bluuebunny/crossref_metadata_2025 huggingface dataset:
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.
1333 Not so Small Biliary Obstruction: A Case of Small Cell Carcinoma of the Ampulla of Vater
Authors: Gomez Paul , Naim Alan , Zucker Kelly , Kiafar Camron , Gomez Adam
Published: October 2019

INTRODUCTION:

Small cell carcinomas commonly arise in the lung, but can also originate in extrapulmonary sites. Extrapulmonary small cell carcinomas are rare and are most frequently found in the female genital tract, gastrointestinal tract and genitourinary tract. Small cell carcinoma of the ampulla of Vater is exceedingly rare, and there are currently less than 15 documented case reports. The neuroendocrine features of small cell carcinoma of the lung and extra pulmonary sites appear to be similar.

CASE DESCRIPTION/METHODS:

64-year-old male with past medical history of hypertension and coronary artery disease presented to our hospital with weight loss and jaundice. At time of admission, labs demonstrated AST 114 U/L, ALT 168 U/L, alkaline phosphatase 715 U/L, total bilirubin 5.8 mg/dL (Dbili 4.5), INR 1.1, platelet 271 k/mm3, and albumin 3.5 g/dL. Notable findings on physical exam included jaundice and scleral icterus. Ultrasound of the biliary tree showed no visible stone. ERCP showed an ulcerated, bleeding ampullary mass. Subsequent cholangiogram revealed a distal bile duct stricture measuring approximately 1.5 cm with shelf effect from extension of ampullary mass into the bile duct. Sphincterotomy was performed, followed by distal bile duct biopsy and stent placement. Pathology report of ampullary mass showed findings consistent with small cell neuroendocrine carcinoma. Immunohistochemical staining showed malignant cells were positive for synaptophysin and chromogranin and negative for cytokeratin AE1/3 and CD45. Patient underwent a Whipple procedure with specimen demonstrating a 4.2 cm × 2.8 cm exophytic mass at the ampulla with invasion into the pancreas. Five of twenty peripancreatic lymph nodes were positive for metastatic small cell carcinoma. Following resection our patient was started on adjuvant chemotherapy with cisplatin and etoposide.

DISCUSSION:

The most common tumor involving the ampulla of Vater is adenocarcinoma. Small cell neuroendocrine carcinoma of the ampulla of Vater accounts for no more than 2% of ampullary malignancies. This case illustrates that although biliary obstruction is commonly attributed to choledocholithiasis and other intrinsic or extrinsic tumors, rarer forms of malignancy should be considered in the differential. Careful assessment of the ampulla during ERCP can aid with diagnosis. Final diagnosis is challenging and can only be confirmed with histopathological examination. Treatment involves a multidisciplinary approach involving surgical resection and chemotherapy.
1336 A Rare Case of a Non-Functioning Pancreatic Neuroendocrine Tumor
Authors: Dasu Neethi R. , Khalid Yaser , Gordon Richard
Published: October 2019

INTRODUCTION:

Pancreatic neuroendocrine tumors (PNETs) are very rare, representing 1–2% of all pancreatic tumors and 7% of all NETs. 85% of PNETs are non-functional PNETs (NF-PNETs). Most are found incidentally at an advanced stage because patients are asymptomatic and the tumors do not secrete hormones that would manifest with symptoms. We report a case of a 32-yr-old female diagnosed with widely metastatic intermediate grade NF-PNET. For pancreatic neuroendocrine tumors that have metastasized, prognosis is poor, with a survival of only 1–3 years.

CASE DESCRIPTION/METHODS:

A 32-yr-old female presented with increasing abdominal girth and lower extremity swelling. A CT of abdomen and pelvis showed a massively enlarged liver with diffuse metastases, pancreatic tail mass, vaginal and peritoneal masses, sigmoid colon mass, and gallbladder masses. Core biopsy of her liver showed a metastatic NF-PNET. Following 6 cycles of chemotherapy with capecitabine and temozolomide, repeat CT showed a significant decrease in her pancreatic mass and metastases. Her chromogranin A level decreased from 5800 to 35 ng/dL. Then she had an exploratory laparotomy, lysis of adhesions, and bilateral oopherectomy. Postoperatively she was changed to octeotide and everolimus until MRI showed a progression of her liver metastases. She was switched to 4 cycles of treatment with doxorubicin, 5-fluorouracil (5-FU), and streptozocin as well as Y90 to her liver. Repeat MRI showed stable disease in her liver.

DISCUSSION:

Based on histology and pathology Ki 67 of 40% and 10 mitoses/10 HPF our patient's tumor was classified as G2 intermediate grade. Our patient had none of the potential risk factors for PNETs. Imaging includes an Octreoscan, negative for our patient, and CT scans in 3 phases. Our patient tested negative for increased levels of all hormones and biomarkers seen with newly diagnosed NETs. Serum levels of CgA are elevated in the 80-90% of patients with advanced NETs and can be used to monitor disease progression and treatment as seen in our case. Surgery is the only treatment proven to prolong survival as seen with our patient. Medical treatment with somatostatin analog octreotide plus chemotherapy regimen of doxorubicin, 5-FU and streptozocin are used for locally advanced and metastatic pancreatic endocrine carcinomas like with our case. This case is important because it illustrates an atypical presentation in a younger patient and it is important for practitioners to be vigilant in diagnosing rare diseases.
1342 A Rare Presentation of Lactobacillus Paracasei Endocarditis With Involvement of Aortic Prosthetic Valve in a Patient With Septic Shock Due to Acute Cholecystitis
Authors: Gueorguieva Iana , Kosti Jorgena , Makandura Mahesha , Ismail Rana
Published: October 2019

INTRODUCTION:

Endocarditis caused by Lactobacilli species is rare and usually manifests in patients with severe comorbidities, congenital heart disease, or prosthetic valves. Lactobacilli species are gram-positive coccobacilli mostly found in the gastrointestinal tract flora. Current literature shows an association of this bacterium with the use of probiotics. We hypothesize a possible association between acute cholecystitis and the occurrence of Lactobacillus endocarditis in a patient with no history of use of probiotics.

CASE DESCRIPTION/METHODS:

A 75-year-old female with a history of Birt-Hogg-Dube Syndrome with multiple spontaneous pneumothoraxes, COPD, aortic stenosis status post prosthetic aortic valve (AV) replacement, presented with acute respiratory failure and septic shock. She was diagnosed with acute cholecystitis, severe aortic regurgitation, and found to have lactobacillus bacteremia. We started her on empiric antibiotic coverage and later switched to penicillin G and gentamicin following three positive blood cultures for Lactobacillus paracasei. A transesophageal echocardiogram showed vegetation with emerging severe aortic regurgitation, degeneration, and perforation of AV leaflet when compared with echocardiogram one year ago. A CT-thorax demonstrated pan lobular emphysema with large cysts and pneumatoceles at the lung bases. Bacteremia persisted for two weeks after initiation of antibiotic therapy. Treatment with penicillin G and gentamicin was deemed necessary for up to 4 weeks after definitive surgical treatment. However, the patient's severe lung condition complicated preoperative clearance, and she was transferred to a specialized center for open-heart AV replacement.

DISCUSSION:

Very few cases of Lactobacillus endocarditis are reported in the literature. A handful of studies describe some implicated pathogenesis of the Lactobacillus in the blood as induced by cholecystitis. Other studies suggest that the increased presence of Lactobacillus in the respiratory tract in cases of severe lung pathology can lead to hematogenous dissemination to the heart valves. However, the association between Lactobacillus species and prosthetic valve material (and implication with endocarditis) is currently well-established. All things considered, and despite the lack of histopathology, we suggest that the source of Lactobacillus was likely the acute cholecystitis, and the presence of lung comorbidities and prosthetic AV further exacerbated the patient's condition.
1347 From the Outside Looking in: Psoriasiform Dermatitis Presenting as a Paraneoplastic Syndrome for Pancreatic Adenocarcinoma
Authors: Narasimha Shruthi , Drame Awa , Shah Jalak
Published: October 2019

INTRODUCTION:

Cancer is a devastating finding but diagnosis can be difficult due to the vague symptoms and often indolent course. Sometimes, medical professionals have clues to possible malignancy precursors but it can easily be overlooked when they are presented in different clinical pictures. Paraneoplastic syndromes range from almost ever organ system but the case discussed here deals with a rare cutaneous manifestation that can be seen with a number of malignancies known as bazex syndrome.

CASE DESCRIPTION/METHODS:

This case involved a 64-year-old female with history of hypertension and paroxysmal atrial fibrillation who came to the hospital with complaints of yellow discoloration of her skin, intermittent fevers and a recent history of right upper quadrant pain with nausea. She was seen by her primary care physician who did basic labs that showed a total bilirubin of 14.3 and she was sent to the emergency room for further evaluation. Of note, patient was previously seen in the hospital 6 months prior for a skin rash on her extremities and abdomen that was biopsied and later described as psoriasiform dermatitis. It was thought to be a drug reaction from a medication but the rash still persisted after discontinuation. On this admission, a CT abdomen showed masses in the pancreatic head and neck with confluent disease encasing the periportal structures that was concerning for malignancy. Biopsy after endoscopic ultrasound showed adenocarcinoma and the patient was evaluated by oncology with gastroenterology and she had biliary sphincterotomy with stenting and was started on modified FOLFIRINOX therapy.

DISCUSSION:

This case highlights a rare finding with the psoriasiform dermatitis rash that erupted a few months prior to formal diagnosis of pancreatic adenocarcinoma, which is concerning for an uncommon paraneoplastic process known as bazex syndrome. What makes this case even more uncommon is that bazex is usually seen in head, neck and lung cancers and is less commonly seen in gastrointestinal malignancies. Clinicians should be made aware of these cutaneous changes, so that they can improve on early evaluation, diagnosis and possible treatment.
1349 A Rare Presentation of Cholecystogastric Fistula With Associated Mirizzi Syndrome Leading to the Diagnosis of Gallbladder Cancer
Authors: Bournique Marissa , Lopez Tania , Kudelko Marc , Thomas Miriam
Published: October 2019

INTRODUCTION:

Biliary fistulas are a rare complication of gallstone disease. Technological advances have allowed for successful management & diagnosis. Mirizzi syndrome is a condition seen in less than 1% of patients with cholelithiasis & malignancy in 5 to 28% of cases. This is a rare presentation of coexistent Mirizzi syndrome and a cholecystogastric fistula leading to the diagnosis of gallbladder adenocarcinoma.

CASE DESCRIPTION/METHODS:

A 68 y/o female presented to the ER with complaints of jaundice & pruritis. Records demonstrated prior CTA&P with cholelithiasis with stranding & nonspecific stranding around the distal stomach. MRI w/wo contrast demonstrated loss of fat plane between gallbladder & stomach suggestive of fistulizing disease & a filling defect within the extrahepatic common hepatic duct related to external compression suggestive of Mirizzi Syndrome, no pneumobilia was seen. EGD/EUS was without direct visualization a fistulous tract, but revealed ulceration of gastric mucosa suggesting extrinsic pressure from a gallstone. ERCP demonstrated a stricture within the proximal CBD. Brushings were negative for malignancy. Lap chole revealed adherence of the stomach to the gallbladder fundus & takedown of cholecystogastric fistula was also performed. Histology from gallbladder specimen returned positive for invasive poorly-differentiated adenocarcinoma with transmural and lymphatic invasion.

DISCUSSION:

The presentation of cholecystogastric fistula generally includes pneumobilia & lacks pruritis suggestive of obstructive jaundice due to drainage of the biliary system via tract formation. Yet during the development of the fistulous tract the presentation varies quite drastically. Surgery is guided by the sub-classification of fistula formation/erosion as noted in the table figure. ERCP allows better characterization of the anatomy, in addition to offering the potential for therapeutic intervention with biliary decompression. There is an important known relationship between Mirizzi syndrome & gallbladder cancer likely related to the recurrent irritation occurring in the region along with chronic biliary stasis therefore frozen sections should be performed in all cases of Mirizzi syndrome to conclusively evaluate for gallbladder cancer. Our case highlights the malignancy potential within the presentation of fistulizing gallbladder disease. With a heightened index of suspicion and advancements in endoscopic and surgical techniques management of these patients will hopefully become less challenging.
1351 The Peculiar Presentation of Extramedullary Plasmacytomas as Acute Pancreatitis
Authors: Jiang Yi , Di Vitantonio Thomas A. , Rotundo Laura , Kutner Matthew , Galan Mark A. , Mirani Neena , Ahlawat Sushil
Published: October 2019

INTRODUCTION:

Extramedullary plasmacytomas (EMP) are a subcategory of plasma cell neoplasms that involve organs outside the bone marrow. Less than 5% of the cases are seen in the gastrointestinal tract. Pancreatic plasmacytomas are an extremely rare condition, with only 50-60 cases previously described in the literature. We report a unique case of concurrent gastric and pancreatic plasmacytomas presenting as acute pancreatitis.

CASE DESCRIPTION/METHODS:

A 62-year-old woman presented to the Emergency Department (ED) for epigastric pain radiating to back for one day. She had a history of multiple myeloma, which had relapsed after autologous stem cell transplant and progressed despite chemotherapy. On exam, she was jaundiced and had an elevated lipase to 1,112 U/L. A computed tomography scan of abdomen showed two masses involving the pancreatic head and body, with biliary and pancreatic duct dilatation. A subsequent endoscopic ultrasound revealed multiple nodules in the stomach. Biopsy of the gastric nodules and fine needle aspiration of the pancreatic lesions revealed atypical plasma cells with immunoreaction to kappa light chain, CD138, and CD56. These findings confirmed the diagnosis of extramedullary plasmacytoma. Acute pancreatitis and obstructive jaundice were diagnosed and an endoscopic retrograde cholangiopancreatography (ERCP) was performed. During the ERCP, a distal bile duct stricture was found and one metal stent was placed. Following the procedure, the patient reported gradual resolution of her abdominal pain and serum bilirubin returned to the normal range within one week.

DISCUSSION:

In previous reports, the majority of pancreatic plasmacytoma cases involved a single lesion located in pancreatic head which presented as painless, obstructive jaundice. We report a case of concurrent gastric and pancreatic plasmacytomas presenting with acute pancreatitis. It is important to recognize the extramedullary manifestations of multiple myeloma in patients presenting with acute pancreatitis and gastrointestinal tract lesions. This observation of aggressive tumor behavior in treatment-resistant multiple myeloma also suggests a potential modulation effect on the tumor and its microenvironment by chemotherapy and stem cell transplant. Further investigation into the underlying mechanism of metastasis in multiple myeloma may shed light on new therapeutic strategies to improve outcomes for future patients.
1357 Pseudocirrhosis in Subacute/Chronic Budd-Chiari Syndrome With JAK 2 Mutation
Authors: Lakshmanan Seetha L. , Toubia Nabil
Published: October 2019

INTRODUCTION:

Budd Chiari Syndrome (BCS) is a rare disorder often characterized by hepatic venous outflow obstruction. Myeloproliferative disorders especially V617F-JAK2 mutations account for almost 40% of these patients. BCS may present differently depending on location, extent, severity of obstruction and existence of collaterals. The course of disease may be fulminant, acute, subacute or chronic, ranging from incidental findings to fatal acute hepatic failure.

CASE DESCRIPTION/METHODS:

A 74-year-old female with remote history of alcoholism, presented to our facility with bleeding per rectum for 2 days. Labs on admission revealed WBC 18.4, HGB 16.8, PLT 588, AST 570, ALT 804 and TB 4.6. Ultrasound Abdomen with Doppler revealed ascites and portal vein thrombosis. Follow-up MRI Abdomen confirmed thrombus in main portal vein and non-opacification of major intrahepatic veins suggesting Budd Chiari Syndrome. Hypercoagulability workup showed positive JAK2 mutation. She was started on IV Heparin immediately. Diagnostic paracentesis revealed transudative fluid and liver elastography confirmed cirrhosis. Grade 2 esophageal varices were seen on EGD with a negative colonoscopy. Other possible etiology of congestive hepatopathy/cirrhosis were excluded. Patient was discharged on daily oral Rivaroxaban with intermittent therapeutic paracentesis. Over next 3 months, she had recurrent admissions for decompensated liver disease. During her last visit however, we noted preserved synthetic liver function and inconsistencies in her presentation thus a liver biopsy was obtained which revealed centrilobular hepatocellular necrosis, sinusoidal dilatation and mild portal fibrosis consistent with hepatic venous outflow obstruction. However, there was no evidence of cirrhosis. She was transferred to an advanced facility for portal vein reconstruction to relieve the obstruction from splanchnic venous thrombosis and possibly reverse the “pseudocirrhosis”.

DISCUSSION:

Pseudocirrhosis is often associated with metastatic cancers in response to chemotherapy induced ischemic injury. Although it radiologically and clinically mimics liver cirrhosis, it can be prevented with early detection. To our knowledge, this is the first reported case of pseudocirrhosis in BCS. Subacute/chronic BCS may present with cirrhotic complications like portal hypertension, ascites and variceal bleeding. Apart from anticoagulation, these patients may require other interventions to bypass the obstruction like Direct Intrahepatic Portosystemic Shunt (DIPS) placement.
1360 Cholecystohepatic Fistula With Liver Abscess From Gallbladder Perforation in a Patient With Ascending Cholangitis
Authors: Lu Ray , Smock Justin W.
Published: October 2019

INTRODUCTION:

Ascending cholangitis can lead to liver abscess (LA). Intrahepatic gallbladder (GB) perforation with fistula formation is an uncommon cause of LA. The co-occurrence of all 3 phenomena is rare.

CASE DESCRIPTION/METHODS:

An 88-year-old man with a history of idiopathic fibrosing pancreatitis diagnosed 3 months prior that required stenting of the common bile duct (CBD) presented with 4 days of malaise, nausea, vomiting, and fever. Endoscopic retrograde cholangiopancreatography (ERCP) showed occluded CBD stent coated with stone material. Successful biliary sphincterotomy and stone extraction was performed. The patient's symptoms initially resolved but recurred later that day. On exam he was tachycardic, tachypneic, and febrile to 103.1 F, with pain in the right upper quadrant (RUQ) of the abdomen without guarding nor rebound. Labs showed WBC 33.4 k/mm3, total bilirubin 2.2 mg/dL, ALP 488 U/L, AST 43 U/L, ALT 39 U/L, and lipase 44 U/L. RUQ ultrasound (US) showed a heterogeneously hypoechoic right liver lesion 6.3 × 7.4 × 4.3 cm in size, consistent with LA and noted to be contiguous with the GB, which had a thickened wall, an irregular sloughing appearance concerning for gangrenous cholecystitis, and contained sludge, gallstones, and air. A percutaneous cholecystostomy drain was placed by interventional radiology (IR). Intra-procedure sinogram and US showed the LA to be connected to the GB. A second percutaneous drain was placed into the LA. Abscess cultures were polymicrobial. The patient was treated with 4 weeks of piperacillin-tazobactam, and then transitioned to amoxicillin-clavulanic acid with plans to continue antibiotic therapy for a total of 6 months. One month after discharge, computed tomography (CT) showed resolution of LA, and both IR drains were removed. Given clinical improvement, cholecystectomy has not been pursued due to advanced age.

DISCUSSION:

When ascending cholangitis and choledocholithiasis are successfully treated through ERCP but the patient does not improve clinically, LA should be considered. Intrahepatic GB perforation leading to fistula formation is a rare cause of LA that most commonly occurs in the elderly, particularly those with a history of chronic biliary disease. Exam and labs can be non-specific, while US and CT are useful diagnostically. Successful treatment involves ERCP, drainage of LA, an extended course of antibiotics, and, while cholecystectomy is preferred, percutaneous cholecystostomy appears to be a viable first option in high-risk patients.
1364 Metastatic Pancreatic Adenocarcinoma Presenting as a Colonic Obstruction
Authors: King Connor , Nehme Fredy , Tawfik Ossama
Published: October 2019

INTRODUCTION:

Pancreatic cancer is the fourth most common cause of cancer-related mortality in the United States largely due to a delayed diagnosis at an advanced stage. Patients with pancreatic tumors commonly present with abdominal pain, anorexia and jaundice. Nearly 50% of patients with pancreatic cancer present with metastatic disease, most commonly to the liver. Rarely it can metastasize to the colon and present with an obstruction.

CASE DESCRIPTION/METHODS:

A 68-year-old male presented with a 2 week history of abdominal pain and constipation. On physical exam, the abdomen was distended with generalized tenderness. Initial computed tomography (CT) scan of the abdomen and pelvis showed abnormal colonic and small bowel distention with induration surrounding the proximal sigmoid colon and distal decompression. There was also evidence of a bulky mass-like appearance of the pancreatic tail and prominent pericolonic lymph nodes surrounding the sigmoid colon. Despite conservative measures, the patient had progressively worsening colonic distention on serial abdominal X-rays and a repeat CT scan was concerning for perforated viscus. The patient underwent emergent exploratory laparotomy and a large inflammatory mass was noted in the sigmoid colon. A subtotal colectomy with end ileostomy was performed. Surgical pathology revealed metastatic mucinous adenocarcinoma to the colonic wall (Figure 1). Several days later, an endoscopic ultrasound with fine needle aspiration was obtained and showing a 31 × 20 mm hypoechoic mass involving the body and tail of the pancreas (Figure 2). Pathology was positive for malignant cells consistent with adenocarcinoma. Histopathological patterns of both tumors were compared and had almost identical features.

DISCUSSION:

Differentiating a primary colonic cancer from metastasis can be challenging and the absence of mucosal involvement favor metastatic disease. In cases where metastasis are favored, immunohistochemistry is helpful to determine the primary origin of the tumor. It is not uncommon to encounter an extrapancreatic adenocarcinoma suspected to originate from the pancreas, with most common locations being liver, lymph nodes, lungs, and adrenal glands. Metastatic pancreatic cancer to the colon is extremely rare and no clear mechanism has been identified. Despite its rarity, pancreatic cancer should be considered in patients presenting with colonic obstruction and a pancreatic mass on imaging.
1366 Primary Antiphospholipid Syndrome, Acute Necrotizing Pancreatitis, and IgA Nephropathy in a Young Male: Single Pathogenesis or Rare Association?
Authors: Chandra Prasad Ram , Patro Pradeepta , Sahoo Srikanta Kumar , Narayan Jimmy , Singh Ayaskanta
Published: October 2019

INTRODUCTION:

Primary Antiphospholipid syndrome (APS) is characterized by recurrent thrombosis, fetal loss and the presence of lupus anticoagulant (LA)/anticardiolipin antibodies. Primary APS is uncommon in males and poses diagnostic difficulties. Primary APS is considered as a rare cause of acute pancreatitis and IgA nephropathy (IgAN). We describe a patient of primary APS (probably the first reported case), who sequentially presented with cerebral venous thrombosis, later with recurrent episodes of acute necrotizing pancreatitis (ANP) followed by IgAN. Despite the initial diagnostic dilemmas, the patient was managed successfully.

CASE DESCRIPTION/METHODS:

A 30 year old male initially presented with sudden onset headache and generalized seizures. CECT brain revealed cerebral venous thrombosis. Cardiac and Pro thrombotic work up (including ANA and LA) was normal. He was treated with antiepileptics and warfarin. 3 months later, he presented with 3 episodes of acute pancreatitis. Each time the patient was managed conservatively with fluids and analgesics. However the last episode was very severe, CECT abdomen was suggestive of severe ANP with extensive splenic vein thrombosis (SVT). Anti-β2glycoprotein1 antibody test was strongly positive. APS was diagnosed according to revised Sapporo criteria. Low molecular heparin and steroids were started. After 5 months, although the patient recovered from ANP, he presented with hypoproteinemia and renal failure. Urine analysis showed RBC casts and proteinuria. Kidney biopsy showed mesangial IgAN with secondary segmental sclerosis. Patient recovered fully and is on follow up for two years. He is doing fine with low dose steroids, ACE inhibitors and warfarin with regular INR (target 2-3).

DISCUSSION:

This patient had multiple hospitalizations, all attributable to the rare manifestations of APS. It is perhaps the first case report where APS, ANP and IgAN are described in a single patient. Clinical suspicion can lead to early diagnosis, which makes it easy to manage such patients. Acute pancreatitis is considered as one of the rarest manifestation of APS, which is distinct from autoimmune pancreatitis. Renal biopsy was suggestive of IgAN, which is unusual and distinct from the APS nephropathy. Thrombosis of the pancreatic and renal microcirculation and vasculitis has been postulated as the underlying pathogenesis. To conclude, APS is a rare, yet treatable cause of acute pancreatitis. Urine examination is a must in cases of primary APS, with or without pancreas involvement.
1373 A Rare Case of Biliary Obstruction in Autosomal Dominant Polycystic Kidney Disease
Authors: Serrao Steve , Rodrigues Medora , Strilaeff Ryan , Che Kendrick
Published: October 2019

INTRODUCTION:

Autosomal Dominant Polycystic Kidney Disease (ADPKD) is the most common genetic disease in adults. We present a rare case of biliary obstruction due to polycystic disease affecting the kidney and liver.

CASE DESCRIPTION/METHODS:

A 18 year old female patient with polycystic disease of the liver and kidney diagnosed in childhood, presented with new onset jaundice and abdominal pain radiating to the back. Initial labs suggested a predominantly cholestatic patter of liver injury with total bilirubin 3.1 mg/dl with a direct bilirubin of 2.3 mg/dl, aspartate aminotransferase 96 U/L, alanine aminotransferase 177 U/L, and alkaline phosphatase 237 international U/L. Lipase was noted to be 443 U/L. The patient did not exhibit signs of cholangitis and was started on intravenous fluids at 2 ml/kg/hr after a bolus of 2 L. The platelets were noted to be 123 with an INR of 1.3. A Magnetic Resonance Cholangiopancreatography showed near-complete replacement of hepatic, bilateral renal and pancreatic parenchyma with greater than 100 simple cysts. Also noted was dilatation of the common bile duct measuring up to 16 mm (Figure1). No internal filling defect was noted. An ERCP was performed. During endoscopy, multiple luminal “bulges” representing extrinsic compression of cysts were noted in the stomach and duodenal. A native ampulla was identified in the second portion and was displaced laterally by extrinsic luminal compression (Figure 2). Cholangiogram revealed dilated CBD and CHD with cystic dilations of the intrahepatic ducts more pronounced in the left lobe of the liver. There appears to be contrast seen in several liver cysts indicating possible communication of biliary tree with the cysts (Figure 3). We entertained the possibility of a choledochal cyst (4a). External compression of the intraduodenal CBD was the etiology of the obstruction and a 10 Fr × 10 cm plastic biliary stent was placed resulting in large volume of bile evacuating from the biliary tree. The placement of the biliary stent resulted in the normalization of total bilirubin to 0.5 mg/dl with, AST 23 U/L, ALT 24 U/L, and ALP 109 international U/L. We recognized the risk of cholangitis due to the stasis of contrast and provided Ceftriaxone 1 g IV intra procedure and Cephalexin once daily for 7 days.

DISCUSSION:

This patient with large number of hepatic cysts replacing the hepatic parenchyma and large renal cysts resulted in distal biliary compression in the intraduodenal portion. This patient is being considered for a liver kidney transplantation.
1374 Pancreatic Cancer: Leading to Failure of Rivaroxaban as an Anticoagulant
Authors: Vekaria Pratikkumar , Raiyani Chandrika , Robitshek Daniel , Raiyani Tejas
Published: October 2019

INTRODUCTION:

Vascular Thromboembolism (VTE) is seen with hypercoagulable state and it can be a reason of significant morbidity and mortality. Cancer is considered as one of the most common causes of hypercoagulability and developing VTE. Since novel oral anticoagulants (NOAC) have been introduced in market for VTE prophylaxis and treatment, their use have been increased significantly as they are easy to use without episodic monitoring. Rivaroxaban is one of the commonly used NOACs and some of studies have shown its good efficacy for VTE.

CASE DESCRIPTION/METHODS:

71 year old female with PMH of persistent A-fib on Rivaroxaban, pulmonary hypertension, HTN, DM-2 who presented to ED with severe pain in both feet. On physical examination, they seemed ischemic with splotchy cyanotic/pallor discoloration on multiple toes of both feet. Patient was already on 20 mg PO daily dose of Rivaroxaban for A-fib which is also prophylactic dose for VTE. Her fibrinogen and D-dimer were elevated at 185 and >5000 respectively. Vascular surgery was consulted and they put patient on heparin drip. CTA Aorta with run off was ordered to find out source of VTE. Unfortunately, CT report showed pancreatic head cancer with metastasis to liver, adrenals and lumber vertebrae which was definitively diagnosed as “Primary Pancreatic Adenocarcinoma” on biopsy. Oncology was consulted and they recommended higher dose of Rivaroxaban 15 mg PO BID on discharge. Still patient came back to ED with worsening of symptoms and pain in both feet due to VTE exacerbation. Patient was then switched to fondaparinux and it helped her from keep getting recurrent VTE exacerbation.

DISCUSSION:

Traditionally, warfarin and enoxaparin have been used as first-line agents for VTE treatment. Their efficacy have been proven with acceptable risks of bleeding. As enoxaparin needs to be injected and warfarin requires regular monitoring of INR, patients were not complaint with using them. Conversely, NOACs were invented to provide innocuous and more compliant alternative to warfarin and enoxaparin. Rivaroxaban has been most commonly used NOAC for this purpose and some of the studies bolsters its efficacy for VTE. Nonetheless, few case reports are out that don't support Rivaroxaban's effectiveness as a prophylactic agents for preventing VTE. We propose that it still requires more post-marketing research in assessing Rivaroxaban's real efficacy/effectiveness as prophylactic agent for VTE in Pancreatic as well as other cancers.
1379 Immune Checkpoint Inhibitor-Induced Acute Pancreatitis and Colitis
Authors: Pagan Andrea , Gill Jeffrey , Arroyo Yadis , Bertran-Rodriguez Carlos E.
Published: October 2019

INTRODUCTION:

Immune checkpoint inhibitors are gaining increasing popularity as an efficacious treatment for advanced malignancies. As their use increases, their side effects are more notable in this specific population. These adverse events can be severe enough to require interruption or withdrawal of immune checkpoint blockade therapy. The most common side effects involve the skin rashes and gastrointestinal symptoms. In order to provide the correct diagnosis and management, physicians should be aware of the different presentations. We present a case of a checkpoint inhibitor induced acute pancreatitis and colitis.

CASE DESCRIPTION/METHODS:

A 76-year-old male with medical comorbidities pertinent for hypertension and COPD that was being treated with Pembrolizumab for Stage IV oligometastatic clear cell renal carcinoma presented to the ED with abdominal pain, nausea, and vomiting. On physical examination, he was tachycardic, normotensive, and afebrile. Laboratory work pertinent for elevated Cr. 1.5 and Lipase at 436. CT abdomen showed edematous pancreas with loss of pancreatic lobulation. Patient was diagnosed with autoimmune mediated acute pancreatitis and was treated with high-dose steroids. Infusions of Pembrolizumab were held until the steroid taper was over. Once infusions were restored, he began having non-bloody diarrhea. Physical examination and laboratory data were normal. His infectious and inflammatory workup for diarrhea was negative. He underwent a colonoscopy with normal appearing mucosa. Random biopsies were obtained. Histological examination on the sigmoid colon showed chronic active colitis with crypts abscess, diagnosing the patient with checkpoint inhibitor-induced colitis. He was restarted on high dose steroids improving his symptoms. Immunotherapy was placed on hold indefinitely.

DISCUSSION:

Gastrointestinal toxicities are among the leading causes of immune-related adverse effects of checkpoint blockade with diarrhea being the most common. Other presentations include hepatotoxicity resulting in transaminitis and acute pancreatitis with the lowest incidence. Most immune-related adverse events appear within 1–2 months after the start of the checkpoint inhibitor. Treatment consists of high dose steroid with a gradual taper. Patients with moderate to severe immune-related adverse events usually require interruption of the checkpoint inhibitor. Early recognition of these toxicities is crucial in minimizing the impact of these complications on planned antineoplastic therapy.