bluuebunny/crossref_metadata_2025_split huggingface dataset:
1424 Follicular Cholangitis Presenting as Cholangiocarcinoma in a Patient With Serologic Evidence of Autoimmune Disease
Authors: Kleesattel David , Safdar Kamran , Shah Shimul , Wang Jiang , Bari Khurram 
Published: October 2019

INTRODUCTION:
Follicular cholangitis is a rare sclerosing cholangitis of the biliary hilum that is difficult to distinguish from primary sclerosing cholangitis, immunoglobulin G4-related sclerosing cholangitis, and most imperatively, hilar cholangiocarcinoma. Pathological findings include fibrotic thickening of bile duct walls containing lymph follicles with germinal center formation. While autoimmune evaluations have previously been negative, we herein report the first case of follicular cholangitis found in the pretense of autoimmune hepatitis.

CASE DESCRIPTION/METHODS:
A 40-year-old female presented following a 2-month history of pruritis, fatigue, and elevation of hepatobiliary enzymes (AST 113 U/L, ALT132 U/L, ALP 499 U/L). Medical history was significant for cholestasis of pregnancy 10 years ago. Laboratory evaluation was positive for an ANA (1:160), an atypical pANCA (1:320), and an elevated F-actin (101 units) while negative for anti-mitochondrial antibody. Serum IgG levels were normal (1250 mg/dl; IgG4 subclass <1). IgM level was elevated at 350 mg/dl. Liver biopsy showed dense portal lymphatic inflammation, including focal germinal center formation as well as paucity of bile ducts without florid ductal lesion or fibrosis. Initial treatment with glucocorticoids, azathioprine and ursodiol did not improve symptoms or liver chemistries. ERCP revealed a tight stricture with direct visualization (spyglass) of a biliary lesion in the distal third of the common bile duct (CBD). Cytology from the stricture returned atypical cells suspicious for adenocarcinoma. Bile duct biopsy revealed severe chronic inflammation and few atypical glands. IgG4 immunostaining was negative. She underwent pancreaticoduodenectomy due to concern for cholangiocarcinoma. Macroscopically, the CBD mucosa was diffusely granular and without mass lesions. Microscopically; bile duct fibrosis, lymphoid hyperplasia with prominent germinal centers, and rare IgG4-positive cells were consistent with severe follicular cholangitis.

DISCUSSION:
Unique to this case is the serological backdrop and histologic findings of autoimmune disease. Whereas previous cases have been strictly ANA negative, there appears to be an unexplored continuum of disease as evidenced by cases of follicular bronchiolitis which have been associated with autoimmune disease and have similar immunohistologic findings. Although the etiology remains unclear, the existence of an autoimmune association raises important considerations for medical therapies.
1426 Type 1 Choledochal Cyst With Anomalous Pancreaticobiliary Malunion Diagnosed on Cholangioscopy
Authors: Ali Mohammed Fahad , El Hage Chehade Nabil , Sowa Piotr , Clary Meredith , Samarasena Jason 
Published: October 2019

INTRODUCTION:
Choledochal cysts are cystic dilatations found within the intra and/or extrahepatic bile ducts. Untreated choledochal cysts can lead to increased risk for strictures, pancreatitis, cholangitis, gallbladder cancer, cholangiocarcinoma and pancreatic cancer. Early and accurate identification of choledochal cysts is crucial to determine appropriate medical and surgical planning. We present a case of a patient with a type 1 choledochal cyst with anomalous pancreaticobiliary malunion.

CASE DESCRIPTION/METHODS:
A 42-year-old female with a PMH of a cholecystectomy and known biliary cyst presented with acute onset abdominal pain. MRCP demonstrated a 5.5 cm fusiform dilatation of the common bile duct (CBD). EUS confirmed a CBD dilatation (Figure 1). ERCP with spyglass cholangioscopy showed an abnormal pancreaticobiliary junction with a common channel about 1 cm long leading to two separate openings, one to the CBD/choledochal cyst and another to the pancreatic duct orifice (Figure 2). Fluoroscopy showed contrast filling a dilated cystic structure in the proximal CBD consistent with a choledochal cyst (Figure 3). A 7 French × 7 cm flex stent was deployed into the CBD. Findings were consistent with a type 1 choledochal cyst and an anomalous pancreaticobiliary malunion. Patient underwent ex-lap with resection of the choledochal cyst with roux-en-y hepaticojejunostomy creation, which she tolerated well.

DISCUSSION:
The Todani classification is based on cyst location and bile duct dilatation. There are 5 types with type 1 as the most common which comprises of 80-90% of all choledochal cysts. Type 1 choledochal cysts involve fusiform dilatation of the entire CBD without any intrahepatic dilatation. Cancer is more common in patients who are older and with type I and IV cysts. These are typically treated with complete cyst resection and Roux-en-Y hepaticojejunostomy. Additionally, 50-80% of patients with choledochal cysts have an anomalous pancreaticobiliary malunion. The KOMI classification is used to classify anomalous pancreaticobiliary malunions into 3 types, which is based on the CBD/pancreatic duct angle and presence of CBD dilation. This case was a KOMI type IIB anomalous pancreaticobiliary malunion given the common hepatic and pancreatic ducts join each other at an acute angle creating a single common channel.
1428 Biliary Obstruction Due to Extrahepatic Biliary Mucinous Cystic Neoplasm: An Uncommon Case
Authors: Hawksworth Sara J. , Pluskota Christopher , Confer Bradley , Sarami Iman , Li Jinhong 
Published: October 2019

INTRODUCTION:
Biliary mucinous cystic neoplasms (BMCNs) are a rare form of premalignant biliary cystic tumors, much like their better-known counterpart, biliary intraductal papillary mucinous neoplasms (IPMNs). BMCNs most commonly occur in females during the 5th decade of life. They account for approximately 1-5% of all liver cysts. The lack of awareness of these lesions poses a challenge for proper and timely diagnosis. Here, we present an interesting case of extrahepatic BMCN.

CASE DESCRIPTION/METHODS:
A 30-year-old female presented with pruritis, dark urine, and light-colored stools. Labs revealed transaminitis and elevated bilirubin concerning for biliary obstruction. Abdominal ultrasound showed cholelithiasis and dilation of the distal common bile duct (CBD) concerning for choledocholithiasis. MRCP was performed and described a 2.2 × 2.4 cm cystic dilatation of the extrahepatic bile duct involving the distal common hepatic duct (CHD) concerning for either type 1B or type II choledochal cyst. Endoscopic ultrasound revealed a CHD cystic lesion with internal debris concerning for either sludge or biliary IPMN (Figure 1). ERCP with cholangioscopy revealed papillary projection in the CHD (Figure 2). Directed biopsies revealed mucinous epithelium with cytologic atypia. The patient was referred for complete resection of the CBD and CHD, cholecystectomy, and Roux-en-Y hepaticojejunostomy. Surgical pathology revealed a tumor consisting of a cystic structure lined by mucinous epithelium with estrogen receptor (ER) positive ovarian-type stroma, and mild to focal moderate dysplasia, consistent with noninvasive extrahepatic BMCN (Figure 3).

DISCUSSION:
BMCNs are often detected incidentally when investigating other common causes of biliary disease. The overall incidence of BMCNs is approximately 1:20,000 to 1:100,000. They are most commonly found within the liver (90%), are rarely extra-hepatic, and they seldom present with obstructive jaundice. The treatment of choice is surgical resection. Diagnosis is made via histological confirmation of ovarian-type stroma. The location of the lesion, in this case, is more common for IPMNs. Both IPMNs and BMCNs carry malignant potential requiring complete resection for diagnosis and treatment. With the risk of malignant transformation as high as 30%, an awareness of these rare neoplasms is very important.
1431 A Bad Connection: Chronic Pancreatitis Leading to a Pancreticopleural Fistula
Authors: Humes Ross , Sadowski Brett , McCarthy John , Young Patrick 
Published: October 2019

INTRODUCTION:
Pancreatic-pleural/mediastinal fistulas are a rare complication of chronic pancreatitis. Fistulous connections occur through fenestrations in the diaphragm. Pancreatic ductal stenting can be valuable, though medical and surgical options may still be required. We describe a case of pancreatic-pleural fistula with mediastinal tracking successfully treated with combined endoscopic and medical management.

CASE DESCRIPTION/METHODS:
The patient is a 53-year-old male with a history of chronic pancreatitis secondary to alcohol abuse, a pancreatic pseudocyst, and compensated heart failure. The patient was admitted with new onset shortness of breath and epigastric abdominal pain. CT showed acute on chronic pancreatitis with a pseudocyst in the uncinate process, lower mediastinal inflammatory changes, and bilateral pleural effusions. Thoracentesis was performed, demonstrating an amylase of >7500. Though MRCP did not show a discrete connection, the clinical presentation was consistent with pancreatic-pleural fistula. ERCP was performed, but the endoscopist was unable to cannulate the pancreatic duct. Patient was placed on octreotide and continued to undergo therapeutic thoracenteses as needed. Patient returned 1 month later with complaints of neck fullness, continued shortness of breath, and abdominal pain. CT was significant for a fluid collection extending from pancreatic head to the mediastinum tracking along the anterior aspect of the esophagus and persistent pleural effusions. MRCP was performed and revealed a fistulous connection between the pseudocyst, pleural space, and mediastinum. Repeat ERCP was done with successful pancreatic stent placement. Patient subsequently had resolution of both mediastinal and pleural fluid collections. The pancreatic stent was removed with durable resolution of fluid collections.

DISCUSSION:
Pancreatic fistulas are reported to resolve with supportive care alone in 80% of external and 50-65% of internal fistulas. In our case, supportive care including octreotide use was insufficient for fistula resolution. The patient was ultimately treated successfully with pancreatic stenting. Had this failed, alternative approaches could have included nasojejunal feeding, endoscopic sealants, or surgical correction. This case highlights a rare, but serious complication of chronic pancreatitis and management options to be considered.
1437 Overlap Syndrome of Primary Sclerosing Cholangitis and Primary Biliary Cholangitis: A Case Report
Authors: Shah Shivani , To Uyen , Silveira Marina 
Published: October 2019

INTRODUCTION:
Primary sclerosing cholangitis (PSC) is characterized by inflammatory changes within the extra-and intrahepatic bile ducts and subsequent development of strictures. Primary biliary cholangitis (PBC) is characterized by progressive destruction of intrahepatic bile ducts, typically associated with a positive anti-mitochondrial antibody. Both of these disease processes are rare, chronic cholestatic liver diseases, with possible outcomes of progression to cirrhosis and need for liver transplant.

CASE DESCRIPTION/METHODS:
A 55-year-old gentleman from West Africa with no significant past medical history presented with intractable pruritus, weight loss, polyuria and polydipsia, found to have new onset diabetes. His other laboratory data revealed cholestasis, with alkaline phosphatase 401 U/L, aspartate aminotransferase 100 U/L, alanine aminotransferase 160 U/L, direct bilirubin 0.37 mg/dL on laboratory examination. Magnetic resonance imaging of his abdomen was unremarkable for any biliary dilatation or etiology of his cholestatic liver injury. Liver biopsy was remarkable for non-specific lymphocytic portal inflammation. Other diagnostic work up was remarkable for negative autoimmune diabetes work up. He was subsequently lost to follow up and re-presented five years later with intractable pruritus. Repeat MRI of his abdomen revealed a beaded and irregular appearance of the extra and intrahepatic bile ducts, concerning for PSC (Figure 1). He was started on cholestyramine with minimal improvement in pruritus. Repeat liver biopsy revealed focal bile duct injury. Laboratory examination revealed an elevation of anti-mitochondrial antibody (AMA). These findings were consistent with a diagnosis of overlap syndrome of PBC and PSC. Treatment with ursodeoxycholic acid (UDCA) was started; however, given lack of improvement in biochemical profile, fenofibrate was added. The patient was subsequently hospitalized for rhabdomyolysis with acute kidney injury in setting of fenofibrate use. The patient developed cirrhosis and subsequent decompensation of hepatic encephalopathy and ascites approximately twenty months after his initial diagnosis of overlap syndrome. He is currently undergoing transplant evaluation.

DISCUSSION:
This is a rare case of overlap syndrome of PBC and PSC. At present, there are no consensus guidelines on the diagnosis and management of this syndrome. Given the rarity of this condition, there is currently no available data on long term outcomes in patients with overlap of PBC and PSC.
1440 An Uncommon Cause of Biliary Obstruction in a Patient With Budd-Chiari Syndrome
Authors: Mago Sheena , Mavilia Marianna 
Published: October 2019

INTRODUCTION:
Biliary varices (BVs) are an infrequent complication of chronic portal hypertension (PHTN). PHTN causes collateral paracholedochal veins to dilate and protrude into the common bile duct (CBD), forming varices. Most cases of BVs are asymptomatic and are therefore likely underdiagnosed. However, protrusion of BVs into the CBD can lead to clinically significant biliary obstruction.

CASE DESCRIPTION/METHODS:
A 34-year-old female with polycythemia vera and Budd-Chairi syndrome status-post portocaval shunt on chronic warfarin presented with a 1-day history of right upper quadrant pain and non-bloody, non-bilious emesis. She was febrile to 39.9 C and had a soft, but distended abdomen without icterus or asterixis. Labs were notable for total bilirubin 4.1 mg/dL, direct bilirubin 1.2 mg/dL, AST 79 U/L, ALT 69 U/L, INR 1.8, and alkaline phosphatase of 207 U/L. Abdominal ultrasound with Doppler demonstrated a patent portacaval shunt, splenomegaly, and stable prominent portosystemic varices causing limited visualization of the CBD. ERCP for further visualization showed a filling defect of the middle third of the CBD. A biliary stent was deployed, causing hemorrhage within the CBD, raising the possibility of an intraductal varix. Hemostasis was achieved with subsequent metal stent placement. Further investigation with venography displayed multiple infrahepatic IVC collateralization and two regions of significant stenosis along the portal-IVC stent and intrahepatic IVC. This resulted in diversion of blood flow through the azygos system and ultimately giving rise to her CBD varices. Balloons were used for venoplasty resulting in successful reduction of the regions of stenosis and improvement of the pressure gradients.

DISCUSSION:
The presence of BVs can complicate surgical and endoscopic procedures of the biliary tract. In this case, endoscopic intubation of the duct during ERCP caused unexpected hemobilia as BVs were not suspected in this patient. Data on management of biliary varices is limited. In our patient, stent placement was performed to both achieve hemostasis and relieve the biliary obstruction. Venography was done to diagnose and treat IVC stenosis. This ultimately improved pressure gradients in the collateral circulation and should prevent progression of BVs. History of PHTN should increase clinical suspicion for BVs in patients presenting with biliary obstruction. If a biliary tract procedure is required, pre-procedure imaging for variceal screening should be considered to prevent complications.
1443 Hypercalcemia as the First Presentation of an Underlying Rare Variant of Adenosquamous Cholangiocarcinoma
Authors: Ramachandran Preethi , Kaur Amandeep , Yadav Ruchi , Joseph Gardith 
Published: October 2019

INTRODUCTION:
Humoral hypercalcemia of malignancy (HHM) is an important paraneoplastic syndrome which is caused by the over secretion of the parathyroid hormone-related peptide (PTHrP) from a variety of cancers. Most commonly HHM is reported in squamous cell carcinomas of the lung, head, neck, skin, esophagus, ovarian and breast cancer. HHM due to intrahepatic cholangiocarcinoma is a rare association and is associated with poor prognosis.

CASE DESCRIPTION/METHODS:
We herein report a case of hypercalcemia presenting as the first presentation of an underlying rare variant of intrahepatic cholangiocarcinoma. Our patient is a 57-year-old male who presented to the emergency room with severe constipation and polyuria. His physical examination was unremarkable except for hepatomegaly. His laboratory values revealed calcium 16.5 mg/dL, creatinine 1.37 mg/dL, ALP of 413 U/L, PTH -2 pg/mL, PTHrP 82 pg/mL. Computed tomography (CT) of the abdomen revealed a 15 cm liver mass with possible satellite lesions. The patient was subsequently treated for symptomatic hypercalcemia with aggressive hydration, zoledronic acid, and calcitonin. Biopsy of the liver lesion revealed poorly differentiated carcinoma with squamous features positive for Immunohistochemical markers CEA, CK19, P63, CK5/6, CK7 which favored pancreaticobiliary malignancy as the primary. Further staging scan with positron emission tomography (PET) revealed extensive hypermetabolic activity in the liver, enlarged upper abdominal lymph nodes, nodularity of the omentum/peritoneum and ascites inferior to the liver. Within a week into the presentation, his bilirubin started to increase, needing emergency percutaneous transhepatic cholangiography (PTC). His repeat CT scans showed progressively increasing tumor size to 19 cm with an associated increase in satellite lesions. This necessitated an urgent inpatient treatment with gemcitabine/cisplatin combination chemotherapy to control the aggressive nature of the malignancy.

DISCUSSION:
Adenosquamous cholangiocarcinoma is a very rare variant accounting for <2% of all liver tumors. It develops due to squamous metaplasia of an underlying cholangiocarcinoma and usually has aggressive clinicopathological features. HMM is a life-threatening yet unrecognized phenomenon of cholangiocarcinoma which represents a poor prognostic marker. Prompt recognition of this complication is important for preventing serious complications associated with hypercalcemia and to improve the quality of life of these patients.
1447 A Rare but Fatal Case of N-methyl-D-aspartate Receptor Antibody Encephalitis Associated With a Pancreatic Neuroendocrine Tumor
Authors: Shim Hong Gi , Lee Raymond , Yacavone Robert F. 
Published: October 2019

INTRODUCTION:
N-methyl-D-aspartate receptor antibody (NMDAR-Ab) encephalitis is an autoimmune inflammatory condition of the brain thought to be paraneoplastic given its strong association with malignancy, but such related tumors are rarely detected in older males. To our knowledge, there is only a single case report of NMDAR-Ab encephalitis related to a pancreatic neuroendocrine tumor (NET). Here, we report a case of NMDAR-Ab encephalitis associated with pancreatic NET that was fatal despite early tumor resection.

CASE DESCRIPTION/METHODS:
A 79-year-old man with hypertension presented with acute onset of confusion, agitation, and urinary and bowel incontinence. He was living independently but had progressive short-term memory loss over the year prior. Electroencephalogram showed diffuse slowing without seizure activity. Magnetic resonance imaging of the brain noted trace sulcal FLAIR hyperintensity in perirolandic regions. Cerebrospinal fluid (CSF) analysis revealed elevated protein (96 mg/dL), low nucleated cell count, no oligoclonal bands, and negative infectious serology and cultures. CSF paraneoplastic panel was positive for anti-NR1 antibody, confirming the diagnosis of NMDAR-Ab encephalitis. Malignancy workup revealed a 1.2 cm mass in the pancreatic tail, and endoscopic ultrasound-guided fine-needle biopsy showed positive staining for synaptophysin and chromogranin A consistent with a well-differentiated NET. The patient rapidly declined from worsening encephalopathy and required percutaneous gastrostomy tube placement. He underwent laparoscopic distal pancreatectomy and splenectomy with intravenous immunoglobulin and glucocorticoids, but a repeat CT abdomen next month showed peritoneal metastases with diffuse uptake on somatostatin receptor scintigraphy and rising serum chromogranin A (603 ng/mL). He had no significant neurologic recovery and eventually died on comfort measures without further interventions.

DISCUSSION:
Though rare, prior cases of NMDAR-Ab encephalitis associated with NET described the importance of early surgical removal of the underlying tumor to provide the best chance of neurologic recovery. Metastatic disease portends a poor prognosis as it renders encephalitis impossible to resolve. Worldwide experience with NMDAR-Ab encephalitis is limited and further observations are needed to refine current treatment strategies.
1450 Acute Ischemic Pancreatitis Secondary to Superior Mesenteric Artery Stenosis: A Common Diagnosis Due to an Uncommon Etiology
Authors: Bajwa Maria , Bella Saif , Bank Leslie 
Published: October 2019

INTRODUCTION:
Pancreatic ischemia is a rare cause for acute pancreatitis which is poorly defined without specific radiological appearance.

CASE DESCRIPTION/METHODS:
We present a case of 57-year-female with 2-week history of abdominal pain. Her history included CHF, carotid stenosis, hypertension, COPD, and invasive Aspergillosis. She underwent cardiac catheterization 6 years ago which showed heavy atherosclerosis without obstruction. Her post procedure course was complicated by cholesterol embolization to left lower extremity. She was a heavy smoker with 1-2 PPD smoking but denied alcohol use. She was diagnosed with invasive Aspergillosis 2 months ago and initially treated with Voriconazole. It was switched to isavuconazole 1 month ago after developing blurring of vision. Patient reported decreased appetite since then with poor oral intake. She presented with 2 week h/o severe abdominal pain, associated with intense nausea and multiple episodes of vomiting. Her initial work up showed leukocytosis and elevated lipase >4000. CT abdomen showed diffuse, homogenous pancreatic enlargement with prominent aortic atherosclerosis and distal aortic aneurysm measuring 3.1 cm. Initially, the antifungal agent, isavuconazole was thought to be the culprit, causing pancreatitis but literature review was negative. IGg4 levels and lipid profile were unrevealing. Pancreatic ischemia was considered as the likely etiology. An abdominal duplex showed superior mesenteric artery stenosis. However, given h/o cholesterol embolization following cardiac catheterization, she was considered high risk for endo-vascular procedure. High intensity statin therapy was prescribed and she was discharged home with close outpatient follow up. Patient was strongly encouraged to quit smoking, as this would likely decrease her risk of major adverse cardiac event.

DISCUSSION:
Three well-identified causes of acute ischemic pancreatitis include hemorrhage, hypotension, & mesenteric vessel occlusion. Pancreatic ischemia leads to appearance of oxygen-derived free radicals which cause endothelial dysfunction, resulting in increased permeability and localized inflammatory response. Our patient had heavy calcified atherosclerosis. She was an active smoker. Dehydration also played a role in the development of ischemic pancreatitis, as the patient endorsed poor oral intake, confounded by nausea, vomiting and diarrhea. Treatment of acute ischemic pancreatitis consists of supportive care with IV fluids and pain control and restoration of pancreatic perfusion.