bluuebunny/crossref_metadata_2025 huggingface dataset:
2568 A Unique Case Presentation of Small Bowel Intussusception
Authors: Kulkarni Archana , Appasamy Ragunath
Published: October 2019

INTRODUCTION:

Intussusception is a life-threatening condition and can cause ischemia of the small intestine if not intervened promptly. When present in adults, intussusception typically is caused by a neoplasm, adhesions of the small bowel, or resultant of weight loss surgeries. Below we describe a unique intervention provided to a case presentation of intussesception.

CASE DESCRIPTION/METHODS:

21 y.o. year old female with a past medical history of chronic abdominal pain, PTSD, suicide attempts, gender dysphoria, gastroesophageal reflux disease, depression, presented to the emergency department with secondary to abnormally low hemoglobin, abdominal pain, nausea and vomiting. She described pain in the epigastric abdominal region. She had no known family history of colon cancer, colonic polyps or other malignancies. She was noted to have a hemoglobin of 4.4 with no overt bleeding. She was tested negative for celiac serologies. She was evaluated with a CT scan of the abdomen and pelvis with IV contrast which showed long segment of proximal jejunal intussusception with associated inflammatory changes. The surgical team approached the gastroenterology team to attempt to reduce the intussusception prior to surgical resection. As a result of which a push enteroscopy was performed where in a normal esophagus, with erythematous mucosa in the gastric fundus and gastric body were seen. The duodenum was normal. An intussusception was encountered after the C-loop of the duodenum, around the proximal jejunum. This was reduced with the scope. A vascular appearing jejunal mass was seen. Biopsy was not attempted given the vascular appearance. A tattoo was applied. The patient subsequently underwent jejunal resection with small bowel anastomosis. The pathology of the mass resulted as benign hamartomatous polyp with surface erosion and no dysplasia. The patient's symptoms and anemia resolved post operatively. In addition she was evaluated with an upper endoscopy and flexible sigmoidoscopy previously which were within normal limits.

DISCUSSION:

Hamartomatous polyps of the small intestine can be associated with Cowden syndrome, Bannayan-Riley Ruvalcaba syndrome (BRRS), and juvenile polyposis syndrome (JPS). The above mentioned patient is unique as she does not meet criteria for any of these syndromes. In addition the emergent symptom relief was provided by endoscopic reduction of the intussusception providing the surgeons for a planned elective procedure rather than an emergent procedure.
2569 Eosinophilic Gastroenterocolitis: A Diagnostic Conundrum and a Rare Cause of Small Bowel Strictures
Authors: Omer Anam , Pinillios Hugo L.
Published: October 2019

INTRODUCTION:

Eosinophilic gastroenterocolitis (EGE) is a rare disorder that can have several gastrointestinal manifestations depending on the site and depth of involvement. The diagnosis involves demonstration of eosinophilic infiltration of bowel wall and exclusion of other causes of peripheral eosinophilia. We present a rare case of eosinophilic gastroenterocolitis that presented as chronic diarrhea and partial gastric outlet obstruction.

CASE DESCRIPTION/METHODS:

A 71-year-old male presented to the clinic for evaluation of a two-month history of watery diarrhea. He had recently been hospitalized for management of profuse diarrhea and dehydration. Extensive infectious work-up during the hospitalization was unrevealing. A colonoscopy with biopsies was negative for microscopic colitis and malabsorptive disorder, however, a prominent population of eosinophils suggestive of an allergic component was seen. The patient's symptoms were partially controlled on anti-motility agents. His current medication list was negative for any offending drugs. Physical examination revealed a mildly distended abdomen. Laboratory investigation showed a total leukocyte count 6.7 × 109/L. Differential count revealed polymorphs −50%, lymphocytes −34% and eosinophils −10%. Computed tomography with contrast showed segmental areas of narrowing in mid descending limb of the duodenum with partial gastric outlet obstruction. Esophagogastroduodenoscopy (EGD) showed multiple diaphragm-like strictures at the duodenal sweep which could not be passed with a regular EGD scope. Histologic examination of the specimens taken from the duodenal strictures revealed mildly increased plasma cells and eosinophils within the lamina propria with villous blunting, without increased intraepithelial lymphocytes. Numerous balloon-dilation sessions were needed to reestablish luminal patency. A diagnosis of eosinophilic gastroenterocolitis was made and the patient was started on a six-food elimination diet with complete resolution of his symptoms. Upon reintroduction, the culprit food were found to be cow's milk and wheat.

DISCUSSION:

EGE is classified based on the depth of eosinophilic infiltration as mucosal, submucosal and serosal. The most frequent sites of involvement include the stomach and the proximal small bowel. Diagnosis is often challenging and delay in treatment may lead to malabsorption and malnutrition. Although evidence of efficacy is lacking, treatment with dietary modification and corticosteroids has shown favorable outcomes.
2573 Appendicitis—Think Sarcoidosis? Appendicular Sarcoidosis Presenting as Acute Appendicitis
Authors: Pendela Venkata Satish , Munoz Anisleidys , Soliman Youssef , Soliman Megan
Published: October 2019

INTRODUCTION:

Sarcoidosis is a chronic inflammatory multisystem disease. Organs typically affected include the lungs, lymph nodes and skin. Gastrointestinal involvement although not uncommon, is usually asymptomatic. Stomach is the most commonly involved gastrointestinal organ, but esophagus, appendix, colon, and rectum have also been implicated. Symptomatic appendicular sarcoidosis is extremely rare. We present a rare case of symptomatic appendicular sarcoidosis.

CASE DESCRIPTION/METHODS:

A 49 year old woman with a history of chronic low back pain and recurrent kidney stones presented to the office with vague abdominal pain for 3 days. Exam revealed a right iliac fossa tenderness. Lab work was significant for mild anemia (Hemoglobin 11 gm/dl) and elevated ESR (30 mm/hr). A CT abdomen showed acute appendicitis, and detected an appendicular mass. A prior MRI of the spine had shown a suspected metastatic lesion. CT chest revealed mediastinal lymphadenopathy, and a spiculated lung nodule. Laparoscopic appendectomy was performed, and the pathology revealed non-necrotizing granulomas raising suspicion for sarcoidosis. Bronchoscopic ultrasound-guided biopsy of the lymph nodes also revealed granulomas. Serum angiotensin converting enzyme levels were elevated (68 U/L). The patient's symptoms improved following initiation of corticosteroids.

DISCUSSION:

There are a number of differentials for granulomatous inflammation of the appendix, notably Crohn's disease, tuberculosis, histoplasmosis and Yersinia pestis infection.
1
Appendicular involvement of sarcoid is very rare and is most often diagnosed incidentally on a PET scan. The clinical presentation of appendicular sarcoidosis may range from pain to perforation and abscess formation.
2
Surgical intervention should not be delayed in patients with sarcoidosis presenting with right iliac fossa pain as there is a high risk of perforation. Such a systemic disease process typically involves other organs, and can be relatively controlled with systemic steroids.
2575 A Rare Case of Extra-Gastrointestinal Stromal Tumor
Authors: Jiyani Rucha , Hirpara Kiran , Desai Jiten
Published: October 2019

INTRODUCTION:

Gastrointestinal stromal tumors (GIST) are tumors of mesenchymal origin rising from the walls of gastrointestinal tract, stomach being most common site followed by small intestine. Those with the similar morphology identified outside the GI tract are termed as Extra-gastrointestinal stromal tumor (EGISTs) and their incidence is about 10%. They are often located in the mesentery, omentum, retro peritoneum, rectovaginal septum, and perivaginal soft tissue and very rarely in prostate. Due to their malignant potential, it is almost essential to diagnose such extra intestinal occurrence. Here, we report a very rare case of EGISTs.

CASE DESCRIPTION/METHODS:

61-year-old male with no past medical history presented with intermittent abdominal pain. Physical exam and vital were unremarkable. CT abdomen revealed multilobulated enhancing lesions in mesentery; largest one was approximately 10 × 9 × 8 cm in size. Bowel loops were unremarkable. Diagnostic laparoscopy showed multiple nodules approximately 800 to 1000 in number with size varying from 1 to 10 cm. Diagnosis of EGISTs was made based on immunohistochemistry (IHC). Gross findings: 1st mass: cut surface- creamish white 2nd mass: cut surface- lymph node structure seen Microscopic exam: Histology from 1st mass showed fascicles and solid sheets of round to oval shaped cells and vesicular nuclei with abundant eosinophilic cytoplasm. Mitosis with scanty lymphocytic infiltration and vascular proliferation were also seen. Histology from 2nd mass showed metastasis to lymph nodes. Patient was treated with Imatinib for 10 weeks and largest tumor was removed surgically. Imatinib therapy was continued post surgery as well.

DISCUSSION:

Due to lack of awareness that GIST can be present in extra intestinal location; they are likely to be misdiagnosed. Pathogenesis and prognosis of EGISTs have not yet been completely understood. Differential diagnosis includes mesothelioma, amelanotic malignant melanoma or metastasis; which can be confirmed out by IHC. (Positive CD117 & CD34 with CK 5/6, pankeratin, desmin, myogenin, s-100 and calretinin being negative in our case). IHC findings favored the diagnosis of EGISTs with metastasis to lymph nodes. As there are specific drugs available for treating EGISTs, it is important to differentiate it from other pathological conditions. This case serves best to promote awareness of GIST in unusual anatomical location and help early diagnosis and prompt subsequent management.
2581 Acute Lymphoblastic Leukemia Presenting With Mesenteric Ischemia
Authors: Baliss Michelle , Krill Timothy , Abdulla Hamza , Parupudi Sreeram
Published: October 2019

INTRODUCTION:

Acute lymphoblastic leukemia (ALL) is a malignancy of B/T-cell lymphoid precursors that predominantly affects the pediatric population. Presenting symptoms are usually related to bone marrow and organ infiltration. Arterial thrombosis is extremely rare and seldom seen as a presenting manifestation of acute leukemia. To our knowledge, we present the first case of mesenteric ischemia due to complete occlusion of the superior mesenteric artery (SMA) as the first manifestation of T-cell ALL.

CASE DESCRIPTION/METHODS:

A 44-year-old male presented with severe upper abdominal pain, vomiting and diarrhea for 1 day. Physical examination was notable for diffuse abdominal tenderness and pain out of proportion to exam. Initial laboratory data showed WBC of 2.02 × 103/mL with 30% blasts. Lactic acid was within normal limits. CT of the abdomen revealed a thrombus in the SMA resulting in complete occlusion (Figure 1). An emergent open SMA thrombectomy was performed following failure of catheter-directed thrombolysis. Diffuse ischemic changes were visualized in the jejunum necessitating a partial small bowel resection (Figure 2). Intraoperative transesophageal echocardiogram was negative for intracardiac thrombus or valvular pathology. A peripheral blood smear revealed a large population of immature cells with fine chromatin and a high nuclear to cytoplasmic ratio (Figure 3). Flow cytometry showed a large population of blasts constituting about 22% of cells, positive for CD5, CD7, and CD10. The immunophenotypic appearance confirmed the diagnosis of T-ALL. He was referred to medical oncology for bone marrow biopsy.

DISCUSSION:

This is the first reported case of acute arterial occlusion in the SMA leading to mesenteric ischemia as the initial manifestation of T-cell ALL. SMA thromboembolism typically occurs in the setting of atherosclerosis or cardiac pathology, which were absent in this case. Venous thromboembolism is a well-documented complication of acute leukemia that negatively impacts mortality. The inhibition of anticoagulant proteins, production of prothrombotic factors, alteration of the vascular endothelium, and increased vascular adhesion of leukemic cells are all potential contributors. However, arterial occlusion is extremely unusual and almost exclusively seen in acute promyelocytic leukemia (APL). This report illustrates that an unexplained arterial or venous thrombotic event in a previously healthy patient could be a sign of an underlying malignancy.
2586 Renal Cell Carcinoma Presenting as Paraneoplastic Syndrome Involving the Gastrointestinal Tract
Authors: Attarha Barrett , Maharaj Satish , Harris Ciel , Schey Ron , Nassri Ammar , Sottile Elisa , Stemboroski Lauren
Published: October 2019

INTRODUCTION:

We present a case of a patient who presented with abdominal pain and a small bowel obstruction and was found to have renal cell carcinoma causing a paraneoplastic vasculitis affecting his bowel.

CASE DESCRIPTION/METHODS:

A 57 year old Caucasian male with no significant past medical of family history presented to the Emergency Department complaining of a 2 day history of severe abdominal pain, nausea, vomiting and a bilateral non-blanching rash on his lower extremities that extended up towards his umbilicus (Figure 1). Initial XR Abdomen revealed a small bowel obstruction. A CT abdomen/pelvis was done which showed focal bowel wall thickening of distal duodenum and proximal jejunum with some mucosal edema and stranding of adjacent mesenteric fat concerning for duodenitis/enteritis. A CT renal mass protocol was ordered which revealed a 3.1 cm enhancing left renal mass highly suspicious for Renal cell carcinoma. An NG tube was inserted for decompression. On day three of admission patient had EGD performed which revealed multiple partially obstructing ulcerations throughout the second and third portion of the duodenum (Figures 2 and 3). Biopsy samples of the duodenum showed severe active duodenitis with erosions that was negative for CMV. A colonoscopy revealed an ulcer at the ileocecal valve which was biopsied and showed acute inflammation. There was non-specific erythematous mucosa throughout the colon. The patient began to experience swelling and pain in his wrist and the small joints of his hand and the rash continued to spread. Rheumatology performed an autoimmune workup which was normal save for mildly elevated C3, C4 and a high CRP. An extensive infectious disease workup was negative. Skin biopsy of the lesion was consistent with medium/large vessel vasculitis and the patient was started on high dose steroids. The patients clinical course improved drastically and he was discharged on a prolonged steroid taper. He underwent a nephrectomy 1 month from hospital discharge with pathology revealing clear cell renal cell adenocarcinoma with clear margins. He was continued on a steroid taper until resolution of his symptoms at 6 months follow up.

DISCUSSION:

Paraneoplastic vasculitis in patients with solid tumors has been described, and can very rarely affect the gastrointestinal tract. It can cause local or diffuse pathologic changes in the gastrointestinal tract, resulting in nonspecific paralytic ileus, mesenteric ischemia, submucosal edema, hemorrhage, or even bowel perforation.
2587 Never Too Old for Meckel's: A Unique Presentation of an Uncommon Source of Gastrointestinal Bleeding in Adults
Authors: Aggarwal Pankaj , Patel Harsh D. , Camero Alfredo , Wright Randy P.
Published: October 2019

INTRODUCTION:

Meckel's diverticulum is a common gastrointestinal congenital malformation, affecting approximately 2% of the population. Related complications such as bleeding commonly occurs in the pediatric age group; although, in adults it is often missed. We present a case of recurrent obscure-overt gastrointestinal (GI) bleeding secondary to a Meckel's diverticulum which remained elusive after multiple diagnostic studies.

CASE DESCRIPTION/METHODS:

30-year-old male with history of recurrent GI bleeding, presents to the outpatient GI clinic for further evaluation after being admitted to the intensive care unit (ICU) 3 weeks prior with massive hematochezia requiring several blood transfusions for a drop-in hemoglobin from 14 gm/dL to <7 gm/dL. The patient had undergone endoscopy, colonoscopy, and video capsule endoscopy (VCE) three years prior for similar symptoms without an identifiable source. A nuclear tagged red blood cell scan was performed showing bleeding in the proximal colon. CT angiography following showed contrast extravasation in the distal jejunum and ileum which was treated with gel foam. The patient had no further episodes of hematochezia and was discharged. After the clinic visit, the patient underwent a Meckel's scan which did not show ectopic gastric mucosa but revealed colonic bleeding proximal to the hepatic flexure. Ileocolonoscopy was normal. Subsequent VCE showed abnormal mucosa in the distal small bowel. He finally underwent a retrograde balloon enteroscopy which revealed a distal small bowel diverticulum with a single adjacent, nonbleeding ulcer (Figure 1). Biopsies of the ulcer showed gastric foveolar metaplasia. Based on the findings, he was referred for a small bowel resection which revealed a jejunal Meckel's diverticulum with gastric oxyntic heterotopia on histology (Figures 2 and 3).

DISCUSSION:

This case highlights the potential morbidity and possible mortality of Meckel's diverticulum as a source of lower GI bleeding. Recent studies have recommended against prophylactic surgical resection in the absence of certain risk factors including age less than 40, male gender, and macroscopic mucosal alteration. Given this patient's significant hematochezia, transfusion requirements, and gross peri-diverticular ulceration, we opted for small bowel resection. Meckel's diverticulum should always be included in the differential for occult or overt GI bleeding, especially in young patients when no other sources of bleeding are readily identifiable.
2593 The Importance of Endoscopy in Diagnosis of Lupus Enteritis
Authors: Koldhekar Amol , Taborda Cesar , Barrett Amanda , Iskandar Heba , Xue Yue
Published: October 2019

INTRODUCTION:

Lupus enteritis is defined as vasculitis or inflammation of the small bowel with supportive image and/or biopsy findings in patients with SLE. Symptoms such as abdominal pain (97% of cases) and vomiting (42%) are non-specific. These symptoms represent a wide differential, including vasculitis, medication reactions, and infection. This can cause diagnostic dilemmas in patients with multiple comorbidities.

CASE DESCRIPTION/METHODS:

A 28-year-old female with SLE, lupus nephritis, and ESRD after failed renal transplant presented with one day of abdominal pain and vomiting. Her medications included mycophenolate mofetil, prednisone, and hydroxychloroquine. Physical examination revealed periumbilical tenderness without rebound or guarding. CT abdomen showed small bowel wall thickening and hyperemia in the proximal jejunum with bowel dilatation and no transition point. Labs were remarkable for elevated CRP and decreased C3 complement. Given her symptoms and abdominal imaging, she underwent small bowel enteroscopy that showed severe inflammation in the proximal jejunum with purple discoloration (Figure 1). Initial jejunal biopsy was concerning for vasculitis. The patient was treated with methylprednisolone. Her symptoms improved within one day. A CTA abdomen two days later showed no vascular abnormalities with interval improvement of jejunal edema. Final pathology of the jejunal biopsy (Figures 2 and 3) showed mildly inflamed and injured small intestinal mucosa with fibrin thrombi in small vessels, lamina propria hemorrhage and patchy lymphangiectasia, consistent with lupus enteritis in this clinical setting.

DISCUSSION:

The clinical presentation of lupus enteritis is non-specific but often associated with abdominal pain and vomiting in SLE patients. The jejunum is involved in 80% of cases. The underlying cause is believed to be immune-complex deposition and complement activation that produce diffuse microvascular injury, thrombosis, and increased vascular permeability. This can result in intestinal necrosis and perforation. Timely diagnosis and treatment lead to excellent response within days. This patient achieved symptomatic improvement after one day of high-dose steroids. Due to her dialysis schedule, CT angiogram of her abdomen was delayed until after symptom resolution. The normal imaging reflected treatment response rather than lack of disease and led to initial non-diagnosis until final pathology resulted. Our case highlights the value of endoscopy in patients with a suspicion of lupus enteritis.
2602 Strongyloidiasis Presenting as Gastric Outlet Obstruction
Authors: Yazdanpanah Fariba , Abraamyan Sirush , Tin Kevin , Rahmani Rabin , Hindy Pierre
Published: October 2019

INTRODUCTION:

Strongyloides Stercoralis is an intestinal parasite mostly seen in tropical areas. Commonly patients are asymptomatic with non-specific gastrointestinal manifestations. We present a case of strongyloidiasis presenting as gastric outlet obstruction.

CASE DESCRIPTION/METHODS:

Our patient is a 67-year- old woman, originally from Jamaica, recently diagnosed with Diabetes Mellitus who presented with 50 pounds weight loss, anorexia postprandial epigastric pain and recurrent episodes of vomiting. Her symptoms have been progressively getting worse and let her to doctor visits over the past several months. On physical exam she was afebrile but appeared cachectic. She did have minimal tenderness to palpation of the epigastric area and had minimal bilateral pedal edema. Laboratory testing revealed normocytic normochromic anemia, thrombocytosis, Hyponatremia and hypoalbuminemia. An Abdominal CT scan revealed dilatation of the second portion of duodenum and tapering of third portion of duodenum plus mild intrahepatic and extrahepatic bile ducts dilatation as well as dilation of the proximal pancreatic duct (Figures 1 and 2). The patient was admitted to the hospital for hydration and subsequently had an upper endoscopy. The procedure revealed marked retention of bilious fluid as well as evidence of severe duodenitis. Narrowing of the lumen of the duodenum was also seen (Figure 3). Biopsies from the duodenum were obtained and confirmed the presence of Strongyloides Stercoralis. Pt was given two days of Ivermectin with dramatic improvement of her symptoms and subsequent discharge.

DISCUSSION:

1. Stercoralis is a nematode with a complex life cycle. The parasite infects humans and replicates within its host for years or even decades (autoinfection). Patients would have positive serologies for S. Stercoralis but usually are asymptomatic. Immunosuppressive medications, HIV, HTLV1, Chronic diseases and in our case the new onset diabetes cause the parasite to replicate and leads to life-threatening dissemination of larvae to multiple internal organs called hyperinfection.
2603 Clostridioides difficile: Is Jejunum a New Reservoir?
Authors: Jha Samir , Thyagaturu Harshith , Aliyev Nurlan , Hyman Charles
Published: October 2019

INTRODUCTION:

Clostridium difficile is the most common cause of healthcare associated infectious diarrhea. Normally inhabiting in the colon, this organism was found in jejunal aspirates, suggesting that the small bowel may act as reservoir. However, review of current literature showed C. difficile enteritis cases have occurred only in the presence of underlying surgical manipulation (resection/partial or total colectomy) or inflammatory bowel disease. Our patient is unique in not having any of the above-mentioned risk or predisposing factor, nor did he have any sign or symptom exhibiting any bowel disease.

CASE DESCRIPTION/METHODS:

A 35-year-old Caucasian male, presented to the hospital with complaint of severe acute abdominal pain, started as dull aching pain, about a week ago, associated with nausea and two episodes of loose stools, back pain and generalized body aches. He had a history of splenectomy due to ruptured splenic artery aneurysm and an episode of diverticulitis treated with intravenous antibiotics, 8 months ago. On the exam, he was found to have temperature of 38.1 deg. C with tachycardia and tachypnea. There was some tenderness to palpation over the epigastric region without any rebound tenderness. A white blood cell count showed leukocytosis of 34,000 cells/mm3. Intravenous Vancomycin and Piperacillin-Tazobactam were empirically started. A CT scan with intravenous contrast (without oral contrast) showed prominence of contiguous length of proximal and mid-jejunum in the left abdomen with concern for inflammation. Stool culture for Salmonella, Shigella, and Campylobacter was unremarkable, and Shiga toxin was negative. Two sets of blood culture obtained 15 minutes apart, didn’t grow any bacteria. Stool evaluation sent for C. difficile toxin Nucleic acid amplification Test (NAAT) was positive. He never had a C. difficile infection in the past. The intravenous antibiotics were discontinued and started on oral Vancomycin for 10 days. He significantly improved with the treatment; symptoms subsided and was discharged home.

DISCUSSION:

C. difficile, commonest cause for diarrhea in healthcare, can involve the small intestine, more so in surgically manipulated bowel or patients with IBD. As small bowel involvement is associated with a higher mortality than colonic involvement, it should be considered as a differential diagnosis for a patient presenting with enteritis, more so in patient with risk factors.
2609 Use of Dotatate Scan to Establish Diagnosis of a Neuroendocrine Tumor
Authors: Cooper John , Sadowski Brett , Tritsch Adam
Published: October 2019

INTRODUCTION:

Neuroendocrine tumors (NETs) are rare malignancies, defined at a cellular level by the presence of somatostatin receptors, which has recently been exploited in the development of positron-emitting somatostatin analogs for use in the 68-Gallium DOTATATE PET scan (hereafter “Dotatate scan”). Compared to previously-used technology, the Dotatate scan has shown dramatic improvements in sensitivity for lesion detection. The Dotatate scan exposes patients to lower doses of radiation, and is completed much more quickly. We present a case of a new diagnosis of a neuroendocrine tumor that illustrates multiple benefits of Dotatate scan including improved lesion detection and characterization which quickly led to changes in the treatment plan for a patient who previously was given a presumptive diagnosis of advanced pancreatic adenocarcinoma.

CASE DESCRIPTION/METHODS:

A 52-year-old man with history of two episodes of pancreatitis presented with one day of acute-onset epigastric pain, diarrhea, and nausea. Initial ultrasound and computed tomography (CT) scan demonstrated numerous hypervascular lesions in the liver, a mesenteric mass with associated lymphadenopathy, and a possible pancreatic tail lesion, leading to clinical concern for pancreatic adenocarcinoma. Upon further questioning, the patient reported 12–18 months of intermittent flushing and rash. A Dotatate scan revealed increased avidity in the hepatic and mesenteric lesions suggestive of carcinoid malignancy. The improved lesion characterizations provided by the Dotatate scan were useful for identifying the larger small bowel lesion as the likely primary tumor, as well as for surgical planning for debulking. Subsequent serum and urine tests were consistent with carcinoid malignancy, and a biopsy of a liver lesion confirmed the diagnosis of metastatic carcinoid tumor. The pancreatic lesion also demonstrated uptake on the scan, most consistent with a metastasis to the pancreas, with a confirmatory biopsy via endoscopic ultrasound pending.

DISCUSSION:

We demonstrate the utility of the Dotatate scan to support the rapid diagnosis of a neuroendocrine tumor, to assist in identification of the likely primary lesion, and to better characterize the extent of disease. Dotatate scans have been infrequently used due to novelty and cost, but cases such as this demonstrate that the cost can be well-justified in the right clinical setting.
2626 A Curious Case of Gastric Outlet Obstruction!
Authors: Saini Mannat K. , Amarnath Shivantha , Ahmed Moiz , Syed Umer , Shankar Uday , Bansal Raghav
Published: October 2019

INTRODUCTION:

The average lifetime risk of a woman developing breast cancer is 12%. Invasive lobular carcinoma (ILC), the second most common histologic type attributes to 10–15% of breast cancer diagnoses. Compared to invasive ductal carcinoma, ILC has a higher chance to metastasize liver, ovaries and even the gastrointestinal tract. We present a unique case of gastric outlet obstruction (GOO) as the initial presentation of metastatic ILC. Histologically, it can be challenging to delineate ILC from primary gastric adenocarcinoma. This was a rare case where the diagnosis was made via the comparison of the endoscopic biopsy with the patient’s previous lymph node pathology.

CASE DESCRIPTION/METHODS:

75-year-old female with history of type 2 diabetes mellitus, and lobular carcinoma treated with a bilateral mastectomy and Anastrozole hormone therapy for 8 years presented with confusion and non-bilious, non-bloody vomiting for two weeks. Vitals indicated orthostatic hypotension and abdominal exam revealed a tender epigastrium without rigidity or guarding. Laboratory findings demonstrated severe contraction alkalosis. Abdominal CT with contrast depicted wall thickening in the second and third portion of the duodenum. Endoscopy revealed an infiltrative circumferential mass that stemmed from the antrum extending through to the proximal duodenum causing GOO. The morphological features of the duodenal mass were consistent with biopsy findings of the patient’s previous sentinel lymph node and mastectomy specimen. The diagnosis was further confirmed with immunostains, which were positive for estrogen receptor and mammoglobin, and negative for progesterone receptor and E-cadherin. Patient received robotic gastrojejunostomy tube placement. Her nutritional status improved and was started on chemotherapy. She had a complete metastatic workup which was unrevealing.

DISCUSSION:

ILC can metastasize later in life to the brain, ovaries and gastrointestinal tract. Isolated duodenal metastasis is limited to only a few case reports. It is theorized in the literature that lack of expression of the adhesion molecule, E-cadherin, may facilitate ILC’s permeation into tissues. This may contribute to metastasis of ILC with a predilection to gastrointestinal and gynecological sites, which generally occurs after a mean duration of 7 years. Our case is rare in that, the patient presented with GOO as the only initial manifestation of duodenal metastasis within 8 years of treatment of ILC without any other evidence metastatic spread.
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.