Biliary Cysts Research Articles

433 SERUM ADIPOKINES CONCENTRATION IN CHILDREN WITH NON-ALCOHOLIC FATTY LIVER DISEASE

Background and Aims: Hepatic cyst infection is a serious complication in autosomal dominant polycystic kidney disease (ADPKD) patients, with a potentially unfavourable outcome if diagnosis and treatment are delayed. The diagnosis of hepatic cyst infection in ADPKD is difficult because symptoms and imaging techniques can be non-specific. Furthermore, there is no specific blood marker for this infection. We hypothesized that Carbohydrate antigen 19.9 (CA19.9), secreted by the biliary epithelium lining the cysts, is overproduced in case of cyst infection. Methods: CA19.9 was measured in the serum of three kidney transplant ADPKD patients who presented with hepatic cyst infection, and in the cystic fluid of 2 of them. CA19.9 was also measured in the serum of 19 consecutive ADPKD and 22 non-ADPKD renal transplant recipients. CA19.9 immunostaining was done in 5 polycystic livers of ADPKD patients and compared with control normal livers. Results: Three ADPKD patients were diagnosed with hepatic cyst infection based on clinical grounds, biological inflammation, and positron emission tomography scan. All had at that time markedly elevated serum CA19.9 levels (963, 2975, and 601U/ml respectively; N< 35U/ml). Two patients required cystic fluid drainage. CA19.9 level was extremely elevated in the cystic fluid (728,200 and 124,200U/ml respectively). With clinical improvement, a striking decrease in serum CA19.9 level was observed in all patients. Serum CA19.9 from 19 consecutive asymptomatic ADPKD patients with liver cysts was significantly higher than in controls (median: 29.55 vs. 10.3U/ml, p< 0.001). Bilirubin level was normal in all patients of both groups. Immunostaining for CA19.9 revealed strong staining in biliary tree epithelia and cysts of human polycystic livers, significantly more intense than in normal livers. Conclusions: CA19.9 is synthesized by the biliary cystic epithelium and can be elevated in ADPKD patients with liver cysts. A marked increase in serum CA19.9 level is observed with liver cyst infection. Our data strongly suggest that the serum level of CA19.9 can be a diagnostic tool for hepatic cyst infection, contributing to early diagnosis and appropriate management. Measurement of baseline serum CA19.9 level in ADPKD patients could serve as a reference value.

Type VI biliary cyst: Report of a case

An isolated cyst of the cystic duct is an extremely rare lesion. Only single case reports are documented in the literature. The most accepted classification system of biliary cysts, the Todani classification, does not include this lesion. We report a case of isolated cyst of the cystic duct. The initial referral was for evaluation of a gallbladder mass discovered during evaluation of abdominal pain. Preoperative diagnosis was challenging as multiple imaging studies were unable to differentiate this lesion from a choledochal cyst. Surgical planning thus included cyst excision and biliary reconstruction. Operative exploration revealed a type VI biliary cyst and cholecystectomy with cystic duct ligation near the common bile duct was curative.

A Case of Congenital Hepatic Fibrosis Presented with Recurrent Acute Cholangitis

Acute cholangitis usually develops in congenital hepatic fibrosis (CHF), accompanied by cystic dilated bile ducts. However, it can also develop in simple CHF and may lead to critical course. A 30-year old man presented with recurrent acute cholangitis without bile duct dilatation. He visited the hospital for febrile sense and abdominal pain in the right upper quadrant. He had been admitted several times for hepatosplenomegaly and cholangitis since childhood and received a liver biopsy 15 years ago. Abdominal computed tomography (CT) and endoscopic retrograde cholangiopancreatography (ERCP) revealed hepatosplenomegaly and a mildly dilated bile duct without stones or biliary cysts. His condition improved after conservative treatment. However, during a two-month follow up period, the patient experienced three episodes of acute cholangitis. A liver biopsy was performed and showed periportal fibrosis and intrahepatic ductular dysplasia, characteristics of congenital hepatic fibrosis. The periportal fibrosis and the infiltration of inflammatory cells were aggravated compared to 15 years ago. There was no evidence of hepatic cirrhosis. He was diagnosed with congenital hepatic fibrosis with recurrent acute cholangitis without intrahepatic duct dilatation, and conservatively treated with antibiotics.

Biliary cystadenoma

The diagnosis of cystadenoma is rare, even more so when located in the extrahepatic bile duct. Unspecific clinical signs may lead this pathology to be misdiagnosed. The need for pathological anatomy in order to distinguish cystadenomas from simple biliary cysts is crucial. The most usual treatment nowadays is resection of the bile duct, together with cholecystectomy and Roux-en-Y reconstruction.

Histopathological Distinction and Evaluation of Biliary and Peribiliary Cysts in Pig Liver

The majority of hepatic cysts identified in animals are considered to derive from the intrahepatic bile ducts (biliary cysts). An alternative origin is the peribiliary glands located in the hilum of the liver and large portal tracts (peribiliary cysts). The distinction between biliary and peribiliary cysts, and whether these have different clinical significance, has rarely been considered previously. This study reports the pathological features of five cystic porcine livers. Four of these five livers had both biliary and peribiliary cysts and the fifth had only biliary cysts. Biliary cysts were not associated with distortion of adjacent hepatic parenchyma, whereas peribiliary cysts appeared to cause local compression and circulatory disturbance. It would therefore appear that peribiliary cysts have greater potential clinical significance than those of biliary origin.

Pharmacological effects are model specific in animal models of portal hypertension

Pharmacological effects are model specific in animal models of portal hypertension

Type I biliary atresia without extrahepatic biliary cyst

Currently, magnetic resonance cholangiography (MRC) is used for the differentiation of biliary atresia (BA) from other causes of infantile cholestasis. The authors present a case of type I BA without an extrahepatic biliary cyst in a 2-month-old girl. MRC clearly visualized the patency of the gallbladder, cystic duct, and hepatic ducts with disappearance of the common bile duct. Intraoperative cholangiography demonstrated a cloudy appearance of the intrahepatic bile ducts, confirming the diagnosis of type I BA. We believe that this is the first reported case of type I BA without an extrahepatic biliary cyst diagnosed by MRC.

A Patient with Choledochal Cyst

Purpose: A 29 year old Hispanic woman presented to the ER with complaint of intermittent right upper quadrant pain of 8 month duration. The pain started when she was 1 month pregnant, was achy in character and associated with nausea and vomiting. After she delivered her baby, she noticed yellowish discoloration of her eyes. She complained of pruritus and anorexia but denied any fever, chills, diarrhea, or loss of weight. She did not have any significant medical illnesses and was not taking any chronic medications at home. She denied smoking, alcohol or drug abuse. Family history was noncontributory. On physical examination, eyes were icteric and right upper quadrant was tender. Laboratory evaluation showed total bilirubin of 6 mg/dL, direct bilirubin of 5.2 mg/dL, elevated transaminases (AST-140 IU/L, ALT-126 IU/L) and elevated alkaline phosphatase (459 IU/L). CT scan of the abdomen showed severe intra and extrahepatic biliary ductal dilatation and a 10.2×11.7 cm cyst that was compressing and displacing the pancreatic head, duodenum and inferior vena cava. MRCP showed significant intra and extrahepatic biliary ductal dilatation with common bile duct massively distended to 10 cm size. A diagnosis of choledochal cyst was made. Choledochal or biliary cysts are cystic dilatations, which may occur singly or in multiples throughout the bile ducts. They are presumed to be multifactorial in etiology. An abnormal pancreaticobiliary junction is present in about 70 percent of patients. Choledochal cysts may be complicated by cholelithiasis, choledocholithiasis, cystolithiasis, pancreatitis, intrahepatic abscess, biliary cirrhosis and malignancy particularly cholangiocarcinoma. The treatment of choice is complete excision of the cyst with construction of a biliary-enteric anastomosis.[figure1][figure2]FigureFigure

Non-Correctable Biliary Atresia with Large Extrahepatic Cyst: A Report of Two Cases

Biliary atresia constitutes a serious problem in early infancy, due to the concomitant cholestasis. The conventional classification falls into 3 main types: type I, atresia of the common bile duct; type II, atresia of the hepatic duct; and type III, atresia of the porta hepatis. We report 2 unusual cases of biliary atresia type III with a cystic structure that could be mistaken for correctable biliary atresia or choledochal cyst.

Intrahepatic biliary cysts in children with biliary atresia who have had a Kasai operation

Purpose In patients with biliary atresia who had undergone a Kasai operation, treatment of intrahepatic biliary cysts (IBCs), particularly when complicated by cholangitis, is often difficult because the clinical implications and the course of IBCs are unclear. Thus, to determine the best treatment guideline, the morphology of IBCs, the clinical course, and the outcomes of such patients were evaluated. Patients and Methods A total of 44 patients with type III biliary atresia who underwent a Kasai operation from 1977 to 2005 were postoperatively examined for IBC by using ultrasonography and computed tomography. We classified the IBCs based on their number and shape. Results Intrahepatic biliary cysts developed in 12 of 54 patients. Three patients with solitary simple cysts and 1 patient with multiple simple cysts had no history of cholangitis. Two patients with multiple simple cysts had cholangitis at the time of IBC diagnosis and were treated with percutaneous transhepatic cholangiodrainage (PTCD). Patients with simple IBCs did not develop persistent cholangitis and their prognosis depended largely on their liver function; 3 of 6 patients remained healthy without cholangitis, whereas 3 patients required liver transplantation (LT) because of progressive liver failure or worsening hepatopulmonary syndrome, and not severe cholangitis. On the other hand, all 6 patients with multiple complicated IBCs had persistent cholangitis, eventually requiring LT. Even after bile flow to the intestine was reestablished after PTCD, both IBCs and cholangitis recurred. These patients required LT because of severe cholangitis. Conclusions Intrahepatic biliary cysts without cholangitis are not a source of infection and require no treatment. Simple IBCs with cholangitis can be controlled by antibiotics and/or PTCD. Patients with multiple complicated IBCs have a poor prognosis, requiring LT to control cholangitis. Although PTCD can control cholangitis in these patients as they wait for LT, PTCD does not alleviate it—LT is the final solution.

Biliary atresia with a “cyst at porta”: management and outcome as per the cholangiographic anatomy

The purpose of this study is to classify biliary atresia (BA) with a "cyst at porta" according to the cholangiographic anatomy and to define management strategy and outcome in each group. A cyst at porta was identified in 13 of 58 babies (22.4%) with BA at first presentation. The cholangiographic anatomy was classified as; Group A (n = 7), type III BA with extrahepatic cyst; Group B (n = 2), type I or II BA with extrahepatic biliary cyst; and Group C (n = 4), type I or II BA with both extrahepatic and intrahepatic biliary cysts. The remaining 45 patients were comprised of type III BA without a cyst. A Kasai's portoenterostomy (PE) was performed for all Group A patients. Groups B and C were treated by hepaticojejunostomy (n = 5) or portoenterostomy (n = 1). All 45 patients with type III BA without a cyst were treated by a Kasai's PE. The median age at surgery was 92 days (ranges 28-342 days). There were three early post-operative deaths, all in patients with type III BA without cyst. Overall 18/55 (32.7%) patients achieved a jaundice free state. In Group A, 5/7 (71.4%) patients had bile flow, 2/7 (28.6%) are anicteric and 2/7(28.6%) had 1-2 episodes of post-operative cholangitis. In Group B, both patients are anicteric and none had post-operative cholangitis. In Group C, all four babies had bile flow but, significant morbidity because of recurrent severe cholangitis. Only one patient reached a jaundice free state. Of the remaining 42 patients with type III BA without a cyst, 27 (64.3%) had bile flow, 13 (31%) became jaundice free and 14 (33.3%) have had 1-2 episodes of post-operative cholangitis. In conclusion, thirteen of 58 (22.4%) babies with BA had a "cyst at porta" at first presentation in this series. The outcome was most satisfactory in type I BA without intrahepatic cystic dilatation (Group B) in terms of achieving a jaundice free state and freedom from recurrent cholangitis. However, intrahepatic biliary cysts (Group C) were associated with recurrent severe cholangitis and a poor eventual outcome despite a good initial bile flow. The outcome in type III BA with extrahepatic cyst was comparable to type III BA without cyst.

Polycystic disease caused by deficiency in xylosyltransferase 2, an initiating enzyme of glycosaminoglycan biosynthesis

The basic biochemical mechanisms underlying many heritable human polycystic diseases are unknown despite evidence that most cases are caused by mutations in members of several protein families, the most prominent being the polycystin gene family, whose products are found on the primary cilia, or due to mutations in posttranslational processing and transport. Inherited polycystic kidney disease, the most prevalent polycystic disease, currently affects approximately 500,000 people in the United States. Decreases in proteoglycans (PGs) have been found in tissues and cultured cells from patients who suffer from autosomal dominant polycystic kidney disease, and this PG decrease has been hypothesized to be responsible for cystogenesis. This is possible because alterations in PG concentrations would be predicted to disrupt many homeostatic mechanisms of growth, development, and metabolism. To test this hypothesis, we have generated mice lacking xylosyltransferase 2 (XylT2), an enzyme involved in PG biosynthesis. Here we show that inactivation of XylT2 results in a substantial reduction in PGs and a phenotype characteristic of many aspects of polycystic liver and kidney disease, including biliary epithelial cysts, renal tubule dilation, organ fibrosis, and basement membrane abnormalities. Our findings demonstrate that alterations in PG concentrations can occur due to loss of XylT2, and that reduced PGs can induce cyst development.

Congenital Solitary Intrahepatic Biliary Cyst in a Newborn: Report of a Case

A solitary liver cyst (SLC) is a nonparasitic cyst of congenital origin. Its cause is unknown, but it may develop in the form of a simple cyst or a biliary cyst. Solitary liver cysts are extremely rare and usually symptomatic in infancy whereas they are most often detected incidentally in adulthood. Despite advanced imaging techniques, the differential diagnosis of SLC may still be difficult. Surgical intervention is required for histopathological verification, to relieve symptoms and prevent complications such as infection, cyst rupture, and hemorrhage. Although total excision of SLC is desirable to impede recurrences, partial excision may be adequate when total excision is impossible. We report the case of a newborn with a progressively enlarging solitary intrahepatic biliary cyst. We discuss the etiology, differential diagnosis, and treatment options for this rare entity.

Improved focal liver lesion detection: comparison of single-shot diffusion-weighted echoplanar and single-shotT2weighted turbo spin echo techniques

The purpose of this study was to compare diffusion-weighted respiratory-triggered single-shot spin echo echoplanar imaging (SS SE-EPI) sequence using four b-values (b = 0, b = 20, b = 300, b = 800 s mm(-2)) and single-shot T2 weighted turbo spin echo (T2W SS TSE) in patients with focal liver lesions, with special interest in small (<10 mm) lesions. Twenty-four patients underwent routine MRI. The five sequences were compared qualitatively for image quality, lesion conspicuity and artefacts. Quantitative analysis was performed for lesion identification and lesion-to-liver contrast-to-noise ratio (CNR). Subgroup analyses were performed for different types of lesions with different sizes. Sequences were compared by rank order statistic (RIDIT) and Kruskal-Wallis test. The best image quality (p<0.05) was achieved with T2W TSE and the best lesion conspicuity (p<0.05) with T2W TSE for biliary cysts and SE-EPI diffusion-weighted imaging (DWI) (b = 20 s mm(-2)) for haemangiomas and metastases. Image artefacts were lowest (p<0.05) with T2W TSE. T2W TSE was found to be the best protocol (p<0.05) for the identification of biliary cysts and SE-EPI DWI (b = 20 s mm(-2)) for haemangiomas and metastases. The lesion-to-liver CNRs were highest on T2W TSE for biliary cysts and on SE-EPI diffusion-weighted imaging (DWI) for haemangiomas and metastases (p<0.05). This study shows the potential of SS SE-EPI DWI (especially with a b-value of 20 s mm(-2)) as a promising technique for detecting small (<10 mm) focal liver lesions.

Spectrum of biliary abnormalities in hepatic hereditary hemorrhagic telangiectasia: demonstration by multidetector computed tomography

We present a case in which multidetector computed tomography demonstrated a specific pattern of biliary disease associated with hepatic hereditary hemorrhagic telangiectasia: multiple intrahepatic stenoses, focal cystic dilatations along the course of the intrahepatic ducts, and large biliary cysts in conjunction with a normal-appearing extrahepatic bile duct.

The Usefulness of Endoscopic Retrograde Cholangiopancreatography and 99mTc-Labeled Albumin Macroaggregates in Diagnosing Hydatid Disease Fistulae

Hydatid disease is an important health problem in areas where it is endemic. There are several therapeutic modalities, the most important being surgery, antibiotherapy, and percutaneous treatment. In recent years percutaneous treatment has become popular, and for this method or surgery it is sometimes lifesaving to know the relation between the biliary ducts and the cyst cavity. The aim of this study was to examine the usefulness of endoscopic retrograde cholangiopancreatography and (99m)Tc-labeled albumin macroaggregates in diagnosing hydatid disease fistulae before percutaneous or surgical treatment. A total of 72 patients diagnosed with hepatic hydatid disease via ultrasound and serologic tests were enrolled in the study. Endoscopic retrograde cholangiopancreatography was successfully performed in all patients. (99m)Tc-labeled albumin macroaggregates also were injected into cysts at a dose of 1.5-2 mCi just before the treatment. All but three patients were treated percutaneously. Scintigraphy of abdominal and thoracic areas was performed with a GE Starcam 3200 XC/T gamma camera at 30 and 120 min after Tc-labeled albumin macroaggregate injections. Endoscopic retrograde cholangiopancreatography revealed communications between biliary ducts and cyst cavities in nine patients (12.5%). However, (99m)Tc-labeled albumin macroaggregates showed not only leakage into the systemic circulation in nine patients but also into the biliary ducts in two (15.4%). In one patient, mild acute pancreatitis occurred as a complication of endoscopic retrograde cholangiopancreatography. No complications of (99m)Tc-labeled albumin macroaggregates injection were seen. Three patients were surgically treated because of clinically manifested cystobiliary fistulae. We conclude that endoscopic retrograde cholangiopancreatography is a gold standard technique for the diagnosis of communication between the biliary duct and the cyst cavity, and (99m)Tc-labeled albumin macroaggregate injection is useful for revealing leakage into the systemic circulation. The diagnosis of biliary fistulae before percutaneous treatment of hydatid disease may enable planning of the optimal therapy.

One case of congenital gallbladder absence complicated with common biliary duct cyst

1.资料:病人,男,17岁,因上腹部疼痛伴皮肤巩膜黄染2d入院.入院查体:皮肤巩膜轻度黄染,右上腹部轻压痛,腹部未及肿块.实验室检查:WBC:17.5×109/L,N96%,GPT:202 U/L,GOT:107 U/L,总胆红素78μmol/L,直接胆红素47.3 μmol/L。

Solitary liver cysts in children: not always so simple

Aim Liver cysts in children are uncommon. Many are simple and solitary and do not require intervention. However, this series demonstrates a broad range of potential pathologies, some of which are life-threatening. Methods All children referred to our unit during an 8-year period (1998-2005) and found to have a solitary liver cyst were prospectively recorded. Clinical, radiologic, and pathologic features were analyzed. Children with an isolated extrahepatic choledochal cyst and polycystic disease were excluded. Results Twenty-one children with a liver cyst were identified. Two had undergone unsuccessful surgical intervention before referral. There were 11 prenatally detected cysts. Median gestational age at detection was 22 weeks (19-35 weeks); only 1 was specifically characterized as a liver cyst prenatally. Six of these required surgery: 2 large simple cysts, 2 intrahepatic choledochal cysts, 1 giant complex biliary cyst causing respiratory distress, and 1 ciliated hepatic foregut cyst. Of the 5 cysts remaining under ultrasound surveillance, 4 decreased in size or resolved. In 10 children presenting between birth and 15.8 years, a liver cyst was diagnosed postnatally: 3 huge cystic mesenchymal hamartomas, 1 type V choledochal cyst, 1 hydatid cyst, and 5 simple cysts. Four of these required surgical resection. Simple cysts tended to be small and could be distinguished from other pathologies using a combination of imaging techniques (ultrasound, magnetic resonance imaging/magnetic resonance cholangiopancreatography [MRCP], radionuclide scan). Only 2 of 12 children with “simple” cysts required surgery for symptoms. However, a wide range of other cyst pathologies were found in 9 children, and although none was malignant, some were life-threatening and 7 required resection. Conclusions Simple solitary nonparasitic liver cysts rarely cause symptoms or require surgery, but the pediatric surgeon should be aware of the wide range of other types of liver cyst in children to ensure appropriate treatment.

Ciliated hepatic foregut cyst with extensive squamous metaplasia: report of a case

Ciliated hepatic foregut cysts (CHFC) are rare cystic lesions of the liver composed of a ciliated pseudostratified columnar epithelium with mucous cells, connective tissue, and smooth muscles bundles. We report the first case of CHFC with extensive squamous metaplasia without dysplasia or carcinoma. A unilocular, avascular, hypoechoic 60-mm liver lesion located in segment IV was detected by ultrasonography in a 31-year-old woman. The cyst was surgically removed and was lined mainly by a regular squamous epithelium without keratin formation. After extensive sampling, a ciliated pseudostratified columnar epithelium with some alcian blue-positive goblet cells was identified. The lesion was totally examined and there was no epithelial dysplasia or carcinoma. Squamous epithelium is very rare in hepatic foregut cysts and may degenerate into squamous carcinoma. Squamous epithelium is also described in biliary cysts. When squamous epithelium is identified in a liver cyst, an extensive sampling is recommended to identify possible foci of squamous carcinoma and to classify more precisely the histological type of the lesion. Because some cases of squamous carcinoma have been described in CHFC, surgical removal of the lesion may be more appropriate than close follow-up or sclerosing therapy.

Inhibition of Intrahepatic Bile Duct Dilation of the Polycystic Kidney Rat with a Novel Tyrosine Kinase Inhibitor Gefitinib

The polycystic kidney (PCK) rat represents a liver and kidney cyst pathology corresponding to Caroli's disease with congenital hepatic fibrosis and autosomal recessive polycystic kidney disease. We previously reported that an epidermal growth factor receptor tyrosine kinase inhibitor, gefitinib (Iressa), significantly inhibited the abnormal growth of biliary epithelial cells of PCK rats in vitro. This study investigated the effects of gefitinib on cyst pathogenesis of the PCK rat both in vitro and in vivo. A three-dimensional culture model of biliary epithelial cells in the collagen gel matrix was used for in vitro analysis. For in vivo experiments, PCK and control rats were treated with gefitinib between 3 and 10 weeks of age. In vitro, gefitinib had strong inhibitory effects on biliary cyst formation of PCK rats. In vivo, treatment with gefitinib significantly inhibited the cystic dilatation of the intrahepatic bile ducts of PCK rats, which was accompanied by improvement of liver fibrosis. By contrast, no beneficial effects were observed on renal cyst development because of the treatment. These results suggest that signaling pathways mediated by epidermal growth factor receptor are involved in biliary dysgenesis of the PCK rat, with the mechanisms of cyst progression being different between the liver and kidney.