Evidence Of Biliary Obstruction Research Articles

The Majority of Patients Who Undergo ERCP When Large Duct Obstruction Is Evident on Liver Biopsy Have Biliary Findings Amenable to Endoscopic Intervention.

(1) Background: Abnormal liver function tests are commonly encountered in clinical practice, often leading to additional workup to determine the underlying etiology of these abnormal laboratory studies. As part of this evaluation, if less invasive imaging studies are performed and are without evidence of biliary obstruction, liver biopsy may be performed, and the finding of large duct obstruction on liver biopsy is commonly encountered. The utility of endoscopic retrograde cholangiopancreatography (ERCP) for evaluation and management of possible biliary obstruction in patients with large duct obstruction on liver biopsy has not been studied to date. (2) Methods: To assess the utility of ERCP in patients with large bile duct obstruction on liver biopsy, we retrospectively evaluated patients with large duct obstruction on liver biopsy from 2010-2019 at our tertiary care and transplant center. Demographic and clinical characteristics were evaluated for all patients, with sub-group analysis for patients who underwent ERCP and those who had intervenable findings at the time of ERCP. Descriptive statistics with proportions, means, and standard deviations were performed for demographics and clinical variables using absolute standardized difference. (3) Results: During the study period, 189 liver biopsies with evidence of large duct obstruction were performed. After exclusion criteria were applied, 166 unique patients were eligible for the study. Ninety-one patients with evidence of large duct obstruction on liver biopsy underwent ERCP and 75 did not. Of the 91 patients who underwent ERCP, 76 patients (84%) had an intervenable finding at ERCP. Patients who underwent ERCP were overall more likely to have had a liver transplant (65% ASD 0.63), have previously undergone cholecystectomy (80%, ASD 0.56), and be immunocompromised (80%, ASD 0.56). (4) Conclusions: ERCP is high yield when large duct obstruction is apparent on liver biopsy, with the majority of patients (84%) who undergo ERCP in this clinical context having a biliary finding necessitating therapeutic endoscopic intervention.

A Comparative Study of Endoscopic Ultrasound Fine-Needle Aspiration (EUS-FNA) and Endoscopic Retrograde Cholangiopancreatography (ERCP)-Based Brush Cytology for Tissue Diagnosis in Malignant Biliary Obstruction.

Background and objective Patients with suspected malignant biliary strictures frequently undergo endoscopic retrograde cholangiopancreatography (ERCP)-based brush cytology and endoscopic ultrasound (EUS)-guided fine-needle aspiration (FNA) for establishing the diagnosis. The outcomes of these tests aid in the further management of the patient. A comparison of these two modalities in establishing the diagnosis is seldom reported. In light of this, we aimed tocompare the diagnostic efficacy between ERCP-based brush cytology and EUS-FNA for tissue diagnosis in malignant biliary obstruction. Our study involved a retrospective audit of all patients admitted to the Vydehi Institute of Medical Sciences and Research Centre for EUS and ERCP from 2015 to 2019. Methodology A Comparative study was conducted in the Department of Medical Gastroenterology at the VydehiInstitute of Medical Sciences and Research Centre over a five-year period. A total of 77 subjects who presentedduring the study period with biliary obstruction based on clinical presentation with altered liver function test in an obstructive pattern and evidence of biliary obstruction in the form of stricture or pancreaticobiliary mass on cross-sectional imaging were included in the study. All the patients included in the study underwent EUS and ERCP. Results The majority of the patientsin the study were in the fifth decade of life with a slight female predominance. The most common CT finding was a periampullary mass with common bile duct (CBD) stricture (59.7%). In the study, EUS-FNA was more sensitive than ERCP-based tissue sampling. The overall sensitivity was 90.63% for EUS-FNA and 65.63% for ERCP sampling. EUS-FNA was found to have diagnostic accuracy of 92.63% in comparison to 71.43% for brush cytology. Conclusions Based on our findings, EUS-FNA is superior to ERCP-based tissue sampling with excellent sensitivity and diagnostic accuracy. Performing EUS before ERCP in allpatients with suspected malignant biliary obstruction would definitely improve diagnostic accuracy and thereby help in the management of such cases.

Primary Sclerosing Cholangitis in a Paediatric Patient: An Atypical Presentation

Primary sclerosing cholangitis (PSC) is an autoimmune chronic liver disease that studies have shown is rare in children. Here, a challenging case of PSC in a 13-year-old male, without preceding manifestations of inflammatory bowel disease and with evidence of biliary obstruction, is reported. The patient presented with progressive scleral icterus; their total bilirubin and alkaline phosphatase levels were raised, with negative autoimmune work-up, and an ultrasound scan of their abdomen was unremarkable. Magnetic resonance cholangiopancreatography revealed marked dilatation of the intra- and extrahepatic bile ducts, with strictures in the hepatic duct and proximal common bile duct (CBD). Endoscopic retrograde cholangiopancreatography revealed a very narrow CBD with high-grade severe biliary stricture at the common hepatic duct. A cholangiogram revealed the beaded appearance of the intrahepatic ducts, and a left percutaneous external biliary drainage tube was placed. Overall, the findings were suggestive of PSC with high-grade CBD strictures. This case is unique due to the absence of preceding clinical manifestations of inflammatory bowel disease and predominantly obstructive symptoms at the time of presentation, which is highly unusual in the paediatric population.

English

BACKGROUND Intra-Operative Cholangiogram (IOC) is a procedure carried out during cholecystectomy with the primary objective of clearly delineating the biliary anatomy. Over decades, routine IOC became selective IOC and now it is being overtaken by less invasive investigations like MRCP and EUS. Role of IOC remains only to intraoperatively confirm or rule out bile duct injury in difficult cases. Laparoscopic IOC is a skilful procedure which requires training and extra added time during laparoscopic cholecystectomy. Once mastered it can be used in many situations for either anatomical reasons or to detect CBD pathology. METHODS All patients getting admitted for laparoscopic cholecystectomy with intermediate risk for choledocholithiasis were enrolled in the study from 2016 to 2019. Procedure was carried out with all necessary consents and precautions. All cases were performed by an experienced GI surgeon and followed up with proper protocol. RESULTS Fifty patients with known intermediate risk for choledocholithiasis underwent laparoscopic cholecystectomy with laparoscopic IOC. Procedure was successfully done in all patients except two, where cystic duct was very thin and cannulation was not possible. Forty-one (82%) patients had deranged liver function test and 9 patients (18%) had history of acute pancreatitis in the past as indications for the procedure. Two patients had dilated CBD (>6 mm) on ultrasound along with deranged liver function tests. An average of 12 minutes was taken to perform the procedure (range: 8 - 15 min). In cases where IOC took longer time was mainly due to technical issues (operability of C-arm). No IOC related complications occurred in any of the patients. Hospital stay was not prolonged in any of the patients due to IOC. None of the patients had any filling defect in CBD. All cases followed till date are asymptomatic. CONCLUSIONS It is a technically feasible procedure that can be performed with limited addition to OT time, minimal failure rate, and complications. All patients with limited criteria for intermediate risk of choledocholithiasis had a normal IOC with no evidence of biliary obstruction in follow up. KEYWORDS Laparoscopic Intraoperative Cholangiogram, Intermediate Risk, CBD Stones

Association between biliary tree manipulation and outcome in dogs undergoing cholecystectomy for gallbladder mucocele: A multi-institutional retrospective study.

To determine whether catheterization of the common bile duct (CBD) is associated with outcome in dogs undergoing cholecystectomy for gallbladder mucocele and to determine whether this association is modified by the catheterization method. Multi-institutional retrospective cohort study. Dogs (n = 252) that underwent cholecystectomy for gallbladder mucocele. Dogs were identified via electronic medical record review at four veterinary teaching hospitals. Baseline dog characteristics, surgical findings, and methods including normograde vs retrograde CBD catheterization, intraoperative outcomes, and postoperative outcomes and complications were recorded. Variables were compared between dogs with and without catheterization. Catheterized dogs had higher American Society of Anesthesiologists scores (P = .04), higher total bilirubin (P = .01), and were more likely to have dilated CBD at the time of surgery (P < .01). Incidence of major and minor intraoperative complications was similar between the two groups. Surgical time was longer for the catheterized group (P = .01). The overall incidence of postoperative complications was similar between the groups; however, postoperative pancreatitis was associated with performing CBD catheterization (P = .01). This association was retained as an independent association in a multivariable model that addressed baseline group differences (P = .04). Likelihood of developing postoperative pancreatitis was not different between normograde and retrograde catheterization (P = .57). Catheterization of the CBD was associated with development of postoperative pancreatitis. This was not influenced by the method of catheterization. The requirement for catheterization of the CBD during open cholecystectomy in dogs should be carefully considered, particularly in dogs without evidence of biliary obstruction because the procedure may induce postoperative pancreatitis.

S1454 Severe Acute Pancreatitis Initial Presentation of COVID-19 Infection

INTRODUCTION: SARS-CoV-2, also known as the COVID-19 has affected more than 7 million people worldwide. While the most scommon initial symptoms are cough and fever, it is known that approximately 50% of patients present with GI related symptoms. We discuss below a case of a COVID-19 patient who initially presented with unexplained severe acute pancreatitis (AP). CASE DESCRIPTION/METHODS: A 71-year-old male presented to the hospital with significant abdominal pain for one day. In the ER, a contrast enhanced CT scan showed peripancreatic inflammation and peripancreatic fluid along the body and tail. The initial lipase was 8400 u/L (73–393 u/L). The patient had no alcohol history or evidence of biliary obstruction. He denied acetaminophen, steroids, or any new medications. The lipid panel was normal along with the LFTs. The BISAP score on admission was 3 for: age >60, BUN >25, and SIRS criteria of fever and leukocytosis. He was treated for severe acute pancreatitis with aggressive IV fluid hydration. On day 4, he had resolution of his abdominal pain and was tolerating a full liquid diet. Despite improvement in his severe acute pancreatitis, the patient developed persistent fevers and increasing oxygenation requirements. A chest X-Ray was then performed with showed infiltrates concerning for pneumonia. He tested positive for COVID-19 and then developed progressive deterioration toward acute hypoxemic respiratory failure. He was transferred to the ICU and intubated on day 19 of his stay and expired a short time after. DISCUSSION: An extensive literature has revealed few cases involving COVID-19 induced AP. While many theories behind this disease process have been discussed, there is no definitive explanation for the pathogenesis. There has been evidence showing ACE2 receptor expression on the pancreas is responsible for damage during a COVID-19 infection. One such study out of China showed 1-2% of non-severe COVID patients and 17% of severe COVID patients exhibited some degree of pancreatic damage. Endothelial damage from the patient's inflammatory response can cause activation of the cytokine cascade. Too much IL-6 activation, as seen with COVID-19, can cause a cytokine storm leading to pancreatitis. While certain viral illnesses have a well-established link to AP, more research needs to be undertaken to determine whether a true relationship exists with the coronavirus. This case highlights that during this global pandemic, unexplained acute pancreatitis may be a presenting symptom of COVID-19 infection.

Survival analysis among unresectable pancreatic adenocarcinoma patients undergoing endoscopic or percutaneous interventions

Most patients with pancreatic cancer are diagnosed at a late stage and are not candidates for surgical resection. Many have jaundice requiring biliary drainage, which can be accomplished using ERCP or percutaneous transhepatic biliary drainage (PTBD). To date, no studies have evaluated the impact of ERCP or PTBD on survival among patients with unresectable pancreatic cancer. The aims of our study were to compare overall survival between patients with unresectable pancreatic cancer receiving ERCP with those receiving PTBD, to compare overall survival between patients who received a biliary intervention (ERCP or PTBD) versus those who received no biliary intervention, and to compare secondary outcomes, such as length of hospital stay and costs, between ERCP and PTBD. We conducted a retrospective cohort study using the Surveillance, Epidemiology, and End Results-Medicare database. Patients with known pancreatic cancer were included if they had a pancreatic head mass and/or evidence of biliary obstruction. We used a time-varying Cox proportional hazards model to estimate overall survival of patients receiving ERCP versus PTBD and overall survival among patients who received a biliary intervention versus no biliary drainage. Secondary outcomes included length of hospital stay, costs, and admissions within 30 days. Of 14,808 patients with unresectable pancreatic cancer, 8898 patients (60.0%) underwent biliary drainage and 5910 patients (39.9%) received no biliary intervention. ERCP accounted for most biliary interventions (8271, 93.0%), whereas 623 patients (7.0%) underwent PTBD. In multivariable analysis, ERCP was associated with reduced mortality compared with PTBD (adjusted hazard ratio [aHR], .67; 95% confidence interval [CI], .60-.75). When ERCP or PTBD was compared with no biliary intervention, both procedures were associated with a survival benefit (aHR, .51 [95% CI, .49-.54] and .53 [95% CI, .48-.59], respectively). Compared with patients receiving PTBD, those who underwent ERCP had shorter mean length of hospital stay (7.0 ± 5.7 days vs 9.6 ± 6.6 days, respectively; P< .001) and lower hospital charges ($54,899.25 vs $75,246.00, P< .001) but no significant difference in hospitalization or 30-day readmissions. ERCP is associated with reduced mortality compared with PTBD in pancreatic cancer patients, highlighting the critical role of ERCP in the management of biliary obstruction from pancreatic cancer.

Cholestasis and disseminated histoplasmosis in a psoriatic patient on infliximab: case report and review of literature

BackgroundHistoplasma capsulatum is the most common endemic mycosis in the United States and frequently presents as an opportunistic infection in immunocompromised hosts. Though liver involvement is common in disseminated histoplasmosis, primary gastrointestinal histoplasmosis of the liver in absence of lung involvement is rare. Similarly, cholestatic granulomatous hepatitis in liver histoplasmosis is rarely seen.Case presentationWe present a rare case of primary gastrointestinal histoplasmosis manifesting with acute granulomatous hepatitis and cholestasis in a 48-year-old female with psoriatic arthritis, receiving methotrexate and infliximab. The epidemiology, risk factors, clinical presentation, diagnosis, and treatment of histoplasmosis is discussed. Furthermore, we review the published cases of biopsy-proven disseminated histoplasmosis with cholestatic jaundice to highlight histoplasmosis involvement in the liver.ConclusionHistoplasmosis should be considered in immunosuppressed patients with fever, chills, abdominal pain and cholestasis with progressive jaundice, particularly in subjects without evidence of biliary obstruction. Future studies are needed to accurately assess the risk of this fungal infection, specifically in patients on immunomodulatory therapy for autoimmune disease.

Utility of ultrasound in acute pancreatitis.

Ultrasound plays an essential role in the initial evaluation of patients with suspected or confirmed acute pancreatitis. In addition to evaluation of the pancreatic parenchyma, ultrasound is used for assessment of the gallbladder, biliary tree, peripancreatic tissues, and regional vascular structures. While enlarged and edematous pancreas are classic sonographic features of acute pancreatitis, the pancreas may appear sonographically normal in the setting of acute pancreatitis. Nonetheless, sonographic evaluation in this setting is valuable because assessment for etiologic factors such as gallstones or evidence of biliary obstruction are best performed with ultrasound. Complications of pancreatitis such as peripancreatic fluid collections, venous thrombosis, or arterial pseudoaneurysm can be identified with careful and focused ultrasound examination. Knowledge of various scanning techniques can help to mitigate some of the commonly encountered barriers to sonographic visualization of the pancreas and right upper quadrant structures. Ultrasound can also be used for guidance of percutaneous treatment such as drainage of fluid collections or pseudoaneurysm thrombosis. Difficulty in differentiating edematous from necrotizing pancreatitis can be mitigated with the use of contrast-enhanced ultrasound to assess pancreatic parenchymal enhancement.

2413 Another Downside for Depression: A Case of SNRI-Induced Liver Injury

INTRODUCTION: Antidepressants are one of the most prescribed medications in the United States with duloxetine being reported as one of the top fifty drug prescriptions. Self-limited liver test abnormalities have been reported with duloxetine use but only a few case reports describe clinically apparent hepatitis following duloxetine use. CASE DESCRIPTION/METHODS: A 35-year-old African American woman with a past medical history of depression presented with few days of worsening upper abdominal pain, nausea and multiple episodes of non-bloody emesis. Initial laboratory tests revealed liver test abnormalities with ALT of 1176 U/L, AST of 596 U/L, bilirubin of 1.6 mg/dL (but with normal ALP) and INR of 1.65. Full workup including viral hepatitis, EBV, CMV, drug toxicology screen (including acetaminophen), lipase, and ceruloplasmin among others turned out to be negative. Autoimmune hepatitis markers were negative other than mild elevation of anti-smooth muscle antibody at 33 units (normal &lt; 19). Abdominal ultrasound with Doppler showed no abnormalities without any evidence of biliary obstruction. The patient denied any alcohol or illicit drug use. Patient endorsed having a recent contact with a sick squirrel so leptospira IgM was ordered which was non-reactive. The patient denied taking any medications other than duloxetine which she was started on within the last two months but she stopped taking it when she started to vomit a few days before presentation. In the second day of hospitalization, patient's LFTs peaked with ALT above 7000 U/L and AST of 5409 U/L but it then started to decrease in the following days with normalization of her bilirubin and INR as well as improvement of her symptoms with supportive care. No corticosteroids or antibiotics were given. Patient's clinical presentation with the timeline of her duloxetine use and laboratory finding indicate that duloxetine was the likely cause of her acute hepatitis. DISCUSSION: Few cases described clinically evident hepatotoxicity following duloxetine treatment. Most duloxetine-induced hepatotoxicity was seen in females with symptoms starting in the first few months following initiation of duloxetine. Typically there was an elevation of ALT &gt; AST and with some cases having bilirubin elevation as well as elevation of one of the autoimmune hepatitis markers in some cases. Clinicians should be vigilant toward possible hepatotoxicity following initiation of antidepressant even in patients with no pre-existing chronic liver disease or alcohol abuse history.

2306 Hydroxychloroquine-Induced Acute Liver Injury

INTRODUCTION: Hydroxychloroquine is a commonly prescribed medication with antimalarial and anti-inflammatory properties. A very rarely reported side effect of this medication is drug induced acute liver injury. CASE DESCRIPTION/METHODS: A 62-year old female with a history of rheumatoid arthritis, on long term prednisone, presented to the emergency department with two day history of arthralgias, generalized weakness, and fever of 104 F. She had no history of alcohol use or any risk factors for viral hepatitis. Clinical exam was unremarkable. Laboratory data showed Aspartate aminotransferase (AST) or 798 IU/L and Alanine aminotransferase (ALT) of 1254 IU/L, with normal serum bilirubin, alkaline phosphatase and coagulation profile. Serologic tests for viral etiology including Hepatitis A, Hepatitis B, Hepatitis C, Cytomegalovirus, Epstein-Barr virus, and Human immunodeficiency virus were non-reactive. Anti-nuclear antibody, smooth muscle antibody, and anti-double-stranded DNA antibody were unremarkable. Her urine and blood cultures showed no growth. Abdominal ultrasound showed an echogenic liver consistent with hepatic steatosis and no evidence of biliary obstruction. Chest X-ray was normal. Upon review of her medications, patient had started hydroxychloroquine two weeks prior to admission. The medication was discontinued upon admission, after which she continued to improve. At follow up ten days later, she remained asymptomatic and her liver function tests continued to improve with AST of 46 and ALT of 161. DISCUSSION: Acute transaminitis due to hydroxychloroquine is rare. Only four reported cases have been noted per literature review. Mechanism of injury to hepatic cells is unknown however the drug is metabolized in the liver and may alter the metabolism of other drugs. Porphyria cutanea tarda is a disease that may be treated with low doses of hydroxychloroquine. It is known that higher doses of hydroxychloroquine usually greater than 100 mg twice a week can trigger acute liver injury in patients with history of Porphyria cutana tarda. These patients present with sudden onset of fever and marked serum enzyme elevations with increased excretion of porphyrins and photosensitivity. The presented patient in the case report did not have underlying Porphyria cutanea tarda.

2397 Successful Reversal of Sickle Cell Intrahepatic Cholestasis Associated Acute Liver Failure With Combined pRBC and Plasma Exchange Transfusion: A Case Report

INTRODUCTION: Sickle cell intrahepatic cholestasis (SCIC) with acute liver failure (ALF) is a severe variant of Sickle cell hepatopathy that has significant mortality despite aggressive supportive therapy. Even though packed red blood cells (pRBCs) exchange has vastly improved outcomes, there is little evidence on alternative therapies to treat those who show inadequate response to pRBC exchange transfusion (ET). CASE DESCRIPTION/METHODS: A 37-year-old African American male with Hemoglobin SS disease complicated by chronic pain and nephropathy ESRD on dialysis, who was referred to our liver transplant program for ALF. Patient presented with lethargy and fever. Upon arrival he was somnolent, jaundiced, cardiopulmonary and abdominal exam was unremarkable, no focal neurological deficits but presence of asterixis was noted. Hb 6 g/dL, WBC 13,000/µL, Platelet 147,000/µL, corrected reticulocyte count 5.9%, LDH 1816 IU/L, Total bilirubin 49 mg/dl, direct bilirubin 37 mg/dL, ALT 112 IU/L, AST 259 IU/L, ALP 296 IU/L, albumin 3.5 g/dL and INR 1.9. CT head was unremarkable. MRCP revealed hepatomegaly, cholecystectomy without evidence of biliary obstruction. Ultrasound duplex liver showed patent hepatic vessels. Other causes of ALF including drugs, alcohol, acetaminophen, and viral hepatitis etiology were excluded. N-acetylcysteine infusion protocol was initiated. Patient received 10 units pRBC ET. Despite successful reduction of HbS fraction from 79.5% to 16%, the patient remained encephalopathic with asterixis and developed severe headache. Furthermore, bilirubin and INR continued to rise (Figure 1). Transjugular liver biopsy revealed portosystemic gradient of 20 mmHg. Liver pathology showed sinusoidal distension by sickled erythrocytes, zone 3 predominant intracanalicular cholestasis with ballooned hepatocytes and hepatocyte siderosis (Figure 2). Total 9L of plasma ET was performed on 2 consecutive days with successful reversal of headache, encephalopathy, and coagulopathy. His bilirubin levels continue to decline. He did not require a liver transplant and was discharged home with prophylaxis pRBC ET to maintain HbS fraction &lt; 30%. DISCUSSION: SCIC with ALF is rare with significant mortality. Plasma ET was used in our patient whom ALF was refractory despite adequate pRBC ET (HbS &lt; 30%). Larger studies are needed to evaluate plasma ET benefit in sickle cell disease patients with end organ damage, regarding treatment outcome as well as transplant-free survival.

3165 The Great Masquerade

INTRODUCTION: Syphilis is a sexually transmitted infection caused by T. pallidum. It is often described as “the great imitator,” affecting multiple organ systems, and has a wide variation in its clinical presentation. Syphilitic hepatitis is a rare and occurs in 0.2-3% of cases of secondary syphilis. We present a case of mixed hepatocellular and cholestatic hepatitis that led to the diagnosis of syphilis in a patient who presented with non-specific symptoms. CASE DESCRIPTION/METHODS: A 39-year-old homosexual male was referred for evaluation of worsening jaundice following a course of acyclovir for genital herpes 3 months prior. He reported non-specific symptoms including acute diarrhea, abdominal pain, myalgia, rash and weight loss with no change in mental status. He had no pertinent past medical or family history and denied any exposure to IV drugs or blood transfusions. Clinical exam showed scleral icterus, a soft and non-tender abdomen and macular rash in the extremities. Laboratory evaluation showed AST 209, ALT 617 and ALP 1195 (peaked at 2170). INR was 1.0 and albumin was 3.1. Viral hepatitis A, B, C, EBV and HIV testing were negative. F-actin IgG was positive (36) and ANA negative. MRI abdomen showed hepatomegaly without evidence of biliary obstruction. Liver biopsy showed moderate portal/periportal edema; mixed acute and chronic inflammation and pericholangitis with cholestasis. Syphilis immunostaining and Warthin-Starry staining were negative. Our patient was initially treated as presumed autoimmune hepatitis with prednisone. He had significant clinical and LFT improvement over 3-4 weeks but developed new-onset arthralgia in the midst of treatment. Rheumatological workup revealed positive rapid plasma reagin with T. pallidumreactivity. He completed a course of benzathine penicillin, following which he had complete resolution of his symptoms and normalisation of his LFTs. DISCUSSION: SH presents a diagnostic challenge in view of its infrequency and there are currently no standardised diagnostic criteria. Liver chemistry typically demonstrates a cholestatic picture with a disproportionately elevated ALP, likely secondary to pericholangiolar inflammation. However, in our patient, there was also moderate lobular activity with plasma cells, corresponding to a moderately elevated ALT. It is imperative that clinicians have a high index of suspicion for syphilis in view of the morbidity and mortality associated with a missed or late diagnosis and the availability of an effective treatment.

Preoperative risk evaluation for pancreatic fistula after pancreaticoduodenectomy.

Pancreatic fistula remains common, with limited ability to risk stratify patients preoperatively. The objective of this study was to identify risk factors for clinically-relevant postoperative pancreatic fistula (CR-POPF) that are routinely available in the preoperative setting. Preoperatively available variables for all pancreaticoduodenectomies from 2014-2017 were examined using a national clinical registry. The cohort was separated into risk factor identification and internal validation subgroups. Among 15 033 pancreaticoduodenectomies, the CR-POPF rate was 16.7%. CR-POPF was more likely in patients that were male (odds ratio [OR], 1.51), obese (body mass index [BMI] > 30, OR, 1.97), had minimal preoperative weight loss (OR, 1.25), had a nondilated pancreatic duct (OR, 1.81), did not have diabetes, (OR, 1.80), did not receive neoadjuvant therapy (OR, 1.78), had no evidence of biliary obstruction (OR, 1.18), or had nonadenocarcinoma pathology (OR, 1.96; all P < 0.01). Patients with three or fewer risk factors had a CR-POPF rate of 7.1%, while those with six or more risk factors had a CR-POPF rate of 26.3% (P < 0.001). Preoperative CR-POPF risk evaluation could be a useful tool in patient counseling and surgical planning, and risk may allow for more well-informed decisions regarding perioperative management, including enhanced recovery protocols and use of somatostatin analogs.

A Case of Multiple Myeloma Associated with Extramedullary Plasmacytoma of the Gallbladder Manifesting as Acute Cholecystitis

Multiple myeloma (MM) is a common hematological malignancy that represents 1% of all cancers. MM is distinguished from other plasma cell disorders by prominent bone marrow involvement and systemic organ damage. Extramedullary plasmacytomas of the gall bladder (GB) or biliary ducts, whether solitary or in association with MM, are very rare. We report a case of a 66-year-old female with a history of refractory MM who presented with right upper quadrant abdominal pain. Her laboratory evaluation revealed an abnormal liver panel and lactic acidosis. Abdominal ultrasonography was consistent with acute cholecystitis with no evidence of biliary obstruction or abnormal liver parenchyma. An open cholecystectomy with liver biopsy was performed. The histological evaluation revealed involvement of the GB submucosa and serosa, as well as the liver parenchyma by abnormal plasma cells with lambda light chain restriction. Congo red stain for the GB sample was positive. The patient declined further treatment for MM and was discharged home with comfort measures.

Hemophagocytic Lymphohistiocytosis Masquerading as Cholangitis

Hemophagocytic lymphohistiocytosis (HLH) is a rare syndrome of unchecked immune activation leading to multi-organ damage with a high mortality. It can be hereditary or triggered by an inflammatory process. Mild hepatitis is present in the majority of patients with HLH, but obstructive jaundice has not been reported. We present the case of a 60 year old male who presented with severe obstructive jaundice from HLH triggered by an underlying lymphoma. A 60 y/o male with a history of DM, CAD and CKD presented with severe jaundice, high fevers and fatigue. His bilirubin was 22 with 20.1 of direct; ALT 33, AST 87, and Alk phos of 114. WBC was 12.4 with left shift. CT abdomen showed mild prominence of CBD and fatty liver without lymphadenopathy. Suspecting cholangitis, antibiotics were started. An MRCP did not show any evidence of biliary dilation or choledocholithiasis and an ERCP showed esophagitis and gastritis but again, no evidence of biliary obstruction. Further work up found a ferritin of 2165 and LDH of 1434. With no lab evidence of hemolytic anemia, a bone marrow biopsy was done, which was inconclusive. Due to progressive liver failure, a liver biopsy was done which showed hemophagocytic histiocytes with evidence of large B cell lymphoma. He had intrahepatic cholestasis due to HLH caused by the lymphoma. Unfortunately with worsening liver and eventual renal failure, he could not receive appropriate chemotherapy, and succumbed to the disease. HLH was considered to be a rare disease in adults; however recently its being increasingly reported, likely due to awareness, rather than rise in incidence. Diagnosis of HLH is a challenge due to nonspecific features mimicking SIRS. Liver injury with mild elevation of transaminases is seen in 85% patients with HLH and about half have hyperbilirubinemia. Florid liver failure is rare, and Post hepatic form of injury mimicking cholangitis has not been reported so far. Prognosis of adult HLH is very poor, with mortality of 41-75% and Delayed diagnosis is considered the most important factor related to treatment failure. Early diagnosis and timely treatment with the HLH-2004 protocol has been shown to improve survival in adult patients with HLH, thus highlighting the importance of recognizing rare presentations of this disease. In patients with liver failure with intrahepatic cholestasis and unclear etiology, HLH should be strongly suspected as timely diagnosis with a Bone marrow or liver biopsy could be life saving.

Leptospirosis: A Rare Cause of ARDS

SESSION TITLE: Critical Care 3 SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: Leptospirosis is a spirochetal infection, which can manifest as a mild flu-like illness or as a life threatening multi-system organ dysfunction. We report a case of Leptospirosis presenting with multiple system organ dysfunction including ARDS. CASE PRESENTATION: A 55-year-old man presented to the Emergency Department with a 3-day history of severe muscle cramps in both legs, generalized aches, fevers, chills, and a productive cough with blood-tinged sputum. He reported a recent trip to Jamaica, where he lacerated his foot. Initial vital signs: HR 98, BP 121/79, Temp 36°C, RR 16, SpO2 98% on room air. Physical exam revealed tenderness in the RUQ of his abdomen and his lower extremities. Chest XR revealed bibasilar reticulonodular opacities. Laboratory studies showed a WBC of 13,000 with a neutrophil predominance, platelet count 75,000, hemoglobin 12.6, creatinine of 2.4, creatine phosphokinase 3353. His differential diagnosis included infection with: Hantavirus, Zika virus, Dengue, Leptospira, and Legionella. He was started empirically on azithromycin and doxycycline. His course was complicated by hypoxic respiratory failure with worsening bilateral infiltrates and a P/F ratio of 177 with an ejection fraction >50% by TTE. Other organ dysfunction also worsened: creatinine 3.6; platelet count 25,000, and total bilirubin 13.3. Hepatobiliary imaging showed multiple liver cysts without evidence of biliary obstruction. He ultimately tested positive for leptospira DNA. He was managed conservatively with respiratory support, IV fluids and doxycycline. He gradually improved and was discharged home. DISCUSSION: Leptospirosis is a rare cause of ARDS and typically presents with mild illnesses. An outbreak in the United States consisted of mild disease, presenting with fevers and myalgias (1), but more severe cases of leptopirosis have presented with acute renal failure, conjugated hyperbilirubinemia, and pulmonary infiltrates without requirement of supplemental oxygen (2). To our knowledge, this is the first reported case of ARDS in the United States secondary to leptospirosis. CONCLUSIONS: Leptospirosis in rare instances can present with ARDS. History of travel to endemic areas should prompt consideration of this as a possible etiology. While leptospirosis can have a complicated course, timely administration of appropriate antibiotics can improve outcome. Reference #1: Update: Leptospirosis and Unexplained Acute Febrile Illness Among Athletes Participating in Triathlons - Illinois and Wisconsin, 1998. (1998, August 28). https://www.cdc.gov/mmwr/preview/mmwrhtml/00054395.htm Reference #2: Pothuri, P., Ahuja, K., Kumar, V., Lal, S., Tumarinson, T., & Mahmood, K. (2016). Leptospirosis Presenting with Rapidly Progressing Acute Renal Failure and Conjugated Hyperbilirubinemia: A Case Report. American Journal of Case Reports, 17, 567-569. doi:10.12659/ajcr.897741 DISCLOSURE: The following authors have nothing to disclose: Martin Senese, Shaeesta Khan, Michael Murray No Product/Research Disclosure Information

Liver Microabscesses Due to Choledocholithiasis Masquerading as Metastatic Liver Lesions

A 56 year-old man with a past medical history of HTN presented with 2.5 weeks of right upper quadrant abdominal pian and a 20lb weight loss. Reported fevers, chills, jaundice, and tea colored urine. Laboratory findings were significant for an elevated total bilirubin of 2.1 mg/dl and an alkaline phosphatase of 918 IU/L. A CT scan of the abdomen and pelvis with IV contrast showed multiple irregularly shaped hypoattenuating masses disseminated throughout the liver with the largest measuring 3.1 cm and a lamellated soft tissue mass within the common bile duct extending into the right and left intrahepatic bile ducts. A subsequent MRI showed several filling defects throughout the biliary tract with ductal dilation. An ERCP was performed which revealed large grape like gallstones filling the common bile duct. While performing cholangioscopy, the stones were fragmented using the holmium laser and removed with balloon sweeps. Brushings of the biliary tree did not reveal any evidence of malignancy. The patient was discharged on intravenous antibiotics. On subsequent follow up, the patient was asymptomatic and his liver was normal appearing on MRI, with no evidence of biliary obstruction, choledocholithiasis, or malignancy. There are 3 major etiologies for a liver abscess: pyogenic (80%), amebic (10%) and fungal (< 10%). A pyogenic liver abscess usually arises from the biliary tract due to the obstruction of bile flow. Typically, a patient will present with continuous or intermittent fever, right upper quadrant pain, weight loss, nausea and vomiting. Jaundice may be present in 25% of patients associated with biliary tract disease. A CT Scan with IV contrast remains the diagnostic modality of choice and can be used for guiding percutaneous aspiration and drainage, when needed. In this case, the patient presented with chronic intermittent fever, right upper quadrant pain, weight loss with no elevation in his white blood count. The CT scan finding of a lamellated mass in the common bile duct and multiple hypoattenuation liver lesions, gave the appearance of an intraductal cholangiocarcinoma, rather than choledocholithiasis which is a rare presentation of liver abscess masquerading malignancy. Because the liver abscesses were caused by the obstruction of bile flow from choledocholithiasis, the patient was treated with broad spectrum antibiotics and ERCP with cholangioscopy, lithotripsy, and stone extraction rather than percutaneous drainage of the liver abscesses.Figure 1Figure 2Figure 2

Hydroxychloroquine Induced Liver Injury

Introduction: Hydroxychloroquine is a commonly prescribed medication with antimalarial and anti-inflammatory properties. Drug induced liver injury is a very rarely reported side effect of this medication. Case Report: 62 year old female with history of rheumatoid arthritis, on long term prednisone, presented to the ER with 2 days of arthralgia, generalized weakness, sweats and fever up to 104F. Chest x-ray and CT of the head showed no acute abnormalities. Upon laboratory evaluation, elevated liver function tests with AST 331 and ALT 379 were noted. Bilirubin and ALP were normal. Prothrombin time not elevated. She was admitted for possible sepsis from an unknown source and placed on empiric antibiotics. Upon review of her medications it became apparent that she had started hydroxychloroquine 16 days prior to admission. Hydroxychloroquine was stopped on hospital day 2 due to concern for drug induced liver injury. Her AST and ALT peaked at 798 and 1254, respectively on hospital day 3 and represent a 17 and 20 fold increase above the ULN. Serology tests for HAV, HBV, HCV, CMV, EBV, and HIV were nonreactive. ANA titer had increased from < 1:40 four months ago to 1:80. Anti-histone antibody, ASMA, and anti ds-DNA antibody were unremarkable. There was an increase in urine porphyrin excretion. Abdominal ultrasound showed an echogenic liver consistent with hepatic steatosis and no evidence of biliary obstruction. No growth was seen on blood cultures and antibiotics were de-escalated. AST and ALT began to trend downward on hospital day 4. Her fever had abated by hospital day 6. AST and ALT were down to 228 and 487 respectively at the time of discharge on hospital day 7. She had remained afebrile without any arthralgia at follow up appointment 10 days after discharge. AST and ALT had improved to 46 and 161 respectively. Discussion: We present this case of apparent hydroxychloroquine induced liver injury to add to the fund of knowledge of drug induced liver injuries. Based on our literature review there appears to be four previously reported cases of hydroxychloroquine induced liver injury. Hydroxychloroquine may trigger an acute worsening of porphyria cutanea tarda in susceptible individuals which could present with sudden onset of fever and marked serum enzyme elevations with increased excretion of porphyrins.

Palliative Treatment for Advanced Biliary Adenocarcinomas With Combination Dimethyl Sulfoxide–Sodium Bicarbonate Infusion and S-Adenosyl-L-Methionine

ABSTRACTAdenocarcinoma of the gallbladder and cholangiocarcinoma account for 4% and 3%, respectively, of all gastrointestinal cancers. Advanced biliary tract carcinoma has a very poor prognosis with all current available modalities of treatment. In this pilot open-label study, the authors investigated the efficacy and safety of a combination of dimethyl sulfoxide–sodium bicarbonate (DMSO-SB) infusion and S-adenosyl-L-methionine (ademetionine) oral supplementation as palliative pharmacotherapy in nine patients with advanced nonresectable biliary tract carcinomas (ABTCs). Patients with evidence of biliary obstruction with a total serum bilirubin ≤300 μmol/L were allowed to join the study. The results of this 6-month study and follow-up of all nine patients with ABTC indicated that the investigated combination treatment improved pain control, blood biochemical parameters, and quality of life for the patients. Moreover, this method of treatment has led to a 6-month progression-free survival for all investigated patients. The treatment was well tolerated for all patients without major adverse reactions. Given that ABTC is a highly fatal malignancy with poor response to chemotherapy and targeted drugs, the authors consider that the combination of DMSO-SB and ademetionine deserves further research and application as a palliative care and survival-enhancing treatment for this group of patients.