Peribiliary Cysts Presenting as Primary Sclerosing Cholangitis

Tamilarasan, Aravind Gokul; Yang, Jessica; Lee, Alice Unah

doi:10.1016/j.cgh.2018.02.005

Cited by 3 publications

(4 citation statements)

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“…Importantly, this entity may mimic a dilated biliary tree and other clinical entities such as primary sclerosing cholangitis and multi-locular malignancy. Peribiliary cysts could also be mistaken for entities such as biliary intraductal papillary mucinous neoplasm, cystic metastases, Caroli's disease, and importantly cholangiocarcinoma [2,3]. Peribiliary cysts have been noted to occur at a higher frequency in patients with cirrhosis with a reported prevalence of up to 9% based on imaging criteria [4].…”

Section: Discussionmentioning

confidence: 99%

“…Peribiliary cysts should be considered in patients with cirrhosis who present with intrahepatic biliary dilatations, with or without obstructive jaundice. Pre-transplant, high-resolution imaging with magnetic resonance cholangiopancreatography is often useful in enabling accurate diagnosis, based on the demonstration of cystic structures adjacent to the biliary tree on both sides of the right and left portal veins [2]. Explant pathology often confirms this clinical suspicion.…”

Section: Discussionmentioning

confidence: 99%

“…These benign lesions may arise from either intrahepatic or extrahepatic bile ducts and typically have no communication with the biliary tree. However, this entity may be confused with alternative diagnoses, resulting in unnecessary investigations, often with significant clinical implications [2,3]. We present the case of a 42-year-old male misdiagnosed with primary sclerosing cholangitis complicated by cholangiocarcinoma, in whom this diagnosis was definitively confirmed by evaluation of explant liver pathology.…”

Section: Introductionmentioning

confidence: 97%

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Peribiliary Cysts Mimicking Primary Sclerosing Cholangitis and Cholangiocarcinoma

Kumar¹

2022

Cureus

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Peribiliary cysts are cystic dilatations resulting from obstruction of extramural peribiliary glands. They are usually benign, may arise from the intrahepatic and extrahepatic bile ducts, and do not communicate with the biliary tree. They may, however, be confused with alternative diagnoses, resulting in unnecessary investigations with profound clinical implications. We present a case of a 42-year-old male, in whom this entity was misdiagnosed as primary sclerosing cholangitis with cholangiocarcinoma.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 97%

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Peribiliary Cysts Mimicking Primary Sclerosing Cholangitis and Cholangiocarcinoma

Kumar¹

2022

Cureus

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“…The clinical importance of these structures arises from the fact that they may mimic other diseases (eg, cholangiocarcinoma, choledochal cyst), or may represent a harbinger of an important underlying illness (eg, portal hypertension), which may lead the clinician to unneeded investigations. 1 2 Our aim was to systematically review the characteristics of these anatomical variants, and propose a clinical classification based on their location.…”

Section: Introductionmentioning

confidence: 99%

Peribiliary cysts: a systematic review and proposal of a classification framework

Bazerbachi

Haffar²,

Sugihara

et al. 2018

BMJ Open Gastroenterol

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ObjectivePeribiliary cysts are usually benign, although severe complications and mortality may occur, and they may be confounded with other diseases. No classification delineating their different characteristics exists.DesignWe performed a systematic review of the frequency and clinical manifestations of peribiliary cysts. Two reviewers identified studies after searching multiple databases on 2 August 2017.ResultsThese cysts were prevalent in cirrhosis (9%). A total of 135 patients were reported in 72 papers with sufficient clinical data (10 countries, 65% from Japan, median age 63 years (range: 4–88), and 80% were males). Symptoms were present in 70%. Misdiagnosis occurred in 40%, and 33% underwent therapeutic misadventures. Cysts were solitary in 10%. Obstructive features and cholangitis were observed in 36% and 12.5%, respectively. Cysts progression was observed in 37.5% over a median of 18 months. Mortality was 24%, mostly due to cirrhosis. A classification framework is proposed, wherein type I includes hepatic, type II extrahepatic and type III mixed hepatic and extrahepatic cysts, each having distinct features. Specifically, type II was more frequent in females, solitary, without cirrhosis, presenting with obstructive jaundice, more difficult to diagnose and required more surgical interventions (all p<0.05).ConclusionAlthough rarely studied in the literature, peribiliary cysts were frequent in studies designed to evaluate their prevalence, and were mostly asymptomatic. Inaccurate diagnosis, therapeutic misadventures, cysts progression and cysts-related complications are frequent. In a novel classification framework based on location, extrahepatic peribiliary cysts have distinct characteristics and require a special approach for diagnosis and management.

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