight patients suffering from unilobar Caroli's disease underwent liver resection during a period of six years. The postoperative course was uneventful, and after a median follow-up of 39.6 months in one case only a temporary jaundice occurred. Liver resection is the method of choice for Caroli's syndrome when feasible. Apart from the curative intention liver resection represents also a prophylactic method since the risk of malignant transformation to cholangiocellular carcinoma in the cystic walls after draining procedures is considerably high. For diffuse spread of Caroli's disease in the liver orthotopic liver transplantation has emerged as therapeutic option.
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PMID:[Therapy of unilobar Caroli syndrome by liver resection]. 782 Oct 45

Forty cases of dilatation of the biliary tract in infants and children were diagnosed and treated at the Veterans General Hospital, Taipei and Taichung between 1980 and 1992. The male to female ratio was 1:2.6 and the age ranged from 1 day to 14 years. Major clinical presentations were abdominal pain (57.5%), jaundice (42.5%), vomiting (37.5%) and abdominal mass (30%); the classical triad of abdominal pain, jaundice and abdominal mass occurred in only three cases (7.5%). Abnormal liver function tests included elevated bilirubin (Bil) (22/37), aminotransferase (30/40), alkaline phosphatase (Alk-P) (34/37), lactic dehydrogenase (LDH) (22/34) and gamma-glutamyl transpeptidase (GGT) (13/15). The amylase in bile was detected in 16 cases with 10 cases higher than 500 Somogyi units/dL. All cases were diagnosed correctly, using real time sonography. Technetium-labeled scintiscan (Tc-99m DISIDA scan) was interpreted correctly in 88% (15/17) and abdominal computerized tomography (CT) in 10 cases (10/10). According to Todani's classification, type I cyst was the most common (35 cases, 87.5%) and the remaining cases were type IV-A (3 cases) and type V (2 cases). Operation was performed in 38 cases except for two who had type V dilatation of biliary tract (Caroli's disease). Complications included rupture of the gall bladder in two patients and common bile duct stone in one; mortality after operation occurred in one case. Surgical specimens of the liver revealed periportal fibrosis in nine instances.
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PMID:Dilatation of the biliary tract in pediatric patients. 836 78

Seventy-four cases of CDC were treated at the Children's Hospital from 1977 to 1995. Female to male ratio was 5:1. Forty per cent of the patients developed symptoms within one year after birth and 75 per cent within 5 years of age. About one third of the cases were treated surgically within one year of age. Only 5 patients or 6.8 per cent had all the clinical triads of jaundice, abdominal pain and mass. Jaundice was the most common symptom in infants below one year of age but abdominal pain was the most common symptom in older children. Three newborn infants had associated biliary atresia. Established liver cirrhosis was noted during surgery in 9 patients who were operated upon within one year of age but none of the older children. All but one had either type I or type IV CDC, while the remaining one had Caroli's disease or type V CDC. Excision was the preferred treatment. Overall operative mortality rate after excision was 6.5 per cent. None of the patients who had internal drainage procedures succumbed. Infants below one year of age appeared to have high morbidity and mortality rates after surgery. Internal drainage procedure may be considered in high risk patients with advanced cirrhosis. Available information suggests that the occurrence of this disease in Thailand is probably more common than in Western countries and etiology of CDC in infants may be different from that in older children or adults.
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PMID:Choledochal cyst: review of 74 pediatric cases. 973 11

Cholangiocarcinoma (CC) is a malignant neoplasm deriving from intra- and extrahepatic bile ducts. It affects both sexes, and is most prevalent at the age 50 to 70. Chronic nonspecific ulcerative colitis, primary sclerosing cholangitis, hepatolithiasis, congenital hepatic fibrosis, and Caroli's disease may lead to the increased incidence of CC. Recently, hepatic cirrhosis in the course of virus-associated chronic hepatitis has been suggested to be involved in the pathogenesis CC. Histologically, 90-95% of CC are well differentiated adenocarcinomas. Usually the tumor grows slowly and metastazes late locally and even less frequently extrahepaticly. CC often causes symptoms by blocking the bile ducts, abdominal pain, weight loss, signs of portal hypertension, rare ascites and thrombophlebitis. Serum chemistry was compatible with obstructive jaundice. The increased expression of CEA, Ca19-9, as well as loss or reduction of sialomucin/sulfomucin concentration in the biliary lining epithelium may be indicative of malignant changes. CC as usually non-vascularized nonencapsulated tumor with a large amount of fibrosis. It is isochogenic in classical USG, CT or MRI. MRCP-magnetic resonance cholangiopancreatography and virtual endoscopy are more helpful methods on the diagnostics of CC. Recently, FDG positron emission tomography has been suggested to be a sensitive technique in identifying small bile duct cancers. Surgical excision of the lesion confirmed localized CC. The adjuvant radio- and chemotherapy and transplantation are not satisfactory. Palliative therapy includes surgical biliary-intestinal bypass procedures as well as operative and nonoperative techniques for biliary intestinal drainage. Recently, the local treatment of CC by photodynamic therapy as a palliative strategy is very promising. Ordinary CC is reported as a neoplasm with a poor prognosis. Post resection 5-year survival is affirmed in about 25% of CC, whereas after palliative treatment only 1 year.
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PMID:[Cholangiocarcinoma--bile ducts cancer]. 1290 Dec 70

In 1958 Caroli was the first to define exactly in literature the congenital dilatations of the intrahepatic bile ducts, concerning a segment, a lobe, or both lobes of the Liver. Later Todani involved it in his classification of the bile duct cysts as type V. Caroli's disease (CD) is quite a rare entity (about 150 cases reported in literature). In CD, stenosis and dilatation cause initially biliary stasis, leading to intrahepatic biliary lithiasis and secondary infection. The spontaneous course of CD is dominated by biliary infection: cholangitis (cholangiohepatitis), septicemia and intrahepatic and subphrenic abscesses. That is why the disease must be recognized before serious complications develop. The authors report about 5 cases of Caroli's disease--2 women and 3 men. Diffuse spread in one patient (25-year-old man) and in four--left lobe involved predominantly. Most common signs and symptoms were: fever, cholangitis, upper quadrant abdominal pain, hepatosplenomegaly, obstructive jaundice, Charcot's triad (in two). Importance for the exact diagnosis involves the use of ultrasonography (US), computer tomography (CT) scan, endoscopic cholangiopancreatography (ERCP), intraoperative cholangiography and cholangioscopy, allowing early diagnosis and therefore a better therapeutic and surgical approach.
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PMID:Caroli's disease. Report of 5 cases and review of literature. 1581 87

Caroli's disease is a rare congenital disorder and occasional cases have been reported from Japan and other parts of Asia. It comprises of congenital dilation of the lower (segmental) intrahepatic bile duct. Cholangitis liver, cirrhosis and cholangiocarcinoma are its potential complication. A case of caroli's disease in an 8-years-old boy with bilobar involvement of liver, (specially affecting right superior lobe) presenting with intermittent abdominal pain, fever and hepatosplenomegaly is reported here.
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PMID:Caroli's disease. 1656 20

Caroli's disease is a rare congenital disease of the liver characterized by cystic dilation of the intrahepatic bile duct. Classic Caroli's disease involves malformations of the biliary tract alone, whereas Caroli's syndrome refers to the presence of associated congenital hepatic fibrosis. Caroli's disease usually presents during childhood and early adulthood. The clinical features of Caroli's disease include jaundice, right upper abdominal pain, and fever due to the associated complications of hepatolithiasis or bacterial cholangitis. Endoscopic or percutaneous cholangiography is the traditional method of diagnosis, but magnetic resonance cholangiopancreatography is emerging as the diagnostic modality of choice. The treatment for Caroli's disease includes supportive care with antibiotics for cholangitis and ursodeoxycholic acid for hepatolithiasis. Surgical resection has been used successfully in patients with monolobar disease. For patients with diffuse involvement, the treatment of choice is orthotopic liver transplantation.
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PMID:Caroli's disease: identification and treatment strategy. 1741 61

In 1958 Caroli described a rare disease with multifocal, segmental and saccular dilation of the large intrahepatic bile ducts which causes stagnation of bile and formation of bile sludge and stones. This results in recurrent abdominal pain, cholangitis and hepatic abscesses. The diagnosis is confirmed with endoscopic retrograde cholangiopancreatography (ERCP) and percutaneous transhepatic cholangiography (PTC) and the purpose of treatment is to restore normal bile flow. Partial resection of the liver has given good results in patient with localized disease. The prognosis is poor despite drainage of bile and 46% of patients die from sepsis, hepatic abscesses, hepatic failure or portal hypertension. There is more than a hundred fold risk of cholangiocarcinoma. We report a case where a male who had a history of recurrent bouts of abdominal pain and pancreatitis was diagnosed with Caroli's disease. He later developed cholangiocarcinoma. Caroli's disease has not, to our knowledge, been reported in Iceland before.
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PMID:[Caroli's disease, case report and review of the literature]. 1782

Caroli's disease is a rare congenital hepatobiliary disease characterized by multifocal segmental dilatation of intrahepatic bile ducts affecting all or parts of the liver. Two forms of Caroli's disease are described, the pure form and that associated with periportal fibrosis. The disease may diffusely affect the liver or be localized to one lobe or segment. Less than 20% of all reported cases of Caroli's disease are monolobar type. We report a case of simple type Caroli's disease confined to right lobe of the liver in a 22 year old man. He was admitted due to right upper abdominal pain and diagnosed by magnetic resonance cholangiopancreatography. He was treated with right hepatic lobectomy and recovered completely.
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PMID:[A case of simple type Caroli's disease confined to right lobe of the liver]. 1815 93


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