Hepatobiliary cysts in patients with autosomal dominant polycystic kidney disease: prevalence and CT findings.

Itai, Yuji; Ebihara, R; Eguchi, Nobuko; Saida, Yoshihisa; Kurosaki, Yoshihisa; Minami, Masahito; Araki, Tsuyoshi

doi:10.2214/ajr.164.2.7839965

Cited by 50 publications

(41 citation statements)

References 4 publications

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“…14,15 Although our patient had multiple renal cysts, they did not meet the criteria of an autosomal dominant polycystic kidney disease. He had a daughter who was diagnosed as having IPH.…”

Section: Discussionmentioning

confidence: 74%

Hepatic peribiliary cysts diagnosed by magnetic resonance cholangiography

Motoo

Yamaguchi

Watanabe

et al. 2001

Journal of Gastroenterology

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We report a 76-year-old man with hepatic peribiliary cysts diagnosed by magnetic resonance cholangiopancreatography (MRCP). On his first admission, in 1991, the patient was misidentified as having localized dilatation of the left intrahepatic bile ducts, from the ultrasound (US) and computed tomographic (CT) findings, or primary sclerosing cholangitis from endoscopic retrograde cholangiopancreatography (ERCP). However, US and CT in 1998 suggested worsening of the lesions. MRCP was performed for the first time, revealing hepatic peribiliary cysts in the hepatic hilum and along the left hepatic ducts. Drip infusion cholangiography (DIC)-CT confirmed the extraluminal compression of the bile ducts, caused by the cysts, without an influx of contrast medium into the hepatic peribiliary cysts. Retrospective evaluation showed increases in the size and number of the cysts in 1998 compared with the findings in 1996.

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“…14,15 Although our patient had multiple renal cysts, they did not meet the criteria of an autosomal dominant polycystic kidney disease. He had a daughter who was diagnosed as having IPH.…”

Section: Discussionmentioning

confidence: 74%

Hepatic peribiliary cysts diagnosed by magnetic resonance cholangiography

Motoo

Yamaguchi

Watanabe

et al. 2001

Journal of Gastroenterology

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“…These 2 types of cysts can be identified by computed tomography. 21,22 The intrahepatic cysts are within the liver parenchyma but not in contact with the larger portal triads, whereas the peribiliary cysts are adjacent to the larger portal triads or in the hepatic hilum. In our study, both types of cysts were detected radiologically and histologically in patients with isolated ADPLD, suggesting that isolated ADPLD and polycystic liver disease associated with ADPKD have similar pathogenesis.…”

Section: Discussionmentioning

confidence: 99%

Clinical profile of autosomal dominant polycystic liver disease

2003

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Most reports on the natural history, manifestations, and treatment of polycystic liver disease are based on the disease as it manifests in patients with autosomal dominant polycystic kidney disease (ADPKD). The purpose of this study was to develop a clinical profile of isolated autosomal dominant polycystic liver disease (ADPLD) using nonaffected family members as controls. The study included 146 probands, known affected relatives, and first-degree relatives of affected individuals. Participants underwent a formalized medical history interview and physical examination, ultrasonographic examination of the liver and kidneys, magnetic resonance angiography of the brain, and echocardiography. Thirty-eight of the 49 individuals diagnosed with polycystic liver disease before participation in the study were or had been symptomatic. Of 97 previously undiagnosed at-risk individuals, 23 were affected, 39 were unaffected, and 35 were indeterminate. Compared with patients with a negative or indeterminate diagnosis, those with polycystic liver disease had slightly higher levels of serum alkaline phosphatase and total bilirubin and lower levels of total cholesterol and triglycerides. Female patients had a significantly higher mean cyst score than male patients. The cysts were found to arise from the dilatation of biliary microhamartomas and from peribiliary glands. Structural mitral leaflet abnormalities were detected more frequently in affected than in indeterminate or nonaffected individuals. A vascular phenotype was detected in 5.6% of the patients with isolated ADPLD diagnosed clinically and/or by linkage analysis but in none of the unaffected patients. In conclusion, isolated ADPLD is underdiagnosed and genetically distinct from polycystic liver disease associated with ADPKD but with similar pathogenesis, manifestations, and management. (HEPATOLOGY 2003;37:164-171.)

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“…In previous studies, multiple hepatic peribiliary cysts have been found in the patients with a variety of liver diseases, such as cirrhosis, hepatocellular carcinoma, portal hypertension, extrahepatic portal venous obstruction, and systemic infection. Moreover, an association with adult-type polycystic disease of liver and kidneys has been reported [7,8], suggesting that some congenital factors might also be associated with the occurrence of peribiliary cysts. Generally, multiple hepatic peribiliary cysts have been considered to be asymptomatic, but some previous reports have indicated that these cysts are not entirely harmless and can even cause obstructive jaundice [3,4,9,10].…”

Section: Discussionmentioning

confidence: 99%