bluuebunny/crossref_metadata_2025_split huggingface dataset:
1260 Am I Seeing Double: A Rare Case of an Intrapancreatic Accessory Spleen Mimicking a Pancreatic Endocrine Neoplasm
Authors: Rozenshteyn Frederick , Hershman Melissa , Weisberg Ilan , Nagirimadugu Ankita , Collins Kelsey , Tangel Matthew , Gromis Jonathan 
Published: October 2019

INTRODUCTION:
Intrapancreatic accessory spleen (IPAS) is a rare but benign lesion associated with aberrant embryologic development. In postmortem studies, accessory spleens have been found in the hilum of the spleen or near the tail of the pancreas in 10-30% of cases. However, IPAS are uncommon and are rarely reported in the literature. The hypervascular pattern of these lesions on imaging may mimic pancreatic neuroendocrine neoplasms, and misdiagnosis may result in surveillance imaging and operative management for an ultimately benign condition. Given 40% of endocrine neoplasms are non-functioning, hormonal markers may not differentiate between the two lesions. With recent improvements in radiologic imaging, non-invasive diagnosis of IPAS has become feasible. On radiologic studies, IPAS will be well-defined, homogenous, and share similar attenuation and intensity as the spleen. Awareness and familiarity with diagnostic features of IPAS is imperative when evaluating a pancreatic mass.

CASE DESCRIPTION/METHODS:
A 74-year-old male with a history of HCV and alcohol abuse presented for evaluation of a pancreatic mass. Two years prior, a CT scan for diverticular bleeding incidentally identified a hypervascular 2.6 cm mass within the pancreatic tail, concerning for a pancreatic neuroendocrine neoplasm. At the time, the patient was asymptomatic, without abdominal pain, weight loss, diarrhea, or lab abnormalities. Due to the concern for malignancy, the patient subsequently underwent EUS with fine needle biopsy, which revealed acute and chronic inflammation without neoplasia and small samples of benign pancreatic parenchyma. Surveillance MRCP was performed, which demonstrated a stable, hyper-enhancing pancreatic tail lesion seen on multiple pulse sequences with dynamic gadolinium enhancement similar to that of the normal spleen. Ultimately, the lesion was diagnosed as an intrapancreatic accessory spleen.

DISCUSSION:
IPAS masses are clinically silent, often found incidentally, and initiate a malignancy workup and at times unnecessary surgery. The consequences of missing a pancreatic malignancy are grave, but recent advances in imaging techniques allow for radiologic differentiation of IPAS from pancreatic neoplasms. For unclear cases, evaluation with nuclear medicine imaging can help detect splenic tissues. Overall, it is important to recognize the benign pancreatic condition of IPAS and differentiate it from diseases that do require surveillance imaging and invasive interventions.
1262 A Rare Case of Pleural Effusion Due to a Pancreatico-Pleural Fistula in the Setting of Pancreas Divisum
Authors: Mukherjee Samiran , Harne Prateek , Sharma Anuj , Masood Umair , Manocha Divey 
Published: October 2019

INTRODUCTION:
A pancreatico pleural fistula is a rare complication of pancreatitis which classically results from a pancreatic duct disruption or a pseudocyst extension into the pleural cavity. The literature present on this condition continues to remain sparse. We attempt to present a rare case of a pancreatico-pleural fistula in the setting of a congenital pancreatic anomaly which initially presented as a recurrent pleural effusion in the setting of chronic pancreatitis.

CASE DESCRIPTION/METHODS:
Our patient was a 51-year-old female who presented with intractable abdominal pain and shortness of breath with a confirmed diagnosis of a pancreatic pseudocyst. On admission, her chest Xray was significant for pleural effusion. Further analysis of the pleural fluid revealed the presence of elevated lipase and amylase levels. The patient eventually underwent an ERCP with contrast that revealed contrast leakage tracking upwards from the dorsal pancreatic duct suggesting a pancreatico-pleural fistula. This was in addition to the inability to fill the ventral pancreatic duct, suggesting a complete pancreatic divisum. A pancreatic duct plastic stent was placed and the pleural effusion gradually subsequently subsided.

DISCUSSION:
The case allows us to consider one of the lesser known etiologies of pleural effusion in the setting of pancreatitis (the other one being transdiaphragmatic lymphatic blockage) and provides us with an opportunity to develop a step to manage the same. This is the only known case report of a pancreatico-pleural fistula with a concomitant complete pancreatic divisum.
1264 Mediastinal Pancreatic Pseudocyst With Expansion Into Bilateral Pleural Spaces
Authors: Smith Cierra C. , Smith Alexander W. , Anderson Jay 
Published: October 2019

INTRODUCTION:
Pseudocysts are a common complication of both acute and particularly chronic pancreatitis. Uncommonly, pseudocysts may communicate with thoracic cavities, including the mediastinum. We describe a case of acute pancreatitis complicated by a mediastinal pseudocyst with bilateral pleural space involvement.

CASE DESCRIPTION/METHODS:
A 51-year-old male with a history of alcohol abuse presented shortly after discharge from a prolonged hospitalization for his first lifetime episode of acute alcohol-induced pancreatitis. A CT of the chest ordered on admission for new onset respiratory distress demonstrated bilateral pleural effusions. Fluid analysis showed a right sided empyema and left sided aseptic exudative effusion. The right empyema resolved after chest tube placement but there was rapid recrudescence of a right pleural effusion after chest tube removal. Repeat fluid analysis was significant for a pancreatic specific amylase level of 2620. Repeat CT imaging demonstrated a posterior mediastinal fluid collection that arose from the pancreas, extended through the esophageal hiatus and was contiguous with new fluid collections in both pleural spaces and this was confirmed with MRCP. The mediastinal pseudocyst was deemed not amenable to endoscopic drainage so ERCP was performed with stent placement to the ventral pancreatic duct along with chest tube placement for the larger right sided fluid collection and subcutaneous octreotide administration, with eventual reduction of pseudocyst size.

DISCUSSION:
Mediastinal pseudocysts are a rare complication of acute pancreatitis more commonly seen in chronic pancreatitis, though exceedingly rare in general. It is theorized that mediastinal pseudocysts occur based on ease of pancreatic fluid movement. The pararenal and pelvic extraperitoneal spaces are typically the easiest spaces for this fluid to penetrate but conditions that create inflammatory fibrosis of these areas may make ascending flow into the mediastinum the path of least resistance. On CT scan and on MRCP, our patient had a mediastinal pseudocyst that communicated directly and inseparably with both the right and left pleural space. To our knowledge, this has not been previously seen in the limited case reports of mediastinal pseudocysts. Despite not being amenable to endoscopic drainage based on location, the use of octreotide coupled with pancreatic duct stenting proved effective in reducing the caliber of this mediastinal pseudocyst on interval imaging.
1266 Beta-Sitosterol-Induced Acute Pancreatitis: A Case Report and Review of the Literature
Authors: Lorenze Alyssa , Hsueh William , Nasr John 
Published: October 2019

INTRODUCTION:
While drug-induced pancreatitis from corticosteroids has been well described in the medical literature, the exact mechanism is unclear. We present the first reported case of drug induced pancreatitis from Beta sitosterol, a naturally occurring plant sterol structurally similar to cholesterol, obtained primarily through Western diet and supplementation.

CASE DESCRIPTION/METHODS:
A 57-year-old male with history of situs inversus and Benign BPH presented from an outside facility with a two day history of generalized abdominal pain. The morning of admission the pain became sharp and severe over the mid-epigastric area, radiating to the right upper quadrant causing the patient to become bedbound. Lab results were noted for WBC 10.2, Hb of 11.1 g/dl, Hct of 31.9%, BUN 14 mg/dl, glucose 143 mg/dl, and calcium of 9 mg/dl. Liver tests including AST, ALT, Total bilirubin, and ALP were within normal limits. Lipase was markedly elevated at 572 U/L (>7x upper limit of normal). CT and US of the Abdomen were remarkable for acute pancreatitis with acute necrotic collection and normal appearing gallbladder and bile ducts without gallstones. The patient was managed with aggressive IV hydration and supportive management. After 48 hours the patient's hemoglobin decreased to 8.9 mg/dl. The patient had significant resolution of symptoms after 4 days and was referred to gastroenterology clinic for follow up. At his follow up appointment, the patient disclosed that he had started a new herbal supplement, B-sitosterol, on the morning that his symptoms began. Patient denied history of smoking, alcohol use, gallstones, recent infection, illicit drug use, trauma, exposure to scorpions, or weight loss. Abdominal MRI and MRCP obtained 4 months after his initial presentation showed interval resolution of pancreatitis and pancreatic fluid collections, normal pancreas, and normal biliary and pancreatic ducts.

DISCUSSION:
We describe a case of acute pancreatitis in a patient with a history of BPH on Beta sitosterol, a phytosterol and common over the counter herbal supplement, who presented with a two day history of progressively worsening abdominal pain and nausea. Following a thorough history and a negative medical workup to exclude other causes, the patient was treated with supportive measures and was advised to discontinue Beta sitosterol. His symptoms drastically improved within days and he continued to remain asymptomatic with normal imaging at his follow up appointment.
1267 Recurrent Mantle Cell Lymphoma to the Gall Bladder: A Peculiar PET CT
Authors: Datta Samit K , Inaganti Anupama , Hilal Jonathan , Chausow Douglas , Guda Nalini 
Published: October 2019

INTRODUCTION:
Mantle Cell Lymphoma (MCL) is a B-cell non-Hodgkin lymphoma that has a variable course. The initial presentation of MCL is usually nonspecific and varies based on the organ involved. Initial treatment includes a variety of chemotherapeutic options; however, almost all patients will have refractory or recurrent disease due to the rapid development of chemotherapy resistance. In this case report, we present a rare presentation of recurrent mantle cell lymphoma in the gall bladder.

CASE DESCRIPTION/METHODS:
Our patient is a 61-year-old female with history of mantle cell lymphoma diagnosed in 2008. At that time patient was treated with Rituxan/hyper-CVAD with an excellent response to treatment. Patient had a disease recurrence in 2012 on axillary lymph node biopsy and received 6 cycles of Bendamustine and Rituximab and achieved remission as determine by PET CT. The patient decided against allogeneic hematopoietic stem cell transplant (HCT) and was started on Rituximab maintenance therapy by 2013. Patient had another recurrence on axillary lymph node biopsy in 2017 and was placed on Ibrutinib with no FDG noted on subsequent PET-CTs in 2/2018 and 5/2018. A surveillance PET CT performed in 12/2018 showed intense avid FDG uptake involving the gallbladder (Figure 1). A MRI was performed that showed an enhancing lesion in the dorsal aspect of the descending duodenum measuring 18 mm. Upper endoscopy revealed a prominent fold with biopsies taken with no histopathologic abnormality. The patient was subsequently taken for uncomplicated cholecystectomy with one lymph node associated and sent for pathology. Pathology results showed the cystic duct lymph node and gallbladder wall involved by MCL with 40 mitoses per 15HPF and Ki-67 proliferative index of 34% (Figures 2 and 3). This suggested a more aggressive MCL. The patient was started on Revlimid and Rituximab, and will be awaiting clinical trial eligibility at a nearby facility.

DISCUSSION:
MCL typically follows an aggressive clinical course with high risk of recurrence regardless of chemotherapeutic regimen. One previous case with MCL to the gallbladder was found incidentally as a lymphomatous polyp. An additional case presented with intussception with a mass noted during surgical repair. Overall, there have been approximately 66 cases of B-cell lymphoma noted to the gallbladder, and two prior cases as above of MCL. Our case represents a rare presentation of relapsed MCL to the gall bladder and the wide possibility of metastasis associated with recurrent MCL.
1268 The Hidden Gallbladder: An Unusual Case of Bouveret Syndrome
Authors: Datta Samit K , Hilal Jonathan , Guda Nalini 
Published: October 2019

INTRODUCTION:
Bouveret syndrome is the most infrequent variant of gallstone ileus characterized by a biliary fistula with the stomach or small intestine. The fistula occurs from recurrent gallbladder inflammation causing adhesion to the GI tract followed by local necrosis. Patients generally present nausea, vomiting, and abdominal pain/distention with variable lab abnormalities, with jaundice and hepatic enzyme alternations in only 1/3 of patients. 43-68% of patients have a history of recurrent biliary colic and given the delay in diagnosis, patients can present very late with dehydration and weight loss. CT scan is diagnostic modality of choice with an overall 93% sensitivity, 100% specificity, and 99% diagnostic accuracy. We present a rare case of Bouveret Syndrome missed by CT scan and diagnosed by ERCP.

CASE DESCRIPTION/METHODS:
Our patient is an 81-year-old male who presented with jaundice and intermittent fever/chills. He reported 7 months of intermittent nausea, vomiting, and abdominal pain and reportedly lost approximately 80 pounds in the past year. The patient denied any prior cholecystectomy. He was noted to have total bilirubin of 8.1 and an alkaline phosphatase of 523. RUQ U/S showed a 1.5 cm common bile duct (CBD) and non-visualization of the gall bladder. CT scan was performed showing CBD of 2.0 cm but no clear cholelithiasis and reported absence of the gallbladder. Patient subsequently had EUS/ERCP. EUS showed a shrunken decompressed gallbladder with cholelithiasis (Figure 1) and bilious output into the lesser curvature of the stomach concerning for fistulous process. ERCP showed dilated CBD with multiple large gallstones with sphincterotomy and balloon extraction (Figure 2). Impaction cholangiogram with cinematic fluoroscopy confirmed a cholecystogastric fistula (Figure 3). The patient was a poor surgical candidate but had improvement in his symptoms and liver function tests and was safely discharged home.

DISCUSSION:
Bouveret syndrome is a very rare complication of gallstone ileus that can have high mortality due to its late and nonspecific presentation. There have been approximately 300 cases of documented Bouveret syndrome with 60% of cases involving a cholecystoduodenal fistula. 5% of cases present with a cholecystogastric fistula. As CT is the mainstay of diagnosis, our case is further unique in that CT scan and RUQ U/S reported an absent gallbladder (which the patient denied), likely due to the contracted nature noted on EUS and required endoscopic visualization and ERCP to confirm.
1271 Arteriobiliary Fistula Repair With Biliary Endoprosthesis: When Trans Arterial Embolization Is Not an Option
Authors: Hayat Maham , Zia Hassaan , Hayat Muhammad Hashim , Bolkhir Ahmed 
Published: October 2019

INTRODUCTION:
Arteriobiliary fistula is an uncommon complication manifesting as hemobilia or an upper gastrointestinal (GI) bleed. Etiologies include intra-hepatic artery chemo-embolization, trauma or previous invasive biliary instrumentation. We describe a case report of using two fully covered metal biliary stents for closure of an acquired arteriobiliary fistula.

CASE DESCRIPTION/METHODS:
20-year-old male with history of neuroblastoma s/p resection and chemoradiation underwent placement of percutaneous transhepatic biliary drain (PTBD) for common bile duct (CBD) strictures. He was admitted to the hospital with acute blood loss anemia, transaminitis and sanguineous output from the biliary drain. Upper endoscopy revealed one clean based gastric ulcer which was injected with epinephrine. Patient was scheduled to undergo replacement of external/internal biliary drain. Intraoperative cholangiogram revealed extensive thrombus within central biliary ducts. It also demonstrated a fistulous connection between the common hepatic duct and the right hepatic artery. Angiogram showed replaced right hepatic artery, originating from superior mesenteric artery with occluded 4 cm proximal end, distal reconstitution was seen via fine collaterals; which made endovascular repair impossible (Figure 1 left). As a temporizing measure, a COOK 16 Fr × 40 cm biliary drain was replaced and advanced beyond the arterial-biliary fistulous connection, serving as a tamponade (Figure 1 right). Over the subsequent days, patient continued to have intermittent sanguineous biliary output. After surgical options were ruled out, two GORE VIABAHN 11 mm × 29 mm balloon expandable fully covered metal stents were deployed within the common bile duct to exclude the biliary-right hepatic artery fistula (Figure 2 left, right). A similar sized biliary drain was replaced subsequently (Figure 3). Hemobilia resolved and hemoglobin remained stable. Patient was discharged successfully to a center of excellence for multivisceral transplant.

DISCUSSION:
The management of hematobilia often includes an initial cholangiogram, if an arterial source is identified, hepatic artery embolization is the treatment of choice. This unique case demonstrates the utility of an endobiliary prosthesis for exclusion of arteriobiliary fistula where endovascular repair was not an option. The shortest possible endoprosthesis should be used to avoid obliteration of other large vessels or bile duct branches. Complications include bacteremia and liver infarction adjacent to stent-grafts.
1272 Atypical Presentation of Intrahepatic Cholestasis of Pregnancy
Authors: Sadarat Fnu , Boroda Konstantin , Hertan Hilary 
Published: October 2019

INTRODUCTION:
Intrahepatic cholestasis of pregnancy (ICP) is a common clinical problem evident in approximately 1% of pregnancies in the United States. The incidence is more common in Chilean and Swedish ethnic groups. Due to the pathognomonic early symptom of severe pruritus, most patients are diagnosed and treated promptly. However, the risk of fetal demise is high, ranging from 2-11%. In very rare cases patients may present with atypical symptoms that can delay diagnosis and appropriate management.

CASE DESCRIPTION/METHODS:
A 40-year-old woman, gravida 4 para 2, presented in her 20th week of gestation with severe right upper quadrant pain radiating to the epigastric region for 3 days. Initial workup showed elevated total bilirubin of 4.3 mg/dl with a direct bilirubin of 3.7 mg/dl; Alanine aminotransferase of 39 U/L and aspartate aminotransferase of 27 U/L. Initial ultrasound of the abdomen showed an enlarged liver with the right hepatic lobe measuring up to 21 cm in length, status post cholecystectomy and normal caliber proximal common bile duct. Patient's bilirubin trended up and she became jaundiced, so MRCP without contrast was obtained. It showed no choledocholithiasis and no biliary ductal dilatation. Hepatitis A, B, C, and E virus PCR, cytomegalovirus IgM, anti-mitochondrial antibody, anti-smooth muscle antibody were all negative as well. The patient never developed pruritus, however, bile acids were checked and found to 190 µmol/L and eventually peaked at 290 µmol/L. She later developed intense pruritus 1 week after initial presentation and abdominal pain had completely resolved. She was started on ursodiol and cholestyramine. The bile acid level eventually decreased to 39 µmol/L 1 month later and her pruritus improved. She continued to follow with maternal-fetal medicine and gastroenterology, there was no harm to the fetus.

DISCUSSION:
ICP usually presents with pruritus of the hands and feet due to elevated bile acid levels in the blood during 2nd and 3rd trimester of pregnancy. Our patient's only symptom was acute right upper quadrant pain with no pruritus, which is very atypical for ICP. There are no case reports of this disorder presenting solely with abdominal pain. It is important to recognize abdominal pain as a possible presenting symptom of ICP in order to diagnose the condition early.
1273 IgG4 Related Cholangiopathy Masquerading as Cholangiocarcinoma
Authors: Iriana Sentia , Parbhu Sheeva , Sossenheimer Michael , Hatch Jenny 
Published: October 2019

INTRODUCTION:
IgG4 related diseases can involve multiple organs, best described in cases of autoimmune pancreatitis (AIP). IgG4-related cholangiopathy is difficult to distinguish from other forms of biliary ductal disorders including primary biliary cholangitis, primary sclerosing cholangitis (PSC), and cholangiocarcinoma (ChCA). Specifically, IgG4 cholangiopathy and ChCA share many clinical and imaging findings which make it difficult to distinguish these entities. We report a rare case of IgG4 cholangiopathy mimicking a ChCA.

CASE DESCRIPTION/METHODS:
A 69-year-old male presented with nausea/vomiting, weight loss, painless jaundice, and CT suggestion of a malignant hilar mass. ERCP with cytology and EUS with FNA from the hilar mass were negative for malignancy. Two further ERCPs with Spyscope hepatic duct bifurcation biopsies showed severe stenosis of the right hepatic ducts and diffuse biliary stricturing suggestive of PSC. Brushings for cytology and fluorescence in situ hybridization (FISH) were obtained, balloon dilation and stenting performed. MRCP noted multifocal intrahepatic biliary strictures, suggestive of PSC; a hilar mass, as seen on CT, was not seen. The MR suggested a solid pancreatic uncinate process mass. A repeat EUS showed changes of chronic pancreatitis but no mass. A comprehensive review of the combination of intrahepatic strictures, pancreatic atrophy and uncinate mass suggested a diagnosis of IgG4 disease; an ANA was positive at 1:160 and IgG4 was elevated at 1,250 mg/dL (normal < 123). The patient was started on prednisone and had complete resolution of abnormal liver tests, clinical symptoms, MR biliary ductal dilatation, and MR pancreatic mass within 3 weeks.

DISCUSSION:
While described in AIP, IgG-4 related diseases can affect multiple organs, including kidneys, liver, salivary gland, orbit, breast, pericardium, aorta, skin, lungs, prostate, meninges, and the pituitary gland. IgG4-related cholangiopathy is a distinct yet rare etiology, characterized by elevated serum IgG4 levels and IgG4-positive plasma cell infiltration with storiform fibrosis/obliterative phlebitis of the bile duct wall. It has been identified in patients with concomitant AIP and is steroid responsive. Early diagnosis of IgG4 cholangiopathy avoids prolonged hospitalizations, unnecessary repeated procedures, and morbidity/mortality due to treatment delay We describe a rare case of IgG4 autoimmune cholangiopathy with delayed diagnosis due to concern for ChCA.