• The Journal of Internal Korean Medicine
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• v.26 no.4
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• pp.912-917
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• 2005

Caroli's disease is a non-obstructive dilatation of the intrahepatic bile ducts. It is a rare congenital disorder that classically causes saccular ductal dilatation, which usually it segmental dilatation. Caroli's disease is associated with recurrent bacterial cholangitis and stone formation. A 57 years-old female inpatient suffering from cholangitis due to Caroli's disease was admitted at Wonkwang University Jeonju Oriental Medicine Hospital. The patient was treated with herbal medicine such as Yongdamsagan-tang and acupuncture. As result, there was improvement of symptoms. The process and content of treatment and the patient's recovery over general symptoms is described.

• Clinical and Experimental Pediatrics
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• v.56 no.11
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• pp.500-504
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• 2013

Here we present the case of an 11-year-old female patient diagnosed with Caroli syndrome, who had refractory esophageal varices. The patient had a history of recurrent bleeding from esophageal varices, which was treated with endoscopic variceal ligation thrice over a period of 2 years. However, the bleeding was not controlled. When the patient finally visited the Emergency Department, the hemoglobin level was 4.4 g/dL. Transhepatic intrajugular portosystemic shunt was unsuccessful. Subsequently, the patient underwent percutaneous transhepatic variceal obliteration. Twenty hours after this procedure, the patient complained of aphasia, dizziness, headache, and general weakness. Six hours later, the patient became drowsy and unresponsive to painful stimuli. Lipiodol particles used to embolize the coronary and posterior gastric veins might have passed into the systemic arterial circulation, and they were found to be lodged in the brain, kidney, lung, and stomach. There was no abnormality of the portal vein on portal venography, and blood flow to the azygos vein through the paravertebral and hemiazygos systems was found to drain to the systemic circulation on coronary venography. Contrast echocardiography showed no pulmonary arteriovenous fistula. Symptoms improved with conservative management, and the esophageal varices were found to have improved on esophagogastroduodenoscopy.

• Journal of the Korean Society of Radiology
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• v.17 no.3
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• pp.385-392
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• 2023

Caroli's disease is a fibrocystic liver disease. Autosomal recessive disorder is characterized by congenital multiple dilatation of the bile duct. Computerized tomography, magnetic resonance imaging, cholangiography and ultrasound are among the methods for diagnosing caroli disease. Computerized tomography is essential for detecting and distinguishing fibroplastic liver disease and is useful for determining intrahepatic bile duct dilatation. However, awareness of the possible side effects of using contrast mediums is necessary. A typical method of magnetic resonance cholangiography is used for magnetic resonance imaging. A non-invasive examination can reduce the pain of the patient, and the anatomical structure of the bile pancreatic duct and the presence or absence of lesions can be easily and quickly observed. Biliary contrast is an effective diagnostic method that can directly visualize various cystic dilatations throughout the enlarged bile duct. However, since this procedure is also an invasive procedure, it is recommended not for diagnosis but for treatment purposes. Ultrasonography can confirm similar findings to computerized tomography. The hepatic artery root is difficult to prove with conventional grayscale ultrasound. However, it is of clinical value in that it can not only describe dilated bile ducts with vascular roots in the tube but also easily identify color Doppler signals in the tube. With the development of video diagnostics, early diagnosis has become possible through computerized tomography, magnetic resonance imaging, cholangiography, and ultrasound. In order to further contribute to the development of video diagnostics so that long-term prognosis can be improved after treatment through early diagnosis, we examined what aspects of each test's caroli disease appear.

• Clinical and Experimental Pediatrics
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• v.45 no.7
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• pp.923-927
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• 2002

Congenital hepatic fibrosis is a relatively rare disease, characterized by bile ductular proliferation and prominent fibrosis in the portal area of liver resulting in portal hypertension. It is frequently associated with other abnormalities such as polycystic kidney, Caroli syndrome, cystic dysplasia of pancreas, intestinal lymphangiectasia, pulmonary emphysema, hemangioma, and cleft palate. We report here a case of congenital hepatic fibrosis associated with renal tubular ectasia in a 3-year-old girl, whose chief complaint was abdominal distension. Her liver function test did not reveal any abnormal findings. Hepatosplenomegaly and multiple dilated bile ducts were seen in the abdominal CT scaning. Esophageal varix was not detected by an endoscopic examination. Microscopically, diffuse portal fibrosis and widening with proliferation of blie ductules in the liver specimen and tubular ectasia in renal cortex were seen.

• Advances in pediatric surgery
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• v.9 no.2
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• pp.81-88
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• 2003

Congenital dilatation of the common bile duct (choledochal cyst) is an uncommon disease. Although the etiology is unknown yet, various theories such as distal obstruction of the common bile duct, congenital weakness of the duct and anomalous union of the pancreaticobiliary duct have been offered to explain the occurrence of choledochal cyst. Thirty - six cases of choledochal cyst over 22 years were analyzed clinically and classified according to Todani's classification and Kimura's anomalous union of pancreaticobiliary duct type. Todani type 1 consisted with 22 cases which were subdivided into 19 cases of type Ia, 1 case of type Ib and 2 of type Ic. Type IVa consisted with 14 cases including one case of Caroli's disease. There were 25 type BP cases and 10 type PB cases and 1 normal pancreatobiliary junction. Serum alkaline phosphatase was increased significantly in almost all cases. Seven patients (19.4%) had associated congenital anomalies such as double gallbladder, left - sided gallbladder, common bile duct web, biliary atresia, accessory hepatic duct, heterotopic pancreas, cleft lip and 2 cases of intestinal malrotation. All patients underwent cyst excision and Roux - en - Y hepaticojejunostomy and cholecystectomy. There was one death due to methicillin resistant Staphylococcus aureus sepsis.