bluuebunny/crossref_metadata_2025 huggingface dataset:
1258 A Case of Direct Necrosectomy via Sinus Tract Endoscopy in the Retroperitoneum: A Unique Approach to a Difficult Problem
Authors: Bader Geoffrey A. , Zibert Kimberly , Miller Charles B.
Published: October 2019

INTRODUCTION:

Pancreatic fluid collections are one of the most common complications of acute pancreatitis. While the majority of pancreatic fluid collections are self-limited, acute necrotic collections have a high likelihood of evolving into walled off pancreatic necrosis, with nearly two-thirds of cases requiring intervention. Endoscopic debridement of centrally located walled off pancreatic necrosis via transmural stents and direct necrosectomy is becoming increasingly popular due to its high success rate and low associated morbidity and mortality. Management of lateral walled off pancreatic necrosis has traditionally been managed surgically. New innovations in multidisciplinary, minimally invasive procedures are becoming increasingly utilized, though case reports are rare. We present a case of sinus tract endoscopy via a surgically placed trocar to debride an extensive collection of walled off necrosis in the left retroperitoneal space.

CASE DESCRIPTION/METHODS:

Our patient is a 65-year-old female with a history of alcohol induced, necrotizing pancreatitis with infected walled off necrotic collections extending along the infrarenal spaces bilaterally. She initially underwent computed tomography guided percutaneous catheter placement, which failed to provide source control. Given the large size and technically difficult location of the infrarenal necrosis, a multidisciplinary approach was pursued. In the operating room, the general surgery service placed a 15 mm surgical trocar into the left retroperitoneal space to successfully access the lesion. The therapeutic gastroenterology team then advanced a pediatric colonoscope via the trocar into the retroperitoneal space. Following the previously placed percutaneous drains, and utilizing various sized snares, 3-prong grasper, and Rat tooth forceps, all necrotic tissue was successfully debrided and retrieved. A wire was left in place and used to successfully place a surgical drain. The patient tolerated the procedure well without acute complications. Repeat cross sectional imaging demonstrated significant interval decrease in size of the necrotic collections. The patient recovered without further intervention and was doing well at 6 month follow up.

DISCUSSION:

Sinus tract endoscopy via surgically or CT guided placement of trocars is an efficacious and safe procedure for lateral walled off pancreatic necrosis. Our case illustrates the evolving role for minimally invasive procedures for pancreatic fluid collections inaccessible to transmural approaches.
1260 Am I Seeing Double: A Rare Case of an Intrapancreatic Accessory Spleen Mimicking a Pancreatic Endocrine Neoplasm
Authors: Rozenshteyn Frederick , Hershman Melissa , Weisberg Ilan , Nagirimadugu Ankita , Collins Kelsey , Tangel Matthew , Gromis Jonathan
Published: October 2019

INTRODUCTION:

Intrapancreatic accessory spleen (IPAS) is a rare but benign lesion associated with aberrant embryologic development. In postmortem studies, accessory spleens have been found in the hilum of the spleen or near the tail of the pancreas in 10-30% of cases. However, IPAS are uncommon and are rarely reported in the literature. The hypervascular pattern of these lesions on imaging may mimic pancreatic neuroendocrine neoplasms, and misdiagnosis may result in surveillance imaging and operative management for an ultimately benign condition. Given 40% of endocrine neoplasms are non-functioning, hormonal markers may not differentiate between the two lesions. With recent improvements in radiologic imaging, non-invasive diagnosis of IPAS has become feasible. On radiologic studies, IPAS will be well-defined, homogenous, and share similar attenuation and intensity as the spleen. Awareness and familiarity with diagnostic features of IPAS is imperative when evaluating a pancreatic mass.

CASE DESCRIPTION/METHODS:

A 74-year-old male with a history of HCV and alcohol abuse presented for evaluation of a pancreatic mass. Two years prior, a CT scan for diverticular bleeding incidentally identified a hypervascular 2.6 cm mass within the pancreatic tail, concerning for a pancreatic neuroendocrine neoplasm. At the time, the patient was asymptomatic, without abdominal pain, weight loss, diarrhea, or lab abnormalities. Due to the concern for malignancy, the patient subsequently underwent EUS with fine needle biopsy, which revealed acute and chronic inflammation without neoplasia and small samples of benign pancreatic parenchyma. Surveillance MRCP was performed, which demonstrated a stable, hyper-enhancing pancreatic tail lesion seen on multiple pulse sequences with dynamic gadolinium enhancement similar to that of the normal spleen. Ultimately, the lesion was diagnosed as an intrapancreatic accessory spleen.

DISCUSSION:

IPAS masses are clinically silent, often found incidentally, and initiate a malignancy workup and at times unnecessary surgery. The consequences of missing a pancreatic malignancy are grave, but recent advances in imaging techniques allow for radiologic differentiation of IPAS from pancreatic neoplasms. For unclear cases, evaluation with nuclear medicine imaging can help detect splenic tissues. Overall, it is important to recognize the benign pancreatic condition of IPAS and differentiate it from diseases that do require surveillance imaging and invasive interventions.
1262 A Rare Case of Pleural Effusion Due to a Pancreatico-Pleural Fistula in the Setting of Pancreas Divisum
Authors: Mukherjee Samiran , Harne Prateek , Sharma Anuj , Masood Umair , Manocha Divey
Published: October 2019

INTRODUCTION:

A pancreatico pleural fistula is a rare complication of pancreatitis which classically results from a pancreatic duct disruption or a pseudocyst extension into the pleural cavity. The literature present on this condition continues to remain sparse. We attempt to present a rare case of a pancreatico-pleural fistula in the setting of a congenital pancreatic anomaly which initially presented as a recurrent pleural effusion in the setting of chronic pancreatitis.

CASE DESCRIPTION/METHODS:

Our patient was a 51-year-old female who presented with intractable abdominal pain and shortness of breath with a confirmed diagnosis of a pancreatic pseudocyst. On admission, her chest Xray was significant for pleural effusion. Further analysis of the pleural fluid revealed the presence of elevated lipase and amylase levels. The patient eventually underwent an ERCP with contrast that revealed contrast leakage tracking upwards from the dorsal pancreatic duct suggesting a pancreatico-pleural fistula. This was in addition to the inability to fill the ventral pancreatic duct, suggesting a complete pancreatic divisum. A pancreatic duct plastic stent was placed and the pleural effusion gradually subsequently subsided.

DISCUSSION:

The case allows us to consider one of the lesser known etiologies of pleural effusion in the setting of pancreatitis (the other one being transdiaphragmatic lymphatic blockage) and provides us with an opportunity to develop a step to manage the same. This is the only known case report of a pancreatico-pleural fistula with a concomitant complete pancreatic divisum.
1264 Mediastinal Pancreatic Pseudocyst With Expansion Into Bilateral Pleural Spaces
Authors: Smith Cierra C. , Smith Alexander W. , Anderson Jay
Published: October 2019

INTRODUCTION:

Pseudocysts are a common complication of both acute and particularly chronic pancreatitis. Uncommonly, pseudocysts may communicate with thoracic cavities, including the mediastinum. We describe a case of acute pancreatitis complicated by a mediastinal pseudocyst with bilateral pleural space involvement.

CASE DESCRIPTION/METHODS:

A 51-year-old male with a history of alcohol abuse presented shortly after discharge from a prolonged hospitalization for his first lifetime episode of acute alcohol-induced pancreatitis. A CT of the chest ordered on admission for new onset respiratory distress demonstrated bilateral pleural effusions. Fluid analysis showed a right sided empyema and left sided aseptic exudative effusion. The right empyema resolved after chest tube placement but there was rapid recrudescence of a right pleural effusion after chest tube removal. Repeat fluid analysis was significant for a pancreatic specific amylase level of 2620. Repeat CT imaging demonstrated a posterior mediastinal fluid collection that arose from the pancreas, extended through the esophageal hiatus and was contiguous with new fluid collections in both pleural spaces and this was confirmed with MRCP. The mediastinal pseudocyst was deemed not amenable to endoscopic drainage so ERCP was performed with stent placement to the ventral pancreatic duct along with chest tube placement for the larger right sided fluid collection and subcutaneous octreotide administration, with eventual reduction of pseudocyst size.

DISCUSSION:

Mediastinal pseudocysts are a rare complication of acute pancreatitis more commonly seen in chronic pancreatitis, though exceedingly rare in general. It is theorized that mediastinal pseudocysts occur based on ease of pancreatic fluid movement. The pararenal and pelvic extraperitoneal spaces are typically the easiest spaces for this fluid to penetrate but conditions that create inflammatory fibrosis of these areas may make ascending flow into the mediastinum the path of least resistance. On CT scan and on MRCP, our patient had a mediastinal pseudocyst that communicated directly and inseparably with both the right and left pleural space. To our knowledge, this has not been previously seen in the limited case reports of mediastinal pseudocysts. Despite not being amenable to endoscopic drainage based on location, the use of octreotide coupled with pancreatic duct stenting proved effective in reducing the caliber of this mediastinal pseudocyst on interval imaging.
1266 Beta-Sitosterol-Induced Acute Pancreatitis: A Case Report and Review of the Literature
Authors: Lorenze Alyssa , Hsueh William , Nasr John
Published: October 2019

INTRODUCTION:

While drug-induced pancreatitis from corticosteroids has been well described in the medical literature, the exact mechanism is unclear. We present the first reported case of drug induced pancreatitis from Beta sitosterol, a naturally occurring plant sterol structurally similar to cholesterol, obtained primarily through Western diet and supplementation.

CASE DESCRIPTION/METHODS:

A 57-year-old male with history of situs inversus and Benign BPH presented from an outside facility with a two day history of generalized abdominal pain. The morning of admission the pain became sharp and severe over the mid-epigastric area, radiating to the right upper quadrant causing the patient to become bedbound. Lab results were noted for WBC 10.2, Hb of 11.1 g/dl, Hct of 31.9%, BUN 14 mg/dl, glucose 143 mg/dl, and calcium of 9 mg/dl. Liver tests including AST, ALT, Total bilirubin, and ALP were within normal limits. Lipase was markedly elevated at 572 U/L (>7x upper limit of normal). CT and US of the Abdomen were remarkable for acute pancreatitis with acute necrotic collection and normal appearing gallbladder and bile ducts without gallstones. The patient was managed with aggressive IV hydration and supportive management. After 48 hours the patient's hemoglobin decreased to 8.9 mg/dl. The patient had significant resolution of symptoms after 4 days and was referred to gastroenterology clinic for follow up. At his follow up appointment, the patient disclosed that he had started a new herbal supplement, B-sitosterol, on the morning that his symptoms began. Patient denied history of smoking, alcohol use, gallstones, recent infection, illicit drug use, trauma, exposure to scorpions, or weight loss. Abdominal MRI and MRCP obtained 4 months after his initial presentation showed interval resolution of pancreatitis and pancreatic fluid collections, normal pancreas, and normal biliary and pancreatic ducts.

DISCUSSION:

We describe a case of acute pancreatitis in a patient with a history of BPH on Beta sitosterol, a phytosterol and common over the counter herbal supplement, who presented with a two day history of progressively worsening abdominal pain and nausea. Following a thorough history and a negative medical workup to exclude other causes, the patient was treated with supportive measures and was advised to discontinue Beta sitosterol. His symptoms drastically improved within days and he continued to remain asymptomatic with normal imaging at his follow up appointment.
1267 Recurrent Mantle Cell Lymphoma to the Gall Bladder: A Peculiar PET CT
Authors: Datta Samit K , Inaganti Anupama , Hilal Jonathan , Chausow Douglas , Guda Nalini
Published: October 2019

INTRODUCTION:

Mantle Cell Lymphoma (MCL) is a B-cell non-Hodgkin lymphoma that has a variable course. The initial presentation of MCL is usually nonspecific and varies based on the organ involved. Initial treatment includes a variety of chemotherapeutic options; however, almost all patients will have refractory or recurrent disease due to the rapid development of chemotherapy resistance. In this case report, we present a rare presentation of recurrent mantle cell lymphoma in the gall bladder.

CASE DESCRIPTION/METHODS:

Our patient is a 61-year-old female with history of mantle cell lymphoma diagnosed in 2008. At that time patient was treated with Rituxan/hyper-CVAD with an excellent response to treatment. Patient had a disease recurrence in 2012 on axillary lymph node biopsy and received 6 cycles of Bendamustine and Rituximab and achieved remission as determine by PET CT. The patient decided against allogeneic hematopoietic stem cell transplant (HCT) and was started on Rituximab maintenance therapy by 2013. Patient had another recurrence on axillary lymph node biopsy in 2017 and was placed on Ibrutinib with no FDG noted on subsequent PET-CTs in 2/2018 and 5/2018. A surveillance PET CT performed in 12/2018 showed intense avid FDG uptake involving the gallbladder (Figure 1). A MRI was performed that showed an enhancing lesion in the dorsal aspect of the descending duodenum measuring 18 mm. Upper endoscopy revealed a prominent fold with biopsies taken with no histopathologic abnormality. The patient was subsequently taken for uncomplicated cholecystectomy with one lymph node associated and sent for pathology. Pathology results showed the cystic duct lymph node and gallbladder wall involved by MCL with 40 mitoses per 15HPF and Ki-67 proliferative index of 34% (Figures 2 and 3). This suggested a more aggressive MCL. The patient was started on Revlimid and Rituximab, and will be awaiting clinical trial eligibility at a nearby facility.

DISCUSSION:

MCL typically follows an aggressive clinical course with high risk of recurrence regardless of chemotherapeutic regimen. One previous case with MCL to the gallbladder was found incidentally as a lymphomatous polyp. An additional case presented with intussception with a mass noted during surgical repair. Overall, there have been approximately 66 cases of B-cell lymphoma noted to the gallbladder, and two prior cases as above of MCL. Our case represents a rare presentation of relapsed MCL to the gall bladder and the wide possibility of metastasis associated with recurrent MCL.
1268 The Hidden Gallbladder: An Unusual Case of Bouveret Syndrome
Authors: Datta Samit K , Hilal Jonathan , Guda Nalini
Published: October 2019

INTRODUCTION:

Bouveret syndrome is the most infrequent variant of gallstone ileus characterized by a biliary fistula with the stomach or small intestine. The fistula occurs from recurrent gallbladder inflammation causing adhesion to the GI tract followed by local necrosis. Patients generally present nausea, vomiting, and abdominal pain/distention with variable lab abnormalities, with jaundice and hepatic enzyme alternations in only 1/3 of patients. 43-68% of patients have a history of recurrent biliary colic and given the delay in diagnosis, patients can present very late with dehydration and weight loss. CT scan is diagnostic modality of choice with an overall 93% sensitivity, 100% specificity, and 99% diagnostic accuracy. We present a rare case of Bouveret Syndrome missed by CT scan and diagnosed by ERCP.

CASE DESCRIPTION/METHODS:

Our patient is an 81-year-old male who presented with jaundice and intermittent fever/chills. He reported 7 months of intermittent nausea, vomiting, and abdominal pain and reportedly lost approximately 80 pounds in the past year. The patient denied any prior cholecystectomy. He was noted to have total bilirubin of 8.1 and an alkaline phosphatase of 523. RUQ U/S showed a 1.5 cm common bile duct (CBD) and non-visualization of the gall bladder. CT scan was performed showing CBD of 2.0 cm but no clear cholelithiasis and reported absence of the gallbladder. Patient subsequently had EUS/ERCP. EUS showed a shrunken decompressed gallbladder with cholelithiasis (Figure 1) and bilious output into the lesser curvature of the stomach concerning for fistulous process. ERCP showed dilated CBD with multiple large gallstones with sphincterotomy and balloon extraction (Figure 2). Impaction cholangiogram with cinematic fluoroscopy confirmed a cholecystogastric fistula (Figure 3). The patient was a poor surgical candidate but had improvement in his symptoms and liver function tests and was safely discharged home.

DISCUSSION:

Bouveret syndrome is a very rare complication of gallstone ileus that can have high mortality due to its late and nonspecific presentation. There have been approximately 300 cases of documented Bouveret syndrome with 60% of cases involving a cholecystoduodenal fistula. 5% of cases present with a cholecystogastric fistula. As CT is the mainstay of diagnosis, our case is further unique in that CT scan and RUQ U/S reported an absent gallbladder (which the patient denied), likely due to the contracted nature noted on EUS and required endoscopic visualization and ERCP to confirm.
1271 Arteriobiliary Fistula Repair With Biliary Endoprosthesis: When Trans Arterial Embolization Is Not an Option
Authors: Hayat Maham , Zia Hassaan , Hayat Muhammad Hashim , Bolkhir Ahmed
Published: October 2019

INTRODUCTION:

Arteriobiliary fistula is an uncommon complication manifesting as hemobilia or an upper gastrointestinal (GI) bleed. Etiologies include intra-hepatic artery chemo-embolization, trauma or previous invasive biliary instrumentation. We describe a case report of using two fully covered metal biliary stents for closure of an acquired arteriobiliary fistula.

CASE DESCRIPTION/METHODS:

20-year-old male with history of neuroblastoma s/p resection and chemoradiation underwent placement of percutaneous transhepatic biliary drain (PTBD) for common bile duct (CBD) strictures. He was admitted to the hospital with acute blood loss anemia, transaminitis and sanguineous output from the biliary drain. Upper endoscopy revealed one clean based gastric ulcer which was injected with epinephrine. Patient was scheduled to undergo replacement of external/internal biliary drain. Intraoperative cholangiogram revealed extensive thrombus within central biliary ducts. It also demonstrated a fistulous connection between the common hepatic duct and the right hepatic artery. Angiogram showed replaced right hepatic artery, originating from superior mesenteric artery with occluded 4 cm proximal end, distal reconstitution was seen via fine collaterals; which made endovascular repair impossible (Figure 1 left). As a temporizing measure, a COOK 16 Fr × 40 cm biliary drain was replaced and advanced beyond the arterial-biliary fistulous connection, serving as a tamponade (Figure 1 right). Over the subsequent days, patient continued to have intermittent sanguineous biliary output. After surgical options were ruled out, two GORE VIABAHN 11 mm × 29 mm balloon expandable fully covered metal stents were deployed within the common bile duct to exclude the biliary-right hepatic artery fistula (Figure 2 left, right). A similar sized biliary drain was replaced subsequently (Figure 3). Hemobilia resolved and hemoglobin remained stable. Patient was discharged successfully to a center of excellence for multivisceral transplant.

DISCUSSION:

The management of hematobilia often includes an initial cholangiogram, if an arterial source is identified, hepatic artery embolization is the treatment of choice. This unique case demonstrates the utility of an endobiliary prosthesis for exclusion of arteriobiliary fistula where endovascular repair was not an option. The shortest possible endoprosthesis should be used to avoid obliteration of other large vessels or bile duct branches. Complications include bacteremia and liver infarction adjacent to stent-grafts.
1272 Atypical Presentation of Intrahepatic Cholestasis of Pregnancy
Authors: Sadarat Fnu , Boroda Konstantin , Hertan Hilary
Published: October 2019

INTRODUCTION:

Intrahepatic cholestasis of pregnancy (ICP) is a common clinical problem evident in approximately 1% of pregnancies in the United States. The incidence is more common in Chilean and Swedish ethnic groups. Due to the pathognomonic early symptom of severe pruritus, most patients are diagnosed and treated promptly. However, the risk of fetal demise is high, ranging from 2-11%. In very rare cases patients may present with atypical symptoms that can delay diagnosis and appropriate management.

CASE DESCRIPTION/METHODS:

A 40-year-old woman, gravida 4 para 2, presented in her 20th week of gestation with severe right upper quadrant pain radiating to the epigastric region for 3 days. Initial workup showed elevated total bilirubin of 4.3 mg/dl with a direct bilirubin of 3.7 mg/dl; Alanine aminotransferase of 39 U/L and aspartate aminotransferase of 27 U/L. Initial ultrasound of the abdomen showed an enlarged liver with the right hepatic lobe measuring up to 21 cm in length, status post cholecystectomy and normal caliber proximal common bile duct. Patient's bilirubin trended up and she became jaundiced, so MRCP without contrast was obtained. It showed no choledocholithiasis and no biliary ductal dilatation. Hepatitis A, B, C, and E virus PCR, cytomegalovirus IgM, anti-mitochondrial antibody, anti-smooth muscle antibody were all negative as well. The patient never developed pruritus, however, bile acids were checked and found to 190 µmol/L and eventually peaked at 290 µmol/L. She later developed intense pruritus 1 week after initial presentation and abdominal pain had completely resolved. She was started on ursodiol and cholestyramine. The bile acid level eventually decreased to 39 µmol/L 1 month later and her pruritus improved. She continued to follow with maternal-fetal medicine and gastroenterology, there was no harm to the fetus.

DISCUSSION:

ICP usually presents with pruritus of the hands and feet due to elevated bile acid levels in the blood during 2nd and 3rd trimester of pregnancy. Our patient's only symptom was acute right upper quadrant pain with no pruritus, which is very atypical for ICP. There are no case reports of this disorder presenting solely with abdominal pain. It is important to recognize abdominal pain as a possible presenting symptom of ICP in order to diagnose the condition early.
1273 IgG4 Related Cholangiopathy Masquerading as Cholangiocarcinoma
Authors: Iriana Sentia , Parbhu Sheeva , Sossenheimer Michael , Hatch Jenny
Published: October 2019

INTRODUCTION:

IgG4 related diseases can involve multiple organs, best described in cases of autoimmune pancreatitis (AIP). IgG4-related cholangiopathy is difficult to distinguish from other forms of biliary ductal disorders including primary biliary cholangitis, primary sclerosing cholangitis (PSC), and cholangiocarcinoma (ChCA). Specifically, IgG4 cholangiopathy and ChCA share many clinical and imaging findings which make it difficult to distinguish these entities. We report a rare case of IgG4 cholangiopathy mimicking a ChCA.

CASE DESCRIPTION/METHODS:

A 69-year-old male presented with nausea/vomiting, weight loss, painless jaundice, and CT suggestion of a malignant hilar mass. ERCP with cytology and EUS with FNA from the hilar mass were negative for malignancy. Two further ERCPs with Spyscope hepatic duct bifurcation biopsies showed severe stenosis of the right hepatic ducts and diffuse biliary stricturing suggestive of PSC. Brushings for cytology and fluorescence in situ hybridization (FISH) were obtained, balloon dilation and stenting performed. MRCP noted multifocal intrahepatic biliary strictures, suggestive of PSC; a hilar mass, as seen on CT, was not seen. The MR suggested a solid pancreatic uncinate process mass. A repeat EUS showed changes of chronic pancreatitis but no mass. A comprehensive review of the combination of intrahepatic strictures, pancreatic atrophy and uncinate mass suggested a diagnosis of IgG4 disease; an ANA was positive at 1:160 and IgG4 was elevated at 1,250 mg/dL (normal < 123). The patient was started on prednisone and had complete resolution of abnormal liver tests, clinical symptoms, MR biliary ductal dilatation, and MR pancreatic mass within 3 weeks.

DISCUSSION:

While described in AIP, IgG-4 related diseases can affect multiple organs, including kidneys, liver, salivary gland, orbit, breast, pericardium, aorta, skin, lungs, prostate, meninges, and the pituitary gland. IgG4-related cholangiopathy is a distinct yet rare etiology, characterized by elevated serum IgG4 levels and IgG4-positive plasma cell infiltration with storiform fibrosis/obliterative phlebitis of the bile duct wall. It has been identified in patients with concomitant AIP and is steroid responsive. Early diagnosis of IgG4 cholangiopathy avoids prolonged hospitalizations, unnecessary repeated procedures, and morbidity/mortality due to treatment delay We describe a rare case of IgG4 autoimmune cholangiopathy with delayed diagnosis due to concern for ChCA.
1275 Intestinal-Type Intracholecystic Papillary Tubular Neoplasm (ICPN) Presenting as Acute Hemorrhagic Cholecystitis
Authors: Gill Jasmeet , Sannapaneni Shilpa , Feldman Mark
Published: October 2019

INTRODUCTION:

The term Intracholecystic Papillary Tubular Neoplasm (ICPN) was coined by Adsay et al (2012). ICPN includes mass-forming (papillary or polypoid), intramucosal and preinvasive gallbladder neoplasms larger than one cm in size.

CASE DESCRIPTION/METHODS:

A 73-year-old man presented with acute and sharp right upper quadrant abdominal pain for one day. He denied nausea, vomiting, diarrhea, fever or chills. Past medical history included COPD, non-Hodgkin lymphoma in remission and hypertension. He was not receiving anticoagulants or antiplatelet medications. On abdominal examination, there was marked tenderness in the right upper quadrant area with a positive Murphy's sign. Laboratory testing revealed leukocytosis of 22,000/µL with neutrophilia and normal liver chemistry tests and a serum lipase level. Abdominal ultrasonography showed a markedly distended gallbladder with mural thickening of up to 1.0 cm and numerous gallstones with a positive sonographic Murphy's sign. Non-contrast CT showed mixed attenuation suggesting hemorrhage into the gallbladder lumen and suggestive of acute hemorrhagic cholecystitis. He underwent open cholecystectomy. Surgical findings were a distended gallbladder with a thickened and inflamed wall. Half of the gallbladder lumen was occupied by stones and the remainder was filled with detached, friable, tan-red hemorrhagic material. Microscopic examination of several of detached fragments and intact mucosa showed an abundant ICPN of intestinal-type origin and a tubular-papillary growth pattern. There was marked acute and chronic inflammation of the gallbladder wall without mural perforation.

DISCUSSION:

ICPNs are usually seen in middle-aged individuals, with a 2- fold higher prevalence in women. Macroscopically, ICPNs are exophytic tumors: tubular/lobulated types are soft, friable and can get easily detached appearing as sludge or debris, while granular or papillary types are sessile and feathery filling the gallbladder lumen. ICPN exhibits a variety of cell lineages such as biliary (50%), gastric (25%), intestinal (8%, as in our patient), oncocytic (6%) and mixtures of lineages (∼10%). In our patient, the two most plausible pathogenic processes are either diagnosis of an incidental ICPN due to the associated acute calculus hemorrhagic cholecystitis or acute “acalculous” cholecystitis from tumor shedding and bleeding into the gallbladder lumen and causing cystic duct obstruction associated with asymptomatic (incidental) cholelithiasis.
1280 Biliary Sludge and Acute Pancreatitis Following Treatment of Sexually Transmitted Infection
Authors: Kwak Hakyong Gloria , Al Momani Laith , Bokhari Ali , Alomari Mohammad , Young Mark
Published: October 2019

INTRODUCTION:

Ceftriaxone has been shown to cause transient biliary and cholecystic sludge evident on imaging in asymptomatic subjects. This sludge may result in obstruction, and hence, acute pancreatitis and cholecystitis. Here we report a case of acute pancreatitis presenting three days following treatment with ceftriaxone which may be attributed to this phenomenon. No other risk factors could be identified.

CASE DESCRIPTION/METHODS:

A 34-year-old male patient with no past medical history presented to the hospital with the complaint of stabbing epigastric abdominal pain radiating to the back with associated nausea and vomiting of one day duration. He was seen by his primary care doctor three days prior to presentation for urethral discharge and was given an intramuscular injection of 250 mg ceftriaxone in addition to 1 g oral azithromycin with complete resolution of symptoms. Physical examination was notable for epigastric tenderness without rebound tenderness or guarding and was otherwise normal. Laboratory work up was remarkable for WBC 15200 per microliter and lipase 1961 U/L. Liver function test, serum calcium, triglycerides, and IgG4 were all within normal limit. Computed tomography (CT) scan of the abdomen confirmed findings of acute pancreatitis with well-defined pancreatic borders. This was followed by a right upper quadrant ultrasound which showed biliary and cholecystic sludge. Upon further questioning the patient denied alcohol and tobacco use, recent travel, or any prior history of mumps and herpes simplex virus infections. Furthermore, no family history of acute pancreatitis was reported. The time frame of symptoms and the absence of other risk factors point towards a drug adverse reaction. The patient was managed conservatively and discharged the following day with significant clinical improvement.

DISCUSSION:

Younger patients, patients receiving a prolonged or larger dose and patients with impaired gallbladder emptying are at a greater risk for developing sludge secondary to ceftriaxone use. This is often asymptomatic with discontinuation of the drug usually resulting in resolution of this phenomenon. This case highlights the fact that ceftriaxone induced biliary sludge may rarely result in potentially serious adverse events including acute cholecystitis and pancreatitis.
1283 Liver Abscesses as a Complication of Side-to-Side Choledochoduodenostomy
Authors: Gonzaga Ernesto Robalino , Bashir Khawaja , Bhatti Hammad , Bracha Adam S. , Vanar Vishwas
Published: October 2019

INTRODUCTION:

Side-to-side Choledochoduodenostomy (CDD) is a low morbidity surgical procedure commonly performed in the pre-Endoscopic Retrograde Cholangiopancreatography (ERCP) era to provide permanent relief and drainage of the common bile duct (CBD) in patients with retained or recurrent CBD stones, ampullary stenosis or stricture of the CBD. In side-to-side CDD, the distal bile duct becomes a poorly drained reservoir or “sump” as bile is unable to be drained from the area. Bile, stones, or debris may accumulate and lead to inflammation and bacterial growth. In many cases the inflammation in the sump can cause biliary obstruction, recurrent cholangitis, or pancreatitis, incurring the name “sump syndrome”. Although it is rare complication, sump syndrome carries a high mortality. In addition, hepatic abscesses have been reported as a complication.

CASE DESCRIPTION/METHODS:

A 61-year-old man, with a surgical history of cholecystectomy 15 years ago, presented with acute epigastric pain and severe sepsis. Laboratory studies suggested a biliary source of infection. A computed tomography showed multiple liver abscesses and pneumobilia. Urgent percutaneous biliary drainage with CBD sweep was performed. Cholangiogram demonstrated side-to-side CDD with residual debris in the sump. ERCP revealed choledolithiasis. Sphincterotomy and balloon extraction was performed to remove residual stones and debris. Distal biliary stricture was found and biliary stent was placed. The presentation of biliary disease complicated by liver abscess secondary to retrograde intrahepatic flow and history of side-to-side CDD with typical ERCP findings, established the diagnosis of sump syndrome.

DISCUSSION:

Hepatic abscesses have an incidence of 3.6 per 100,000 in the US. The majority of these cases are secondary to biliary tree disease, with pyogenic abscesses being the most common. There is limited data regarding abscesses as a late complication of sump syndrome, with few cases reported in the literature. This complication may be due to an obstruction between proximal CBD to duodenum, which leads to regurgitation of bile into intrahepatic bile ducts. Although the incidence of those abscesses is low, mortality remains as high as 12%. It is important to recognize the underlying cause of hepatic abscesses, as timely drainage and antibiotic therapy are essential to prevent significant morbidity. Furthermore, the restoration of normal bile flow from CBD via an ERCP is needed to eliminate any culprit source of infection and reduce recurrence.
1287 Metastatic Cholangiocarcinoma in a Healthy 27-Year-Old Man: A Case Report
Authors: Gribbin Caitlin , Niec Rachel , Mehta Amit , Tafesh Zaid , Wan David , Crawford Carl V. , Soumekh Amir
Published: October 2019

INTRODUCTION:

Cholangiocarcinoma is a rare, aggressive cancer of the biliary tract most often found in men between the ages of 50 and 70. Risk factors include genetic disorders, such as Lynch syndrome and cystic fibrosis, primary hepatobiliary disease, such as primary sclerosing cholangitis (PSC), and chronic liver disease. This malignancy is highly uncommon in young individuals without PSC. Here we describe the case of a young man with cholangiocarcinoma with recurrent ascites in the setting of a portal vein thrombosis (PVT).

CASE DESCRIPTION/METHODS:

A 27-year-old man with a history of metastatic cholangiocarcinoma status-post hemihepatectomy with Roux-en-Y hepaticojejunostomy and adjuvant chemoradiation presented with rapidly accumulating ascites secondary to a portal vein thrombosis (PVT) discovered on abdominal imaging. A large-volume paracentesis was performed followed by thrombus recanalization and placement of a portal vein stent. The patient was discharged and presented two weeks later with recurrent ascites and evidence of occlusion of the portal vein stent as well as a new thrombus in the superior mesenteric vein (SMV) and a possible hepatic abscess seen on abdominal imaging. Clinical examination demonstrated a distended abdomen with a positive fluid wave. Laboratory studies revealed elevated white blood cell count (WBC) as well as Klebsiella, Candida, and gram-positive rods (GPR) bacteremia. The benefits versus risks of thrombectomy for recurrent PVT occlusion were considered as though this procedure could ameliorate ascites, the risk of rapid reocclusion was deemed to be high. Furthermore, the patient was at risk for intra- and post-operative complications due to an anticoagulant regimen and unstable clinical status. Ultimately, the patient received repeat LVP and remained on anticoagulants with no additional interventions for recurrent PVT. The patient and his family were discharged home with supportive care.

DISCUSSION:

Cholangiocarcinoma is a rare, aggressive malignancy of the biliary tract predominantly found in middle-aged men with a high risk of metastasis and a rapidly progressive course. The diagnosis is made on imaging and confirmed on biopsy. Treatment is dependent on disease staging and includes surgery, chemotherapy, and radiation. The disease is rapidly progressive and may predispose patients to pulmonary vein obstruction, leading to portal hypertension and associated sequelae secondary to tumor burden or PVT.
1295 A Unique BRIC Case With Response to Glucocorticoids
Authors: Lorio Eric , Valadez David , Alkhouri Naim , Loo Nicole
Published: October 2019

INTRODUCTION:

Benign Recurrent Intrahepatic Cholestasis (BRIC) represents a rare class of autosomal recessive chronic cholestasis disorders usually presenting with intense pruritus and jaundice. BRIC type 2 (BRIC 2) results from a defect in the ABCB11 gene, which encodes the ATP dependent bile salt export pump (BSEP).
1
Here we report a unique case of a 27-year-old heterozygous female presenting with BRIC 2 and a response to steroids that may have implications for future treatment.

CASE DESCRIPTION/METHODS:

A 27-year-old female presented with 4 weeks of progressive jaundice, pruritus, and weakness. A prior episode one year earlier was notable for similar symptoms that resolved with supportive measures and a liver biopsy that showed nonspecific cholestasis with features suggesting extrahepatic duct obstruction. Presenting labs were notable for AST 63, ALT 74, total bilirubin (TB) 9.1, direct bilirubin (DB) 6.7, GGT 22, and alkaline phosphatase 227. Hepatitis serology, α-1 antitrypsin, hemochromatosis, and autoimmune workup were negative. Abdominal ultrasound and CT were unremarkable. MRCP revealed mild liver enlargement (19 cm), but no biliary stricture or dilatation. Liver biopsy showed extensive canalicular cholestasis with associated mild hepatocyte pericentral and perisinusoidal fibrosis. Her symptoms and labs continued to worsen, with TB peaking at 14.2 and DB at 11.5. On day 18, she was started on methylprednisolone 40 mg IV daily and achieved modest symptomatic improvement. On day 21, TB had downtrended to 8.1, and she was discharged home with a five-day course of prednisone. Genetic testing revealed heterozygosity for ABCB11.

DISCUSSION:

This report demonstrates a fairly typical presentation of BRIC, but in an older patient than typically seen. Though BRIC classically presents with its first episode prior to second decade of life, it can present in patients of any age.1 Our patient's heterozygosity for CFTR, NPHP4, and A1ATD (SERPINA1) may explain the severe nature of her disease expression, as heterozygosity in each of these genes has been associated with cholestasis. Her steroid response suggests a potentially novel treatment for BRIC and highlights the need for further consideration of steroid therapy in BSEP-related diseases.
1296 Elevated CA 19-9 Associated With Heavy Black Tea Consumption
Authors: Jiang Alice C. , Chan Edie Y. , Jakate Shriram , Shah Nikunj
Published: October 2019

INTRODUCTION:

CA 19-9 is an important tumor marker for pancreatic cancer, but is also seen in non-malignant conditions such as cholelithiasis, cholangitis, pancreatitis, and other non-pancreaticobiliary conditions such as interstitial lung disease. This is a case of markedly elevated CA 19-9 found in the setting of a simple biliary cyst in the liver and heavy black tea consumption.

CASE DESCRIPTION/METHODS:

A 34-year-old woman with no significant past medical history presented to the ER for acute onset RUQ pain and nausea with vomiting. She had an ultrasound showing a 10.8 × 16.1 × 9.5 cm cystic lesion. Follow-up CT described the lesion as a large non-enhancing, hypoattenuating mass within the medial segment of the left lobe, most likely representing a cyst. AST, ALT, alkaline phosphatase, total bilirubin, albumin, INR and CBC were normal. AFP was <2.0 and CA 19-9 was 45,266 U/mL. On further interview, she reported drinking large amounts of black tea daily. She underwent a left lobectomy, with pathology showing simple biliary cyst. After surgery, her symptoms resolved and she reduced tea consumption to once weekly. Her CA 19-9 level decreased to 29 U/mL by 1 month post-resection, and remains normal 1 year later.

DISCUSSION:

The first case report of an association between heavy black tea consumption and elevated CA 19-9 hypothesized that the high gallated flavenoid content in black tea can impact a range of molecular targets influencing cell growth through the angiogenesis pathway.
1,2
In a study of 270 healthy individuals with heavy black tea consumption, 43.3% had elevated levels of CA 19-9 between 69-105 U/mL. The remainder of individuals had levels of 40 U/mL or lower. The proposed mechanism was stimulated production of CA 19-9 in epithelial tissues. While the exact mechanisms remain unknown, the presence of heavy black tea consumption should be considered in patients with elevated CA 19-9 in the absence of pancreaticobiliary disease because it is an easily modifiable factor.
1299 Sorafenib Induce Acute Pancreatitis: A Seldom Reported Side Effect
Authors: Then Eric O. , Lopez Michell , Ofosu Andrew , Are Vijay S. , Culliford Andrea , Gaduputi Vinaya
Published: October 2019

INTRODUCTION:

Sorafenib is a multikinase inhibitor that has been used successfully in patients with hepatocellular carcinoma. Given its recent approval in the market, many of its side effects are still being uncovered. Here we present a rare case of Sorafenib induced acute pancreatitis.

CASE DESCRIPTION/METHODS:

The patient is a 60-year-old male with a medical history of hepatocellular carcinoma (stage IV with metastasis to bone), Hepatitis C virus, hypertension and rheumatoid arthritis who presented to the emergency department complaining of slowly progressive epigastric abdominal pain for 2 weeks. Of note the patient was started on Sorafenib 400 mg twice daily three weeks prior as an outpatient. Notable laboratory workup included a lipase of 189 U/L and a triglyceride level of 70 mg/dL. Notable imaging included a computed tomography of the abdomen which showed mild peri-pancreatic haziness consistent with acute pancreatitis and a ill defined hypodense lesion in the right hepatic lobe that was unchaged from previous imaging. The patient was then admitted under the impression of acute pancreatitis secondary to Sorafenib. Prior to reaching this diagnosis, alternate etiologies of acute pancreatitis were ruled out. This included ingestion of alcohol, hypertrigliceridemia, cholelithiasis and other medications. During his admission the patients diet was held and he was given early intravenous fluid resuscitation. Two days later his abdominal pain resolved and he was safely discharged home with follow up appointments. On follow up visit the patient was restarted on Soraenib at a lower dose (200 mg twice daily) with no recurrence of symptoms.

DISCUSSION:

Sorafenib induced pancreatitis is a rare complication that has seldom been reported in the literature and whose pathogenesis has not been completely elucidated. One theory suggests that it results from pancreatic ischemia that occurs secondary to sorafenib's anti-androgenic effect on vascular endothelial growth factor (VEGRF). A second theory suggests that sorafenib's effect on gastrointestinal dysmotility results in reflux of duodenal contents into the pancreatic duct. Diagnosis of sorafenib induced pancreatitis can be done after ruling out secondary causes of pancreatitis (ie: alcohol, hypertriglyceridemia). Treatment of sorafenib induced pancreatitis includes cessation of the offending agent. There have been reported cases where sorafenib was resumed at a lower dose after initial cessation, with no clinical sequelae as was the case with our patient.
1300 Disconnected Pancreatic Duct Syndrome a Rare Complication
Authors: Truscello David , Pandey Nishi , Shah Maulik , Itidiare Michael , Chiesa Drew J. , DeRosa Justin
Published: October 2019

INTRODUCTION:

What follows is a case study of a patient with a pancreatic pseudocyst who developed a complication of Disconnected Pancreatic Duct Syndrome (DPDS). Pseudocysts are a collection of fluid and tissue that forms as result of trauma or repeated spells of pancreatitis. A rare complication of these cysts is disruption of the pancreatic duct termed (DPDS). This case report will discuss the diagnosis and management DPDS along with highlighting the use of endoscopic stenting for the management.

CASE DESCRIPTION/METHODS:

The patient is a 49-year-old male who presented to the hospital with sudden onset epigastric pain and distention. His past medical history includes chronic pancreatitis, and alcoholic cirrhosis with ascites, requiring large volume paracenteses. On admission, the patient's physical exam was remarkable for diffuse abdominal tenderness and ascites. Labwork was significant for a lipase of 329 U/L, albumin of 2.5 U/L, and a hemoglobin of 8.4 g/dL. Given the patient's abdominal tenderness, a paracentesis was performed with a resulting cell count concerning for Spontaneous Bacterial Peritonitis. A week later, patient had marked improvement in his abdominal exam. However, a CBC drawn on day 7 revealed a hemoglobin of 6.9 g/dL. A CT scan was ordered revealing an 8 cm inflammatory mass/pseudocyst in the head of the pancreas compressing the main portal vein. Another paracentesis was performed which showed high amylase consistent with a pancreatic source. Given these findings, GI performed a magnetic resonance cholangiopancreatography which showed pancreatic duct disconnection complicated by a large 6.4 × 5.5 cm cystic collection at the pancreatic head. An ERCP was performed with the implementation of a lumen apposing stent.

DISCUSSION:

DPDS is usually diagnosed with contrast enhanced CT which shows an absence of the pancreatic neck, body or tail. ERCP is usually able to show cut-off of the pancreatic duct or extravasation of contrast. ERCP with stenting is one minimally invasive intervention that has shown to reduce the recurrence rate of fluid collection. There are two challenges that are commonly encountered: high rates of stent migration and inability to pass a stent between the two disconnected ends of the duct. Due to the fact that there is still a lot that is not known about this disease state the consensus on optimal management remains to be determined. DPDS is a disease state that can be difficult to manage which is why early identification is the mainstay of successful treatment.