Dilatation Of Intrahepatic Ducts Research Articles

Recurrent extrahepatic cholangiocarcinoma after bile duct resection for intraductal papillary mucinous neoplasm of the bile duct.

Intraductal papillary neoplasm of the bile duct (IPNB) is a rare but recognized precursor to invasive carcinoma of the biliary tree and is characterized by dilated intrahepatic ducts with mucinous or papillary epithelium. Progression of papillary biliary neoplasia from benign disease to malignancy is thought to be similar to the pathway of an intraductal papillary neoplasm of the pancreas. The mainstay of therapy has been surgical resection. Surgical series demonstrate high rates of invasive cancer arising from IPNB; rates range from 40% to 70%.

Severe emesis and acholic stools in a 70-year-old man

A 70-year-old man with an unremarkable medical history presented to the emergency department with a 2-week history of dark urine, pale stools and diffuse abdominal discomfort. He could not tolerate...

Acute kidney injury due to high-output external biliary drainage in a patient with malignant obstructive jaundice: a case report

BackgroundPersistent high output is a rare but potentially serious complication of percutaneous biliary drainage.Case presentationA 68-year-old Sinhalese woman with a palliative self-expanding metal stent placed for an inoperable hilar cholangiocarcinoma presented with worsening obstructive jaundice. Ultrasonography showed intrahepatic duct dilatation with the self-expanding metal stent in situ. Since this was indicative of a blocked stent, percutaneous transhepatic cholangiogram-guided internal biliary stenting through the self-expanding metal stent was attempted and failed. Therefore, an external biliary drain was left in the dilated biliary system. Post procedure, she developed a high biliary output of 3–4 liters per day and went into oliguric acute kidney injury with metabolic acidosis, most probably due to inadequate fluid replacement and hypovolemia.ConclusionAlthough the mechanism by which this occurs in some cases is unclear, early identification and prompt fluid resuscitation prevent acute kidney injury. The adoption of new strategies for internal drainage of long complex strictures will both prevent and ameliorate this problem.

Transabdominal Ultrasonography in Symptomatic Choledocholithiasis - Usefulness in Settings with Limited Resources.

Objective:Ultrasonography remains the initial imaging modality in the management of biliary disease. This study is designed to evaluate the accuracy of transabdominal ultrasonography in diagnosing biliary pathology in patients with choledocholithiasis.Methods:This was a retrospective study of a continuous sample of patients over a period of 3 years ending in January 2016; these patients were referred for endoscopic management of choledocholithiasis to a tertiary care hospital in Colombo, Sri Lanka. Ultrasound reporting was carried out by different consultant radiologists at both the index and the referring hospitals. The findings of endoscopic retrograde cholangiograms were compared with the ultrasound scan (USS) results.Results:A total of 247 patients were included in the study. USS was 97.4% accurate in detecting intrahepatic duct dilatation (IHDD). Stone counts and the location of stone(s) in the USSs correlated strongly with the number of stones delivered during endoscopic removal and their location in cholangiograms (P < 0.001). The difference in mean diameter of the common bile duct (CBD) of patients with choledochal cysts (CCs) (18.57 mm) and of patients without them (12.39 mm) is statistically significant (P < 0.001). At 14.5 mm, the negative predictive value for a CC is 99.02%.Conclusion:Ultrasonography is a reliable tool in predicting IHDD, stone count, and the location of stones in the biliary tree, particularly in a resource-poor setting. A CBD diameter of 14.5 mm in transabdominal ultrasound scan can be used as a cutoff for predicting extrahepatic CC.

Remodeling of Intrahepatic Ducts in a Model of Caroli Syndrome: Is Scar Carcinoma a Consequence of Laplace’s Law?

Caroli syndrome, characterized by saccular dilatation of intrahepatic ducts and congenital hepatic fibrosis, is without therapy in part due to its ultra-rare prevalence and the apparent lack of availability of a suitable experimental model. While the PCK rat has long been used as a model of fibropolycystic kidney disease, hepatobiliary biophysics in this animal model is incompletely characterized. Compared to age-matched, wild-type controls, the PCK rat demonstrated severe hepatomegaly and large saccular dilated intrahepatic ducts. Nevertheless, hepatic density was greater in the PCK rat, likely due to severe duct wall sclerosis accompanied by scarring across the hepatic parenchyma. Extracellular matrix accumulation appeared proportional to duct cross-sectional area and liver volume and appeared compensatory in nature. The PCK rat livers exhibited both cholangiocarcinoma and hepatocellular carcinoma coincident with areas of increased extracellular matrix deposition. Together, these data suggest that the PCK rat model mimics at least in part the spectrum of hepatobiliary pathology observed in Caroli syndrome and highlights the attendant risk associated with this disease.

Partial response after treatment with Conversion chemotherapy

Rationale:Many studies have reported radical resection for liver metastasis and the primary tumor could represent an important prognostic factor in patients affected by colorectal liver metastases (CRLM). However, resection of huge liver metastases from colon cancer has been seldom reported.Patient Concerns:A 58-year-old man presented with huge liver metastases from colon cancer. Laboratory tests revealed elevated tumor markers and a wild-type mutation in the K-RAS gene. A computed tomography scan demonstrated unresectable liver masses with a 16.5-cm maximum diameter and intrahepatic duct dilatation due to compression by the liver metastases.Diagnosis:The patient was diagnosed with stage IV descending colon carcinoma with multiple huge hepatic metastases.Interventions:He was administered 3 treatment courses, including 9 cycles of combined chemotherapy with mFOLFOX6 plus cetuximab (mFOLFOX6 + Cet), and the liver masses reduced. After a preoperative assessment by a multidisciplinary team when the 9 cycles of systemic chemotherapy had been completed, the patient underwent hepatectomy, followed 4 months later by a laparoscopic colectomy. We used a reverse strategy (liver-first) for the patient.Outcomes:In this case, liver-first treatment (systemic chemotherapy of mFOLFOX6 + Cet) was an effective treatment for unresectable CRLM. No postoperative complications occurred. The patient continued to receive postoperative chemotherapy (mFOLFOX6 + Cet) at the latest follow-up. During the 17 months of follow-up, tumor recurrence was un-noted.Lessons:Treating colorectal cancer patients with huge hepatic metastases is possible, and surgeons should consider various treatment options in the management of these patients.

Hepatolithiasis following “non-Roux-en-Y” hepatico-jejunostomy for common bile duct injury

Background: Hepatolithiasis (HL) is defined as gallstones present in all bile ducts peripheral to the confluence of the right and left hepatic ducts, irrespective of the coexistence of gallstones in other parts of the biliary tract. HL is frequently found in patients with recurrent pyogenic cholangitis. Methods: Case presentation. Results: A 65-year-old woman presented with intermittent fever, jaundice, abdominal pain, and nausea. Eighteen years ago she underwent open cholecystectomy for acute cholecystitis. In the first post-operative days she was noted to be icteric. Abdominal ultrasound showed intrahepatic biliary ductal dilatation. Re-operation was indicated due to suspicion of iatrogenic common bile duct injury, which was confirmed intra-operatively at time of surgical re-exploration. Hepatico-jejunostomy (HJ) “non-Roux-en-Y” was performed. Despite this surgical biliary bypass, this patient continued to have recurrent bouts of cholangitis over the ensuing eighteen years. Given the patient’s recurrent symptoms and results of MRI consistent with HL, surgical treatment was indicated. A left hepatectomy was performed with Roux-en-Y HJ biliary reconstruction. Post-operative course was uneventful. Patient is symptoms free without any attack of cholangitis. Conclusion: The primary goal in treating HL is to eradicate existing infection, recurrent cholangitis and subsequent hepatic fibrosis, prevent progression of the disease to cholangiocarcinoma. Hepatectomy and/or Roux-en-Y HJ is the best choice for treatment of HL.

Differences in early imaging features and pattern of progression on CT between intrahepatic biliary metastasis of colorectal origin and intrahepatic non-mass-forming cholangiocarcinoma in patients with extrabiliary malignancy.

To assess the differences in early imaging features and progression pattern on CT between intrahepatic biliary metastasis (IBM) and non-mass-forming cholangiocarcinoma (NMFC) in patients with extrabiliary malignancy. This retrospective study included 35 patients who were surgically and pathologically confirmed with IBM (n = 14) or NMFC (n = 21) at the time of or after surgery for extrabiliary malignancy. Two observers evaluated the following aspects of biliary lesions on initial or follow-up CT images: location, characteristics of intrahepatic duct (IHD) dilatation, presence of duct wall thickening, and periductal infiltration lesion or periductal expansile mass. All IBMs were associated with colorectal cancer (p = 0.032). As early imaging features on CT, smooth tapered localized IHD dilatation without duct wall thickening and peripheral duct involvement were observed significantly more often in IBM, and IHD dilatation with abrupt tapering or irregularity of transition site and bile duct wall thickening were significantly more common in NMFC (all p < 0.05). Regarding progression pattern, periductal expansile mass was present only in IBM, whereas periductal infiltrative lesion was present only in NMFC (p < 0.001). In the differentiation between IBM and NMFC in patients with extrabiliary malignancy, the differences in early imaging features and progression pattern of the two diseases revealed in this study would be helpful for diagnosis.

Sarcomatoid Carcinoma of the Pancreas: An Unusual Cause of Abdominal Pain

Introduction: Sarcomatoid carcinoma of the pancreas (SCP) is a rare and highly malignant variant of pancreas carcinoma that usually has a very poor prognosis. Epithelial-mesenchymal transition (EMT), a transition between different phenotypes, is regarded as an essential process in tumor progression. When EMT occurs, epithelial tumor cells lose their restricted phenotypes, concomitant with the loss of epithelial characteristics and the acquisition of motile behavior. These changes, which contribute to the transition from an epithelial morphology towards a more mesenchymal fibroblastic phenotype, have been reported to occur in some special tumors, such as cholangiocarcinomas; this change is also defined as sarcomatous change. Case Report: A 37-year-old-female with a history of hypertension presented to the hospital with the chief complaint of epigastric abdominal pain. Her abdominal pain progressively worsened and she came back to the hospital 3 weeks later. She also complained of pale, foul-smelling, floating stools that began 3 days prior to presentation with a decrease in her appetite. Routine laboratory showed: hemoglobin, 12.8 g/dL; WBC, 5,100 cells/uL; platelet, 244,000/mm3; with elevation in AST, ALT, alkaline phosphatase; total bilirubin, 4.0 mg/dL; direct bilirubin, 3.1 mg/dL; and LDH 306 u/L and negative viral markers. Tumor marker assays showed elevated CA19-9. Results: CT abdominal scan showed a hypoechoic mass in the uncinate process of the pancreas (Figure 1) with intrahepatic biliary ductal dilatation and dilation of the proximal common bile duct (CBD). The patient underwent an esophagogastroduodenoscopy with brush biopsies of the CBD with stent placement, as well as, an endoscopic ultrasound fine needle aspiration of the pancreatic mass. The patient underwent a pancreaticoduodenectomy to help diagnosis and remove the tumor. The histopathological diagnosis was as follows: ductal adenocarcinoma with sarcomatoid features with the invasion of the tumor in the extra-pancreatic common bile duct, stage IIB (Figure 2A & 2 B).1372_A.tif Figure 1: CT abdomen with contrast showing the hypoechoic mass in the head of the pancreas with intrahepatic biliary ductal dilatation.1372_B.tif Figure 2: Pathology specimen showing adenosquamous carcinomaDiscussion: Adenosquamous carcinoma of the pancreas is associated with high rates of recurrence and metastasis and carries a poor prognosis, much worse than even that of pancreatic adenocarcinoma, which has a reported median overall survival of no more than 5 months. SCP is a rarely appreciated subset of pancreatic malignancy that does not necessarily portend to a uniformly dismal prognosis.1372_C.tif Figure 3: Sarcomatous changes.

Ewing Sarcoma/Primitive Neuroectodermal Tumor of the Liver: A Late Presentation With an Extremely Rare “Large Cell” Variant

Ewing Sarcoma (ES)/primitive neuroectodermal tumour (PNET) is a mesenchymal tumor typically belonging to the family of “small round-cell tumors”. Typically, patients present in the first three decades of life. Although PNETs can occur in numerous solid organs, they are extremely rare in the liver. We report a case of a 33-year-old male who presented with worsening right upper quadrant abdominal pain and jaundice for one month. His laboratory studies were as follows: Total Bilirubin 12.2 Direct Bilirubin 7.6 Alkaline Phoshatase 484 Aspartate Aminotransferase 144 Alanine Aminotransferase 153. Computed Tomography scan of the abdomen revealed multiple liver lesions, the largest measuring 10.4 x 7.1 cm (Figure 1), as well as intrahepatic ductal dilatation. A biopsy of this lesion was consistent with sheets of large atypical epithelioid cells with vesicular nuclei, prominent nucleoli, and vacuolated cytoplasm diffusely infiltrating the liver parenchyma with patchy necrosis (Figure 2). These findings represent ES/PNET, but the histologic appearance shown (Figure 1) represented a rare “large cell” variant.2345_A Figure 1 No Caption available.2345_B Figure 2 No Caption available.The Ewing Family of Tumors includes ES and PNET.1 There is considerable overlap between the two, sharing a common and unique translocation t(11; 22) (q24; q12).2 ES/PNET is a member of the family of “small round cell tumors”. Although it can occur in numerous solid organs, it is an exceptionally rare tumor entity. Liver involvement is even rarer and is usually in the form of metastasis.2 The majority of patients with PNET are younger than 30 years of age.3 Three unique aspects of our case are as follows: late presentation, a “large cell” variant indicating an atypical ES/PNET, and liver involvement. To the best of our knowledge, this is only the fifth case reported involving primarily the liver. Furthermore, this is the only reported case involving the liver in an adult over the age of 30 years with a “large cell” variant. Most patients are diagnosed when metastasis has already occurred and prognosis is extremely poor.

Unusual Discovery During ERCP: Bile Duct Adenoma

Cholangiocarcinoma represents an uncommon heterogenous group of tumors that can affect the liver, the hilum, the bile duct or the gall bladder. We report a patient with hilar cholangiocarcinoma (Klatskin's tumor) and an extremely rare adenomatous polyp in the mid portion of the common bile duct seen incidentally during cholangioscopy. A 74-year-old male presented to the office with acholic stools for the past 2 weeks, jaundice, and muscle pain since starting Crestor. He had a remote history of alcohol abuse but quit >10 years ago. He denied any other risk factors for liver disease and there was no family history of liver disease. Physical exam was unremarkable except for significant jaundice. Four days earlier T. bilirubin was 12.9, alkaline phosphatase 357, AST 85, and ALT 182. Four months prior T.bilirubin was 1.0, alkaline phosphatase 257, AST 41, and ALT 51. Abdominal ultrasound 1.5 years ago showed no evidence of cholecystolithiasis, the common bile duct measured 8 mm without intrahepatic dilation. The liver had homogeneous echotexture, and no mass was seen. Additional new studies showed: AFP - 4.0, Ca 19-9 - 132, GGT — 698. CT with contrast revealed significant right and left intrahepatic biliary ductal dilation, no extrahepatic biliary ductal dilation with hyperenhancement throughout the common hepatic duct that focalizes to a 2cm mass suspicious for hilar cholangiocarcinoma. ERCP was performed: The cholangiogram revealed a tight short stricture just below the bifurcation. A papillotomy was performed and cholangioscopy was carried out, the strictured area appeared friable and irregular. It was biopsied. In the mid CBD an area of elevated tissue (polyp) was identified (photo A) and biopsied. Histological exam of the hilum biopsy showed adenocarcinoma(photo B) and the biopsy of the polyp showed adenoma and cholesterolosis (photo C). The patient was referred to a university hospital for possible surgery and oncologic treatment. We report a rare finding: an adenomatous polyp with cholesterolosis in the common bile duct. CBD polyps may be hyperplastic, hamartomatous, inflammatory, cholesterol, papillary neoplasms or adenomatous. With greater use of cholangioscopy more polyps may be detected which in time may bring a better understanding of the natural history of biliary neoplasms and improve therapeutic options for the patients with these rare but deadly cancers.1389_A.tif Figure 1: CBD polyp on cholangioscopy1389_B.tif Figure 2: Pathology of the hilar adenocarcinoma1389_C.tif Figure 3: Pathology of the CBD adenoma

Herbal Remedy or Medical Tragedy?

This is the case of a 63—year—old Hispanic man who came to the emergency room due to right upper quadrant discomfort and yellowing of the skin for the past week. Patient denies fever, chills, nausea, vomiting, diarrhea, sick contacts at home or recent traveling however refers he began taking saw palmetto for benign prostatic hyperplasia a few months ago. Physical exam was remarkable for generalized jaundice and moderate abdominal right upper quadrant tenderness. Laboratory tests revealed elevated bilirubin in 4mg/dL, alkaline phosphatase of 181 u/L and elevated hepatic enzymes consistent with a cholestatic pattern. Abdominal CT without intravenous contrast and abdominal sonogram both unremarkable. MRCP revealed mild intrahepatic and proximal extrahepatic ductal dilatation with common bile duct measuring 2.8cm most likely secondary to prior cholecystectomy since no evidence of obstructive lesions. Toxicology negative. Work up for pre—existing liver disease was negative for viral hepatitis and autoimmune markers. Drug—induced liver injury (DILI) can develop secondary to the use of many drugs including both prescription and over—the counter. Drugs associated with DILI may cause hepatic injury in a dose—dependent way or an unforeseeable manner. DILI accounts for approximately 10 percent of all cases of hepatitis and it is the most common cause of acute liver failure in the United States. DILI is characterized by the type of hepatic injury occurred including hepatocellular injury, cholestatic injury or mixed. In this case, the DILI is considered cholestatic since the ratio of ALT/ALP is less than 2. The presence of jaundice (bilirubin >2 times the upper limit of normal) and elevation in serum aminotransferases (>3 times the upper limit of normal) is associated with a worse prognosis, as in this case. Saw palmetto is one of the most commonly use herbal remedies to treat benign prostatic hyperplasia. Even though in clinical trials liver injury associated to saw palmetto is rare, there are a few case reports in which direct liver injury secondary to Serenoa repens, colloquially known as “Saw Palmetto” is demonstrated. As it occurred in reported cases, liver injury in our patient resolved upon removal of the offending agent which was demonstrated in laboratory workup performed three months later.

Challenges in the Diagnosis of Biliary Malignancies: Klatskin Tumor, A Case Report

Klatskin described cholangiocarcinomas arising at the level of the hepatic duct bifurcation in 1965, hence its name (1). They comprise 3% of all the gastrointestinal malignancies from the biliary tract. In the United States the higher prevalence ranks among African Americans and Asian Pacific Islanders (2). Due to the location of these lesions and non-specific symptoms, they are usually caught on very late stages, making treatment and prognosis an issue. At the same time, diagnosis can be challenging as invasive procedures like endoscopic retrograde cholangiopancreatography (ERCP) and tissue biopsy are required for a better characterization of any lesion in the biliary tree. We report the case of a 74 years old male who presented with jaundice, abdominal pain, dark loose stools and weight loss for three months. He was noted to have a total bilirubin of 14mg/dl, alkaline phosphatase 888mg//dl. A computer tomography showed a perforation vs malignancy in the gallbladder surrounded by pericholic abscess. He received a course of antibiotics to prevent cholangitis. A drain was placed relieving the obstruction; the cholangiogram revealed an obstruction at the confluence of the left and right hepatic ducts. The Radiology service considered a MRCP would not be beneficial due to high amount of gas and stones. Surgery determined that a biopsy could not be done due to inaccessibility and also he was a poor surgical candidate for any intervention. Tumoral markers were elevated. We could not establish the diagnosis of cholangiocarcinoma by tissue biopsy although the tumor markers and location of the lesion were highly suggestive for Klatskin tumor. Due to poor prognosis, the patient opted for hospice care. As mentioned before, the diagnosis of cholangiocarcinoma can be challenging due to its location and, like in this case, the possibility of synchronous malignancies (gallbladder). A proper diagnosis can be made through lesion biopsy. Unfortunately, just 26% of patients are surgery candidates either due to poor prognosis, late stage of diagnosis or multiple comorbidities (3). ERCP/MRCP are very useful imaging techniques that allow a fairly direct visualization of the biliary tree, however, due to the amount of gas and stones, this modality is not always optimal. The prognosis of these malignancies is very bad, around 7 months since the time of diagnosis. Poor prognostic factors are: the presence of metastasis, perineural invasion or involvement of the cystic duct.(4)2907_A Figure 1. Severe intrahepatic biliary ductal dilatation. Pericholecystic abscess.2907_B Figure 2. Obstruction at the level of hepatic cysts bifurcation concerning for malignancy.

Finding a Signet Ring in the Biliary System

65 year old female comes in with painless jaundice and weight loss of greater than 40 lbs in the past year, Patient found to have signet ring carcinoma of the stomach with metastasis to the ampulla of vater. SRCC in the ampulla of vater is a rare entity.1 While the incidence of gastric cancer is decreasing, the incidence of signet ring cell carcinoma (SRCC) is only increasing with about a 10-fold increase from 1970 to 2000.2 65 yo F with PMH of HTN, DM, non-ischemic cardiomyopathy s/p AICD 2013 (EF 45% 9/2015), CVA with expressive aphasia, cervical cancer s/p hysterectomy admitted for painless jaundice (total bili of 21) and unintentional weight loss (about 40lbs within the past year). CT abd/pelvis w/out contrast and RUQ ultrasound showed intrahepatic and extrahepatic biliary ductal dilatation (Image 1,2 and 3). EUS was significant for wall thickening in the entire body and antrum of the stomach with wall thickness measuring 14mm in the antrum and 19mm in the body; impression consistent with Linitis plastica or infiltrating gastric cancer. EUS guided FNA showed signet ring carcinoma of stomach with local metastasis to ampulla of vater. ERCP unable to be performed due to edematous mucosa; had percutaneous biliary drain placed through IR. Bili trended downward after placement of drain. She had surveillance CT chest and abdomen/pelvis w/ contrast for further staging (negative for any metastasis or lymphadenopathy). Patient was not a candidate for chemotherapy/RT/surgery. Only 26 cases previously have been described with SRCC in the ampulla of vater.1 Prognosis in early gastric cancer is better in SRCC compared to non SRCC gastric carcinomas while prognosis is less clear between SRCC and non-SRCC gastric cancers in advanced gastric cancer.2Prognosis with SRCC in the ampulla is even less clear, with >5 year survival reported in only 4/26 cases.1 Our patient was advanced gastric cancer of SRCC and given metastasis and poor functional status was not a candidate for further therapeutic measures.21365_A.tif Figure 1: No Caption available.1365_B.tif Figure 2: No Caption available.1365_C.tif Figure 3: No Caption available.

Escherichia coli Bacteremia Following Liver Biopsy: A Case Report of Hemobilia Related Cholangitis From Transjugular Liver Biopsy

Purpose: To present an orthotropic liver transplant (OLT) patient who had transjugular liver biopsy and developed sepsis secondary to cholangitis and bacteremia in the setting of hemobilia. Case: The patient was a 61 yo M with a history of OLT with Roux-en-Y hepaticojejeunostomy (HJ) in 2008 for cryptogenic cirrhosis. The patient did well for ten years but was referred for liver biopsy to assess for graft rejection. The biopsy was performed in a transjugular approach without immediate complication. Two days post biopsy, he developed rigors, nausea with vomiting, right upper quadrant abdominal pain, leukocytosis, and fever. Blood cultures grew E. coli. Liver ultrasound demonstrated mild left lobe intrahepatic bile ductal dilatation. MRCP was consistent with hemobilia (Image 1, Image 2). Bilirubin increased post biopsy from 1.5mg/dl on admission to 10mg/dl. A percutaneous biliary drain was placed and culture of the drain fluid grew extended-Spectrum Beta-Lactamase (ESBL) E. coli. The patient was treated with meropenem with resolution of infection and hyperbilirubinemia. The patient never developed melena and the hemoglobin remained stable. Discussion: Hemobilia is a rare complication of liver biopsy whether it is performed with a percutaneous or transjugular approach. Hemobilia can cause biliary infection due to retention of “clotted blood” within the common bile duct, particularly if there is underlying pathology which impairs drainage. In a patient with Roux-en-Y (HJ), there is a lack of a native ampulla which usually serves as a competent valve. This altered anatomy could predispose to reflux of bowel contents into the biliary tree, allowing bacteria to colonize, particularly if there is underlying biliary dilation or stricture. While hemobilia is a rare complication of liver biopsy we believe that underlying Roux-en -y (HJ) may predispose the patient to a septic complication should it occur. Our experience suggests that in order to minimize morbidity from liver biopsy that patients with Roux-en-Y (HJ), with or without evidence of impaired biliary drainage, should be considered for pre-operative prophylactic antibiotics cover enteric flora.2246_A Figure 1. T1 cross-sectional image from abdominal MRI MRCP w/o contrast showing mild prominence of the intra and extrahepatic biliary ducts2246_B Figure 2. T1 image from abdominal MRI MRCP w/o contrast showing mild prominence of the intra and extrahepatic biliary ducts2246_C Figure 3. T2 image which demonstrates proteinaceous signal within the intra and extrahepatic bile duct to the level of the hepaticojejunostomy consistent with hemobilia

A Very Rare Complication of Hepatitis a Infection: Acute Myocarditis

Hepatitis A is a common viral infection with a benign course but in rare cases can progress to acute liver failure. A rare manifestation of hepatitis A is acute myocarditis. We report a case of a 30-year-old female, without significant past medical history who presented with four days of fatigue, myalgias, nausea, non-bloody, non-bilious emesis and non-bloody diarrhea along with anorexia. Physical exam was significant for mild scleral icterus and right upper quadrant abdominal tenderness, without peritoneal signs or fluid wave. She had intact mentation, no asterixis or stigmata of liver disease. Lab work revealed severe elevations in liver transaminases, AST = 6,769U/L, ALT = 8.479U/L., INR = 2.0 and acute viral hepatitis panel was positive for hepatitis A IgM only. EBV IgG and IgM were positive but heterophile assay and EBV PCR was negative (Figure 1 and 2). Abdominal US revealed homogenous hepatic echotexture without evidence of focal hepatic lesions. There was mild intrahepatic biliary ductal dilatation with common bile duct measuring 2mm. On her 3rd day of admission the patient developed chest pain and nonspecific ECG changes. Her troponin rose to 16.4 ng/mL, and an echocardiogram revealed global hypokinesis and a depressed ejection fraction (EF) at 30%. A coronary CT angiography showed no evidence of significant coronary artery disease. The patient was managed supportively and symptoms and laboratory findings slowly improved over the next 7 days. Her chest pain resolved and follow up echocardiogram showed improved EF to 45%. To our knowledge, there are 3 case reports of myocarditis associated with hepatitis A (Figure 3). Like previous case reports, a presumptive diagnosis of myocarditis secondary to hepatitis A was made, despite endomyocardial biopsy (EMB) being the gold standard for definitive diagnosis. However, EMB is not always indicated especially if it would not change management. Despite improving liver indices, the patient developed myocarditis. Therefore, it may be worthwhile to monitor patients for cardiac abnormalities despite signs of improving transaminases. Comparison of these case reports also highlights that higher elevations in transaminases may be associated with more severe cardiomyopathy. However, given the rarity of this manifestation, true associations are yet to be elucidated. The purpose of this case report is to increase awareness of this disease entity, so it can be recognized and considered in clinical practice.2305_A Figure 1. Key laboratory values on admission.2305_B Figure 2. Trend of liver function tests and troponin obtained during hospital course. Day 1 is day of admission. Day 17 is outpatient follow up.2305_C Figure 3. Other reported cases of myocarditis in hepatitis A.

Idiopathic Intrahepatic Cholestasis Associated With Monoclonal Gammopathy of Undetermined Significance

Cholestasis is a common presenting symptom in various hematologic diseases. We present a patient who had non-obstructive jaundice without demonstrable malignant cell infiltration and intact intrahepatic bile ducts, which was related to monoclonal gammopathy of undetermined significance (MGUS). A 58- year- old male had history of painless jaundice, anorexia and weight lost for 2 months. He had dark urine and pale stool. The patient denied alcohol and medicine intake. Physical examination found unrecognized fever, pale conjunctiva, icteric sclera and splenic dullness on percussion. Otherwise were unremarkable. Laboratory workups were significant for hemoglobin of 8.9, a TB of 65.7, a DB of 34.1, a SGOT of 170, a SGPT of 109, a ALP of 144. Peripheral blood smear was compatible with cold-type AIHA. CT of the whole abdomen showed normal parenchyma of liver without intrahepatic duct and CBD dilatation. The spleen size was 11 cm. No intra-abdominal lymph node enlargement. Workups for liver disease including viral hepatitis (HAV, HBV, HCV, HEV), ANA, ASMA, and AMA were negative. No atypical cell was detected from bone marrow study. Serum free light chain was significantly high level for Kappa monoclonal gammopathy. Liver biopsy found mixed inflammatory cells infiltration in hepatic lobules and portal tract. There was no significant bile duct loss. No malignancy was detected. The diagnosis of idiopathic intrahepatic cholestasis associated with MGUS was made. Intravenous dexamethasone 20 mg/day was prescribed. The LFTs improved dramatically after treatment and was allowed to be discharged with prednisolone 60 mg/day. Most cases of paraneoplastic-related cholestasis are caused by cytokines-induced vanishing bile duct syndrome. However, liver biopsy results from our patient did not show significant decrease in number of bile duct. Cholestasis in our patient was likely caused by dysregulation of bile duct transporters insulted from inflammatory mediators. This phenomenon is called idiopathic intrahepatic cholestasis, which is extremely rare paraneoplastic event. To the best of our knowledge, our patient has been the first case reported in MGUS. Treatment with systemic steroids is highly effective. In conclusion, paraneoplastic syndrome presenting as idiopathic intrahepatic cholestasis is associated with MGUS. Systemic steroid is effective for the treatment of this condition.2399_A Figure 1. The hepatic lobuls showed severe cholestasis with feathery degeneration. There was mild mixed inflammatory cell infiltrate in hepatic lobules and portal tracts. Bile duct was observed without bile duct injury.2399_B Figure 2. Immunohistochemical study for CK7 demonstrated insignificant bile duct loss.2399_C Figure 3. The liver function test improved dramatically after steroids administration.

Endoscopic Management of Biliary Stent Occlusion With Bipolar Electrocautery

Persistent biliary obstruction due to stent occlusion is a major cause of morbidity and mortality in patients with malignant obstruction of the bile duct. The standard of care for endoscopic management of these occlusions involves mechanical cleaning with a balloon or additional stent insertion. We present the case of a patient with biliary metal stent occlusion that was successfully treated with the aid of endoscopic bipolar electrocautery. A 73-year-old man with history of metastatic gastrinoma complicated by malignant biliary obstruction presented to the hospital with sepsis and was admitted to the MICU. During his workup, he was found to have rising total bilirubin levels, despite previously having four stent-in-stents placed to maintain patency of the CBD. Imaging was concerning for worsening CBD and intrahepatic biliary duct dilation. During ERCP, the stents were found to be occluded by sludge and debris. After attempted manual debridement with endoscopic forceps, a catheter could not be advanced through the stent occlusion. A bipolar electrocautery probe was then used to tunnel through the occlusion using soft coagulation. After tunneling, a balloon was passed through the inner stent for manual debridement. Occlusion cholangiogram after cleaning demonstrated patency of the duct with good drainage. Self-expanding metal stent placement is the standard therapy for endoscopic treatment of malignant biliary obstruction. Unfortunately, ensuring patency of these stents is difficult. Simple stent occlusions can be treated with a stent-in-stent approach or with mechanical cleaning with a balloon catheter. However, problems can arise that make this approach technically infeasible. With significant tumor ingrowth into the lumen of the stent, or with densely packed debris, a guidewire cannot be passed through the stent. Several studies have highlighted the use of radiofrequency ablation to tunnel through the occlusion. An alternative that has not been studied is the use of bipolar electrocautery to deliver thermal energy directly within the occluded stent. Our case demonstrated that an electrocautery probe can be used in a controlled manner to debride biliary duct occlusions, after which a balloon can be passed for more thorough cleaning. The wide availability of the gold probe catheter, ease of use, and minimal cost, make this a compelling alternative to radiofrequency ablation. Further study is warranted to assess the efficacy and safety of this approach.1414_A.tif Figure 1: Fully occluded uncovered SEMS seen protruding from the ampulla of Vater1414_B.tif Figure 2: Gold Probe catheter was used to burrow through the debris within the stent1414_C.tif Figure 3: After debridement, bile was visualized flowing freely through the stent

Mirror Image Technique in Choledocholithiasis: ERCP in Situs Inversus With a Forward Viewing Endoscope

Situs inversus is a congenital disease manifested by complete or partial reversal of anatomy. It occurs during early gestation and typically results in normal function. However, performing procedures on such patients is technically challenging due to their abnormal anatomies. Herein we describe a challenging endoscopic case of choledocholithiasis in a patient with situs inversus. A 45-year-old male with history of abdominal situs inversus from polysplenia syndrome presented for epigastric pain, nausea and vomiting. Vital signs were stable and exam was notable for diffuse tenderness in the epigastrium without jaundice or Murphy's sign. Pertinent labs showed a negative troponin, AST 399 U/L, ALT 385 U/L, alkaline phosphatase 425 U/L, total bilirubin 2.5 mg/dL, direct bilirubin 1.8 mg/dL, and WBC 13.6 K/uL with neutrophilic predominance. CT scan revealed abdominal situs inversus with multiple splenules, gallbladder distention without wall thickening or pericholecystic fluid, and an obstructing stone with common bile duct (CBD) dilatation to 2.1 cm. ERCP was performed by placing the patient in the right lateral decubitus with the endoscopist standing to the right of the patient. A TJF scope was advanced into the duodenum but retracted and was unable to be re-advanced due to the anatomy. The TJF scope was switched out for a forward viewing endoscope which was successfully advanced to the major papilla. The bile duct was then cannulated with the short-nosed traction sphincterotome. Injected contrast showed adequate ductal flow and dilated main bile duct with a 12 mm stone. Biliary sphincterotomy was made with traction sphincterotome using ERBE electrocautery and a plastic double pigtail stent was placed into the CBD allowing free-flow of the bile. Repeat ERCP for mechanical lithotripsy was scheduled and the patient was discharged. Situs inversus is a rare congenital disease that presents with partial to complete reversal of anatomy. Our case utilizes the mirror image technique employed in prior reports. This technique positions the patient in the right lateral decubitus and the endoscopist on the right of the patient. Prior cases were notable for ERCP failure rates of up to 25% in situs inversus. This case was successfully performed by utilizing mirror image technique with a forward viewing 1T scope. Though such cases are rare, we present this case to highlight the difficulties met by changes in anatomy and techniques used to overcome such challenges.2160_A Figure 1. Abdominal CT demonstrating stone obstructing common bile duct (Red Arrow).2160_B Figure 2. Abdominal CT demonstrating intrahepatic ductal dilatation (Red Arrows), contrast filled stomach (Blue Arrow), and liver situated on left side of the body (Green Arrows).2160_C Figure 3. Abdominal CT demonstrating extrahepatic ductal dilatation (Red Arrow), and multiple splenules (Blue Arrows).

Surgical Clip Migration Leading to Obstructive Choledocholithiasis

Choledocolithiasis is a known complication related to gallstone disease, but rarely occurs after cholecystectomy. Recurrent choledocolithiasis can occur anywhere from one to thirty years after a cholecystectomy. Few case reports have documented a surgical clip as a potenital nidus formation for choledocolithiasis with a proposed mechanism of involution of the cystic duct leading to necrosis and migration of the surgical clip into the common bile duct following a cholecystectomy. This case demonstrates a surgical clip that is found inside a choledocolith years after a cholecystectomy. A 56-year-old female with history of cholecystectomy two years ago presented with chest pain and found to have significantly elevated liver enzymes (total bilirubin 2.9 mg/dl, AST 1126 u/L, ALT 1998 u/L). Cardiac workup was unremarkable. A chest CTA in the ER did not find any pulmonary embolism but found a linear metallic structure in the distal common bile duct (CBD). Next, an ultrasound (US) was performed which reported the CBD at 10 mm and no evidence of dilated intrahepatic ducts. There was no mention of a choledocolith on both the CTA and US reports. On physical exam, the patient was tender in the epigastric region. It was decided to proceed with an endoscopic retrograde pancreatography (ERCP) for further evaluation. During the procedure, a metallic object was noted on fluoroscopy and appeared to be located in the center of a mobile choledocolith. Additional stationary metallic objects consistent with surgical clips were seen related to the past cholecystectomy. A sphincterotomy and multiple balloon sweeps were performed with successful extraction of biliary sludge and a large choledocolith. The choledocolith was retrieved and found to have a surgical clip in the center. Following the ERCP, the patient's symptoms and liver enzymes improved. The patient was seen in the clinic at follow up with complete normalization of her liver enzymes and was asymptomatic. This case demonstrates a rare complication that can occur years after a cholecystectomy related to surgical clip migration into the common bile duct which then serves as a nidus for choledocolith formation. Therefore, it is important to understand the mechanism of how a surgical clip is able to migrate into the common bile duct so that late presentations of stone formation can be more proactively identified and addressed or even prevented in the future.1340_A.tif Figure 1: Linear metallic object in the distal common bile duct1340_B.tif Figure 2: Mobile choledocolith with centrally located linear dense object1340_C.tif Figure 3: Extracted choledocolith with surgical clip