A case of multiple hepatic peribiliary cysts which contributed to the obstructive jaundice and led to liver failure at the young man with von Recklinghausen's disease

Yokomichi, H; Tsuji, Keiji; Hayashi, Yuzo; Kaneko, Mayumi; Nakadoi, Koichi; Ishida, Yoshihisa; Kuwabara, Taku; TSUMUNA, T; Sumida, Tadayoshi; Nagata, Shinji

doi:10.1016/j.hepres.2006.04.002

Cited by 7 publications

(4 citation statements)

References 11 publications

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“…There have been only six reported cases in which obstructive jaundice developed, and in all six, there were multiple lesions on a background of severe liver disease with a poor prognosis. [7][8][9] In our case, the peribiliary cyst was unlike those previously reported because it was macroscopically solitary and the patient's liver was healthy. Moreover, the obstructive jaundice was successfully relieved by cystectomy, and the clinical course after surgery was uneventful.…”

Section: Discussioncontrasting

confidence: 72%

A Case of Peribiliary Cyst Presenting with Obstructive Jaundice

Ikenaga

Chijiiwa

Otani

et al. 2009

Journal of Gastrointestinal Surgery

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A 77-year-old woman with a complaint of itching was shown to have an elevated serum bilirubin level. She had no history of liver disease. Computed tomography and magnetic resonance cholangiopancreatography revealed a 17-mm-diameter cystic lesion obstructing the main hepatic duct at the hepatic hilum. Drip infusion cholangiographic computed tomography and endoscopic retrograde cholangiography showed that the cyst did not communicate with the biliary tree; thus, a peribiliary cyst was diagnosed. Cystectomy was performed, and the jaundice resolved. Peribiliary cysts are generally asymptomatic and rarely cause obstructive jaundice. They are usually multiple and caused by an underlying liver disorder with a poor prognosis. Our case suggests that peribiliary cysts can arise in healthy liver and cause symptoms. Cystectomy is the treatment of choice if the cyst is solitary.

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Section: Discussioncontrasting

confidence: 72%

A Case of Peribiliary Cyst Presenting with Obstructive Jaundice

Ikenaga

Chijiiwa

Otani

et al. 2009

Journal of Gastrointestinal Surgery

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“…Generally, peribiliary cysts are considered to be clinically harmless. However, to date only four cases of obstructive jaundice due to multiple peribiliary cysts excluding post‐liver transplantation cases have been reported in the English literature 9,13–15 . To the best of our knowledge, there has been only one report of a peribiliary cyst accompanying hepatolithiasis, 16 while there has been no report of multiple peribiliary cysts accompanying both hepatolithiasis and obstructive jaundice due to cysts simultaneously.…”

Section: Discussionmentioning

confidence: 99%

An autopsy case of obstructive jaundice due to hepatic multiple peribiliary cysts accompanying hepatolithiasis

Kai

Eguchi

Kumagai

et al. 2007

Hepatology Research

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A 73-year-old Japanese male, who had a history of alcoholic cirrhosis, was admitted to Saga University Hospital to receive treatment for hepatocellular carcinoma. The patient was treated to maintain his liver function, however, the total bilirubin level continued to increase gradually. Endoscopic retrograde cholangiography demonstrated an obstruction of the bilateral intrahepatic ducts. Although endoscopic nasobiliary drainage was performed, the patient died two months after admission. At the autopsy, multiple peribiliary cysts were found to almost completely obstruct the bilateral intrahepatic bile ducts. In addition, hepatolithiasis was found in the right hepatic duct. To date, only a few such cases of multipleperibiliary cysts with obstructive jaundice have been reported, and no such case accompanied by hepatolithiasis. It is also important to note that all the reported cases resulted in a poor prognosis. We herein report a very rare autopsy case with obstructive jaundice due to multiple peribiliary cysts accompanying hepatolithiasis. Generally, multiple peribiliary cysts are considered to be clinically harmless, but once they cause obstructive jaundice, their presence suggests the occurrence of end-stage cirrhosis and a poor prognosis.

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“…However, diffusive hepatic lesions plus splenomegaly can also be found in a variety of other conditions including portal hypertension caused by thrombosis or dysplasia of the portal venous system, congenital disorders such as Niemann-Pick disease, congenital hepatic broses other than CS, and even malignancies like lymphoma [15]. Hepatic cysts with splenomegaly have a narrower differential diagnosis, mainly other hepatic cystic diseases including polycystic kidney disease [16], hepatic peribiliary cysts (which only rarely lead to splenomegaly) [17], and, even more rarely, malignant cystic diseases of the liver and spleen, which are easily differentiated [18]. Thus, hepatic cysts plus splenomegaly on US might provide a useful clue to physicians and thus might shorten the diagnostic delay of CS.…”

Section: Discussionmentioning

confidence: 99%

Factors contributing to diagnostic delay of Caroli syndrome: a single-center, retrospective study

Shi

Huang

Feng

et al. 2020

Preprint

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Background: Caroli syndrome (CS) is a rare congenital disorder without pathognomonic clinical symptoms or laboratory findings; therefore, the diagnosis is often delayed. The objective of this study was to investigate the diagnostic delay and associated risk factors in CS patients. Methods: This was a retrospective analysis of 16 CS patients admitted to a single tertiary medical center on mainland China. The diagnostic timelines of CS patients were reviewed to demonstrate the initial findings of CS at diagnosis, the risk factors associated with diagnostic delay, and potential clues leading to early diagnosis. Results: The median diagnostic delay was 1.75 years (range: 1 month to 29 years, interquartile range: 6.2 years) in 16 enrolled CS patients. Sex, age, and initial symptoms were not associated with diagnostic delay. 87.5% of CS patients were diagnosed by imaging, and the accuracies of ultrasonography, computed tomography (CT), and magnetic resonance cholangiopancreatography were 25%, 69.2%, and 83.3%, respectively. The median diagnostic delays for patients with or without CT performed at the first hospital visited according to physician and radiologist suspicion of the diagnosis were 7.4 months and 6 years, respectively (p=0.021). Hepatic cysts with splenomegaly were detected by ultrasound in over half of CS patients. Conclusions: The majority of CS patients were not diagnosed until complications of portal hypertension had already developed. Recognition and early suspicion of the disease were important factors influencing diagnostic delay of CS. Hepatic cysts plus splenomegaly detected by US might raise the clinical suspicion to include CS in the differential diagnosis.

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A case of multiple hepatic peribiliary cysts which contributed to the obstructive jaundice and led to liver failure at the young man with von Recklinghausen's disease

Cited by 7 publications

References 11 publications

A Case of Peribiliary Cyst Presenting with Obstructive Jaundice

A Case of Peribiliary Cyst Presenting with Obstructive Jaundice

An autopsy case of obstructive jaundice due to hepatic multiple peribiliary cysts accompanying hepatolithiasis

Factors contributing to diagnostic delay of Caroli syndrome: a single-center, retrospective study

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