bluuebunny/crossref_metadata_2025_split huggingface dataset:
1398 A Stone in the Wall: Extraction of an Embedded Choledocholithiasis in the Proximal CBD by Cholangioscopy
Authors: Alsheikh Mira , El Douaihy Youssef , Andrawes Sherif , Haddad Fady , Kamar Khalil 
Published: October 2019

INTRODUCTION:
Primary choledocholithiasis are stones formed in the common bile duct as opposed to secondary stones when they originate in the gallbladder and pass through. Symptomatic choledocholithiasis are usually managed by cholecystectomy but the CBD stones post cholecystectomy are not uncommon. The rate of recurrence of symptomatic CBD stone range from 4% to 24%. Having a stone impacted in the wall has never been reported before.

CASE DESCRIPTION/METHODS:
A 74-year-old man with a history of cholecystectomy for symptomatic cholelithiasis presented for one-year history or right upper quadrant colicky pain. He had recurrent CBD stones for four years before presentation. He underwent multiple endoscopic retrograde cholangiopancreatography (ERCP) with sphincterotomy and stones removal multiple times. He had a CBD stent. Medical history is also relevant for atrial fibrillation on rivaroxaban and CAD on aspirin. Upon initial evaluation vitals were stable, physical examination was only relevant for RUQ abdominal tenderness. Imaging including computed tomography scan and an ultrasound revealed stable CBD dilation at 1.1 cm with no stones or intrahepatic biliary duct dilation. ERCP was performed with a cholangiogram showing multiple small filling defects in the CBD consistent with small stones. Sludge and stones were removed using a biliary stone extraction balloon. The duct was swept multiple times and no more stones were noted. The pain did not resolve. A repeat ERCP with a cholangioscopy using the spyglass system was performed. It revealed a normal CBD ductal walls, however closer to the bifurcation of the cystic duct, a small stone was noted; it was impacted in the wall. It was retrieved using a spyglass forceps.

DISCUSSION:
CBD stones are common after cholecystectomy. They are formed in the bile duct in situ and usually occur 12 months after surgery. Risk factors are the presence of multiple CBD stones and type 1 or 2 periampullary diverticulum. Cholangioscopy is sometimes needed as in our case to check for any stone that is impacted or left in the proximal common bile duct.
1402 Metastatic Merkel Cell Carcinoma With Isolated Pancreatic Metastases
Authors: Lingamaneni Prasanth , Vohra Ishaan , Upadhyay Shristi , Farooq Muhammad Zain , Gupta Shweta 
Published: October 2019

INTRODUCTION:
Merkel cell carcinoma (MCC) is a rare neuroendocrine skin malignancy. It has a high risk of nodal metastases (25%) but distant metastases are uncommon (8%). Liver and lung are the most common metastatic sites reported. Pancreatic metastases are uncommon and are associated with other sites of metastases. We report a rare case of Merkel cell carcinoma with isolated pancreatic metastases.

CASE DESCRIPTION/METHODS:
A 53-year-old Caucasian man presented with a 9-month history of an enlarging crusting lesion on the anterior chest wall. Physical examination showed a 15 × 20 cm crusty erythematous skin lesion in the upper mid chest. He also had palpable right axillary and left pre-clavicular lymphadenopathy. He underwent wide local excision with bilateral axillary and left neck lymph node dissection. Surgical pathology revealed node positive stage III MCC with a focus of squamous cell carcinoma. All margins were negative. He was started on adjuvant Radiation Therapy (RT) after discussion in the multidisciplinary tumor board but presented after 1-fraction with new upper abdominal discomfort. A CT scan showed areas of hypoattenuation in the pancreas with enlarged peri-pancreatic lymph nodes (Figure 1). EUS showed similar findings (Figure 2). EUS guided FNA was consistent with MCC. He underwent six cycles of chemotherapy with cisplatin/etoposide with complete resolution of the pancreatic masses and lymphadenopathy (Figure 3). However, 1 month later he passed away due to sudden cardiac arrest.

DISCUSSION:
Merkel cell carcinoma (MCC) is a neuroendocrine neoplasm of the skin, arising from Merkel cells at dermo-epidermal junction. It is a rare cancer with incidence of 0.7 per 100,000 persons. It predominately effects elderly, light-skinned and immunocompromised people. Merkel cell polyomavirus may play a role in the pathogenesis. Common sites of distal involvement include liver, lungs and bone. Pancreatic metastasis is rare with only a few cases reported, most of which were associated with other sites of metastases. EUS is a less-invasive means of confirming diagnosis and ruling out pancreatic adenocarcinoma. There is no standard modality of management for MCC with pancreatic metastasis. Chemotherapy, radiation therapy, surgical removal and immunotherapy with PD-L1 inhibitors are options for management. Overall prognosis is poor, with median survival around 7.5 months. Our patient responded very well to therapy but passed away from cardiac arrest 9 months after initial diagnosis.
1403 Portal Vein Compression in Light of Chronic Pancreatitis
Authors: Hoskere Dhanush , Ramasamy Kovil , Dedousis John , Tsompanidis Antonios 
Published: October 2019

INTRODUCTION:
In patients with pancreatitis and pancreatic pseudocysts, the risk of splenic vein compression is well known. The portal vein is a rare location for compression. Here, we have a rare case of a Pakistani male found to have portal vein compression by the pancreatic head.

CASE DESCRIPTION/METHODS:
44-year-old Pakistani male with past medical history of cirrhosis, chronic pancreatitis with surgical resection of pancreatic pseudocysts, diabetes mellitus type II, and cholecystitis with cholecystectomy presented with worsening sharp periumbilical abdominal pain, nausea, and non-bloody/non-bilious vomiting for 3 days. Pain was exacerbated by food consumption and relieved with tramadol. Patient denied associated fever, chills, and diarrhea. Physical exam findings included soft abdomen with diffuse abdominal tenderness worse in the epigastric and right upper quadrant areas. Patient had voluntary guarding. Patient did not have scleral icterus or jaundice on exam. Laboratory findings included leukocytosis of 12.4, elevated lactate at 3, elevated triglycerides at 823, unremarkable lipase at 160, and unremarkable bilirubin at 1.5. Patient had unremarkable AST, ALT, and alkaline phosphatase. Patient was treated for chronic pancreatitis by restarting home pancrelipase and slowly progressing diet. Abdominal CT showed markedly abnormal pancreas including inflamed pancreatic head and body with pseudocyst formation present. The portal vein was compressed by the pancreatic head. Recommendation for patient was for portal venous stenting, however, patient did not want intervention at that time.

DISCUSSION:
Portal hypertension is a serious condition, leading to complications including varices, ascites, and hepatic encephalopathy. Cirrhosis is the most common cause of portal hypertension in the western world. In developing countries, there is a higher incidence of extrahepatic causes, such as external compression. The splenic vein is the most common location for extrinsic compression; however, this case demonstrates the rare incidence of portal vein compression. Thus, the risk of compression as the cause of portal hypertension must be taken into consideration in the immigrant population. In addition, this patient did not exhibit symptoms significant for portal hypertension complications. Since many of these patients are asymptomatic in relation to portal hypertension, these patients should be screened for portal vein compression to help prevent complications that can arise from portal hypertension.
1404 Sclerotic Bone Metastasis From Cholangiocarcinoma: A Case Report
Authors: Shah Hardikkumar , Barham Shaker , Spira Etan 
Published: October 2019

INTRODUCTION:
Cholangiocarcinoma (CCA) account for 3% of gastrointestinal malignancies. Commonly it metastasizes to liver, peritoneum and lymph nodes. Rarely it metastasizes to bone with lytic lesions. We are presenting a case of metastatic CCA with sclerotic bone metastasis.

CASE DESCRIPTION/METHODS:
59-year-old female with no medical history presented with diffuse abdominal pain for 2 weeks. She denied other complaints, illicit drug use, personal or family of history of malignancy. She was a 40-pack year smoker and social drinker. Vitals and physical exam were unremarkable except minimally diffuse tender abdomen. Labs revealed elevated AST, ALT, ALP and Bilirubin which were 524, 640, 2300 and 1.7 respectively. CT abdomen with IV contrast showed intrahepatic biliary ductal dilatation and prominence of the common hepatic duct with abrupt tapering of the duct proximal to a small, high density mass versus short segment ductal wall thickening, normal Pancreatic duct and widespread sclerotic bone lesions. MRCP showed 3 cm long nonspecific stricture involving the mid CBD regional to the insertion of the cystic duct. No associated mass or abnormal enhancement with moderate intrahepatic biliary duct dilatation in both liver lobes. CA 19-9, CEA, and AFP were elevated to 492, 700, and 7 respectively. Bone scan showed extensive diffuse increased activity over multiple focal areas within the calvarium, sternum, bilateral ribs, scapulae, entire spine, pelvis, diffusely involving most femurs. ERCP with spyglass was done along with brush biopsy and metal stent placement. Biopsy samples were not adequate for tissue diagnosis. Iliac crest biopsy was sent for pathology, immunohistochemistry (IHC), flow cytometry and chromosome analysis which showed metastatic adenocarcinoma, by IHC (pankeratin+ CK7+, CK20+, MOC31+, CK19+, CA19-9+) most compatible with pancreatobilliary primary. Flow cytometry did not show any evidence of myeloproliferative disorder and chromosome analysis could not be done due to nondividing cells. Pt underwent palliative chemotherapy for metastatic CCA with gemcitabine and cisplatin.

DISCUSSION:
Metastatic CCA to the bone is rare. In the few case reports describing this finding, bony lesions are usually lytic in nature. Sclerotic bone metastasis from CCA has never been reported in literature. Prognosis is extremely poor due to the minimal response to conventional chemotherapy or radiotherapy, symptom control, particularly pain, is the mainstay of treatment for improving the quality of life of the patients.
1407 Foreign Body Erosion into the Biliary Tree—How to Avoid a Hepaticojejunostomy
Authors: Ghattu Meghana , Rolston Vineet , Lee Ann Y. , Bryk Tammy , Ho Sammy , Tzimas Demetrios 
Published: October 2019

INTRODUCTION:
Cholecystectomy remains one of the most common surgical procedures in the United States with a rate of greater than 750,000 annually. Of these cases, approximately 1-2% are complicated by a bile leak. Although the rate of bile leaks is relatively low, the morbidity and mortality can be very high with patients rapidly deteriorating after presentation usually due to sepsis. Endoscopic retrograde cholangiopancreatography (ERCP) can detect greater than 95% of leaks and provide therapeutic intervention aimed at eliminating the pressure gradient across the Sphincter of Oddi which promotes the flow of bile into the duodenum to heal the injured portion of the biliary tree. This case aims to show the significant clinical benefit of endoscopic management of bile leaks, although with the novel use of direct cholangioscopy.

CASE DESCRIPTION/METHODS:
This is a case of a 45-year-old woman with no significant past medical history who presented to an outside hospital with cholecystitis and underwent open cholecystectomy. Surgery was complicated by duodenal injury which was primary repaired and a Jackson-Pratt (JP) drain was left in place. Bilious fluid returned from the JP drain on post-operative day two and she underwent an ERCP which confirmed a bile leak and a sphincterotomy was performed and a stent was placed. She was referred to our center for stent removal four months later and at the time her cholangiogram was negative for leak so a stent was not replaced (Figure 1). Within a week of the procedure she had bilious return from the JP drain and a tube study preformed via the JP drain was read as a “normal T-tube” cholangiogram (Figure 2). As there was suspected erosion of the JP drain into the biliary tree and high concern for creating a biliary injury when removing the drain, direct cholangioscopy was used for safe removal and identification of the leak (Figure 3). The biliary injury was sealed with a fully covered self-expanding metal stent with excellent results and no further evidence of ongoing leak.

DISCUSSION:
ERCP remains the preferred treatment option for bile leaks and prevents patients from having to undergo corrective operation.4 In this case there was high concern for creating a biliary injury when removing the drain, thereby direct cholangioscopy was used for safe removal and identification of the leak. To our knowledge this is the first report of JP drain erosion into the CBD with use of direct cholangioscopy and ERCP as endoscopic management for removal and treatment of biliary injury.
1411 A Rare Cause of Acute Pancreatitis in a Transgender Female
Authors: Shipley Lindsey C. , Steele David T. , Wilcox Charles M. , Burski Chad M. 
Published: October 2019

INTRODUCTION:
While hypertriglyceridemia and certain medications are known causes of acute pancreatitis, estrogen resulting in secondary hypertriglyceridemic acute pancreatitis (HTG-AP) is rare. To our knowledge, this is only the third case of a transgender female presenting with acute pancreatitis secondary to estrogen-induced hypertriglyceridemia.

CASE DESCRIPTION/METHODS:
A 31-year-old transgender female presented to the hospital with epigastric pain, fever, chills and decreased appetite. Laboratory work-up was significant for lipase >7,500 u/L, triglycerides >7,000 mg/dL and calcium of <4 mg/dL. The patient denied alcohol use, diabetes, hypothyroidism or family history of pancreatitis. Initial management included aggressive intravenous fluid resuscitation. CT scan of the abdomen was performed and was notable for a large peripancreatic fluid collection most consistent with walled off necrosis (Figures 1 and 2). Three retroperitoneal drains were placed into the fluid collections with clinical improvement. Upon further questioning, it was discovered that several months prior to admission, the patient had started taking oral estrogen therapy obtained via an online store to transition from male to female, which was ultimately felt to be the most likely cause of her very severe hypertriglyceridemia. Two weeks after discharge, patient had IR drain exchange and repeat CT scan showed almost complete resolution of the fluid collections and her drains were removed.

DISCUSSION:
Medications are often described as the etiology for why people develop pancreatitis. However, to clearly prove a medication as the inciting factor, reinitiation of that medication needs to occur in order to establish a temporal relationship. Estrogen is a rare cause of drug-induced pancreatitis with approximately 40 cases reported worldwide. It is one of the few medications that has a described mechanism, hypertriglyceridemia, for the development of pancreatitis. Of the 40 cases described only 2 published case reports involve transgender females (7) (Table 1). The number of transgender patients presenting to the healthcare system for care is growing (9.2 per 100 000). Importantly, transgender females require supraphysiologic doses of estrogen therapy to maintain secondary sexual characteristics. Accounting for these factors, it is important for healthcare providers to understand their risk for and ways to prevent acute pancreatitis from occurring in their transgender patient population (8).
1413 Twelve-Year-Old Male With Esophageal Dysphagia: A Diagnostic Dilemma!
Authors: Shipley Lindsey C. , Talathi Saurabh , Wilson John T. , Ahmed Ali M. , Tipnis Neelesh 
Published: October 2019

INTRODUCTION:
Esophageal dysphagia is defined as any difficulty or abnormality of swallowing with delay in transit of solids or liquids in the esophageal phase. We present an interesting case of a young male evaluated for esophageal dysphagia and failure to thrive who was, after an extensive workup, found to have an esophageal duplication cyst.

CASE DESCRIPTION/METHODS:
A 12-year-old male was transferred from a local hospital with a 4-week history of sub-sternal pain, worsening dysphagia, odynophagia, a sensation of “food stuck in his chest” and an 8-pound weight loss. He was seen by his primary physician and started on ranitidine for possible gastrointestinal reflux disease (GERD) without improvement in his symptoms. He was admitted a week later due to worsening symptoms, new onset nausea, non-bloody, non-bilious vomiting and a fever (102.5 F). Due to persistent symptoms, an upper gastrointestinal series with contrast was performed, which was concerning for an esophageal diverticulum, an esophageal manometry and ultimately, esophagogastroduodenoscopy (EGD). An EGD revealed congested and friable mucosa throughout the esophagus, and a large opening in the posterior aspect of the middle esophagus. Due to inability to intubate this opening with a regular size endoscope, a neonatal upper endoscope was used. The opening was intubated, and the esophageal cyst was visualized (Video 1). Histology revealed normal basal thickness, papillary congestion and focal hemorrhage in the proximal esophagus. Due to parental preference, the patient was referred to another institution for a second opinion, where patient received fenestration of the cyst and is doing well.

DISCUSSION:
Esophageal duplication cysts are congenital abnormalities that arise during embryologic development and uncommonly present as dysphagia in an adolescent. Within the GI tract, only 2% arise from the esophagus. Duplication cysts often present as a diagnostic dilemma and thus, a high index of suspicion is needed. If a patient is not responding to standard therapy for esophageal dysphagia or has recurrent respiratory symptoms/infections, it may be necessary to pursue imaging or an endoscopic procedure. Alarm signs indicating a need for advanced diagnostics include failure to thrive, iron deficiency anemia, hematemesis, hemoptysis, weight loss or unresponsiveness to medical therapy. Larger multicenter prospective trials are needed to evaluate the utility of endoscopic ultrasound in both diagnostic and therapeutic management of esophageal duplication cysts.
1414 Prognosis and Management of Rare Pancreatic Tumors: A Case Series to Highlight the Importance of Accurate Pathologic Diagnosis in Pancreatic Malignancy
Authors: Enslin Sarah , De Las Casas Luis , Kaul Vivek 
Published: October 2019

INTRODUCTION:
Most pancreatic tumors are adenocarcinomas. Less than 6% of all pancreatic cancers are anaplastic (undifferentiated, ACP). ACPs with osteoclast-like giant cells (OGC) occur in less than 1% of all tumors and are classified separately from other subtypes of ACP by the WHO. Here we present 2 rare & unusual histologic subtypes of pancreatic cancer, both diagnosed by EUS-FNA/FNB, with varying prognosis & management.

CASE DESCRIPTION/METHODS:
Case 1: An 81-year-old male presented with indigestion and 10-pound unintentional weight loss. Abdominal CT scan showed a 6.6 cm pancreatic body-tail mass with splenic artery encasement and splenic vein occlusion. EUS confirmed the lesion with internal necrotic/cystic areas and celiac trunk involvement. FNA was performed with a 22-gauge needle; cytology confirmed undifferentiated carcinoma with osteoclast-like giant cells (OGC) (Figure 1). PET/CT revealed a hypermetabolic pancreatic lesion (SUV 21) and a 1.5 cm right hepatic dome lesion (SUV 10.2). He was deemed not a surgical candidate and was treated with palliative chemotherapy (gemcitabine). Restaging CT scan showed marked tumor progression with a 17 cm pancreatic tumor and a 4 cm liver tumor. He entered hospice less than three months after diagnosis. Case 2: A 74-year-old male presented with abdominal pain and weight loss. Laboratory evaluation revealed total bilirubin of 1.00 mg/dL, AST 40 U/L, ALT 87 U/L, and alkaline phosphatase 138 U/L. CT scan showed a pancreatic head mass measuring 4.3 cm abutting the portal vein. Tumor markers were normal. EUS revealed a 4.5 cm pancreatic head lesion with portal vein invasion and double duct sign. FNB was performed with 22-gauge needle; histology revealed anaplastic giant cell carcinoma (ACP) (Figure 2). After multidisciplinary discussion, pancreaticoduodenectomy was recommended. Intraoperatively, he was found to have unresectable locally advanced tumor. Palliative chemotherapy was started.

DISCUSSION:
OGCs & ACPs are rare histological subtypes of pancreatic cancer with aggressive clinical behavior. In both our patients, there was evidence of vascular and/or intraductal invasion by tumor at diagnosis. Data suggests OGCs may have a better prognosis than ACPs and ductal adenocarcinomas. Pancreas cancer multidisciplinary teams should be aware of these rare tumor subtypes and the treatment and prognostic implications thereof.
1422 Pancreatogenic Diabetes (Type 3C) Presenting With Diabetic Ketoacidosis
Authors: Melki Gabriel , Laham Linda , Karim Gres , Komal Fnu , Barham Shaker , Hanna Maggie , Yuridullah Ruhin , Vora Aarohi , Grossman Matthew , Baddoura Walid 
Published: October 2019

INTRODUCTION:
Diabetes type 3c (DM3c) accounts for 5% of diabetics in the US. It is due to chronic pancreatitis leading to the destruction of islet cells. Glucagon and insulin levels are both decreased due to destruction of alpha and beta cells, respectively. This makes the development of diabetic ketoacidosis (DKA) a rare occurance in these patients, as glucagon is one of the key hormones in the production of ketone bodies. We report a case of Type 3C diabetes presenting with DKA.

CASE DESCRIPTION/METHODS:
A 50-year-old male with chronic alcoholic pancreatitis s/p pseudocyst drainage presented to the ED complaining of epigastric pain for two months. The patient was on oxycodone for pain control and pancreatic enzymes. V/S: BP 100/92, HR 94, RR 16, and O2 sat 97%. Physical exam was notable for epigastric tenderness. Prior imaging studies revealed diffuse pancreatic calcifications. Cardiac enzymes were normal and lipase level was 156. Blood glucose level was notably elevated at 703. Other lab abnormalities included serum bicarbonate of 16, ketones in the urine and acetone in the blood, anion gap of 27 and HbA1C of 16% (prior values were normal). The patient was admitted to the medical ICU for management of DKA. He was started on IV fluids and IV insulin. An autoimmune workup for diabetes was negative. Once anion gap closed, patient was transitioned to SC insulin and subsequently discharged.

DISCUSSION:
DM3c involves inflammation of the pancreatic islet cells with irreversible fibrotic changes, due to the underlying chronic pancreatitis. Early on, the beta cells and the pancreatic polypeptide cells are affected, but as the disease progresses, glucagon secreting alpha cells are destroyed, making it rare for these patients to present in DKA. However, an upregulation of cortisol, growth hormone and catecholamines make ketone production possible. Due to exocrine pancreatic deficiency, incretin secretory function is lost; thus pancreatic enzyme supplementation can be beneficial for glycemic control. Diagnosing DM3c requires imaging evidence of chronic pancreatitis, pancreatic exocrine insufficiency, glucose intolerance and absence of type 1 DM autoimmune markers. Recognition of DM3c is essential for optimal management of these patients.