Multiple hepatic cysts along the intrahepatic bile duct--case report

A case of multiple hepatic cysts of the periductal gland located along the left intrahepatic bile duct is described. Ultrasonography and computed tomography disclosed many cystic lesions along the left portal vein in the left lateral segment of the liver. Percutaneous transhepatic cholangiography showed many compressed lesions. The resected specimen revealed multiple cysts of 2-7mm in diameter along the intrahepatic bile duct. Microscopically, cysts within the large Glisson's capsule were intermixed with lobuli of the periductal glands, thus suggesting periductal gland origin. Histopathological features of these cysts were similar to those of "multiple hilar cysts" reported by Nakanuma, but lack of portal hypertension and underlying chronic liver disease is the significant characteristics in this case which is different from "multiple hilar cysts".     

Murine autosomal recessive polycystic kidney disease with multiorgan involvement induced by the cpk gene

BACKGROUND: Autosomal recessive (AR) polycystic kidney disease (PKD) is characterized in humans and mice as a rapidly progressive, collecting duct cystic disease usually leading to uremia in the neonatal or infantile period. In humans, ARPKD renal pathology can be variable in severity and is associated with the development of prominent bile duct and liver pathology. The C57BL/6J-cpk/cpk mouse model of ARPKD is the most extensively studied murine model of inherited infantile PKD; however, these mice lack extrarenal pathology. METHODS: In the present study, the cpk gene was backbreed onto CD1 mice to examine the development of cpk-induced ARPKD in this outbred mouse background. Resulting cystic offspring were examined morphologically and their serum urea nitrogen levels were assessed. RESULTS: The rapid development of PKD in CD1 mice homozygous for the cpk gene appears to be slightly more rapid but otherwise comparable to that seen in inbred C57BL/6J mice. In CD1-cpk/cpk mice, the principal renal pathological finding is collecting duct cysts, which are lined by a relatively uniform epithelium. This epithelium appears to be relatively undifferentiated based on almost total absence of intercalated cells. Proximal tubule cysts are prominent in the first postnatal week while collecting duct cysts predominate in the later stages of the disease. Extrarenal manifestations of the cpk gene are evident in the CD1 strain and include cysts of pancreatic, common bile, and major hepatic ducts. Intrahepatic bile ducts also have focal dilations. Primary (thymus) and secondary (spleen) lymphoid tissues become hypoplastic as azotemia progresses. The strain-related variability in renal and liver changes in cpk-induced ARPKD may reflect the influence of other genes (possibly modifier genes) expressed in this mouse strain. In older CD1-cpk/+ mice, renal (proximal tubular) cysts and prominent liver cysts (lined by a biliary epithelium) develop, indicating that the heterozygous state (cpk/+ genotype) causes renal and hepatic pathology. CONCLUSIONS: The cpk gene, when placed on an appropriate mouse strain background, causes multiorgan disease that more closely mimics human ARPKD than when the cpk gene is expressed on the C57BL/6J strain. A gene dose effect is present as cystic pathology is present in kidney and liver of both suckling homozygous (cpk/cpk) and old heterozygous (cpk/+) mice.     

Serum bile acids and ursodeoxycholic acid treatment in cystic fibrosis-related liver disease

Increased circulating levels of hepatotoxic bile acids may contribute to the cholestasis characteristic of cystic fibrosis-related liver disease. The aims of this study were to compare serum bile acid profiles in patients with cystic fibrosis with and without liver disease, and to evaluate the effect of treatment with ursodeoxycholic acid, a non-hepatotoxic bile acid, on liver biochemistry and serum bile acids in patients with cystic fibrosis-related liver disease. Fasting and postprandial serum bile acid levels were analysed in 15 patients (nine males; median age 18 years) with cystic fibrosis-related liver disease and compared with serum bile acid levels in 18 cystic fibrosis patients (12 males; median age 22 years) without liver disease and 10 control subjects. Fasting and postprandial serum levels of primary and secondary serum bile acids were analysed using high-performance liquid chromatography. Liver biochemistry and serum bile acids were measured in six cystic fibrosis patients with liver disease before and 6 months after treatment with ursodeoxycholic acid 20 mg/kg/day and compared with six control patients with cystic fibrosis-related liver disease. Total fasting and postprandial serum bile acid levels were significantly (P < 0.01) elevated in patients with liver disease compared to those without liver disease and controls. The fasting glycine conjugates of cholic acid, chenodeoxycholic acid and deoxycholic acid, and the fasting and postprandial taurine conjugates of cholic acid and chenodeoxycholic acid were significantly (P < 0.05) elevated in liver disease patients compared to patients without liver disease and controls. After 6 months' treatment with ursodeoxycholic acid, although the serum was significantly saturated with ursodeoxycholic acid and significant improvements in liver biochemistry were observed in the treatment group, there was no significant reduction in the levels of individual serum bile acids. Although circulating levels of potentially hepatotoxic serum bile acids are elevated in patients with cystic fibrosis-related liver disease, improvements in liver biochemistry associated with ursodeoxycholic acid treatment cannot be attributed solely to alterations in levels of endogenous bile acids.     

Asymptomatic intrahepatic choledochal cyst associated with chronic active hepatitis C

A rare case of asymptomatic congenital choledochal cyst (Todani Type V) associated with chronic active hepatitis C is reported. A 45 year-old man was admitted for work-up of localized cystic dilatation of the intrahepatic bile ducts after effective interferon treatment for chronic active hepatitis C. Radiologic examination revealed localized cystic dilatation of the intrahepatic bile ducts in the atrophic left lobe. A left hepatic lobectomy was performed because malignancy had not been excluded. Macroscopic examination of the resected specimen revealed cystic dilatation of the intrahepatic bile ducts. Histologically, there was no evidence of malignancy. Hepatic lobectomy is the treatment of choice for Type V cysts if the disease is confined to a single lobe. Choledochal cysts should be excised to eliminate the potential for cholangitis, lithiasis, and carcinoma.     

Peroxisome proliferator-activated receptor gamma activators inhibit gene expression and migration in human vascular smooth muscle cells

Mesenchymal hamartomas of the liver are the second most common benign liver tumor of childhood. The experience with this tumor at Egleston's Children Hospital at Emory University from 1989 to 1994 is reviewed. Eight patients presented with abdominal distention or an upper abdominal mass. Six patients presented at a mean age of 8 months, and two patients presented at 17 and 23 years of age, respectively. Four patients displayed normal alpha-fetoprotein levels, whereas one patient had an elevated level. Liver function studies were normal in all patients. Abdominal ultrasonography and CT scans revealed a cystic, septated mass within the liver or on a pedicle in all patients. Five patients had simple excision of the tumor, and two had major hepatic resections. The cysts were multiloculated and lined with cuboidal bile duct epithelium surrounded by stroma containing proliferating bile ducts, blood vessels, and compressed liver tissue with no calcifications. In one patient, some pathologists favored the diagnosis of malignant myxoid fibrous histiocytoma because of similar-appearing stroma. Follow-up (mean, 35 months) revealed one symptomatic recurrence after initial resection was incomplete. There were no other recurrences and no malignant transformations. A septated, noncalcified, cystic hepatic mass in an infant with normal liver function studies and characteristic ultrasound or CT is likely a benign mesenchymal hamartoma that can be cured by total local excision.     

Hepatic peribiliary cysts: multiple tiny cysts within the larger portal tract, hepatic hilum, or both

PURPOSE: To describe characteristic imaging features of hepatic peribiliary cysts. MATERIALS AND METHODS: Four patients with hepatic cysts in which the radiologic (n = 3) or histologic (n = 1) findings were consistent with peribiliary cysts of the liver (multiple small cysts seen exclusively in the larger portal tract, hepatic hilum, or both at gross examination and dilatations of extramural peribiliary gland at histologic examination) underwent computed tomography (CT) and ultrasound (US). In three patients, CT was performed after drip infusion of cholangiographic contrast material. RESULTS: Contrast material-enhanced CT clearly depicted many tiny cysts along the larger portal veins up to the third- or fourth-order branch (n = 3). US depicted multiple cysts in the echogenic portal tract definitely (n = 2) or equivocally (n = 2). On cholangiographic contrast-enhanced CT scans, cystic areas were located adjacent to or surrounding the bile ducts, and the possibility of biliary dilatation, communication, or both was disproved. CONCLUSION: Hepatic peribiliary cysts can be diagnosed with US and enhanced CT, especially with CT performed after administration of cholangiographic contrast material.     

Polycystic kidney and liver disease in cats

This paper reviews 27 cases of polycystic disease of the kidneys and/or liver in cats. The multiple cysts in the kidneys were rounded in all but one case, as described in adult polycystic kidney disease in humans. In 68% of the cats presented with polycystic kidneys, there were also cystic changes of the liver (uni- or multilocular cysts and/or congenital hepatic fibrosis (CHF)). In 1 cat polycystic changes of kidneys and liver were accompanied by cysts in the pancreas. In 5 cases there was severe pancreas fibrosis. Twenty-one of the 27 cats were Persian or Persian-crossbred.     

The effect of the extent of A1 pulley release on the force required to flex the digits. A cadaver study on the thumb, middle and ring fingers

BACKGROUND: Laparoscopic management of cystic disease of the liver, including severe polycystic disease, is evolving. METHODS: Wide unroofing, or "fenestration," as is required for a successful result in open cases, leads to complete resolution of the cysts. This can even occur in chronic cysts, with wide-enough unroofing, given time. RESULTS: In polycystic disease, adequate fenestration of superficial, cysts allows deeper cysts to prolapse and be similarly fenestrated, thus reducing pressure effects on the liver and restoring normal function. CONCLUSION: However, because of the distortion of anatomy by this disease, it is important that an experienced liver surgeon perform such a complex procedure, as operative complications could be severe.     

Bile duct cysts in adults

INTRODUCTION: Bile duct cysts are rare, congenital dilations of the intrahepatic and/or extrahepatic biliary tract. Most of them present during childhood. The classical triad right upper quadrant pain, jaundice and abdominal mass is present only in a few instances. We report here the bile duct cysts which were diagnosed at our institution from 1989 to 1996. METHODS: 3245 consecutive endoscopic retrograde cholangiopancreatograms (ERCP) were evaluated retrospectively. Diagnosis was made when localized cystic dilations of the intrahepatic and/or extrahepatic biliary tract were present. Diffuse dilations of the intrahepatic and extrahepatic biliary tract were excluded. RESULTS: Bile duct cysts were found in 20 patients (17 females, 3 males) among 3245 ERCPs. Their mean age was 56 +/- 20 (median 64, range 10 to 83) years. The cyst types (according to the Alonso-Lej classification with the Todani modification) were type I in 11 (55%), type II, III and IV in two instances each (10%), and type V (or Caroli's disease) in 3 patients (15%). Leading symptoms were cholestasis in 14 patients, 10 of whom had abdominal pain, jaundice in 4 patients, and single cases of pancreatitis, cholangitis, and abdominal mass. In 2 patients the diagnosis was made incidentally. 10 patients had bile duct stones. We performed endoscopic sphincterotomy in 15 patients with concretions or persistent symptoms, 3 patients had cyst resection. One of these, with a type I cyst, already had a disseminated cholangiocarcinoma. 10 of 17 patients without cyst resection are currently symptom-free after complete removal of all gallstones. One male patient with cholecystolithiasis, who is not operable due to advanced liver disease, has recurrent cholangitis, 4 patients have died from causes unrelated to the bile duct cysts, and 2 patients are lost to follow up. CONCLUSION: Bile duct cysts in adults are rare. There is a preponderance in the female gender, and the most common type is the extrahepatic (choledochal) cyst. The leading symptoms are cholestasis and right upper quadrant pain. There is an increased risk of cholangiocarcinoma. In young patients the cysts should be entirely removed to prevent malignancy. Older persons are usually symptomless after complete removal of gallstones.     

Association between pancreatic cystadenocarcinoma, malignant liver cysts, and polycystic disease of the kidney

Polycystic kidney disease is an autosomal dominant disease that may be associated with cystic disease of the liver. In women, the cysts may develop early and be more troublesome than in men. Cystadenocarcinoma of the pancreas is uncommon, comprising 1% of primary pancreatic malignancies. This case report is the first to describe a familial association between polycystic kidney disease and cystadenocarcinoma of the pancreas and liver in the English medical literature. A patient with autosomal dominant polycystic kidney disease (ADPKD) and multiple hepatic cysts developed cystadenocarcinoma of the pancreas with multiple malignant liver cysts. The patient's mother, sister, and niece had ADPKD, and the patient's sister also died of pancreatic cystadenocarcinoma. We believe that the development of these two disease entities in which the primary pathology is cyst formation has a genetic association.     

The use of the liver-spleen-scan for the diagnosis of congenital Intrahepatic cysts of the bile duct (M. Caroli) (authors transl)

After the short review of the pathogenesis, diagnosis and therapy for congenital intrahepatic cysts of the bile-duct (morbus Caroli), reference was made to the diagnostic significance of liver scintigraphy on the basis of two casuistic reports. Liver scintigraphy permits an exact localization of intrahepatic cysts as well as information regarding the course of the hepatopathy connected with morbus Caroli. In addition, 131-J-bromsulfon scintigraphy of the liver is a simple method for controlling the patency of the bile ducts. Our observations tend to indicate that preoperative and postoperative scintigraphic controls of the liver are practical for this disease.     

Cancer arising in choledochal cyst and management

Biliary cancer develops in 20-30% of the patients with choledochal cyst and pancreatobiliary malunion. Some bile acid fractions and refluxed pancreatic enxymes into the bile duct is probably responsible for carcinogenesis. Cancer often develops in the extrahepatic bile duct and gallbladder, and rarely in the intrahepatic duct. In cystic dilatation, cancer often occurs in the common bile duct, while in diffuse or non-dilated type it occurs in the gallbladder. Cancer usually occurs in younger patients than does biliary cancer in general population, and the average age is in the 40s. The risk of malignancy in cysts with internal drainage is higher than that in primary cysts, and early removal of the retained cyst should be performed as quickly as possible. Although the prognosis of biliary cancer is usually dismal, aggressive procedures are recently gaining better results than that by conventional methods. The prevention of cancer is the procedure of choice by early excision. Removal of the whole extrahepatic bile duct is necessary, even in case of malunion with no biliary dilatation. Cancer rarely arises in the intrahepatic duct after excisional surgery, due to long standing biliary stricture. Wide anastomosis with ductoplasty should be essential. Cancer also occurs in the remnant duct. Excision of the distal duct in the pancreas is also necessary.     

Sibs diagnosed prenatally with situs inversus totalis, renal and pancreatic dysplasia, and cysts: a new syndrome?

We describe two sib fetuses with situs inversus, cystic dysplastic kidney and pancreas, bowing of the lower limbs and clavicles, severe intrauterine growth retardation (IUGR), and oligohydramnios. Early prenatal diagnosis of pancreatic and dysplastic renal cysts and situs inversus totalis were made in the 18-week-old fetus. This syndrome differs from that of Ivemark and related syndromes because of the presence of situs inversus totalis and absence of hepatic fibrosis and cysts. The parents were first cousins, and did not have any cysts of kidney, liver, or pancreas detected by ultrasonography.     

New Gaba-containing analogues of human growth hormone-releasing hormone (1-30)-amide: I. Synthesis and in vitro biological activity

BACKGROUND AND METHODS: Congenital cystic dilatation of the bile ducts represents an uncommon anomaly of the biliary system. We report on 6 patients suffering from cystic biliary duct dilatations which were treated in our hospital between 1980 and 1992. Clinical signs included upper abdominal pain, white or clay-colored stool, icterus and/or palpable tumor. According to the classification of Todani, 4 children had type Ia cysts, 1 child a type Va cyst and 1 child a type Ia cyst with extrahepatic biliary atresia. RESULTS: Among the diagnostic methods sonography is preeminent and permitted demonstration of intra- and extrahepatic biliary duct dilatations in all of our patients. In 2 patients small cystic dilatations could be distinguished from hepatic vessels by colour-coded Doppler sonography. CONCLUSION: The treatment of choice is the resection of the dilated extrahepatic biliary ducts followed by hepaticojejunostomy using the Roux-en-Y-technique.     

Nonparasitic cysts of the liver: results and options of surgical treatment

Nonparasitic cysts of the liver (NPHC) are highly variable in respect to appearance and therapeutic approach. The treatment of these cysts varies according to the nature and appearance of the disease. Based on the variable nature of disease and the various therapeutic options, all of which were attempted in our patients, the most suitable mode of treatment for different forms of NPHC are discussed. Ninety-one patients with NPHC who had been treated surgically from 1977 through 1995 were examined retrospectively. Asymptomatic peripheral cysts measuring up to 10 cm do not require further treatment. Computed tomography (CT)-guided aspiration (n = 9) should be regarded as a palliative measure. Within a short period, CT-guided aspiration led to recurrence of symptoms in seven of our patients. Standard treatment of NPHC is fenestration with widest possible excision of the cystic wall, which can be performed laparoscopically (n = 10) or by the conventional surgical mode (n = 54). One patient was initially operated by the laparoscopic technique but developed bleeding, which necessitated conversion to the open mode. Three patients underwent synchronous laparoscopic cholecystectomy. Recurrence rates were similar: 11% in the laparoscopically treated group and 13% in the group that underwent conventional open surgery. Conventional surgical treatment was always successful in cases of solitary cysts. However, in cases of multiple cysts measuring more than 5 cm, conventional surgery was followed by recurrence of symptoms in 26% of patients (7/27), who then had to undergo a second operation. Partial resection of the liver (n = 9) was successfully performed in cases of polycystic disease (n = 5) with concomitant enlargement of the organ as well as in cases of large solitary cysts of the left lobe of the liver (n = 4). In patients in whom we found that the cysts communicated with the ductal system (n = 3), we performed a cystojejunostomy to drain the bile. The complication rate was low. In addition to frequent postoperative ascites, which necessitated no further intervention, we observed infectious complications in four patients. Twenty patients (22%) expired during a mean follow-up period of 6.2 years. Interestingly, deaths were frequently associated with malignancy (11/20). After fenestration of multiple cysts measuring > 5 cm, the patients are at high risk for recurrence. Hence partial resection of the liver is an excellent therapeutic alternative in selected patients with polycystic disease and massive enlargement of the organ in whom the disease could not be controlled by simple fenestration. The results of this study show that laparoscopic fenestration should replace the conventional surgical technique as the gold standard in cases of NPHC because the laparoscopic technique is less stressful for the patient and is associated with a rate of success similar to that of the conventional technique.     

Jaundice associated with polycystic liver disease. Relief by surgical decompression of the cysts

Jaundice is an unusual feature of polycystic liver disease. In a 46-year-old woman with polycystic liver disease and jaundice, the bilirubin level was 42.0 mg/100 ml. Because of the rapid rise in bilirubin level, relief of supposed obstruction of intrahepatic bile ducts was attempted by unroofing the hepatic cysts. Following operation the bilirubin level returned to normal, and the patient has remained well since.     

Diagnostic testing for Prader-Willi and Angelman syndromes: response

Between 1992 and 1997, every year, approximately 30,000 examinations of the liver and bile ducts were done. Addition of colour Doppler imaging improves safety of procedures and allowed to perform ultrasound guided biopsy in 73 patients. On the basis of our experience we state that colour Doppler sonography guided percutaneous fine needle biopsy of the liver is useful, sufficient and safety diagnostic method of abscesses, cysts and malignant tumours of the liver.     

Benign schwannoma of the liver with cystic changes misinterpreted as hydatid disease

The rare event of a benign mesenchymal tumor of the liver is described since its cystic transformation resembled hydatid disease through the presence of Echinococcus. Ultrasound and computerized tomography showed a cystic mass within the liver of a 57 year-old woman with upper abdominal pain. This was interpreted as hydatid disease and an evacuation procedure was performed. The histopathology of a minute specimen was interpreted as consistent with chronic inflammation in a cyst wall. Five years later, a recurrence of the parasite was suspected, and complete excision of the mass and resection of a bile fistula was performed. The histopathological examination revealed a large benign schwannoma with regressive cystic changes, proven by positive immunoreaction for the neurogenic marker S-100 protein. Revision of old paraffin blocks of tissue taken during the first operation was able to retrospectively confirm the identical tumor by the same markers. Occurrence of schwannomas in parenchymatous organs or the retroperitoneum is extremely rare and may lead to asymptomatic growth with cystic changes, causing considerable difficulties in imaging procedures. Overall, the primary complete excision of cystic masses within the liver seems to be the best approach in discovering their real nature and to ultimately cure them.     

A case of Caroli's disease: usefulness of color Doppler sonography for evaluating the malformation of the intrahepatic bile ducts

Caroli's disease is a rare congenital disorder with multiple cystic dilatations of the intrahepatic bile ducts. In addition to the nonobstructive localized dilatation of the bile ducts, intraluminar bulbar protrusions of the wall and bridge formation across dilated lumina have been demonstrated on sonography and computed tomography. We present a case in which color Doppler sonography clearly showed a blood flow signal toward the periphery of the liver in the protrusion of the dilated intrahepatic bile ducts. Color Doppler sonography is a useful noninvasive method for evaluating the malformation of Caroli's disease.     

Intrahepatic biliary cystadenoma demonstrated by intraoperative cholangiography

Biliary cystadenomas are rare neoplasms usually found in the liver. These neoplasms have a strong tendency to recur and undergo malignant transformation, and so differentiating between cystadenomas and other cystic lesions of the liver is very important. We describe herein the characteristics of these neoplasms and report the first case of an intrahepatic biliary cystadenoma being demonstrated by intraoperative cholangiography. In our case, intraoperative cholangiography was very useful in differentiating a cystadenoma or cystadeno-carcinoma from other cystic mass lesions of the liver. Not only did it reveal a communication between the intrahepatic bile duct and the cystadenoma, but it also allowed obtain fluid for cytology.