Intrahepatic Biliary Dilatation Research Articles

Computed Tomographic Findings in Lemmel Syndrome: A Rare Cause of Obstructive Jaundice.

Obstructive jaundice resulting from a duodenal diverticulum is known as Lemmel syndrome. Lemmel syndrome should be included in the differential diagnosis in patients presenting with obstructive jaundice in the absence of choledocholithiasis, mass, or a stricture. To describe the computed tomography (CT) findings in patients with Lemmel syndrome. Eighteen cases with incidentally detected peri-ampullary duodenal diverticula were retrospectively reviewed over a period of one year. Out of these, eight patients who presented to the hospital with jaundice with or without acute abdominal symptoms were included in the study. CT scans were acquired using the Philips Incisive CT 128 Slice machine, and the findings were recorded. Jaundice was the presenting complaint in eight patients (100%) followed by fever in four (50%) patients and abdominal pain in three (37.5%) patients. Vomiting was observed in one patient (12.5%). All patients had evidence of obstructive biliopathy on baseline blood investigations. On CT, all patients had periampullary duodenal diverticula arising from the second part of the duodenum along with dilated common bile duct and mild intrahepatic bilobar biliary dilatation. Mild wall thickening and/or enhancement of bile ducts were seen in four patients, while evolving cholangitic abscesses were seen in two patients. Findings of acute pancreatitis and pneumobilia were observed in each patient. Lemmel syndrome should be suspected in a patient with features of obstructive jaundice when choledocholithiasis, pancreatobiliary tumors, and strictures have been ruled out. Computed tomography remains the preferred imaging modality because of its easy accessibility, less time consumption, and non-invasive nature.

Mass Forming Intrahepatic Cholangiocarcinoma- An Imaging Mimicker Of Hepato-Cellular Carcinoma And Abscess

Cholangiocarcinoma (CCA) is the second most common primary malignancy of liver. Intrahepatic mass-forming (IMCC) is one of the three patterns of cholangio carcinoma. On imaging IMCC usually presents as irregularly outlined homogenous mass with low attenuation and minimal peripheral enhancement, mild intrahepatic biliary dilatation (IHBD). Sometimes, biliary dilatation, capsular retraction and satellite nodules are other features helping in diagnosis of IMCC. Out of 56 cases we evaluated for liver neoplasms on MDCT, the most common lesions encountered are Hepatocellular carcinoma (n=47, 83.9%) followed by Cholangiocarcinoma. All the 5 cases of IMCC are larger tumors with homogenously hypodense on NECT. On contrast there was rim enhancement in most of these cases. One case had delayed wash out mimicking HCC. Capsular retraction indicated the diagnosis of IMCC. The 5TH case was confused with abscess but absence of double rim sign indicated alternate diagnosis. Hence absence of double rim and washout in delayed images differentiate IMCC from liver abscess and HCC.

Antenatally Detected Choledochal Malformation: What Are We Waiting For?

IntroductionAn increasing proportion of congenital choledochal malformation (CCM) are being detected on antenatal ultrasound. However, the actual timing of its surgical correction remains controversial with some series showing an excess of complications the earlier the operation. The aim of this study was to characterize the pathophysiological aspects of this cohort from the perspective of age at surgery in order to inform a more rational basis for clinical decision-making. MethodsWe analysed a prospective database of CCM acquired over a 26-year period (Jan. 1997 to Dec. 2022) for patient demography; details of pre- and post-natal imaging; age at surgical intervention; operative complications (Clavien-Dindo classification) and outcome. Data are quoted as median (IQR). All comparisons are non-parametric. A P value of 0.05 was accepted as significant. ResultsThere were 58 (72% female) children with an antenatally-detected CCM from a total of 265 (21.8%) in the series. These were classified as Type 1C (n = 47; 81%), type 4C (n = 3; 5%) and Type 5 (n = 8; 14%). There were no Type 1F lesions in this cohort. Median age at surgery was 113 (IQR 57–198) days.Postnatal cyst (US) size varied from 12 to 130 mm but there was little evidence of a relationship between this and post-natal liver biochemistry (e.g. bilirubin rS = 0.01, P = 0.44; AST rS = 0.14, P = 0.19). Choledochal pressure measurements (n = 46) showed resting pressures of 12 (9–21) mmHg with no significant correlation with age (P = 0.4) or aspartate aminotransferase (P = 0.2) or γ-glutamyl transferase (P = 0.06).The cohort was divided into 2 groups (Early and Late) based on the median age at surgery (all open) (113 days). Biliary obstruction was more common in the Early group (10 vs. 2; P = 0.01). Two perforations occurred, both in the Early group.With a deliberate policy of regular ultrasound-based follow-up we have seen no anastomotic complications (leak, stenosis, persistent intrahepatic biliary dilatation or stones) or post-operative cholangitis in any child [median follow-up 3.42 (1.30–8.05) years]. ConclusionsThis is one of the largest series documenting the outcomes of antenatally-detected CCMs, certainly in Europe and North America. Such lesions are invariably cystic in nature, and either Types 1C, 4C or 5. The absence of complications using a policy of early intervention (where possible) in experienced hepatobiliary units was shown. Evidence LevelII.

Study of Intrahepatic Biliary Architecture in Patients Operated for Extrahepatic Biliary Atresia using Magnetic Resonance Cholangiopancreatography.

The aim is to study intrahepatic biliary architecture in patients following Kasai's portoenterostomy for extrahepatic biliary atresia using magnetic resonance cholangiopancreatography (MRCP). It is a prospective observational study in a cohort of patients who have survived with a complete jaundice-free period for at least 1 year. MRCP was done to look for various intrahepatic architectural changes during their last visit. Findings were correlated with liver functions and growth and development. Twenty-one patients were included in the study. The male-to-female sex ratio was 1.6:1, the median age at surgery was 75 days (18-140 days), and the median age at magnetic resonance imaging (MRI) was 4 years (18 months-18 years). More than 2 years of follow-up was in seven patients. Left lobe hypertrophy was observed in six patients, right lobe hypertrophy was in three patients, intrahepatic biliary tract dilatation was in five patients, and altered signal intensity between intrahepatic ducts was seen in five patients. In addition, hypointense foci in the spleen were seen in one patient. Twelve children had normal weight for their age, ten children had the normal height for their age, and gamma-glutamyl transferase was elevated in all children. Only four children had a history of fever and jaundice. The patients who survive after successful surgical intervention have near-normal liver gross architecture as on MRI/MRCP. Occasional findings of dilatation and lobar atrophy/hypertrophy are possible, especially in those with a history of cholangitis. The survivors also have acceptable growth parameters and normal liver functions.

THU369 A Rare Case Of IgG4-related Autoimmune Pancreatitis And Diabetes Mellitus

Abstract Disclosure: M. Zahid: None. J. Shakil: None. Introduction: IgG4 related disease (IgG4-RD) is a rare multiorgan inflammatory condition characterized by dense lymphoplasmacytic infiltrates, presence of IgG4+ plasma cells and fibrosis. Here we present a case of young male with painless jaundice and hyperglycemia diagnosed with IgG4 related autoimmune pancreatitis and DM. Clinical Case: 46-year-old male with no significant history presented to outpatient clinic for pruritis and dark-colored urine of 2-week onset. He reported 17-pounds weight loss in the preceding month. While undergoing outpatient workup, patient developed epigastric pain prompting visit to the hospital. On examination, patient was afebrile, tachycardiac, icteric and had epigastric tenderness. Labs revealed normal electrolytes and renal function. LFTs were significant for total protein 7.6 g/dl (n=6.3-8.3 g/dl), AST 77 U/L (n = 10-50 U/L), ALT 127 U/L (n = 5-50 U/L), alkaline phosphatase 458 U/L (n = 40-129 U/L) and direct bilirubin >10.0 (n = 0-0.3 mg/dl). Lipase was 99 U/L (n - 13-60 U/L), blood glucose was 292 mg/dl and A1C was 6.5% (n = 4.0-5.6%). US abdomen revealed diffuse gallbladder wall thickening with moderate sludge and intrahepatic and extrahepatic distal biliary obstruction and dilatation. MRCP showed common bile duct and intrahepatic biliary ductal dilation. Pancreas was diffusely irregular with areas of hemorrhagic pancreatitis. ERCP showed dilatation of upper and middle third of main bile duct, left and right hepatic ducts and all intrahepatic branches with a stricture in distal bile duct. Biliary sphincterotomy and placement of temporary plastic biliary stent was performed and ampullary biopsies were taken. IgG4 level was checked due to suspicion for IgG4-RD and was 293 mg/dl (n: 1-123 mg/dl). Pathology revealed mucosa with epithelial reactive changes. CD138 stain showed several mature plasma cells within the inflammatory infiltrate. In addition, immunostain for IgG4 shows several IgG4 positive plasma cells forming small clusters confirming diagnosis of IgG4-RD. Post procedure, his abdominal pain and LFTs started improving. Patient was started on prednisone for IgG4-RD and insulin for persistent hyperglycemia. His prednisone dose was later tapered after starting methotrexate. Patient’s most recent LFTs have normalized except mildly elevated ALT and A1C improved to 6.2%. Conclusion: Autoimmune pancreatitis and cholangiopathy are the pancreatobiliary manifestation of IgG4-RD and most often present as painless jaundice mimicking pancreatic cancer. Endocrine insufficiency usually manifests as diabetes mellitus/pre-diabetes and may also lead to autoimmune hypophysitis and Reidel’s fibrosis. Other organ systems that may also get involved include lacrimal and salivary glands, lungs, kidneys, nervous system, and lymphatic/vascular system. Early recognition and ongoing surveillance are key to appropriate management of this condition. Presentation: Thursday, June 15, 2023

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.

Recurrent Choledocholithiasis: Unveiling the Diagnostic Challenges of Profound Transaminitis Post Cholecystectomy.

BACKGROUND Profound transaminitis (>1000 international units per liter [IU/L]) is typically associated with ischemic and viral or toxic hepatitis. Pancreaticobiliary causes are less likely to be included in the workup, especially in patients who have undergone cholecystectomy. We present a case of recurrent choledocholithiasis in a 52-year-old woman 7 years after cholecystectomy, presenting with severe transaminitis, illustrating the diagnostic challenges of this presentation. CASE REPORT A 52-year-old woman presented to the Emergency Department (ED) with acute upper abdominal pain. Computed tomography (CT) of the abdomen without contrast showed no abnormalities and mild common bile duct (CBD) dilation was noted on ultrasound (US) abdomen. Laboratory studies were significant for elevated transaminases greater than 1000 units/L and alkaline phosphatase (ALP) greater than 200 units/L. She was diagnosed with acute hepatitis of unknown etiology without undergoing further investigation of the biliary tract and was discharged after improvement with supportive therapy. She returned 4 months later with similar symptoms and laboratory findings, but with more CBD dilation and intrahepatic biliary dilation on CT and US. Endoscopic retrograde cholangiopancreatography (ERCP) was performed, and multiple stones and sludge were removed from the CBD. CONCLUSIONS This report has shown that pancreaticobiliary causes should be included in the workup of severe transaminitis, even in patients with a remote history of cholecystectomy. Failure to do so may subject patients to extensive, unnecessary workup and delay correct management.

An Unusual Variant of Anomalous Pancreaticobiliary Junction.

A 27-year-old previously healthy African American female presented to the Emergency Department with an acute onset of epigastric abdominal pain and nausea. Laboratory studies proved unremarkable. CT scan demonstrated intrahepatic and extrahepatic biliary ductal dilation with possible stones within the common bile duct. The patient was discharged with a surgery follow-up appointment. Laparoscopic cholecystectomy with intraoperative cholangiography was performed 3weeks later due to concern for choledocholithiasis. The intraoperative cholangiogram showed multiple abnormalities, concerning for an infectious or inflammatory process. Magnetic resonance cholangiopancreatography (MRCP) demonstrated a suspected anomalous pancreaticobiliary junction and cystic lesion near the pancreatic head. Endoscopic retrograde cholangiopancreatography (ERCP) for cholangioscopy showed normal-appearing pancreaticobiliary mucosa with 3 tributaries directly from the pancreas entering the bile duct and an ansa orientation to the pancreatic duct. Biopsies of the mucosa were benign. Annual MRCP and MRI to assess for findings concerning for neoplasm given the anomalous pancreaticobiliary junction were recommended.

Effectiveness of ultrasound screening in right upper quadrant pain: A comparative study in a basic emergency service.

The use of ultrasound screening is primarily facilitated by point-of-care ultrasound (POCUS) and its integration into healthcare systems is a result of the versatility of this imaging technique. This study intends to compare the accuracy and pertinence of sonographic findings obtained by a sonographer in a Basic Emergency Service (BES) with that of radiologists at referral hospital (RH) in Portugal. Twenty patients with right upper quadrant (RUQ) pain and suspected cholecystitis or biliary pathology underwent sonography screening using POCUS in the BES. They were then forwarded to the RH where a radiologist performed a conventional ultrasound exam on the same patients. The results of both exams were compared to determine if the findings obtained in the BES were confirmed by the radiologist in the RH. In our sample, 60% of cases were related to biliary pathology, 20% were liver-related, 10% had hepatopancreatic biliary etiology, and 10% had unknown etiology. A strong association between the sonographic findings in the BES and the RH was found in the variables "Sonographic Murphy sign" (V = 0.859; p = 0.001), "Cholelithiasis/Gallbladder sludge" (V = 0.840; p = 0.001), and "Intrahepatic biliary tract dilatation" (V = 0.717; p = 0.006). Adequate measures of agreement between the findings of the radiographer and radiologist were obtained for the "Sonographic Murphy sign" (k = 0.664; p = 0.001) and the presence of "Cholelithiasis/Gallbladder sludge" (k = 0.712; p = 0.000). Major biliary abnormalities were detected in patients with RUQ pain in BES using sonography. The correlation between the sonographic findings obtained by the sonographers at BES and those obtained by radiologists at the RH in Portugal was strong, showing that POCUS screening could be extended to other similar settings; however, more studies are needed.

Analyse des différences cliniques et radiologiques entre abcès hépatiques amibiens et à pyogènes à partir d’une étude cas-témoin

IntroductionAmoebic liver abscess (ALA) is the fourth cause of mortality by parasitic infection. This study aimed to assess clinical, radiological and therapeutic characteristics of patients admitted for amoebic liver abscess compared to pyogenic abscess in a French digestive tertiary care-centre. Material and methodThe charts of patients hospitalized for a liver abscess between 2010 and 2020 were retrospectively assessed then separated in two groups: amoebic liver abscess and pyogenic liver abscess from portal underlying cause. Clinical and radiological data were collected for univariate comparison. ResultsTwenty-one patients were hospitalized during the time of the study for ALA, and 21 patients for pyogenic liver abscess with a portal mechanism. All patients hospitalized for ALA lived in and/or had travelled recently in an endemic area. In comparison with patients hospitalized for pyogenic abscess, patients admitted for ALA were younger (44years old vs. 63years old, P<0.001), had less comorbidities (5% vs. 43% of patients with at least one comorbidity, P<0.01), a longer median duration of symptoms (10days vs. 3days, P=0.015), abdominal pain (86% vs. 52%, P=0.019), and a slighter leucocytosis (9600G/L vs. 15,500G/L, P=0.041) were more frequent. On the abdominal tomodensitometry, density of ALA was higher (34 vs. 25 UH, P<0.01), associated with a focal intra-hepatic biliary dilatation and less often multiloculated. ConclusionWhile rare in western countries, amoebic liver abscess care should not be underestimated. The presence of a solitary liver abscess of intermediate density on computed tomography, occurring on a patient returning from an endemic zone should lead the physician to a possible diagnosis of ALA.

Multidisciplinary management in the treatment of intrahepatic cholangiocarcinoma.

Multidisciplinary management in the treatment of intrahepatic cholangiocarcinoma.

Natural history and prognosis of pediatric PSC with updates on management.

Natural history and prognosis of pediatric PSC with updates on management.

PRIMARY SCLEROSING CHOLANGITIS AND AUTOIMMUNE HEPATITIS: OVERLAPPING CHALLENGES IN DIAGNOSIS AND TREATMENT

Abstract Overlap syndromes (OS) describe the simultaneous presentation of more than one immune-mediated liver disease in a singular patient, and present unique challenges both in diagnosis and treatment. Here we report a case of primary sclerosing cholangitis (PSC) and autoimmune hepatitis (AIH) OS, ultimately treated with Vancomycin. A 30-year-old female with known ulcerative pancolitis (UC) and alcoholic cirrhosis in the setting of AIH presented to the ED with severe abdominal pain, bloody diarrhea, and bilious emesis. Initial labs showed ESR 48 mm/hr, CRP 32.97 mg/L, fecal calprotectin 1131.2 µg/mg, AST 45 IU/L, and total protein 9.1 g/dL. Clostridium Difficile and stool studies were negative. Patient was admitted with concern for acute UC flare. CT scan showed mild worsening of intrahepatic and extrahepatic biliary ductal dilatation suspicious for PSC, and a cirrhotic liver with signs of portal hypertension and splenomegaly (Fig. 1a). Endoscopy revealed multiple non-bleeding duodenal ulcers and grade II esophageal varices. Colonoscopy found moderately active UC Mayo score 2, worsened from prior exam. MRCP was significant for cirrhotic liver morphology with nodular surface contour, and intrahepatic and extrahepatic biliary dilatation with beading and irregularity of the intrahepatic biliary tree and cystic duct, consistent with PSC (Fig. 1b). She was diagnosed with PSC-AIH OS and underwent treatment with Prednisone, Azathioprine, and Ustekinumab with minimal improvement. Only after the addition of Vancomycin was there normalization of biochemical markers and clinical status. Autoimmune liver disease is generally categorized into hepatic (AIH) or cholestatic (PSC and PBC) predominance. PSC is known to be associated with IBD, specifically UC. It is hypothesized that PSC evolves in UC patients due to uptake into the enterohepatic circulation of proinflammatory cells from the colon; these agents typically concentrate in the biliary system where they ultimately cause the biliary ductal damage frequently seen with PSC. By targeting enteric bacteria, Vancomycin decreases the absorption of these inflammatory biliary toxins. For PSC patients with UC and co-occurring AIH, the addition of Vancomycin could significantly reduce mortality and disease burden. Several case reports and one pilot study have investigated Vancomycin’s role in the treatment of PSC. However, it remains a vastly underutilized therapy, and its role specifically in PSC-AIH OS has not been previously reported. There remains an evident need for a large double blind randomized placebo-controlled clinical trial to further support the addition of Vancomycin to the standard treatment regimen for PSC, and to establish clear parameters on effective dosage and length of treatment. In the meantime, clinicians should be aware of the possible use of Vancomycin to treat patients with PSC +/- AIH and IBD. Figure 1a: CT abdomen pelvis with IV contrast highlighting stigmata of cirrhosis with portal hypertension and splenomegaly. Image also shows worsening of intrahepatic and extrahepatic biliary ductal dilatation in the setting of PSC given the patient’s underlying history of ulcerative colitis. Figure 1b: MRCP showing beading and irregularity of the intrahepatic biliary tree and cystic duct, in keeping with known history of primary sclerosing cholangitis. This image also highlights the dilation of the intrahepatic and extrahepatic biliary system.

Histological Changes in Portal Cavernoma Cholangiopathy.

Portal cavernoma cholangiopathy (PCC)' refers to abnormalities of the extrahepatic and intrahepatic bile ducts in patients with portal hypertension. Although there is data on clinical and imaging aspects of PCC, the description of liver pathology has been strikingly deficient. The purpose of this study was to examine the histopathological characteristics of PCC. A retrospective study of patients clinically diagnosed with extrahepatic portal vein obstruction (EHPVO) with portal cavernoma cholangiopathy, was conducted. Vascular anatomy was characterized by computerized tomographic angiography, and endoscopic retrograde cholangiography (ERC) and magnetic resonance cholangiography (MRC) were used to characterize the biliary anatomy. Histological features were analyzed by two hepatopathologists in a blinded manner, with mutual discussion to resolve any discrepancies. A total of 50 patients with portal cavernoma cholangiopathy were included in the study. The mean age of the patients was 26.2 ± 11.6 years. Radiologically, bilobar intrahepatic biliary dilatation was seen in 98% with common bile duct abnormality in 100% of patients, along with extrinsic ductal impressions in 77 % of cases. Liver tests were deranged total bilirubin 1.5 mg/dL (IQR 0.8-2.4) and alkaline phosphatase 109.5 IU/L (IQR 70-193). Histologically; dilated multiple portal venous channels (72%), hepatic artery thickening (70%). The presence of aberrant vascular channels around portal tracts (54%), elastosis of portal veins (50%), and bile ductular reaction in (44%) were the other prominent findings. A 12% of cases show focal thin bridges. Advanced fibrosis was not seen in any of the cases. One-fourth of the cases showed concomitant minimal to mild hepatocyte steatosis. Histologically, intrahepatic portal vein and portal tract abnormalities were noted in cases with portal cavernoma cholangiopathy, associated with mild derangement of liver tests.

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

Acalculous variant of Mirizzi syndrome: Imaging and clinical characteristics

PurposeMirizzi Syndrome is a rare disease that causes biliary obstruction in the setting of an impacted stone in the gallbladder neck or Hartmann's Pouch which exerts mass effect on the common duct; however, we have noticed inflammatory biliary narrowing in the absence of an offending gallstone in the setting of acute cholecystitis. The purpose of this study is to report the clinical and MRCP findings in a series of 10 patients with this variant of Mirizzi Syndrome. Materials and methodsA search of our institution's PACS and electronic medical record identified 10 patients with a diagnosis of acute cholecystitis and narrowing of the common duct on imaging in the absence of an impacted gallstone. Imaging and clinical findings were confirmed by two board-certified abdominal radiologists. ResultsAll patients presented with abdominal pain and an average elevated total bilirubin of 3.0 mg/dL. Seven patients had MRCP findings of complete narrowing of the CBD. Nine patients had intrahepatic biliary ductal dilation. All nine patients with gadoliniumenhanced MRCP displayed biliary wall thickening with enhancement adjacent to the gallbladder. Nine patients underwent cholecystectomy, one patient underwent percutaneous cholecystostomy. Average bilirubin upon discharge was within normal limits at 0.9 mg/dL after intervention. Two patients had follow-up MRCP showing resolution of biliary narrowing. ConclusionA variant of Mirizzi Syndrome occurs in the absence of an offending gallstone in the gallbladder neck or cystic duct to explain the biliary narrowing. We postulate that acute cholecystitis can cause a local inflammatory narrowing resulting in biliary obstruction.

Concurrence of novel mutations causing Gilbert’s and Dubin–Johnson syndrome with poor clinical outcomes in a Han Chinese family

Dual-hereditary jaundice (Dubin–Johnson syndrome (DJS) and Gilbert’s syndrome (GS)) is a rare clinical entity resulting from defects of the ATP binding cassette subfamily C member 2 (ABCC2) and UDP glucuronosyltransferase family 1 member A1 (UGT1A1) genes with autosomal recessive inheritance. In this study, we aimed to investigate the mutation profiles and characterize the phenotypes in a Han Chinese family with DJS and GS. Genetic screening for variants in the ABCC2 and UGT1A1, immunohistochemistry for expression of ABCC2, and histopathological examination were carried out. The proband and his brother had unconjugated and conjugated hyperbilirubinemia after birth. The proband’s sister had only conjugated hyperbilirubinemia after birth. The proband developed into pleural effusions and ascites, pericardial thickening, intrahepatic and extrahepatic biliary duct dilatation, and enlarged gallbladder at age 50. Hepatocellular carcinoma occurred in the proband’s brother at age 46. Seven compound defects of the ABCC2 gene [c.2414delG, p.(Ile1489Gly), p.(Thr1490Pro), and p.(Ile1491Gln)] and the UGT1A1 gene (c.-3279T>G, p.(Gly71Arg), and p.(Pro451Leu)) were identified in family members. Accumulation of pigment in hepatocytes characteristic of that in DJS was present in the proband and his brother. Expression of ABCC2 protein was markedly diminished in the patient’s liver. Our results show a different genetic profile of DJS and GS in a Han Chinese family, indicating a more complex pattern of dual-hereditary jaundice among different populations. The present study illuminates the underpinnings of DJS and GS and extends the mutation profiles and phenotypes of these two syndromes in dual-hereditary jaundice.

S3033 HIV-AIDS: A Rare Cause of Cholestatic Cholangiopathy

Introduction: HIV/AIDS has been shown to affect hepatic parenchyma and biliary tree leading to inflammation and biliary strictures. With the advent of highly active antiretroviral therapy (HAART), it is rarely encountered, except in cases of resistance or medication non-compliance. Case Description/Methods: A 48-year-old man with history of HIV and poor compliance with HAART presented with abdominal pain, nausea and diarrhea. Physical exam showed scleral icterus and mild abdominal tenderness, with labs showing a T.bili. 16.9, AST 212, ALT 133, and ALP 3885. MRCP revealed intrahepatic biliary ductal dilatation of both lobes of liver and mild dilatation of the proximal extrahepatic CBD (9 mm) without cholelithiasis, biliary sludge or ductal stone suggesting nonspecific cholestasis. ERCP was performed which revealed papillary stenosis and moderately enlarged CBD (12 mm). Irregularities were noted in bilateral intrahepatic branches but not in ducts. A pancreatic and biliary sphincterotomy was performed. With minimal decrease in T.bili. and ALP, repeat MRCP was performed 3 months later showing persistent intrahepatic biliary ductal dilatation with normal CBD. Repeat ERCP showed patent prior sphincterotomy and the biliary tree was swept with nothing found. Further workup revealed a CD4 count of 17 and serology positive for cryptosporidium antigen. A diagnosis of HIV-cholangiopathy was made and the patient was restarted on HAART and nitazoxanide with gradual decline in T.bili and ALP. (Figure) Discussion: HIV/AIDS-cholangiopathy is a rarely encountered biliary syndrome, usually manifesting in severely immunosuppressed AIDS patients. Several opportunistic pathogens have been implicated in its pathogenesis with Cryptosporidium parvum being the most commonly associated pathogen. C. parvum predominantly affecting the biliary tract leading to apoptotic cell death of the cholangiocytes. Biliary involvement has been described in four cholangiographic patterns – papillary stenosis, extrahepatic strictures, intra and extrahepatic sclerosing lesions, and acalculous cholecystitis. Diagnosis can be established usually by MRCP or ERCP which shows papillary stenosis with intrahepatic ductal dilatation. Alternating multifocal intrahepatic strictures can also be seen giving a beaded appearance. The cornerstone of management involves the management of opportunistic infections with the initiation of HAART. The prognosis is usually poor due to advanced stages of immunosuppression and multiple opportunistic infections.Figure 1.: CT Abdomen pelvis showing intrahepatic biliary ductal dilation.

S1822 Recurrent Pancreatitis Secondary to Common Channel Volvulus Through Petersen’s Space Defect in a Patient With Roux-en-Y Bypass

Introduction: Petersen’s space hernia is an internal hernia that can occur after Roux-en-Y gastrojejunostomy. The intestinal loops herniate through a defect between the retroperitoneum, the transverse mesocolon and the small bowel limbs. We present a case of recurrent pancreatitis in a patient with Roux-En-Y bypass found to have common channel hernia through a Petersen’s space defect. Case Description/Methods: We present the case of a 34-year-old female with a history of Roux-en-Y surgery in 2018 and subsequent recurrent pancreatitis who presented to the emergency department with a chief complaint of severe epigastric and left lower quadrant abdominal pain associated with hematemesis. Patient reported 3 episodes of pancreatitis within 1 year previously. CT abdomen and pelvis showed mildly dilated common bile duct and intrahepatic biliary dilatation with no evidence of pancreatitis. Significant lab work included elevated lipase at 184 U/L. Patient was admitted to the medical service. Gallbladder ultrasound revealed no evidence of cholelithiasis, a prominent CBD of 9 mm and redemonstrated mild intrahepatic biliary dilatation. MRCP revealed a mesenteric swirl in the mid abdomen which was suspicious for an internal hernia in the setting of antecolic Roux-en-Y gastric bypass. It also showed focally dilated intrahepatic with underlying segmental atrophy. General surgery consultation was sought, with eventual plans for diagnostic laparoscopy after ruling out marginal ulcer via EGD. An EGD was performed which did not show evidence of marginal ulcer. Patient then underwent diagnostic laparoscopy which revealed a 360-degree volvulus of the common channel through a Petersen’s space defect; this was carefully reduced, and the Petersen’s space defect was closed. Patient also underwent laparoscopic cholecystectomy. Patient did not have any further episodes of pancreatitis after surgery. Discussion: This case demonstrates recurrent pancreatitis in a patient with a history of Roux-En-Y bypass found to have a common channel volvulus through a Petersen’s space defect. It is our understanding that the volvulus likely caused compression of the pancreaticobiliary system, thus causing recurrent pancreatitis. Reduction of the volvulus and closing of the Petersen’s defect resulted in complete resolution of recurrent pancreatitis in the patient.

S3234 A Cancer Encasing the Porta Hepatis: An Aggressive Intra-Abdominal B Cell Lymphoma Presenting as a Post-Obstructive Jaundice With Reactivation of Hepatitis C Virus

Introduction: Hepatitis C virus (HCV) is a lymphotropic virus that promotes HCV-mediated B-cell proliferation and transformation to Non-Hodgkin Lymphoma (NHL). Here we present a case of reactivation of HCV presenting as an aggressive intra-abdominal B cell lymphoma. Case Description/Methods: A 68-years-old-male with history of eradicated HCV with recombinant interferon-alfa and resolved HBV infection presented with painless jaundice and pruritus. CT abdomen revealed a 9.6 x 11.5 x 9.5 cm heterogeneous mass causing CBD and intrahepatic biliary duct dilation. Labs revealed reactivation of HCV infection with worsening of liver function tests with total bilirubin of >40mg/dl. FNA biopsy confirmed mature B-cell lymphoma. Given persistent worsening jaundice despite biliary stent, trial of high dose steroid was initiated to improve lymphoma burden in order to get a window to initiate chemotherapy. After tumor board discussion, he received radiation therapy for tumor shrinkage for this aggressive lymphoma. Prior to initiation of planned R-CHOP chemotherapy, he developed severe sepsis with hepatic abscess. Patient and family opted for hospice care and comfort care was initiated. Discussion: Chronic HCV is a fast-growing epidemic in the U.S.A which carries an enormous threat to general population and healthcare. HCV becomes chronic in >70% of infected patients with risk for cirrhosis, hepatocellular carcinoma and lymphoma. HCV can cause NHL via lymphocyte proliferation from viral antigenic stimulation, HCV replication inside B-cell and mutation via B-cell damage. Among patient who achieve sustained viral response (SVR), reactivation occurs in 1%, 11% and 15% among low-risk monoinfected HCV, high-risk monoinfected HCV and HIV/HCV coinfected group respectively. Treatment of HCV with resolution of NHL has been reported and there is some literature with sequential chemotherapy followed by antiviral therapy (AVT) showing promising results in HCV-NHL cases. Concurrent use of AVT and chemotherapy is primarily discouraged due to hematological toxicity. More evidence and studies need to be explored for such aggressive HCV-NHL management. Patient with SVR should have at least an annual testing and periodic follow-up to access for HCV reactivation or reinfection. Hepatitis C education regarding risk factors reduction and transmission prevention should be encouraged at every healthcare level and in general community and only then we can achieve WHO aim of eradication by 2030.