bluuebunny/crossref_metadata_2025_split huggingface dataset:
1305 A Genetically Rare Cancer Singled Out Just in Time: Pancreatic Neuroendocrine Tumor
Authors: Alegre-Boschetti Sergio M. , Betancourt-Guzmán Natalia , Diaz Carolina , De Jesús Henry , Fernández Carlos , Magno Priscilla 
Published: October 2019

INTRODUCTION:
Pancreatic neuroendocrine tumors (PNETs) are rare neoplasms that account for 1-2% of all pancreatic tumors. PNETs can manifest at any age, although, they occur commonly after the fourth decade of life. We herein report a patient diagnosed and managed for nonfunctioning PNET. It is important to acknowledge that these malignant tumors might have an insidious presentation.

CASE DESCRIPTION/METHODS:
46-year-old male patient presented with unintentional 25-lb weight loss over 8 months with associated symptoms of weakness and decreased oral intake. Patient without major medical history and family history of gastric cancer, as well as, breast cancer. His physical examination was unremarkable. Initial laboratory workup including CBC, CMP, serum CA-19-9, IgG4, serum chromogranin A and serum neuron specific enolase were unremarkable. CT scan of the abdomen and pelvis, ordered for evaluation of unexplained weight loss, demonstrated a large lobulated mass replacing most of the pancreatic body and tail with loss of cobblestoning with several distinct peripancreatic soft tissue nodules. PET/CT revealed severe hypermetabolic lesion involving the body and tail of the pancreas. An endoscopic ultrasound with fine-needle biopsy of the pancreatic mass (3 cm × 2 cm) revealed a PNET. Immunohistochemical panel such as CK7, synaptophysin, and chromogranin were positive and CD34 as well as Pan-CK were non-contributory. Patient underwent a successful distal pancreatectomy, splenectomy, and removal of regional nodes without any complications. Histology confirmed a well differentiated PNET intermediate-grade (G2) with 2-20 mitosis/2 mm2, Ki-67 index of 3%-20%, and the tumor only involved the pancreas. A somatostatin receptor scintigraphy scan, using octreotide, was normal without evidence of any somatostatin avid lesions. The patient is currently under the care of the Gastroenterology and Hematology & Oncology service.

DISCUSSION:
PNETs are generally indolent malignancies and clinical manifestations can vary from abdominal pain, weight loss, anorexia, nausea, jaundice, palpable mass on physical exam or symptoms attributed to metastatic disease. No risk factors like smoking, diabetes, nor history of chronic pancreatitis were identified. It is of great importance to raise awareness of malignancy symptoms. This case demonstrates how a complete history and physical exam can lead to early identification of a curable malignancy, having a positive impact on the patient survival and quality of life.
1314 Lemmel Syndrome in a Centenarian
Authors: Mirza Zeyn , Fernández Carlos , Tirado Francisco , Martin-Ortiz Jose 
Published: October 2019

INTRODUCTION:
Obstructive jaundice can be caused by intraluminal forces (choledocholithiasis), extraluminal forces (carcinoma) or transmural forces (choledochal cysts). In addition to the above, a rare cause of obstructive jaundice includes periampullary diverticula - Lemmel's syndrome. This syndrome involves a periampullary duodenal diverticulum that can compress the common bile duct resulting in obstructive jaundice. These diverticula are extraluminal and their prevalence increases with age and given that the majority are asymptomatic, they can be overlooked in imaging studies. It is estimated that 5% of patients experience acute symptomatology due to these diverticula.

CASE DESCRIPTION/METHODS:
This is the case of an over 90-year-old male with medical history of hypertension and Alzheimer's dementia who came to the ER due to right upper quadrant abdominal pain the day prior to admission. Physical wasexam remarkable for epigastric and right upper quadrant tenderness. Labs with transaminitis (ALT:1294 U/L, AST: 1166 U/L), alkaline phosphatase 366 U/L, total bilirubin 3.2 mg/dL, amylase: 123 U/L, lipase: 98 U/L). Abd/pelvic CT scan reported prominence of the intrahepatic and extrahepatic biliary system. The common bile duct measuring 8-9 mm, proximal pancreatic duct dilated approximately 4 mm, and the common bile duct extending to a large duodenal diverticulum without evidence of choledocholithiasis. Considering the patients advanced age, he was treated with conservative medical management including antibiotic therapy.

DISCUSSION:
This case highlights the importance of taking a holistic approach to patient care especially patients of advanced age who may have findings suggestive of a diagnosis that is overlooked as it is uncommon. The literature states that the detection rate of duodenal diverticula ranges from 1% to 27%. In addition, the majority of peri-ampullar diverticula are asymptomatic however 5% of cases can present with bleeding, jaundice or intestinal obstruction. Misdiagnosis of this condition may lead to erroneous management and delay in treatment in patients who are able to receive aggressive management.
1316 A Rare Congenital Anomaly Unveiled in an Octogenarian
Authors: Mirza Zeyn , De Jesús Henry , Estremera Rodolfo , Martin-Ortiz Jose , Toro-Lugo Doris 
Published: October 2019

INTRODUCTION:
Congenital agenesis of the gallbladder is a rare anomaly that occurs during embryonic development with an average incidence of 0.02% at birth. The clinical presentation is varied as patients may be asymptomatic; therefore, isolated gallbladder agenesis is usually discovered incidentally on imaging. Up to 50% of patients may present with symptoms similar to a biliary colic, such as right upper quadrant pain, nausea, vomiting and jaundice. We present an interesting case of an octogenarian who was first diagnosed with gallbladder agenesis after presenting with obstructive jaundice due to choledocholithiasis.

CASE DESCRIPTION/METHODS:
This is the case of an 88 year-old male with past medical history of hypertension and chronic kidney disease, presented to the emergency department with right upper quadrant abdominal pain, nausea and non-bloody vomits. He appeared acutely ill with pain in the right upper quadrant. Laboratory tests were remarkable for a cholestatic pattern. Total bilirubin 5.1 mg/dL, alkaline phosphatase 676  IU/L, AST 167 IU/L, and ALT 111 IU/L. There was prior history of exposure to Hepatitis A. Hepatitis B and Hepatitis C were negative. In view of the clinical presentation, an abdominal ultrasound was done. There was evidence of choledocholithiasis with extensive intra and extrahepatic duct dilation. At the gallbladder fossa, there was “WES sign” indicating a gallbladder full of stones. The patient was admitted to the hospital and underwent ERCP with a sphincterotomy with stones extraction. The gallbladder was not visualized. An abdominopelvic CT and HIDA scan confirmed its absence.

DISCUSSION:
Gallbladder agenesis is a rare entity and seldomly diagnosed for the first time in the ninth decade of life. It is estimated that 25%-50% of this subset of patients may develop choledocholithiasis. The association between these two entities is hypothesized to be similar to post-cholecystectomy syndrome: biliary dyskinesia may favor stone formation. In addition, hypertonic contractions of the sphincter of Oddi may lead to common duct dilation, biliary stasis and also predispose to calculi formation. This case provides an important point to increasing awareness of this condition to avoid misdiagnosis, unnecessary procedures and prolonged hospitalizations. In patients who present with biliary colic, jaundice with equivocal gallbladder imaging findings, the possibility of gallbladder agenesis should be entertained.
1318 Pancreaticopleural Fistula in a Patient With Recurrent Alcoholic Pancreatitis: Combined Endoscopic and Medical Management
Authors: Ferreira Kaio S. , Arsenault John J. , Pan Jason , Harmatz Alexander , Moss Steven F. 
Published: October 2019

INTRODUCTION:
Pancreaticopleural fistula (PPF) is a rare complication of pancreatitis, resulting from disruption of the pancreatic duct. This injury may be secondary to acute/chronic pancreatitis, trauma, or surgery. Here we describe a patient with a PPF, and our workup and treatment for this rare condition.

CASE DESCRIPTION/METHODS:
A 61-year-old male with COPD, alcohol misuse, and history of recurrent acute alcoholic pancreatitis complicated by pseudocysts was admitted with one month of worsening shortness of breath from a large left pleural effusion, which reaccumulated within four days after a 1.6 L thoracentesis. On examination he had mild abdominal tenderness in the RUQ and epigastrium. Labs were notable for serum amylase 1105 U/L, lipase >1000 U/L, and WBC 10.8. He underwent another thoracentesis, removing 1.5 L of fluid which was an exudate with amylase greater than 2000 U/L. CT of the chest and abdomen showed a subdiaphragmatic LUQ fluid collection with communication to the pancreatic body and duct. A chest tube was then placed to prevent fluid re-accumulation. MRCP confirmed a PPF from the proximal pancreatic body duct to the left pleura. He was started on octreotide. ERCP confirmed a fistula originating from the distal pancreatic duct but attempts to pass a wire into the dorsal pancreatic duct were unsuccessful, and a ventral pancreatic sphincterotomy was performed. His chest tube was removed after one day, and he was discharged to continue octreotide at home. Unfortunately, he returned the following day with complaints of dyspnea. Pancrelipase was then added in an attempt to minimize pancreatic exocrine secretions. On follow-up 1 and 2 months later, the pleural fluid is much smaller, and the patient's dyspnea has resolved.

DISCUSSION:
Here we present a novel management of PPF without pancreatic duct stent placement and instead with pancreatic sphincterotomy to divert pancreatic secretions away from the fistula tract and medical therapy (octreotide and pancrelipase) to reduce the pancreatic secretion. The diagnosis was reached through a combination of clinical history, lab studies (particularly, the elevated amylase in the pleural fluid), and CT and MR imaging. This was further confirmed with ERCP, which is useful especially if a stent can be placed to divert secretions away from fistula tract. Ultimately, this diagnosis requires a high index of suspicion, particularly in patients who present with recurrent effusions in the setting of pancreatitis.
1320 Out of Gas: A Rare Case of Spontaneous Hepatic Portal Venous Gas
Authors: McDonald Emmanuel , Pacha Faris , Skrove Jamie L. , Rana Zaid , Roberts Carey , Billari William , Rocher-Gomez Maray , Sobrado Javier 
Published: October 2019

INTRODUCTION:
Hepatic Portal Venous Gas (HPVG) was first described between 1955-1960 and is identified by the presence of tubular, branching translucencies in the non-dependent part of the liver, distributed within 2 cm of the periphery owing to centrifugal blood flow. The exact mechanism for the formation of gas in the portomesenteric venous system is still unclear. Bowel ischemia is the primary etiology of HPVG (70% cases), and when together they are related to transmural necrosis in 91% and mortality in 85%. Thus HPVG is an indication for surgery in the presence of mesenteric ischemia.

CASE DESCRIPTION/METHODS:
A 69-year-old male with a history of atrial fibrillation and congestive heart failure presented to the ER complaining of weakness, palpitations and dark stools. On physical exam, he was found to have BRBPR and tachycardia with an irregularly irregular rhythm. Days in to the admission, the patient was found to be hypotensive with sinus bradycardia and acutely altered. He was intubated and paced. CT scans showed portal venous gas within the superior mesenteric vein and right common iliac vein, causing hepatic portal vein gas, suggestive of ischemic disease, which led to a recommendation for surgery. A repeat showed thickened loops of ascending, transverse and proximal descending colon in the distribution of the superior mesenteric artery along with free air in the abdomen anteriorly. A colonoscopy showed an extended area of colitis in the ascending colon, transverse colon, splenic flexure, and descending colon with erythema and ulceration concerning for ischemic colitis. Multiple biopsies showed inflammatory related ulceration. Based on the clinical improvement of the patient, medical management was recommended. A repeat CT of the abdomen revealed spontaneous resolution of the hepatic and biliary pneumatosis. Upon recovery, the patient denied previous history of sphincterotomy or any hepatic or biliary manipulation.

DISCUSSION:
HPVG can be a diagnostic sign, indicating serious intra-abdominal pathology requiring emergent surgical intervention in patients. As CT scans and ultrasound modalities increase, we have the capability to detect severe disease earlier, thus allowing immediate treatment and reducing mortality rates. As with this case, the need for surgical intervention is becoming questionable, even in the presence of ischemia. As clinicians we must be abreast of all information and treatment options. Medical management may allow for less complications and shorter recovery times.
1323 Hemosuccus Pancreaticus Secondary to Hepatic Artery Pseudoaneurysm Presenting as Acute Gastrointestinal Bleed
Authors: Fischer Kyle , Jordan Melissa , Frandah Wesam 
Published: October 2019

INTRODUCTION:
Hepatic Artery Psuedoaneurysms (HAPs) are typically caused by iatrogenic injury from instrumentation during invasive procedures or trauma. Psuedoaneurysms are described as a disruption/tear through all three layers of the arterial wall, with a fibrous capsule intact. Detection of HAP is important, as the rupture rate is around 76%.

CASE DESCRIPTION/METHODS:
A 77-year-old male with PMH of chronic pancreatitis with pseudocyst formation and necrotizing pancreatitis presented to the emergency department with 36 hours of black stools and diffuse abdominal pain. Admission labs showed a hemoglobin of 11.4 gm/dl, AST of 113 U/L, ALT 197 U/L, lipase 535 U/L. Given his past history and acute transaminitis, CT abdomen/pelvis was completed and showed 1.7 × 1.5 cm pseudoaneurysm of the common hepatic artery (Figure 1). Melena progressed to frank bright blood in the stools. Hemoglobin dropped to 9.7 gm/dl. Given these findings, vascular surgery and gastroenterology were consulted. He was started on Protonix and Octreotide and over the next two days, his hemoglobin stabilized but continued to have melanotic stools. Patient underwent esophagogastroduodenoscopy (EGD) which was negative for bleeding. Given the HAP and lack of findings during the EGD, he was taken for Hepatic artery stenting (Figure 2). Plavix was initiated post stenting. Post stenting his melena resolved. Patient was sent home with follow up as outpatient and CT scan in 1 month. CT abdomen completed 1 month after stent placement showed a patent stent within the common hepatic artery without an extravasation of contrast into previous pseudoaneurysm.

DISCUSSION:
This case illustrates the importance of keeping a broad differential in a patient with complex past gastrointestinal history, presenting with a common chief complaint. In the case presented, it is hypothesized that the origin of his HAP was secondary to his chronic pancreatitis leading to hemosuccus pancreaticus resulting in acute gastrointestinal bleed. This case was unique in that most hemosuccus pancreaticus involve splenic artery pseudoaneurysm instead of HAP. Overall, identification of HAP is important given the high risk of rupture and mortality rate ranging from 25-70%. Definitive treatment surgically or endovascular is necessary for all HAPs. As presented in the case, diagnosis of HAP is critical as it can be the source of a sentinel bleed in patients presenting with an acute gastrointestinal bleed and should be considered in the differential for all gastrointestinal bleeds.
1327 Gallstone Ileus Leading to Small Bowel Perforation: Case Report
Authors: Baldwin Nicholas , Shoreibah Mohamed , Collins Hampton 
Published: October 2019

INTRODUCTION:
Gallstone ileus (GI) is a rare but potentially lethal complication of cholelithiasis. It is only responsible for 0.5% of mechanical small bowel obstructions in the general population; but it disproportionately affects the elderly female population, causing roughly 25% of mechanical small bowel obstruction in females over age of 65. Stones must be at least 2-2.5 cm to result in bowel obstruction, typically lodging in the terminal ileum. In rare cases, larger stones can become lodged elsewhere within the bowel, leading to severe complications such as bowel perforation.

CASE DESCRIPTION/METHODS:
A 77-year-old male with a 6-month history of weight loss presented with non-bloody, nonbilious emesis without abdominal pain three days after an admission for an empyema. Physical exam was notable for a mildly tender abdomen upon presentation without rebound or guarding. He was treated with anti-emetics and IV fluids with mild improvement in symptoms. Liver function panel was significant for alkaline phosphatase of 411 U/L (normal 37-117), while total bilirubin was 0.4 (normal 0.3-1.4). Ultrasound of the abdomen revealed pneumobilia which was previously seen six months earlier on a CT abdomen. Subsequent MRCP revealed a large gallstone in the distal ileum with proximal dilation of the small bowel. Nasogastric decompression was initiated, and patient began passing flatus and had small bowel movements. Later that evening, the he started retching and had severe abdominal pain with rebound tenderness and lactic acidosis (LA 3.1). He became hypotensive and was taken for an emergent laparotomy where a 4 cm gallstone was found impacted in the mid ileum and removed along with 6 cm of perforated small bowel.

DISCUSSION:
Presenting symptoms of gallstone ileus are often non-specific, frequently leading to a delay in diagnosis and treatment. Although no fistula was identified in this patient by imaging, the most common cause of gallstone ileus in most patients is formation of a cholecystoduodenal fistula. This is thought to result from inflammation associated with cholecystitis along with pressure from a large stone within the gallbladder itself. Nonemergent treatment for larger stones often includes more invasive treatment, such as enterolithotomy or enterotomy with stone extraction. However, as we see in this case, obstructing stones can result in bowel perforation, requiring emergent small bowel resection or even death.
1329 Choledochocele: An Unlikely Culprit of Acute Relapsing Pancreatitis
Authors: Collins Kelsey , Chuang Philip , Rozenshteyn Frederick , Constantino Joseph , Kasmin Franklin , McCabe Evin 
Published: October 2019

INTRODUCTION:
Choledochocele, a type III biliary cyst representing 1-5% of all biliary cysts, is a congenital or acquired cystic dilatation of the intraduodenal bile duct more commonly seen in patients of Asian descent. Patients are usually asymptomatic, but can occasionally present with abdominal pain, jaundice, and a palpable abdominal mass; with malignancy less frequently seen than in other types of biliary cysts. Acute, relapsing pancreatitis is an uncommon presentation. We present a case of recurrent pancreatitis secondary to a choledochocele.

CASE DESCRIPTION/METHODS:
An 84-year-old woman with a history of DM, HTN, and recurrent gallstone pancreatitis s/p cholecystectomy 2 weeks prior presented with generalized abdominal pain for 1 day. She was febrile to 100.2, with tenderness to palpation of the epigastrium. Lipase was elevated to 1388. CT Abdomen/Pelvis was remarkable for mild common bile duct (CBD) dilation with bulging into the ampulla, a cystic structure in the uncinate process measuring 2.1 cm, and edematous changes in the peripancreatic space around the head and uncinate; consistent with pancreatitis. An MRCP was obtained, and showed no evidence of choledocholithiasis, dynamic bulbous dilatation of the distal CBD at the ampulla of unclear etiology, and interval enlargement of a 2.1 cm simple-appearing pancreatic uncinate cyst. She underwent EUS, which was significant for a choledochocele; followed by ERCP with sphincterotomy and unroofing of the choledochocele with a needle knife, as well as pancreatic duct (PD) stent placement. She was then discharged with follow-up but returned 6 days later, with progressively worsening RUQ pain with nausea and vomiting. Imaging revealed a 2.8 cm peripancreatic fluid collection associated with pancreatic stranding and PD stent dislodgement. She had EUS and FNA of the pancreatic cyst, which showed infected pancreatic necrosis. She improved with Vancomycin and was soon discharged home with outpatient follow-up.

DISCUSSION:
Choledochocele is an uncommon cause of acute, relapsing pancreatitis, which can cause significant pancreatic pathology over time. If the cyst becomes symptomatic, treatment is required. Since first described by J Siegel in 1981, first-line management has involved sphincterotomy with unroofing. In cases of large >3 cm cysts, or cysts that did not respond to endoscopic management, surgery must be considered.
1331 Choledochal Cyst in an Adult Female
Authors: Thurber David , Mossad David , Krishnamurthy Padmini 
Published: October 2019

INTRODUCTION:
The incidence of biliary cyst (BC) is 1:100,00 to 150,000 making it a rare medical condition. BC are most commonly diagnosed in the pediatric population but can occasionally present in adults. A multi-disciplinary team approach including a gastroenterologist and a hepatobiliary surgeon is ideal in treating patients with this diagnosis.

CASE DESCRIPTION/METHODS:
We present a case of a 38-year-old female with past medical history significant for gastroesophageal reflex disease and migraine who was admitted for evaluation of worsening epigastric pain. Patient was noted to be one week after a total abdominal hysterectomy with bilateral salpingo-oophorectomy. A comprehensive metabolic panel was ordered on admission displaying liver chemistries elevated with AST of 258 IU/L (Ref: 9-45 IU/L), ALT of 260 IU/L (Ref: 17-63 IU/L), alkaline phosphatase of 230 IU/L (45-117 IU/L) and total bilirubin of 3.2 mg/dl (Ref: 0.2-1.0 mg/dl). The patient obtained a RUQUS, which did not show any gallbladder stones or sludge. Patient was still having pain throughout her admission, so a magnetic resonance cholangio-pancreatography (MRCP) was ordered for further evaluation. The MRCP revealed a fusiform dilation of proximal half of the common bile duct (CBD) with a diameter of 15 mm and normal intrahepatic ducts. The pancreatic duct could not be visualized on MRCP and hence unable to comment on length of common channel. There was no evidence of stone or stricture in the CBD. At this point, the patient was diagnosed with Type I choledochal cyst. Patient then underwent a laparoscopic extrahepatic bile duct excision with Roux-en-Y hepatico-jejunostomy and cholecystectomy. On follow up evaluation, the patient had been doing well with normal liver chemistries.

DISCUSSION:
Although rare, choledochal cysts can cause many complications and need to be treated as soon as possible. These complications are due to bile stasis and/or reflux of pancreatic enzymes into the CBD resulting in recurrent cholangitis and other problems including CBD strictures, rupture, stones and secondary biliary cirrhosis. Additionally, there is an overall 10-30% risk for cholangiocarcinoma in patient's with this diagnosis. This case is a good example of successful treatment of a choledochal cyst in a young female.