Bilateral Intrahepatic Bile Ducts Research Articles

A choledochal cyst type IVa in a child treated with Roux-en-Y hepaticojejunostomy.

A choledochal cyst is a bile duct anomaly that disrupts the transportation of bile from the liver to the gallbladder and small intestine. Choledochal cysts are rare, occurring in approximately one out of every 100,000 to 150,000 children in Western countries, with a girls-to-boys ratio of 4:1. Immediate surgery to excise the cyst and construct a biliary-enteric continuity is necessary to treat this condition. This case-report aimed to present a child with choledochal cyst type IVa who underwent a Roux-en-Y hepaticojejunostomy. A 3-year-11-month-old girl with an abdominal mass experienced jaundice, nausea, and vomiting over the past two years, which worsened in the last month. Abdominal ultrasonography indicated intrahepatic biliary dilatation. Abdominal computed tomography scan results confirmed a choledochal cyst type IVa, characterized by fusiform cyst dilatation at the bilateral intrahepatic bile duct, common hepatic duct, cystic duct, and common bile duct. The cyst exerted pressure on the pancreas and small intestine. Before the surgery, the patient was treated with ceftriaxone 100 mg/kg/day and gentamicin 5 mg/kg/day. Roux-en-Y hepaticojejunostomy was performed, involving the complete excision of the extrahepatic bile duct to reconstruct the biliary system. During the surgery, a retroperitoneal cyst measuring 20 cm x 10 cm with a volume of 200 ml was discovered. Following the surgery, the patient showed clinical improvement. Patient follow-ups indicated that no complications such as wound infection, acute pancreatitis, and the formation of pancreatic or biliary fistula occurred. This case highlights that Roux-en-Y hepaticojejunostomy proves to be an effective surgical approach for managing choledochal cyst type IVa in children, helping to prevent further complications.

Retroperitoneal germ cell tumor mimicking neoplasm of ampulla of Vater

A 20-year-old man previously healthy, presented to the emergency department for evaluation of obstructive jaundice, backache and weight loss. Abdominal ultrasonography demonstrated a dilated common bile duct (up to 2 cm), increased gallbladder dimensions (10.3 x 4.9 cm) with biliary sludge. Computed tomography of the abdomen showed a retroperitoneal tumor with 18 x 14 cm dimensions, and numerous large retroperitoneal lymph nodes. The liver demonstrated a bilateral intrahepatic bile duct dilatation. An endoscopic retrograde cholangiography was performed. The study revealed an ulcerative neoplasm of ampulla of Vater, and the cholangiogram obtained images of dilated extra-hepatic and intra-hepatic bile ducts with a large stenosis in the distal third of the common bile duct. After biopsies were analyzed, the definite diagnosis was a retroperitoneal extragonadal germ cell tumor (subtype: embryonal carcinoma) with infiltrative invasion to duodenal wall. A testicular ultrasonography ruled out a testicular tumor. Endoscopic stenting was necessary for decompress the unresectable malignant bile duct obstruction. Patient was referred for cisplatinum based chemotherapy.

A Unique Triad of Ulcerative Colitis With Autoimmune Hemolytic Anemia and Primary Sclerosing Cholangitis

Inflammatory Bowel Diseases (IBD) lead to chronic intestinal inflammation along with extra-intestinal manifestations which could present prior to, subsequent with or after the diagnosis of IBD. We present a unique case of Ulcerative Colitis (UC), Autoimmune Hemolytic Anemia (AIHA) and Primary Sclerosing Cholangitis (PSC) diagnosed simultaneously in a patient. The unique triad of these diseases gives important clues to the immunological factors involved in the pathogenesis of these diseases. An 18-year-old male presented with 1 week of bloody diarrhea, abdominal cramps and nausea. Vitals were stable. Initial labs showed Hb 9.5 g/dL, MCV 102 fL, WBC 22.4 x 103/μL, total bilirubin 2.4 mg/dL, direct bilirubin 0.7 mg/dL, alkaline phosphatase 436 units/L, ALT 133 units/L and AST 83 units/L. Hepatitis panel and Clostridium difficile toxin assay were negative. Further work-up revealed reticulocyte count > 17%, LDH 529 units/L, haptoglobin < 10 mg/dl and positive direct Coomb's test. Peripheral smear showed hemolysis by a warm antibody. Hydrocortisone was started for AIHA. CT abdomen showed mild colonic wall thickening. Colonoscopy showed moderate pancolitis and biopsies showed findings consistent with UC. Mesalamine was started. MRCP showed irregular beaded appearance of intra and extrahepatic bile ducts. ERCP was performed and biopsies taken from the ducts showed chronic inflammation but no evidence of malignancy. ASMA and ANCA were positive. Liver biopsy ruled out overlap syndrome with autoimmune hepatitis. The patient clinically improved and was discharged to follow-up with a gastroenterologist. In patients with UC, extra-intestinal manifestations involving cutaneous, hepatobiliary and ocular systems are common. The concomitant presence of PSC is 2-7% and AIHA is 0.2 -1.7% in patients with UC. The extra-intestinal manifestations of UC could be due to associated autoimmune diseases or they could be immune-mediated due to damaged mucosal wall. The exact pathogenesis of PSC and AIHA is unknown. The absorption of toxins and antigenic substances from the mucosa in the circulation mediates inflammation in the biliary system leading to PSC. This also leads to warm anti-E antibody production which cross-react with intestinal microbiome and the E antigen at the surface of RBCs. In UC patients, a vigorous workup for early detection of other autoimmune diseases is important to improve the quality of life and to prevent mortality associated with them.Figure: Peripheral smear showing polychromatic RBCs and microspherocytes.Figure: Axial CT demonstrating mild wall thickening and featureless appearance of the rectosigmoid colon compatible with chronic ulcerative colitis (left). Descending colon at 4X magnification showing crypt distortion, cryptitis and crypt abscess (right).Figure: Left: Coronal thin section T2 weighted image showing a beaded appearance of the left hepatic and intrahepatic bile ducts with alternating strictures (arrows) and dilatation (arrowheads). Right: A) Fluoroscopic image from an ERCP depicting beading of the bilateral intrahepatic bile ducts due to segmental strictures (thin arrows). Guidewire in the left intrahepatic bile duct (thick arrow). B) ERCP image after biliary stent placement (arrowhead) with decompression of the biliary tree.

Bilateral Intrahepatic Bile Duct Compression by Giant Non-parasitic Hepatic Cyst: A Rare Cause of Obstructive Jaundice

Bilateral Intrahepatic Bile Duct Compression by Giant Non-parasitic Hepatic Cyst: A Rare Cause of Obstructive Jaundice

Compresión bilateral de vías biliares intrahepáticas por quiste hepático no parasitario gigante: una causa infrecuente de ictericia obstructiva

Compresión bilateral de vías biliares intrahepáticas por quiste hepático no parasitario gigante: una causa infrecuente de ictericia obstructiva

A case of IgG4-related disease coexisted with rectal cancer.

A 67-year-old man was referred to our hospital with suspicion of rectal tumor, hilar tumor, and urinary tumor. Colonoscopic findings were intermittent nodular lesions with redness which were atypical to primary rectal cancer. Endoscopic retrograde cholangiopancreatography showed narrowing of the bilateral intrahepatic bile duct. However, the findings were improved 1 month later. Blood biochemistry showed high level of serum IgG4 up to 1140 mg/dl. The patient matched to comprehensive diagnostic criteria for IgG4-related disease as a possible diagnostic case. Laparoscopic low anterior resection with creation of ileostomy was performed for rectal cancer. Histological findings revealed cancer cells spread horizontally at submucosal layer and subserosal layer. There was marked infiltration of the plasma cells and lymphocytes at tumor stroma, and more than half of the plasma cells were positive for IgG4. After surgery, the level of serum IgG4 was decreased to 597 mg/dl. Although the association with IgG4-related disease and colorectal disease is unclear, the tumor progression was atypical for rectal cancer. Some report that the disease may rise up the risk of a malignant disease. It is necessary to perform systemic examination keeping in mind for concurrence of malignancy.

Isolated hepatobiliary cryptococcosis manifesting as obstructive jaundice in an immunocompetent child: case report and review of the literature

The majority of patients with symptomatic cryptococcosis have an underlying immunocompromising condition. In the absence of coexisting immunocompromising condition, Cryptococcus neoformans is rarely considered in the differential diagnosis of obstructive jaundice that occurs in children with hilar masslike lesion. Here, we report a 5-year-old boy without immunoglobulin or lymphocyte abnormalities who developed a hepatobiliary infection with C. neoformans. Ultrasonography and computed tomography showed dilatation of the bilateral intrahepatic bile ducts and a low-attenuated mass in the hepatic hilum. Microscopic examination of tissue samples revealed abundant numbers of encapsulated yeast cell suggestive of C. neoformans. After 4 months of antifungal therapy (liposomal amphotericin B for 2 weeks and oral fluconazole for 3 months), the disease was effectively controlled. Unnecessary operation could be avoided by an early and accurate diagnosis. By sharing our experience, we suggest hepatobiliary surgeons and gastroenterologists should have a suspicion of this unusual entity to make earlier diagnosis and treatment.

Well-Differentiated Endocrine Carcinoma Originating From the Bile Duct in Association With a Congenital Choledochal Cyst

We encountered a rare case of a well-differentiated endocrine carcinoma originating from the bile duct in association with a congenital choledochal cyst (CCC). The patient is a 28-year-old woman referred to our clinic for pruritus. Laboratory data showed mild elevation of serum hepatobiliary enzymes. Computed tomography and magnetic resonance imaging demonstrated pancreatobiliary maljunction and a Todani type IV-A CCC from the inferior bile duct to the bilateral intrahepatic bile ducts. A solid tumor was detected in the middle portion of the common bile duct. Pancreatoduodenectomy and total extrahepatic bile duct resection was performed. Based on pathologic and immunohistochemical examinations, a diagnosis of well-differentiated endocrine carcinoma was made according to the World Health Organization criteria. To our knowledge, this is the third report of a neuroendocrine tumor originating from the bile duct in association with a CCC.

Diagnostic usefulness of precise examinations with intraductal ultrasonography, peroral cholangioscopy and laparoscopy of immunoglobulin G4‐related sclerosing cholangitis

Herein, a case of immunoglobulin G4 (IgG4)-related sclerosing cholangitis is reported. IgG4 was diagnosed based on observations from peroral cholangioscopy and laparoscopy, and these methods are proposed for definitive and precise diagnosis of this disease. A 76-year-old male patient with inguinal Paget's disease had intrahepatic bile duct dilatations detected with computed tomography at his periodic check-up. Magnetic resonance cholangiography showed stenosis of the upper common bile duct and poststenotic dilatation of left intrahepatic bile ducts. The portal tract and bilateral intrahepatic bile ducts were surrounded by a low-density area, facing a tumor-like lesion at segment 2. Cytological examinations of the stenotic and dilated lesions revealed no cellular atypia. Histological examination of the tumor showed normal liver tissue with infiltration of lymphocytes, indicating an inflammatory pseudotumor. Peroral cholangioscopy excluded the possibility of biliary cancer and indicated that the stenotic legion was of submucosal, not mucosal, origin. Laparoscopic observations showed discoloration with wide yellowish-white lobular markings and wide depressed lesions at segments 2 and 7. Liver histology showed mild cholangitis with infiltration of IgG4-positive plasma cells around the bile ducts. Serum IgG4 levels were elevated. From these findings, the patient was diagnosed with IgG4-related sclerosing cholangitis. After treatment with prednisolone, blood liver enzymes and IgG4 rapidly normalized, bile duct dilatations improved, and the hepatic pseudotumor disappeared. The cholangitis did not recur. In this case, biliary cancer was ruled out by observation with peroral cholangioscopy, and the spread of cholangitis in the liver periphery was verified with laparoscopy; this information could not be obtained with other modalities.

Liver cryptococcosis manifesting as obstructive jaundice in a young immunocompetent man: Report of a case

A 25-year-old immunocompetent Japanese man was referred to us for investigation and treatment of obstructive jaundice. Computed tomography (CT) showed dilatation of the bilateral intrahepatic bile ducts and a low-attenuated mass extending along the portal tract, from the hepatic hilum to the periphery, suggestive of cholangiocellular carcinoma (CCC) with periductal infiltration. However, the culture of bile obtained by percutaneous transhepatic biliary drainage revealed Cryptococcus neoformans. Microscopic examination of a biopsy specimen of the mass-like lesion in the liver revealed fungal organisms encapsulated in multinucleated giant cells. A small area of infiltration in the left lung was also detected on chest CT, and a diagnosis of liver and lung cryptococcosis was made. After 8 months of antifungal therapy, both the liver and lung lesions had vanished and the biliary stricture resolved. Unnecessary surgical intervention was avoided by an early and accurate diagnosis and effective antifungal therapy. The bile culture was the key to the definitive diagnosis in this case.

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

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.

Atypical MRI features of a biliary cystadenoma revealed by jaundice

We report on the case of a patient admitted for epigastric pain. An abdominal ultrasound revealed a voluminous cystic lesion of the left hepatic lobe. In magnetic resonance imaging, the mass had a liquid-liquid level that was spontaneously hyperintense on T(1)-weighted images and hypointense on T(2)-weighted images. Magnetic resonance cholangiography identified bilateral intrahepatic bile duct dilatation. A left hepatectomy finally revealed a mucinous cystadenoma with pseudo-ovarian stroma that had a pedunculated intraductal extension to the biliary convergence.

An Icteric Type Hepatocellular Carcinoma with No Detectable Tumor in the Liver: Report of a Case

A 70-year-old man was admitted to our hospital with obstructive jaundice. Computed tomography revealed a tumor in the left intrahepatic bile duct extending to the common bile duct without any significant lesions in the liver. Cholangiography showed a filling defect due to an intraductal tumor. Cytology of the bile juice was negative and tumor markers were carcinoembryonic antigen 5.7 ng/ml, carbohydrate antigen 19-9 49 U/ml, alpha-fetoprotein 9 ng/dl, and PIVKA-II 19 200 AU/ml. With a preoperative diagnosis of hilar bile duct carcinoma, a laparotomy was performed. The common bile duct was filled with a tumor and it extended into the bilateral intrahepatic bile ducts. The intraductal tumor was removed together with the extrahepatic bile ducts. An intraoperative histological examination of the tumor showed a well-differentiated hepatocellular carcinoma. No lesions were detected in the liver by ultrasonography, palpation during the operation, or a computed tomography scan after the operation. At 1 year postoperatively, no recurrence has been seen in this patient.

Clinical evaluation of the Haber RAMPTM catheter for endoscopic bilateral metal stent placement in patients with malignant hilar strictures

Background: Endoscopic bilateral expandable metal stent (EMS) placement has been performed in malignant hilar strictures. To perform EMS placement successfully, insertion of guidewires into the bilateral intrahepatic bile ducts is an important and necessary step. In the present study, we evaluated the usefulness of the Haber RAMPTM catheter (HRC) for the selective insertion of guidewires.Methods: EMS placement was performed in 17 patients with malignant hilar strictures. As the HRC has three lumens connected to apertures facing in different directions, the direction of the guidewire can be controlled at the catheter tip. Insertion of guidewires into bilateral intrahepatic bile ducts was attempted with and without the HRC.Results: The success rate of guidewire insertion into the bilateral intrahepatic bile ducts was 100% (7/7) when the HRC was used, whereas it was 50% (5/10) without the HRC. In the five patients with insertion failure, reinsertion was successfully performed using the HRC. The time required for the bilateral insertion of guidewires was reduced using the HRC. Bilateral EMSs placement was successful in 14 of the 17 patients (82%).Conclusions: The HRC was very useful for the accurate and rapid bilateral insertion of guidewires.