Intrahepatic Biliary Radicals Research Articles

Early onset Caroli’s disease with associated renal cystic disease presented with recurrent fever and epigastric pain: a case report

BackgroundCaroli’s disease is characterized by non-obstructive dilatation of intrahepatic biliary radicals with formation of calculi followed by recurrent episodes of cholangitis. It is a rare congenital malformation and often remains silent, diagnosed accidentally. But if kept unattended, and without any early intervention, it may lead to fatal residual complications. Hence, its early recognition is of utmost importance to prevent recurrent cholangitis, hepatic abscess, liver cirrhosis and cholangiocarcinoma. Hence, we feel that this case must be reported so as to increase awareness among physicians regarding this entity.Case presentationWe hereby report a case of 10-year-old boy who reported with complaints of recurrent episodes of fever, breathing difficulty and multiple episodes of epigastric abdominal pain with apparently healthy siblings. His routine investigation, laboratory parameters were within normal limits. Ultrasonography abdomen showed liver with normal echo-texture with varying sized multiple thin walled cysts involving both the lobes, right more than left with saccular dilatation of 2nd and 3rd order intra-hepatic biliary radicals without calcification without any colour flow on Doppler evaluation. The common hepatic and common bile ducts were normal in calibre and lumen. Magnetic resonance imaging abdomen had similar findings and upper GI endoscopy was also normal. He was finally diagnosed to be a case of Caroli’s disease and was managed with broad spectrum antibiotics, ursodeoxycholic acid, multivitamins, and calcium supplements. He made an uneventful recovery thereafter. Parents were counselled about the risk of cholangitis and cholangiocarcinoma and was referred to tertiary care centre for genetic counselling and for future need of liver transplantation.ConclusionAge presentation of Caroli’s disease varies. Majority present during adolescence and early adulthood. This often poses a diagnostic challenge owing its rare entity and silent presentation. A strong index of suspicion and prompt diagnosis is warranted to prevent its fatal residual complications.

TYPE IV-A CHOLEDOCHAL CYST IN NEWBORN

A congenital cystic dilatation of the bile duct is called a choledochal cyst; it may involve the extrahepatic, intrahepatic, or both biliary radicles. It is an uncommon illness that affects people more frequently in developing nations. We describe a highly uncommon case of a male newborn who has Type IV-A choledochal cyst. The denitive diagnosis was made based on magnetic resonance cholangiopancreatography (MRCP) which showed left sided dilated intrahepatic biliary radicals with fusiform dilatation of common bile duct (CBD) and common hepatic duct (CHD). The decision to follow up with a subsequent surgical plan was taken because there were no complications and the bilirubin level returned to normal after conservative treatment.

Type IVA Choledochal Cyst in Adult: A Case Report

Choledochal cyst is a congenital disease of the biliary tract with extrahepatic and/or intrahepatic biliary tree dilatation. There is a female majority (4:1). Controlling biliary sepsis and curing pancreatitis are prerequisites of surgery. Here, a 35-year-old female patient, came with complaints of epigastric and right upper abdominal pain for three days. On examination per abdomen was soft, non distended, right hypochondriac tenderness was present, no guarding, bowel sounds were audible. Magnetic Resonance Cholangiopancreatography (MRCP) was done which showed fusiform dilatation of Common Bile Duct (CBD), Common Hepatic Duct (CHD), right and left hepatic duct, along with mild dilatation of central intrahepatic biliary radicals (4.8-5 mm)- suggestive of choledochal cyst type IVA. Hepatico-duodenostomy was done. The entire extrahepatic biliary tree should be removed when the choledochal cyst is diagnosed whether or not symptoms are present. Despite being congenital, the condition is typically discovered in adulthood as well. The treatment of choledochal cysts has evolved. The current standard of care is cyst excision and hepaticojejunostomy

Outcome of Digital Cholangioscopy and Laser Lithotripsy for Impacted Biliary Stones

Abstract Background and Aims Impacted stones in the bile duct are difficult to extract, and are predictor of failure of conventional endoscopic retrograde cholangiopancreatography techniques including mechanical lithotripsy and large balloon dilatation. Intracorporeal lithotripsy may be an effective technique for these stones. The aim of this study is to report the efficacy and safety of intracorporeal laser lithotripsy for impacted stones in the bile duct. Method This study is retrospective analysis of prospectively collected data. Patients with impacted stones in the bile duct underwent cholangioscopy with spyglass DS system and laser lithotripsy. Outcome measures were proportion of patients with complete clearance of bile duct after the first session, number of sessions required for complete clearance, and the complications. Results Forty-three patients (27 female) with mean age of 56.12 ± 15.16 years underwent digital cholangioscopy and laser lithotripsy. Mean bilirubin value was 1.8 ± 1.6 mg/dL, 20 (46.51%) patients had single stone, 35(81.39%) patients had only bile duct stones, and 8(18.61%) patients had additional stones in cystic duct or intrahepatic biliary radical. Mean size of largest stone was 16.2 ± 4.4mm. Average duration of the procedure was 69.11 ± 28.12minutes, and complete clearance was achieved in 41/43 (95.34%) patients after the first session. Mean number of sessions required for complete clearance was 1.02 ± .26. Postprocedure cholangitis occurred in one patient. Conclusion Intracorporeal laser lithotripsy is an effective and safe modality for the clearance of impacted bile duct stones.

Incidental Detection of Caroli Syndrome With Unusual Findings

A 30-year-old man, while being evaluated for chronic nonspecific abdominal discomfort, was incidentally found to have dilated intrahepatic biliary radicals with stones on routine ultrasonography. On blood investigations, the only remarkable findings were mild thrombocytopenia and elevated level of serum alkaline phosphatase. An unenhanced computed tomography demonstrated a marked cystic dilatation of intrahepatic biliary radicals with numerus stones, predominantly in the right posterior division (Figures A and B).

An Unusual Case of Haemobilia

Introduction: Haemobilia a rare cause of acute abdomen presents with Quincke’s triad: right upper quadrant pain, jaundice and gastrointestinal bleeding. Hemorrhagic cholecystitis is a rare cause of haemobilia. Case Presentation: A 30 year-old gentleman presented with right upper quadrant pain for 5 days, associated with fever, vomiting and jaundice. Patient was pale and icteric and right hypochondrium was tender with palpable gallbladder. His hemoglobin was 7.7 gm/dl with deranged liver function test (LFT). Abdominal ultrasonography demonstrated edematous and contracted gallbladder wall with echogenic lesion within the lumen. Computed tomography (CT) scan of abdomen revealed a mildly distended gallbladder, bilateral intrahepatic biliary radical dilatation and dilated common bile duct (CBD) with hyperdense sludge in the gallbladder and CBD. Patient developed per rectal bleeding on the fourth day of admission. Upper GI endoscopy showed blood pooling from ampulla of Vater. Abnormal blushing of gallbladder wall with abnormal enhancement at neck of gallbladder arising from cystic artery, was noted on angiography, suggestive of hemorrhagic cholecystitis. During surgery thickened gallbladder wall, engorged cystic artery and blood mixed with clot was seen in the gallbladder lumen. Cholecystectomy with choledochoscopy was performed. Choledochoscopy demonstrated normal extrahepatic biliary channel. Post-operative period was uneventful, blood hemoglobin level was static and LFT improved. Patient was discharged on fourth post-operative day. Conclusions: Haemobilia can be fatal condition hence mandates urgent diagnosis and treatment. Hemorrhagic cholecystitis is not to be forgotten as a cause of haemobilia.

Single-use duodenoscope in the management of an elderly patient with difficult bile duct stones: laser lithotripsy using a disposable cholangioscope.

Video 1Single-use duodenoscope in the management of an elderly patient with difficult bile duct stones using laser lithotripsy and a disposable cholangioscope.

Therapeutic Potential of Human Umbilical Cord Blood Derived Mesenchymal Stem Cells in Murine Model of Hepatic Schistosomiasis

Background and Aim: The pathogenesis of schistosomiasis is mainly due to the host’s immune response against Schistosoma eggs trapped in tissues causing a granulomatous reaction. Recently, stem cell therapy has drawn much attention due to their potential for self-renewal and differentiation. The present study was designed to investigate the regenerative and antifibrotic potential of Human Umbilical Cord Blood derived Mesenchymal Stem Cells (HUCB-MSCs) in hepatic granuloma due to schistosomiasis mansoni in the experimental murine model. Material and Methods: Swiss Albino mice (n=70) were divided into 3 groups; Group A: 30 mice infected with S. mansoni cercariae and subjected to intrahepatic injection of HUCB-MSCs by a single dose of 1 million HUCB-MSCs at 12 weeks post infection (wpi), G (A1): 10 mice sacrificed at 16 (wpi), G (A2):10 mice sacrificed at 18 (wpi), G (A3): 10 mice sacrificed at 20 (wpi). Group B: 30 mice infected with S. mansoni cercariae (positive control), G (B1):10 mice sacrificed at 16 (wpi), G (B2):10 mice sacrificed at 18 (wpi), G (B3):10 mice sacrificed at 20 (wpi). Group C: 10 uninfected mice (negative control). Results: Treatment with HUCB-MSCs, showed an improvement in the histopathological picture of the liver with diminution in the size of granulomas and the overall fibrotic content. Hyperplasic changes in intrahepatic biliary radicals obviously decreased in test groups. Conclusion: The human umbilical cord blood-derived MSCs are promising and effective therapy for the treatment of diseased or damaged liver tissues.

Ultrasound abdomen as a tool to predict difficult cholecystectomy

Background: Laparoscopic cholecystectomy is a commonly performed procedure in general surgical practise, which can sometimes be tedious and can have high morbidity. Ultrasound abdomen is a routinely performed radiological investigation for every case of cholecystectomy. This study intends to find correlation between the ultrasound findings and the perceived difficulty during surgery.Methods: This is a prospective study of 100 patients who underwent cholecystectomy after a preoperative ultrasound. Difficult cholecystectomies were defined and sonographic findings and intraoperative difficulties recorded and tabulated.Results: Fifty one cholecystectomies were classified as difficult cholecystectomy (20 converted to open procedure and 31 took more than 90 min to complete laparoscopically). The commonest ultrasound finding that was encountered was multiple calculi (62%) followed by gallbladder (GB) wall thickness of >4 mm (33%), stone size >1 cm (22%), contracted GB (17%), intrahepatic biliary radicals (IHBR) dilatation (15%) and the least common finding was empyema of the gallbladder seen in 7% of the cases.Conclusions: This study is a reflection of surgeries performed by a single team over 3 years which included 100 cases. It was started with an idea to identify the findings on a preoperative ultrasound that predicted a difficult cholecystectomy (laparoscopic or open). In our experience we found that empyema GB, pericholecystic fluid, IHBR dilatation and wall thickness of GB are excellent predictors of a difficult cholecystectomy.

Cholestasis In Infants With Down Syndrome Is Not Due To Extrahepatic Biliary Atresia: A Ten-Year Single Egyptian Centre Experience.

PurposeWe aimed to define the clinical presentations, course and outcome of cholestasis in infants with Down syndrome (trisomy 21) who presented to the Pediatric Hepatology Clinic, New Children Hospital, Cairo University, Egypt.MethodsRetrospective analysis of data of cohort of infants with Down syndrome and cholestasis who followed up during 2005–2015.ResultsAmong 779 infants with cholestasis who presented during 2005–2015, 61 (7.8%) had Down syndrome. Six dropped out. Among the 55 who followed-up for a mean duration +SD = 12.1 ± 16.7 months, none had extrahepatic biliary atresia (EHBA), 37 (63.3%) had neonatal hepatitis and 18 (32.7%) had non-syndromic paucity of intrahepatic biliary radicals. Fourteen (25.4%) had associated congenital heart disease. Only 35 (63.3%) cleared the jaundice. Twenty-nine (52.7%) received ursodeoxycholic acid (UDCA); of them, 13 cleared the jaundice, one improved, 14 progressed and one died, compared to 22 who cleared the jaundice of the 26 who did not receive UDCA. Only three of those who did not receive UDCA progressed and none died. UDCA carried a 3.4-fold risk of poor prognosis (p= 0.001). UDCA use was associated with more complications (p= 0.016) in those with Down syndrome and cholestasis.ConclusionWe did not come across EHBA among neonates and infants with Down syndrome in 10 years. Non-syndromic paucity is associated with favorable outcome in infants with Down syndrome. UDCA use in cholestasis with Down syndrome is associated with poor outcome.

Cystic biliary atresia or atretic choledochal cyst: A continuum in infantile obstructive cholangiopathy

Introduction: Both cystic biliary atresia and choledochal cyst present as infantile obstructive cholangiopathy. Methods: We detail an infant with congenital biliary dilatation and obstructive cholangiopathy where clinicoradiological features (antenatally imaged subhepatic cyst, early onset jaundice, intrahepatic dilated biliary radicals) suggested a choledochal cyst but operative findings (lack of a distal communication of cyst with duodenum) and histomorphological features (cicatricial collagen and myofibroblastic hyperplasia in the cyst wall; ductal plate malformation, ductular cholestasis and strong expression of CD56 in the liver) were those associated with biliary atresia. Conclusion: The observations support the contention that BA and CC may be interim entities in a continuum of manifestation of the same pathology.

Cysto-biliary communication (CBC) in hepatic hydatidosis: predictors, management and outcome

Background: Liver hydatidosis is a common health problem in endemic areas. Cystobiliary communication is the most common complication of liver hydatid.Methods: Cases of liver hydatid operated during the period June 2012 to July 2018 were retrospectively assessed. Patients diagnosed with cystobiliary communication (preoperatively, intraoperatively or postoperatively) were analysed. Demographics, laboratory tests were noted. Computed tomographic (CT) findings including size, location, Gharbi’s type, presence of intrahepatic biliary radical dilatation, CHD and CBD dilatation were noted. Intraoperative findings were noted. Postoperative outcomes and any intervention if done were noted. Results were analysed.Results: Around 108 patients with liver hydatid underwent surgical intervention. Of which 20 (18.5%) patients were found to have cystobiliary communication. Mean cyst size was 8cms. Location of cyst in segment IV and V commonly. 8 patients were diagnosed preoperatively by elevated bilirubin and alkaline phosphatase and on contrast enhanced computerized tomography. Out of 8, 4 patients underwent preop ERCP and stenting, followed by surgery and the other 4 underwent direct surgery with CBD exploration. 4 were diagnosed intraoperatively and the fistula site sutured. But 2 patients had postop biliary fistula which required postop ERC and stenting. Remaining 8 presented postoperatively with biliary fistula. Of 8 patients, 2 had major and 6 had minor fistulas. Postoperative mortality was 0%.Conclusions: Cystobiliary communication is more common in males with large cyst size, located in the central segments of liver close to biliary confluence, Gharbi type IV cysts. Timely diagnosis and appropriate management decrease the morbidity and mortality.

CT Evaluation of Disease Pattern in Hilar Cholangiocarcinoma

Purpose: The objectives of our study were to map the various imaging patterns of hilar cholangiocarcinoma and identify commonest pattern. Material and methods: In this retrospective study a search of the case records of the hospital using the key word cholangiocarcinoma in hospital information system and morphology code M-8160/3 yielded 288 patients of cholangiocarcinoma in the period April 2001 to June 2016. One hundred and fifty nine patients of intrahepatic and distal cholangiocarcinoma were excluded. Out of the remaining 129 patients with diagnosis of hilar cholangiocarcinoma, nine patients were excluded because available records were not complete. The remaining 120 patients formed the study sample. Results: All the 120 (100%) patients showed dilated intrahepatic biliary radicals. Periductal-infiltrating tumor was present in 117 (97.50%) cases. According to Bismuth-Corlette classification on imaging, type I tumor was present in three (02.50%), type II in 13 (10.83%), type IIIa in 32 (26.66%), type IIIb in 39 (32.50%), and type IV in 33 (27.50%) patients. The involvement of adjacent liver parenchyma was present in 60 (50.00%) patients. Atrophy of a lobe was present in 75 (62.50%) patients. Portal vein was involved in 91 (75.83%) patients. These findings were common in mass-forming tumor. Conclusion: Diagnosis of this rare entity can be done on imaging by identifying its typical pattern. In the majority cases tumor appears to be centered on the right or left hepatic duct with involvement of the ipsilateral portal vein, atrophy of hepatic lobe on that side and invasion of adjacent liver parenchyma. Periductal-infiltrating tumor was commonest morphological form and type IIIb is the commonest pattern.

Von Meyenburg complex (VMC) – A case report

Abstract von Meyenburg complex (VMC) is a ductal plasia of terminal or end branches of intrahepatic biliary radicals. These are multiple biliary hamartomas (MBH) which are of total benign in nature. Very rarely, patient reports with some complaints related to liver or any other alarming complaints. This is found as accidental finding in routine ultrasound and contrast enhanced computed tomography (CECT) studies. We present one such case of a 73-year-old male who came for routine ultrasound examination and was found to be having multiple small cysts in liver and kidneys. He was subjected to triple phase CECT abdomen for further evaluation. VMC was diagnosed as incidental finding and was further confirmed by magnetic resonance imaging (MRI). The entity becomes important as this has to be differentiated from other lesions especially metastasis.

Primary hydatid cyst of head of pancreas communicating with common bile duct containing stones

A 28 year female presented with dull pain in the upper abdomen for 1 year and distension/ fullness in abdomen and itching all over the body for 1 month. Examination showed icterus and a palpable gall bladder. Serum bilirubin was 4 mg/DL, serum alkaline phosphatase was 700 IU/L, Alanine transaminase was 103 IU/L and Aspartate transaminase was 201 IU/L. Magnetic Resonance Cholangio-Pancreaticography (MRCP) showed a thick walled cystic lesion with some solid component, measuring 8 X 7 X 6.1 cm at lower end of Common Bile Duct, with internal membranes and calcifications (Figure 1). Common Bile Duct (23mm), Common Hepatic Duct (13mm) and Intra Hepatic Biliary Radicals were dilated. Gall bladder showed multiple calculi. A provisional diagnosis of Hydatid cyst of head of pancreas was made. Hydatic serology was not done due to its non-availability. Patient was given 400 mg of Albendazole, once a day for 3 weeks and then was taken up for surgery.

Carcinoma of the cystic duct presenting as obstructive jaundice

Cystic duct carcinoma is a rare disease. We present here a case of 33-year-old female patient who presented with obstructive jaundice. An abdominal ultrasound and computed tomography scan showed dilatation of intrahepatic biliary radicals with abrupt narrowing of the common bile duct and markedly distended gall bladder suggestive of obstructive pathology. Following an exploratory laparotomy, a markedly distended gall bladder, cystic duct specimen and infrapyloric lymph nodes were sent for histopathological examination. Histopathological examination revealed a well-differentiated adenocarcinoma of the cystic duct with no lymph node metastasis.

English

The present study was carried out to study the cases of obstructive jaundice caused by gall bladder cancer and cholangiocarcinoma. Twenty cases were included in the study. The major aims of the study were to delineate the clinical features and laboratory tests to know the extent of liver damage in patients with obstructive jaundice caused by gall bladder cancer and cholangiocarinoma and to evaluate further the diagnostic accuracy and safety of procedures like ultrasonography, computerized tomography scan, percutaneous transhepatic cholangiography and diagnostic laparoscopy. An attempt was made to evaluate these techniques for the early detection of metastasis and to know the feasibility of surgery, definitive or palliative to relieve obstructive jaundice and its outcome including morbidity and mortality. The study was carried out in MB Government Hospital associated with R.N.T. Medical College, Udaipur. Material and method used in this study include various laboratory investigations including haemogram, blood sugar, urea, creatinine, liver function test, urine examination etc. Investigations like ultrasonography, CT scan, x- ray abdomen and chest and ECG were carried out in hospital. Various procedure e.g., percutaneous transhepatic biliary drainage, diagnostic laparotomy and surgical procedure were performed in hospital. Statistical analysis of various data done which include spectrum of gall bladder cancer and cholangiocarcinoma, distribution of site of pathology in cholangiocarcinoma, age wise distribution, sex wise distribution, area wise distribution, occupation wise distribution, symptomatology, physical findings, laboratory investigation findings, ultrasonography and CT scan reports and various procedures like percutaneous transhepatic biliary drainage, diagnostic laparoscopy and surgery. Jaundice and lump in abdomen were the commonest presenting symptoms in the presenting series. All the twenty patients with gall bladder cancer and cholangiocarcinoma had extreme hyperbilirubinaemia with significant elevation of direct fraction of serum bilirubin level. On ultrasonography and computerized tomography scan 45 percent patients revealed gall bladder stones associated with gall bladder cancer and cholangiocarcinoma. Both of these investigations detected hepatomegaly, dilated intrahepatic biliary radicals and dilated hepatic ducts in 100 percent patients with obstructive jaundice caused by gall bladder cancer and cholangiocarcinoma. In detection of site of mass, liver infiltration, lymph node involvement, ascites and other organ involvement both ultrasonography and CT scan were almost equally effective. Percutaneous transhepatic cholangiography (PTC) was 100 percent effective in showing dilated biliary tree above the level of obstruction in the patients with obstructive jaundice due to gall bladder cancer and cholangiocarcinoma. Diagnostic laparoscopy yielded additional information of the liver and biliary tract and allowed accurate evaluation of extent and degree of neoplasm and involvement of lymph nodes and other organs. Treatment offered was mainly palliative in nature in majority of cases. Non- operative palliation included Percutaneous Transhepatic Biliary Drainage (PTBD) and operative palliation included cholesystectomy along with Roux-en-Y hepatico or choledocho-jejunostomy.

Analysis of bile in various hepatobiliary disease states: A pilot study.

Aim:Our study aims to find various enzymatic and biochemical components of bile and their clinical or prognostic correlation with regard to progression and severity of hepatobiliary diseases.Materials and Methods:It was a cross-sectional study where all the patients suffering from choledochal cyst (CDC), extrahepatic portal venous obstruction (EHPVO), and infantile obstructive cholangiopathy undergoing diagnostic preoperative cholangiogram; and patients with history of total parenteral nutrition (TPN) undergoing surgery for some other condition were included in the study. Intraoperatively, bile was collected from the gallbladder and sent for estimation of amylase, lipase, sodium, potassium, calcium, chloride, bicarbonate, total bilirubin, pH, cholesterol, triglycerides, and total bile acid.Results:A total of 80 patients were included in the study (20 in each of the four disease-based groups). Amylase, lipase, and pH were significantly different among the patients of CDC when compared with the presence or absence of dilated intrahepatic biliary radicals. Similarly, amylase, lipase, and pH were also significantly different among the patients of EHPVO when compared with presence or absence of biliopathy. Levels of cholesterol and bile acid were significantly higher in patients who were evaluated after 1 year following TPN than those who were evaluated before 1 year. The patients of infantile cholangiopathy, who had history of fever, had significantly higher level of calcium.Conclusion:The components of bile show close correlation with various clinical and prognostic markers, there is a very close correlation between these parameters and the clinical severity, disease progression, and final outcome.

Caroli’s syndrome with ureterovesical calculi in a young child

An 18-month-old boy was admitted with fever, abdominal pain, and hepatomegaly. CT scan revealed dilatation of intrahepatic biliary radicals with central dot sign, bilateral nephrocalcinosis, multiple ureteric and urinary bladder stones and right-sided obstructive hydroureteronephrosis due to a ureterovesical calculus of about 1.5 cm. Liver biopsy showed early hepatic fibrosis.

Endoscopic ultrasound in biliary fasciolosis

A 16-year-old woman presented with recurrent episodes of severe upper abdominal pain and vomiting of 3 years duration. Multiple upper gastrointestinal scopies and ultrasonographies (USG) were normal. CT scan of abdomen had revealed mild intrahepatic biliary radical dilatation up to the lower end of common bile duct (CBD), but no stones or mass lesion. Her amylase and lipase levels during attacks of pain were normal. She was labeled as suffering from depression and was on antidepressants. Her liver function tests were normal except deranged alkaline phosphatase (178 IU/L). Clinical examination was unremarkable. Endoscopic ultrasound (EUS) revealed dilated CBD with a 3–4 cm floating and moving structure in the mid CBD coming down into the lower CBD with appearance like fluke (Fig. 1a). A biliary sphincterotomy was performed and a leaf like live worm was extracted with balloon catheter (Fig. 1b). Microbiological examination revealed diagnosis of Fasciola gigantica. She received a course of antihelminths and is asymptomatic on follow up of 9 months. Conventional USG in biliary fasciolosis usually reveals biliary dilatation, irregular thickened walls and worms are seen as vermiform structures without acoustic shadowing. USG is believed to be helpful in diagnosis of biliary fasciolosis, it may miss distal CBD lesions. EUS is an accurate investigation for imaging the lower biliary tree. Its role in the diagnosis of biliary fasciolosis has been documented earlier [1].