bluuebunny/crossref_metadata_2025 huggingface dataset:
1356 A Case of IgG4-related Sclerosing Cholangitis Initially Mistaken for Cholangiocarcinoma
Authors: Agrawal Rohit , Attar Bashar M. , Majeed Muhammad , Randhawa Tejinder , Vohra Ishaan , Gandhi Seema
Published: October 2019

INTRODUCTION:

IgG4 related disease is an orphan immune-mediated fibro-inflammatory disorder histopathologically characterized by lymphoplasmacytic infiltrates, storiform fibrosis and phlebitis with elevated serum IgG4 levels. It has a diverse range of presentation and often mimics malignant, infectious and other inflammatory conditions. We present a case of IgG4 related cholestasis- obstructive typethat was initially worked up for cholangiocarcinoma.

CASE DESCRIPTION/METHODS:

A 42-year-old male was admitted for right upper quadrant discomfort, dark urine, pale stools and pruritus for 2 weeks. He had lost around 30 pounds in the last 3 months. Physical examination revealed jaundice, submandibular lymphadenopathy and right upper quadrant tenderness. Laboratory findings were total bilirubin of 4.7 mg/dl, direct bilirubin 2.5 mg/dl, alkaline phosphatase (ALP) 733 U/L, gamma-glutamyltransferase 767 U/L, aspartate aminotransferase 240 U/L and alanine aminotransferase of 572 U/L. Computed tomography of the abdomen revealed intrahepatic biliary dilatation, thickened common bile duct, enlargement and hypo-enhancement of the pancreatic body and tail which was confirmed by magnetic resonance cholangiopancreatography (Figure 1). Endoscopic retrograde cholangiopancreatographydemonstratedstricturing in the intrahepatic, common hepatic and common bile ducts, and a stent was implanted. Histopathology of submandibular lymph node biopsy revealed small non-necrotizing granulomas and biliary brushings had no evidence of malignancy with features of acute and chronic inflammation. IgG4 levels were >300 mg/dl. Given the high suspicion for IgG4 related disease, he was treated with prednisone 40 mg daily for 4 weeks. Follow up liver chemistries revealed an ALP of 135 U/L with marked improvement of symptoms.

DISCUSSION:

IgG4 related autoimmune pancreatitis (AIP) presents as cholestasis- obstructive typedue concomitant sclerosing cholangitis (SC). It is crucial to differentiate this entity from malignancy due to the availability of effective treatments. Although the typical histopathology was absent in our patient on both lymph node and pancreatic needle biopsy, it is important to highlight that fibrosis is rarely seen in lymph nodes and needle biopsy can miss the involved tissue due to its patchy involvement. In summary, we emphasize on the limitations of biopsy and propose treating patients with steroids when the clinical picture if highly suggestive of IgG4 related disease.
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.
1398 A Stone in the Wall: Extraction of an Embedded Choledocholithiasis in the Proximal CBD by Cholangioscopy
Authors: Alsheikh Mira , El Douaihy Youssef , Andrawes Sherif , Haddad Fady , Kamar Khalil
Published: October 2019

INTRODUCTION:

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

CASE DESCRIPTION/METHODS:

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

DISCUSSION:

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

INTRODUCTION:

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

CASE DESCRIPTION/METHODS:

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

DISCUSSION:

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

INTRODUCTION:

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

CASE DESCRIPTION/METHODS:

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

DISCUSSION:

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

INTRODUCTION:

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

CASE DESCRIPTION/METHODS:

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

DISCUSSION:

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

INTRODUCTION:

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

CASE DESCRIPTION/METHODS:

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

DISCUSSION:

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

INTRODUCTION:

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

CASE DESCRIPTION/METHODS:

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

DISCUSSION:

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

INTRODUCTION:

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

CASE DESCRIPTION/METHODS:

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

DISCUSSION:

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

INTRODUCTION:

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

CASE DESCRIPTION/METHODS:

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

DISCUSSION:

OGCs & ACPs are rare histological subtypes of pancreatic cancer with aggressive clinical behavior. In both our patients, there was evidence of vascular and/or intraductal invasion by tumor at diagnosis. Data suggests OGCs may have a better prognosis than ACPs and ductal adenocarcinomas. Pancreas cancer multidisciplinary teams should be aware of these rare tumor subtypes and the treatment and prognostic implications thereof.