bluuebunny/crossref_metadata_2025_split huggingface dataset:
2630 Late Presentation of Caroli’s Syndrome: A Rare Cause of Cholangiocarcinoma
Authors: Hassan Ammar , Weissman Simcha , Pinkhasov Mark , Sciarra Michael 
Published: October 2019

INTRODUCTION:
Caroli’s syndrome (type 2) is a rare, complex form of Caroli’s disease (type 1) with an estimated incidence of 1/1,000,000. The syndrome manifests as saccular dilatations of the intra-hepatic biliary ducts and is associated with fibrosis and portal hypertension, distinguishing it from the disease form. Additionally, it predisposes patients to regionalized malignancy. Generally this presents before the age of 40. Intriguing however in our case, was the late presentation of the disease. The aim of this case report is to educate clinicians as to an obscure presentation of this rare disease, in an attempt to prevent cirrhosis, as well as subsequently increase the early detection of cancer.

CASE DESCRIPTION/METHODS:
We present a 78-year-old Asian female with a history of hypertension and cholelithiasis whom presented to the hospital with abdominal pain, jaundice, and fever. Magnetic resonance cholangiopancreatography (MRCP) revealed saccular intra-hepatic ductal dilations with a distal common bile duct (CBD) stone (Figure 1). Subsequent endoscopic retrograde cholangiopancreatography (ERCP) with biopsy, and balloon sweeping, removed the stone. Upon histologic examination of the targeted liver biopsy severe fibrosis was observed, confirming the diagnosis of Caroli’s syndrome (Figure 2). Two years later the patient returned to the office with new onset weight loss and nausea. CA19-9 was elevated at 1191. Magnetic resonance imaging (MRI) of the abdomen showed biliary duct dilation and enlarged lymph nodes (Figure 3). Endoscopic ultrasound (EUS) guided fine needle aspiration (FNA) of the celiac lymph nodes was subsequently performed. Upon immunohistochemistry, the pathology showed tumor glands positive for CK7 and CDX2--consistent with metastatic adenocarcinoma of pancreaticobiliary origin.

DISCUSSION:
Caroli’s syndrome is a rare, autosomal recessive disorder that typically manifests in a patients early twenties. Interestingly, our patient did not develop any symptoms until the age of 78. Distinguishing Caroli’s syndrome from recurrent pyogenic cholangitis is key and can be done in the setting of saccular dilatations on imaging as in our case. If the disease is diagnosed prior to the development of cirrhosis or cancer, patients may benefit from liver transplant. Individuals with Caroli’s disease are at a higher risk for developing cholangiocarcinoma. Prompt diagnosis of Caroli’s syndrome and awareness of the risk for cancer is vital, as the potential for malignancy and metastasis is significant.
2633 A Challenging Case of Ileo-cecal Intussusception in Pregnancy
Authors: Shah Aun R. , Landsman Marc , Sandhu Dalbir 
Published: October 2019

INTRODUCTION:
Intussusception is an extremely rare cause of intestinal obstruction in adults; being reported in up to 1% of cases of obstruction and in contrast to children almost always is secondary to an identifiable lesion forming a lead point. Its incidence is even lower in pregnancy with studies reporting incidence rates ranging from 0.067% to 0.0015%. Rarity of the condition combined with non-specific clinical presentation make diagnosis difficult in adults. This is particularly challenging in pregnant patients due to the overlap of symptoms with pregnancy and limited findings on abdominal examination. High morbidity and mortality for both mother and child warrant prompt investigation and surgery.

CASE DESCRIPTION/METHODS:
We describe the case of a 39-year-old female who presented in the third trimester with abdominal pain and an episode of hematochezia. Ultrasound findings were suggestive of ileo-colic intussusception that was confirmed with subsequent magnetic resonance imaging. The lead point was formed by an adenocarcinoma of the colon measuring 4 cm. Our patient underwent a right-sided colectomy with primary ileocolostomy. The postoperative course was uneventful for both mother and fetus, and the patient was discharged a week after the surgery.

DISCUSSION:
Intussusception very rarely presents during pregnancy. Ultrasonography of the abdomen is the initial test of choice in most cases and the findings of single or double anechoic rings surrounding a central echogenic focus is the classic hallmark. CT scanning is considered to be superior in adults with a reported diagnostic yield of over 50%. MRI, however, is the preferred imaging modality in pregnancy as it helps avoid the risk of radiation to the fetus and has comparable sensitivity to CT. Most cases are diagnosed by exploratory laparotomy which itself is associated with a high risk of miscarriage and pre-term birth, with some studies reporting miscarriage rates as high as 8%. Adults almost always require surgery as untreated bowel obstruction carries a maternal mortality of up to 6% and fetal mortality as high as 26%. Surgical reduction of intussusception can be tried but most patients require bowel resection, which is also the procedure of choice if a mass is identified at the leading point. Nearly 50% of these masses will be malignant. This report highlights the importance of abdominal imaging and urgent intervention in suspected cases of intussusception in pregnancy.
2641 Duodenal Crohn’s Disease Presenting as Gastric Outlet Obstruction Requiring Partial Gastrectomy
Authors: Castano Juan , Rapoport Grigoriy , Al Jobori Hussein , Behara Subrahmanyam 
Published: October 2019

INTRODUCTION:
Crohn’s disease (CD) is a chronic relapsing inflammatory disease which mainly affects the gastrointestinal tract. Although about 30–50% of these patients develop proximal disease, only about 0.5–4% of patients have clinically significant disease involving the stomach and/or duodenum. We present a case of isolated duodenal CD presenting as gastric outlet obstruction (GOO) requiring gastrectomy.

CASE DESCRIPTION/METHODS:
An 18-year-old woman without medical history presented to clinic with a 4-year history of postprandial abdominal pain, nausea, nonbloody emesis, decreased appetite, and unintentional weight loss. EGD demonstrated pyloric stenosis confirmed with upper GI series. Due to persistent symptoms with worsening weight loss on follow up, patient underwent repeat EGD that showed pyloric stenosis and erosive duodenitis. Duodenal biopsy showed active duodenitis with mucosal ulceration and granulation tissue. Pertinent labs were positive for ASCA IgA and ASCA IgG but negative for ANCA. As C-reactive protein was normal, patient was not begun on therapy. One month later, she underwent subtotal gastrectomy with partial small bowel resection with pathology showing marked active and chronic enteritis with ulceration and granulomas consistent with CD. On follow up, patient’s symptoms had resolved and she had largely returned to her baseline weight gaining about 30lbs.

DISCUSSION:
Published cases of proximal CD typically present with a several years history of progressively worsening gastritis-like epigastric pain. GOO developed in several cases, however, usually after numerous years. GOO symptoms frequently included bloating, vomiting, postprandial pain, and weight loss with inability to tolerate oral intake similar to our patient. Like other few published cases, our patient’s initial presentation of CD was GOO, though weight loss was quite severe in our patient. To our knowledge, rapidly progressive obstructive symptoms developed in two published cases. Duodenal CD treatment includes medical and endoscopic therapy. Medical therapy includes acid suppression with a proton-pump inhibitor and anti-TNF therapy. Endoscopic dilation may be possible if a stricture is present. Surgical intervention is undertaken when conservative therapy fails which includes resection, bypass, or strictureplasty. Duodenal CD causing GOO poses a diagnostic challenge and often requires a combination of endoscopic, radiological, histologic, and biochemical findings for accurate diagnosis and treatment.
2644 ACE Inhibitor-Induced Visceral Angioedema - A Rare Phenomenon
Authors: Sehmbey Gurbir , Mann Sarabdeep , Seetharam Anil 
Published: October 2019

INTRODUCTION:
Angiotensin Converting enzyme (ACE) inhibitors are widely used to treat hypertension and congestive heart failure. Common adverse reactions include cough, hypotension and dizziness. Angioedema is a rare reaction that occurs in less than 1% of patients. Most cases of angioedema are involving the lips, tongue, face and airway. More rarely, visceral angioedema can also occur with jejunal involvement.

CASE DESCRIPTION/METHODS:
A 24-year-old female with recently diagnosed hypertension treated with lisinopril presented for evaluation of abdominal pain, vomiting, and diarrhea. On examination, she had moderate epigastric tenderness with normal bowel sounds and no organomegaly. Labs revealed white blood cells 20 k/mm3, elevated C-reactive protein 34.2 mg/L and normal erythrocyte sedimentation rate 7 mm/hr. Complement levels including C1 esterase inhibitor, C1q and C4 were found to be normal. Stool culture also remained negative. Computed tomography (CT) of the abdomen revealed moderate ascites, marked mucosal thickening of jejunal and ileal loops with adjacent mesenteric edema (Figure 1). Magnetic Resonance Enterography did not reveal any pathological process. A paracentesis was performed with the ascetic fluid analysis revealing a low serum-ascites albumin gradient without infection. Endoscopy with small bowel enteroscopy revealed normal duodenum and jejunum (Figure 2). Jejunal biopsy returned negative for an infectious or inflammatory process. Due to negative diagnostic workup, a diagnosis of ACE inhibitor-induced visceral angioedema was suspected. Patients symptoms quickly improved 24 hours after cessation of lisinopril and she was discharged home. On follow up 4 weeks later, the patient remained symptom-free.

DISCUSSION:
Visceral angioedema is a rare side effect of ACE inhibitors most commonly affecting the small bowel. The jejunum is most often affected followed by ileum and duodenum. High levels of bradykinin can cause increased vascular permeability within the bowel mucous membrane causing edema. Symptoms commonly include abdominal pain, anorexia, vomiting and diarrhea. Symptoms typically begin 72 hours after starting the ACE inhibitor. Imaging studies show circumferential small bowel thickening with mesenteric edema and ascites. Endoscopic examination is usually normal. Diagnosis is made by exclusion of other etiologies and improvement of symptoms within 48 hours of drug cessation. Treatment includes discontinuation of the offending drug and supportive therapy until symptoms are resolved.
2652 Primary Malignant Peripheral Nerve Sheath Tumor of Small Bowel and Colon Presenting With Metastases to Bone, Lung, Liver, Spleen, and Brain
Authors: Lakkasani Saraswathi , Shaaban Hamid S. , Guron Gunwant , Fedida Andre 
Published: October 2019

INTRODUCTION:
Malignant Peripheral Nerve Sheath Tumor (MPNST) is a rare diagnosis, with an incidence of 1:100,000 per year and 4–10% of soft tissue sarcoma, contains properties both neural crest cells and mesenchymal cells. Gastrointestinal MPNSTs are rare disease conditions with only 10 cases reported worldwide in the literature.

CASE DESCRIPTION/METHODS:
A 71-year old Portuguese male, presented with 8-week history of abdominal pain, anorexia with 20Ib weight loss. Vital signs were normal. On physical examination, a firm, tender, fixed abdominal mass in the RLQ was palpated. CT scan of the abdomen showed a 10 cm necrotic mass on the right side of the abdomen with communication to the small bowel was detected with a suggestion of a walled off perforation. Colonoscopy revealed a flattening of the terminal ileum suggesting an extrinsic compression. Biopsy of tumor consisting of mixed spindle and pleomorphic cells with focal rhabdoid features, high grade nuclear features and brisk mitotic activity. A week later, he presented with symptoms of small bowel obstruction, the CT scan at this time showed a 8 cm left sided abdominal mass with communication to the small bowel with dilation of the jejunum suggesting a new partial obstruction in addition. The patient underwent a palliative debulking of the tumor. Multiple large obstructive masses were found in the Ligament of Treitz,significant portion of the small bowel, right colon, left upper quadrant and omentum. For palliation purposes, these masses were resected and anastomosis was performed. Gross examination of the surgical specimens revealed grayish-tan cystic masses filled with brownish tan hemorrhagic material. Histology of the masses were similr to previous mass. On immunohistochemistry, showed patchy weak S100 immunoreactivity and focal BCL-2 and Neuron Specific Enolase stain immunoreactivity. Ki-67 proliferation index was 70%. The pathologic diagnosis of MPNST was established. He developed multiple bone, pulmonary, liver, splenic and hemorrhagic brain metastases. He died under hospice care within six months of his initial presentation.

DISCUSSION:
MPNST, occurs mostly in association with Neurofibromatosis Type 1 (8–13%). The tumor typically originates from the nerve sheaths of the major nerves in the extremities (40%), trunk/ retroperitoneal (38%) and head and neck region (21%). However, recent case reports have also documented MPNST of the liver, thyroid gland, skin, eighth cranial nerve and rarely the greater omentum without a history of radiation or NF1.
2656 A Rare Case of Gastrointestinal and Hepatic Extra-Pulmonary Involvement of Sarcoidosis Mimicking Crohn’s Disease
Authors: Maruggi Chiara , Ahmad Dina , Nezami Behtash G. , Sclair Seth , Cummings Linda , Sinh Preetika 
Published: October 2019

INTRODUCTION:
Sarcoidosis is a systemic disease characterized by noncaseating granulomas. While pulmonary and lymphatic involvement is common, gastrointestinal involvement is rare. We report a case of diffuse gastrointestinal and hepatic involvement of sarcoidosis initially mimicking Crohn’s disease.

CASE DESCRIPTION/METHODS:
A 62 year-old man with history of chronic thrombocytopenia and sarcoidosis, presented for further evaluation of suspected Crohn’s disease after undergoing high-risk surveillance colonoscopy. The colonoscopy was significant for congested mucosa in the terminal ileum and colon as well as multiple colonic erosions (Figure 1). Pathology showed chronic active colitis with crypt abscesses in the left colon and poorly formed granulomas without dysplasia in the right colon suggestive of Crohn’s disease. The patient reported no gastrointestinal or systemic complaints and stated good control of his underlying sarcoidosis. An esophagogastroduodenoscopy (EGD) was then completed and showed moderately-congested mucosa with many non-bleeding gastric body, antral and superficial duodenal ulcers positive for Helicobacter pylori. Despite normal liver chemistries, given the presence of indirect signs of portal hypertension (splenomegaly, thrombocytopenia), a transjugular liver biopsy was pursued which was significant for portal hypertension as well as moderate chronic portal hepatitis with cirrhosis, non-necrotizing granulomas consistent with sarcoid involvement, and no histologic features of PBC (Figure 2). Labs, including hepatitis B and C serologies, ANA, and anti-mitochondrial antibody, were grossly unremarkable. After establishing an elevated portal pressure gradient, a repeat EGD was done approximately six-months later which was significant for grade I esophageal varices, erythematous and nodular mucosa in the gastric antrum and erythematous duodenopathy; biopsies were negative for H. Pylori. The patient was then referred back to pulmonology for further assessment of his pulmonary disease prior to consideration of treatments for his progressed liver disease.

DISCUSSION:
Typically presenting with pulmonary involvement, gastrointestinal involvement of sarcoidosis, particularly of the small bowel, is a very rare extra-pulmonary manifestation of sarcoidosis. Hepatic involvement with progression to cirrhosis is uncommon. Above, we present a very interesting case with both diffuse gastrointestinal and hepatic sarcoidosis subsequently noted to have progressive liver disease with portal hypertension.
2657 Novel Use of Radiofrequency Ablation in the Treatment of AVMs of Small Bowel Mucosa
Authors: Lysek Michael , Kumar Manoj , Fahmawi Yazan , Mizrahi Meir 
Published: October 2019

INTRODUCTION:
Radiofrequency ablation (RFA) is a well-authorized treatment in a number of gastrointestinal conditions such as dysplastic Barrett’s, gastric antral vascular ectasia and biliary stricture. We are presenting a case series focused on the use of RFA in the treatment of small bowel AVMs through small bowel enteroscopy, using a novel through the scope radio frequency ablation catheter.

CASE DESCRIPTION/METHODS:
Cases: This case series encompasses five cases using a new through the scope radio frequency ablation caterer to treat AVMs found in the small bowel and cecum. The age for these patients ranged from 56 to 72 years old, with two being males and three being females. Our patients presented for various reasons, including abdominal pain, abnormal CT findings, and gastrointestinal bleeding. Colonoscopies and endoscopies on these patients were unremarkable. All patients underwent single balloon enteroscopy, and multiple arteriovenous malformations (AVMs) were found. AVMs were successfully ablated using a novel through the scope radio frequency ablation catheter (RFA-SB). AVMs in these patients were found in the cecum, jejunum, ileum and stomach. 12 J/cm2 energy was applied with each paddle application. All patients recovered well after the procedure and follow up CBC showed improvement in their hematocrit. No immediate or late procedure related complications were reported.

DISCUSSION:
AVMs are one of the common causes of gastrointestinal bleeding. Ablation of AVMs in the small bowel has typically utilized argon plasma coagulation (APC) as a treatment modality. This case series looks at the use of a new RFA-SB as a new treatment modality for small bowel AVMs. This series is novel, due to the length of the new RFA catheter, as it is able to pass through a 200 cm small bowel enteroscope with better maneuverability and providing a uniform therapy than traditionally used APC technique without healthy tissue damage and achieving satisfactory hemostasis.
2660 Duodenal Perforation Caused by Inferior Vena Cava Migration
Authors: Daid Sarav , Seo Dong Joo , Mangala Yashvin Onkarappa , Toubia Nabil 
Published: October 2019

INTRODUCTION:
The Inferior Vena Cava filter is known as an effective method for preventing pulmonary thromboembolism in patients with deep vein thrombosis. Usually, the remaining IVC filters are asymptomatic and do not cause clinical problems. We report two cases of duodenal perforation caused by a remaining IVC filter which is very rare.

CASE DESCRIPTION/METHODS:
Case 1: 67 year old female patient with past medical history of heartburn,IVC filter placement presented at the GI outpatient clinic with complains of abdominal pain and heartburn for 6 months.After initial negative work up, Upper Endoscopy done showed small polyp at the metallic clip adjacent to the ampulla.Endoscopic ultrasound subsequently revealed a metal object traversing the duodenal wall. No biopsies or additional manipulation of the metal object were done until we did a CT scan which showed two distal anterior legs of the IVC filter extending into the transverse duodenum.This patient was referred to vascular surgeon for IVC filter removal but the patient's symptoms started resolving and after discussion with the patient it was decided not to proceed with the surgery and manage her conservatively. CASE 2: 37 y/o female seen in GI clinic for repeated episodes of nausea, vomiting, and abdominal discomfort which had been going on for 4 months and after negative initial work up, patient was scheduled for an Upper endoscopy which showed a metal like foreign body in the second portion of the duodenum and irritation of the opposite wall of the second portion of the duodenum with nodularity and represented a prong of the IVC filter which migrated into duodenum.Patient was referred to vascular surgeon for IVC filter removal and after the procedure post operatively she was kept on intravenous antibiotics and was discharged after she was able to tolerate regulate diet and was ambulating well.

DISCUSSION:
IVC filter perforation of the duodenum is fairly uncommon. It can manifest with abdominal pain, gastrointestinal bleeding, cava-duodenal fistula, or small bowel obstruction. Diagnostic evaluation include plain abdominal radiographs, CT scan and/or Upper Endoscopy.To prevent potential sequelae caused by IVC filter leg penetration, like that reported in this case, the removal of the IVC filter, when possible, is preferred.Also manipulations of the foreign body should be avoided until further confirmation is done by abdominal imaging.
2663 A Rare Presentation of Duodenal Carcinoid Tumor Complicated by Hemorrhagic Shock
Authors: Baseer Yaseen 
Published: October 2019

INTRODUCTION:
A carcinoid tumor is a tumor that develops from enterochromaffin cells. These cells produce hormones and chemicals that have a profound impact on the circulatory system, gastrointestinal tract, and the lungs. Duodenal carcinoid tumors are rarely encountered in the clinical setting making up only 2–4% of all carcinoid tumors. They are also difficult to diagnose due to their nonspecific clinical presentation which include symptoms such as abdominal pain, jaundice, pancreatitis and weight loss. According to the general guidelines for the treatment of duodenal carcinoid tumors, endoscopic therapy is recommended for tumors measuring less than 1 cm in diameter, whereas surgical resection may be preferable for those measuring 1 cm or more. However, treatment guidelines for duodenal carcinoids are not yet standardized as a result of the lack of consistent data. The most feared complication of carcinoid tumors include carcinoid crisis but milder presentations may include anemia, shortness of breath and diarrhea.

CASE DESCRIPTION/METHODS:
A 79 year old male with a history of hypertension, hyperlipidemia, spinal stenosis, aortic stenosis and carcinoid tumor at the gastric bulb who previously refused interventional surgery presented to the hospital with a primary complaint of shortness of breath. Initial hemoglobin on presentation was 5.1 and patient noted to be in hemorrhagic shock with vital signs that included heart rate of 116 and blood pressure of 91/52. Patient was subsequently admitted to the intensive care unit and transfused a total of 6 units of packed red blood cells over the course of his 8-day hospital course. An octreotide scan performed during the hospital stay showed a carcinoid tumor at the gastric bulb that was not actively bleeding. This may be explained by the patient’s late presentation to the hospital after the carcinoid tumor bleeding had resolved. The patient at the conclusion of the hospital stay continued to decline any surgical intervention.

DISCUSSION:
This case highlights an unusual presentation of a patient with a history of carcinoid tumor that presented in hemorrhagic shock secondary to a duodenal carcinoid tumor. Previous research has shown complications of carcinoid tumors that range from diarrhea and shortness of breath to carcinoid crisis. However, hemorrhagic shock has not been demonstrated to be an association with carcinoid tumor. Further large scale retrospective studies may be needed to better evaluate to the full scope of carcinoid tumor complications.