bluuebunny/crossref_metadata_2025_split huggingface dataset:
1358 A Case of Hypertriglyceridemia Acute Pancreatitis Requiring Plasmapheresis Treatment
Authors: Penninti Pranav , Hopp Grace 
Published: October 2019

INTRODUCTION:
Hypertriglyceridemia is an uncommon cause of acute pancreatitis, comprising 2-7% of cases. The traditional approach to treatment is an insulin drip. In severe cases with signs of organ dysfunction, plasmapheresis/exchange (PLEX) is occasionally trialed, although the data behind this is extremely limited. We present a case of hypertriglyceridemia-induced acute pancreatitis (HTG-AP), resistant to insulin therapy and requiring PLEX for acute management.

CASE DESCRIPTION/METHODS:
A 36 year old male with diabetes mellitus, hypertension and hyperlipidemia presented to the ED with acute onset mid-epigastric abdominal pain with radiation to the back. On examination, he was tachycardic, appeared visibly uncomfortable and exhibited diffuse tenderness to palpation with voluntary guarding. The patient's lipase was elevated to 220 U/L (>3x the upper limit of normal). A CT abdomen/pelvis revealed pancreatic inflammation and he was diagnosed with acute interstitial pancreatitis. He denied alcohol use. A right upper quadrant ultrasound lacked evidence of gallstones. His records demonstrated poorly controlled hyperlipidemia with triglyceride levels ranging from 362 mg/dL to 1648 mg/dL over 10 years. He reported compliance with his Gemfibrozil and fish oil supplements. On admission, his triglyceride level was 1277 mg/dL. He was placed on an insulin drip for 48 hours without significant change in his clinical picture or triglyceride level, which nadired at 802 mg/dL. The decision was made to initiate PLEX. After his first treatment, his triglyceride level improved to 449 mg/dL and after a second it decreased to 315 mg/dL with corresponding improvement in symptoms. He was discharged on Fenofibrate, Atorvastatin and fish oil supplements. An appointment was scheduled with Endocrinology for further genetic testing and management of his dyslipidemia.

DISCUSSION:
HTG-APcarries significant morbidity and mortality, up to 40 per 100,000 persons in the Western population, with serious complications typically occurring at triglyceride levels above 1000 mg/dL. Aside from supportive care, an insulin drip is accepted as mainstay therapy. However, our patient did not respond to over 48 hours of standard therapy with profound improvement after PLEX therapy. The evidence for the use of PLEX in this context is solely through observational data. Our patient had a 75% reduction in serum triglycerides with PLEX therapy. We believe that there is a need for randomized trials to elucidate the benefits and risks of PLEX in HTG-AP.
1361 Facilitating Direct Cholangioscopy and Lithotripsy in a Roux-en-Y Bypass With a Stuck Dilation Balloon
Authors: Abadir Alexander , Sowa Piotr , Samarasena Jason , Lee John 
Published: October 2019

INTRODUCTION:
A 66-year-old woman with a history of Roux-en-Y gastric bypass, thromboembolic CVA on dual antiplatelet therapy, and known common bile duct (CBD) stones presented an outside facility 2 months prior complaining of chronic intermittent abdominal pain and jaundice. She was in sepsis due to cholangitis and MRCP showed a 2.1 cm CBD stone. ERCP was unsuccessful due to her altered anatomy; therefore, she was treated medically and referred for repeat attempt at ERCP, lithotripsy and bile duct clearance.

CASE DESCRIPTION/METHODS:
ERCP was performed using a double balloon enteroscope (DBE). Ampulla was seen next to a periampullary diverticulum. Retrograde cannulation and cholangiogram showed 2 large stones (Figure 1). Sphincterotomy and balloon dilation was done to 10 mm and stone extraction attempted but failed due to their large size. The procedure was repeated with plans to perform cholangioscopy and electrohydraulic lithotripsy (EHL). The ampulla was further dilated using a 14 mm balloon to allow direct cholangioscopy using the DBE. Unfortunately, attempt at removing the dilating balloon afterwards failed due to the small lumen size of the scope. Therefore, the scope was removed while keeping the overtube in place. The balloon at the tip of the DBE was removed to facilitate direct cholangioscopy and the scope was readvanced through the overtube to the ampulla. The DBE without the scope balloon passed easily into the CBD. Two stones, a black pigmented and a brown pigmented, were seen (Figure 2) and fragmented using EHL. Stone fragments were cleared in the usual fashion and direct cholangioscopy confirmed bile duct clearance (Figure 3).

DISCUSSION:
ERCP is difficult in patients with prior Roux-en-Y gastric bypass. The newest approach is to place a lumen apposing metal stent (LAMS) from the gastric pouch to the remnant stomach then perform antegrade ERCP in the usual fashion. If this is not possible (e.g., unfavorable anatomy, patient, or surgeon refusal) then the only approach is retrograde using a DBE; our patient underwent ERCP prior to the introduction of LAMS. DBE ERCP is cumbersome at best due to the small channel size and limited maneuverability of the scope as well as lack of an elevator. For these reasons, the only way to perform lithotripsy is by direct cholangioscopy after large balloon dilation.
1365 A Rare Case of Neuroendocrine Tumor Originating in Liver Diagnosed by ERCP Using a Spyglass Direct Visualization System
Authors: Seo Dong Joo , Daid Sarav , Mangala Yashvin Onkarappa , Malik Amer 
Published: October 2019

INTRODUCTION:
Neuroendocrine carcinoma is predominantly found in the gastrointestinal tract (54-75%) with 44%, 19%, 16%, 10% and 7% in the small intestines, rectum, appendix, colon and stomach respectively. Primary liver neuroendocrine carcinoma comprises around 0.3% of all neuroendocrine tumors. This is a case of a neuroendocrine carcinoma with a focus of cholangiocarcinoma presenting as a biliary stricture diagnosed by ERCP using the spyglass visualization system.

CASE DESCRIPTION/METHODS:
A 75 year old female with a prior history of rheumatoid arthritis presented with persistent nausea, vomiting, diarrhea and abdominal pain and a 20 lb weight loss since December 2017. An ultrasound revealed intrahepatic biliary ductal dilation in the left lobe with no visible mass and a coarse hepatic echotexture. A subsequent MRI of the abdomen demonstrated left lobe intrahepatic biliary ductal dilation with a hypointense 1 cm region within the liver parenchyma associated with a biliary stricture. The biliary stricture in the left hepatic branch was visualized with the spyglass direct visualization cholangioscope introduced through an ERCP scope. Biopsies obtained through the cholangioscope revealed high grade dysplasia. The patient underwent a left lobectomy of liver with hepatic artery and portal lymph node dissection. Lymph nodes were negative for malignancy. The biliary stricture was from a high grade neuroendocrine carcinoma with a microscopic focus of cholangiocarcinoma with extensive lymphovascular and perineural invasion. The margin of the bile duct and hepatic hilum were involved by neuroendocrine carcinoma. Post operative PET scan showed a small focus of activity without any signs of distant metastasis.

DISCUSSION:
SpyGlass direct visualization system is a relatively new technique in ERCP. Recently published prospective data confirm that the overall success rates for adequate tissue sampling is nearly 90%. ERCP has limitations for sampling lesions in the bile duct without direct visualization. Conventional ERCP biliary sampling yields are 20% to 30% for cytology brushing to approximately 50% with a combined brush/standard biopsy forceps approach. If sampling is negative, the choice is either radical surgery or continual observation with an MRI. Nowadays, ERCP with spyglass technique and targeted direct visualization biopsy affords vastly higher yields. Direct visualization is now the gold standard to obtain biopsy samples in cases of biliary strictures.
1370 An Extremely Rare Presentation: Primary Neuroendocrine Tumor
Authors: Estremera-Marcial Rodolfo , Marzan-Loyola Adriana , Cerra-Franco Javier , Diaz Carolina , Magno Priscilla 
Published: October 2019

INTRODUCTION:
This is the case of a 63-year old asymptomatic male with history hypertension who was found with an abnormal gallbladder in a routine abdominal sonogram for kidney cysts. The gallbladder walls appeared thickened with low and intermediate level intraluminal echoes interpreted as tumefactive sludge. Given asymptomatic presentation, no further workup was solicited.

CASE DESCRIPTION/METHODS:
A follow up abdominal MRI displayed further gallbladder asymmetry with focal thickening of the right lateral aspect of the proximal fundus. A PET CT failed to show significant FDG concentration at the soft tissue density on the fundus. Upon evaluation, the patient denied abdominal pain, jaundice or weight loss, among others. All laboratories were within normal limits including CEA and CA 19-9. The patient was referred to surgery service who initially stated no surgical management due to asymptomatic presentation and negative PET CT. However, cholecystectomy was performed by a hepatobiliary surgeon upon further increase in size of the gallbladder lesion. Surgical pathology sample showed sheets of small atypical cells, focally growing in a cord like and nested pattern. Immunology stains were done which showed a poorly differentiated component with positive markers for chromogranin and synaptophysin. These findings were suggestive of a poorly differentiated neuroendocrine carcinoma. Serum chromagranin A and 5- HIAA in urine collection were requested after resection and were normal.

DISCUSSION:
Primary neuroendocrine tumors of the gallbladder are exceedingly rare, comprising 0.2% of all neuroendocrine tumors. Most neuroendocrine tumors of the gallbladder are diagnosed as an incidental finding on cholecystectomy since most patients present with non-specific symptoms including upper abdominal pain, discomfort, jaundice and weight loss. Carcinoid syndrome is seldom present as it is associated with less than 1% of neuroendocrine tumors of the gallbladder. The rarity of this tumor, along with its asymptomatic presentation and poor prognosis, supports the claim that further research is needed to achieve early detection and better outcomes. This case also serves to illustrate that an asymptomatic presentation, negative PET CT, and increasing gallbladder thickening on repeated imaging should not be overlooked.
1372 A Rare Case of Purtscher's Retinopathy in the Setting of Acute Alcoholic Pancreatitis
Authors: Joshi Tejas V. , Bilalis Mathoula , McPeters Ryley , Thorne Tyler , Landreneau Stephen , Amoss John 
Published: October 2019

INTRODUCTION:
Purtscher's retinopathy (PUR) is a rare condition first described in 1910. Occlusive microvasculopathy is characterized by sudden visual loss with multiple areas of retinal whitening in the posterior pole. Associated with acute pancreatitis, connective tissue disorders, renal failure, childbirth, bone marrow transplantation. Proteases like trypsin activate the complement system cascade causing coagulation and leukoembolization of the retinal precapillary arterioles. The emboli lodge in the arterioles producing the clinical appearance of PUR with impaired visual acuity and visual field loss. We present a case of a young female with repeat acute pancreatitis who developed sudden visual changes seconday to PUR.

CASE DESCRIPTION/METHODS:
28 yo F with PMH of Etoh abuse, gastritis, Mallory-Weiss tear and prior admissions for pancreatitis presented to ED with emesis and abdominal pain for one day. Exam revealed a diffusely tender abdomen. Labs included lipase (939 U/L) (aspartate aminotransferase = 351 U/L, alanine aminotransferase = 179  U/L, alkaline phosphatase = 133  U/L) and hyperbilirubinemia (1.8 mg/dL). Abdominal U/S showed peripancreatic edema correlating acute pancreatitis along with hepatomegaly and steatosis. On day 1 of admission pt complained of visual disturbances upon awakening. Acute visual changes included blurriness, flashes and floaters which completely resolved by the evening. Bedside ophthalmologic exam revealed multiple cotton wool spots bilaterally. Ophthalmology evaluation revealed macular edema and neurosensory detachment of the macula bilaterally secondary to PUR. Visual field testing showed loss of central field bilaterally with preserved peripheral vision. Acuity was 20/200 bilaterally and received intraocular steroid injections with weekly follow up. Exam at 8 weeks demonstrated improvement to visual acuity to 20/40 bilaterally.

DISCUSSION:
Purtscher Flecken are variable, polyglonal regions of retinal whitening with a clear zone and are pathognomonic. The treatment for the ocular complications of PuR have not been proven and prognosis depends on the areas of the retina that are affected. Isolated case reports have shown rapid improvement in vision with high dose IV steroids. Steroids help preserve the neuronal membrane and suppress granulocyte aggregation caused by complement activation. Patient's complaining of changes in vision following an episode of acute pancreatitis should undergo eye examination in order to rule out Purtscher-like retinopathy.
1376 Pseudoaneurysm of the Cystic Artery: An Unusual Case of Hemobilia in a Teenage Patient
Authors: Kang Sohyun , Miller John W. , Lee Edwin J. , Hughes Michelle L. , Aranez Jose L. , Ullah Asad 
Published: October 2019

INTRODUCTION:
Pseudoaneurysm of the cystic artery is a rare cause of hemobilia and often derives from the inflammatory changes in cholecystitis. Knowledge regarding this topic is limited with rare occurrence reported in the literature. This may be the first reported case of a cystic artery pseudoaneurysm causing biliary obstruction in a pediatric patient. This case emphasizes the importance of considering hemobilia whenever gastrointestinal bleeding is associated with cholestatic liver injury as well as including cystic artery aneurysm in the differential diagnosis of hemobilia.

CASE DESCRIPTION/METHODS:
A 16-year-old obese male scheduled for elective cholecystectomy for symptomatic cholelithiasis was admitted for worsening abdominal pain associated with nausea and vomiting. After admission, patient remained afebrile with resolving symptoms, but rising cholestatic markers. MRCP showed Common Bile Duct (CBD) dilation to 1 cm but without CBD stones. EUS was performed to evaluate for choledocholithiasis but was limited by the unexpected finding of fresh blood in the duodenum. CBD dilation to 8 mm was seen but was without evidence of CBD stones. Cholestatic injury worsened, prompting ERCP as distal CBD stones were still the leading concern. Upon examination prior to cannulation, passage of blood and blood clots was seen from the native ampulla. Cholangiogram showed multiple filling defects within the CBD suggestive of blood clots, sphincterotomy was performed and multiple sweeps with the balloon catheter removed only blood clots with no stones seen. CT angiography was performed and found a focal arterial density collection in the posteromedial gallbladder wall, most likely due to a pseudoaneurysm of the cystic artery. Interventional Radiology performed coil embolization of the cystic artery prior to cholecystectomy. Laparoscopic cholecystectomy was converted to open laparotomy after the discovery of extensive pericholecystic adhesions to the gallbladder as well as a cholecystocolonic fistula. The fistula was repaired and the gallbladder was removed with ligation of the cystic artery just distal to the coils. Symptoms resolved and the patient's laboratory values normalized.

DISCUSSION:
A rare case of cholestatic liver injury and hemobilia secondary to cystic artery pseudoaneurysm in a pediatric patient is reported. As demonstrated in this report, immediate angiography and embolization of the involved artery followed by cholecystectomy may be the optimal treatment regardless of patient age.
1386 Narrow Tunnel, Zero Visibility: Role of Lumen-Apposing Metal Stent in the Treatment of Short Luminal Stricture Prior to Endoscopic Retrograde Cholangiopancreatography Procedure
Authors: Shapira Gilad , Kumar Manoj , Fahmawi Yazan , Mizrahi Meir 
Published: October 2019

INTRODUCTION:
Managing short-segment benign gastrointestinal (GI) strictures can be challenging if they become refractory to conventional endoscopic therapies. Even though lumen-apposing metal stents (LAMS) are approved for access to and drainage of pancreatic fluid collections, endoscopists are using it in off label manner to treat these short GI luminal strictures. Here we present a patient with short duodenal stricture who was treated with LAMS, resulting in improvement of duodenal patency to accomplish ERCP.

CASE DESCRIPTION/METHODS:
69-year-old male with signs of obstructive jaundice (choledocholithiasis) could not get ERCP due to a large duodenal ulcer and associated duodenal stricture. He underwent percutaneous biliary drainage (PTC), treated with antibiotics and later internalization of biliary plastic stent through interventional radiology. Three months later, follow up endoscopy showed healed duodenal ulcer but persistent duodenal stricture. CT revealed proximal migration of the biliary stent. Esophagogastroduodenoscopy was done and under endoscopic and fluoroscopic guidance, a 20 mm LAMS was placed across the duodenal stricture (measured to be <1 cm) with its Distal flange of LAMS was deployed first followed by proximal flange successfully. Two weeks later, the site was found to be patent with the LAMS intact. LAMS was removed with rat tooth forceps without any complication and ERCP was accomplished easily through improved duodenal lumen. Migrated biliary stent was removed and duct was cleared of stones and sludge followed by placement of 10 mm by 60 mm fully covered biliary metal stent and treatment with antibiotics.

DISCUSSION:
Our case represents another success story of treatment of short GI luminal stricture with LAMS in a patient who required ERCP and eventually had it after duodenal stricture treatment. The short length and wide flanges make LAMS potentially an attractive alternative treatment for short GI luminal stricture without any significant complications and better patient tolerance. Long term data and efficacy still needs to be studied. However, it is likely to continue to substitute as an alternative to traditional endoscopic option for short GI luminal stricture.
1387 Late Recurrence of Metastatic Pancreatic Angiomyolipoma in Tuberous Sclerosis Following Bilateral Nephrectomy and Transplant
Authors: Sinha Ayusa , Tufail Kashif , Johal Amitpal S. , Khara Harshit S. 
Published: October 2019

INTRODUCTION:
Angiomyolipomas are typically benign tumors often associated with tuberous sclerosis, most commonly occurring in the kidneys. Some variants have malignant potential. Surgical resection remains the most effective form of treatment; however, recurrence after nephrectomy is rare, especially in the pancreas.

CASE DESCRIPTION/METHODS:
We report a unique case of a 59-year-old female with tuberous sclerosis who underwent bilateral nephrectomy for malignant, hemorrhagic angiomyolipomas with subsequent renal transplant. Almost 10 years after initial discovery of renal angiomyolipoma, patient presented to gastroenterology clinic with elevated liver function tests and was subsequently referred for endoscopic ultrasound (EUS) to evaluate for biliary obstruction as the etiology. If no obstruction was found, an EUS guided liver biopsy was requested. The EUS showed a normal non-dilated common bile duct (CBD) with no evidence of obstruction. EUS guided liver biopsy was then performed using a 19G core biopsy needle. Incidentally, she was found to have pancreatic tail mass lesion which measured 13 mm in diameter. This was not visualized on her previous surveillance CT scans. EUS fine needle aspiration was performed on the pancreatic tail mass with a 25G needle. The liver biopsy showed mild iron overload and minimal macrovesicular steatosis. The cytology from the pancreatic lesion was reported as an angiomyolipoma. The patient was subsequently referred to surgical oncology for evaluation of distal pancreatectomy.

DISCUSSION:
This is the second reported case of a pancreatic angiomyolipoma in a tuberous sclerosis patient, however guidelines on surveillance are lacking. Radiological imaging has not been an effective form of detecting pancreatic angiomyolipomas. This case highlights the importance of continued surveillance, and the possible role of EUS in that surveillance, for recurrent angiomyolipoma following nephrectomy in patients with tuberous sclerosis.
1389 Unusual Presentation of an Intraductal Papillary Neoplasm of the Bile Duct (IPNB) Masquerading as Mirizzi Syndrome Correctly Diagnosed With SpyGlass Cholangioscopy
Authors: Fayyaz Mohammad , Schwarz Roderich 
Published: October 2019

INTRODUCTION:
Intraductal papillary neoplasm of the bile duct (IPNB) is relatively rare in the Western world with higher incidence reported in Far Eastern countries because endemic hepatolithiasis and clonorchiasis. We describe an unusual presentation of the tumor masquerading as Mirizzi syndrome.

CASE DESCRIPTION/METHODS:
A 64-year-old lady with a history of cholelithiasis presented to the hospital with acute onset of severe epigastric and right upper quadrant pain with associated symptoms of nausea and vomiting. She was afebrile and was noted to have epigastric and right upper quadrant tenderness. She had abnormal liver enzymes with AST of 958, ALT of 994, alkaline phosphatase of 170 and bilirubin of 1.5 that increased to 3.9. An ultrasound revealed cholelithiasis and a positive sonographic Murphy's sign. There was intrahepatic and extrahepatic biliary ductal dilation. Debris was noted within a 9 mm diameter bile duct. A HIDA scan revealed non-visualization of the gallbladder at 4 hours compatible with acute cholecystitis/cystic duct obstruction. MRI with gadolineum and MRCP revealed a 17 mm irregular shaped filling defect within the cystic duct with extension into the junction of the common hepatic and common bile duct most consistent with a large stone, Mirizzi syndrome to be considered. It also revealed cholelithiasis and gallbladder wall edema consistent with acute cholecystitis. A ERCP was performed that revealed a filling defect causing obstruction at the CHD/CBD/cystic duct junction thought to be caused by a large stone impacted within the cystic duct. A sphincterotomy was performed and multiple balloon sweeps were done without retrieval of a stone. A 10 F/9 cm size plastic stent was placed with complete resolution of her symptoms. She was scheduled for A SpyGlass cholangioscopy for further evaluation and possible electrohydraulic lithotripsy/stone extraction. SpyGlass cholangioscopy interestingly revealed a tumor occluding the cystic duct and extending into the CHD and CBD causing luminal narrowing. The tumor had frond-like papillary projections characteristic of an IPNB. Biopsies obtained with SpyBite biopsy forceps confirmed IPNB. The patient underwent tumor resection along with cholecystectomy and Roux-en-Y hepaticojejunostomy with excellent recovery.

DISCUSSION:
Peroral cholangioscopy with biopsies provided a definite diagnosis in this patient with an acute Mirizzi syndrome like presentation of IPNB leading to appropriate surgical management.