bluuebunny/crossref_metadata_2025_split huggingface dataset:
1795 Jugular Venous Pulsations With Neoplastic Implications: A Case of Unilateral External Jugular Venous Pulsations in the Setting of a Mediastinal Mass
Authors: Malashanka Sofya , Quinones William , Obuch Joshua 
Published: October 2019

INTRODUCTION:
Jugular venous pulsations (JVP) usually have associated cardiac or pulmonary pathology. In this case report we explore a patient with JVP due to an otherwise asymptomatic mediastinal mass.

CASE DESCRIPTION/METHODS:
A 44 year old male with no significant prior history presented to the ER after 5 months of intensifying unilateral pulsations (Figure 1), He denied neck pain, visual changes, dysphagia/odynophagia, palpitations, dyspnea, or weight loss. Lab evaluation revealed normal CBC, CMP, TSH, pro-BNP, and troponin level. Neck CT showed no vascular dilation or masses. Chest CT revealed a large lobulated mass in the posterior mediastinum arising from the gastroesophageal junction (GEJ) (Figure 2a–c). Follow up PET CT showed FDG avidity. EGD revealed a large diverticulum adjacent to the GEJ as well as diffuse gastritis with biopsies positive for H.Pylori. Endoscopic ultrasound (EUS) was performed (Figure 3a, 3b) for tissue acquisition with pathology showing a benign smooth muscle proliferation with immunophenotypic profile positive for desmin, smooth muscle actin and vimentin (SMA), and negative for S100, CD117 and DOG-1, consistent with leiomyoma. The patient underwent esophagectomy with gastric pull-through. Final pathology showed low-grade spindle cell neoplasm with similar immunohistochemical staining as EUS specimens consistent with a leiomyoma. Postoperative course was complicated by chylothorax requiring thoracic duct embolization as well as mild gastric outlet obstruction.

DISCUSSION:
This case of unilateral JVP in the setting of a posterior mediastinal mass illustrates the need for a low threshold for imaging in the setting of otherwise unexplained exam findings. Esophagogastric leiomyomas are rare and may have an association with H.Pylori infection as seen in this patient, which warrants exploration between the cause and effect of H. Pylori and leiomyomas (1). While hypermetabolic tumors on PET scan typically indicate malignancy, there are reports of leiomyomas appearing hypermetabolic on PET scan (2) as seen in this patient.
1796 Chronic Cough Leads to Unexpected Diagnosis: Sarcomatoid Squamous Cell Carcinoma of the Proximal Esophagus
Authors: Shah Natasha , Modilevsky Biana , Qazi Baseer , Chi Kenneth D. 
Published: October 2019

INTRODUCTION:
Sarcomatoid squamous cell carcinoma (SSCC) of the esophagus is a rare malignancy with an incidence of 2.0%. This entity was previously thought to be associated with an overall good prognosis compared to squamous cell carcinoma, however, recent studies revealed the disease to be of higher malignant potential. We report a case of a patient who was diagnosed with SSCC of the esophagus presenting with atypical symptoms.

CASE DESCRIPTION/METHODS:
A 61 year old female with a 30 pack year smoking history presented with chronic cough and 30 pound weight loss over 3 months. She denied dysphagia. Vitals were significant for temperature of 39oC and respiratory rate of 22. Serology revealed leukocytosis, microcytic anemia, and low albumin. Chest X-ray revealed right lobe opacification and antibiotic therapy was initiated. A computed tomography (CT) of the chest revealed a large fungating central cavitary mass in the right lung apex that appeared to be arising from or invading the proximal esophagus (A). Esophagogastroduodenoscopy (EGD) visualized a non-bloody, fungating mass nearly obstructing the proximal esophagus (B). Endoscopic ultrasound (EUS) revealed a 6.5cm hypoechoic mass that extended beyond the esophageal wall (C). Multiple hyperechoic liver lesions greater than 1cm in diameter were found, raising suspicion for metastases. Forceps biopsy of the esophageal mass showed spindle shaped cells and cytokeratin positive epithelial cells consistent with SSCC. Positron emission tomography (PET/CT) scan revealed a right upper lobe necrotic lung mass and findings suggestive of metastases to bone and pancreas. Due to the extent of disease, she was neither a surgical candidate nor stable enough for chemotherapy and therefore, underwent palliative care. The patient expired shortly after her diagnosis.

DISCUSSION:
We highlight a rare pathology and its radiographic and endoscopic images in a patient who did not present with the typical symptom of dysphagia. Only 10% of these cases consist of asymptomatic patients. Therapy includes esophagectomy versus chemoradiotherapy depending on the extent of the disease. At this time, due to the rarity of the disease and the complexity of its diagnosis on biopsy, only a handful of case reports have identified patients with this etiology and very few large cohort studies have been done to further assess overall prognosis and survival rates.
1802 A Hard Truth to Swallow
Authors: Steele Joshua , Birris Thomas 
Published: October 2019

INTRODUCTION:
Pseudoachalasia is a condition typically caused by lower esophageal sphincter (LES) obstruction in the setting of invasive neoplasm. In rare cases, a syndrome known as paraneoplastic achalasia can present similarly, but is not associated with tumor invasion into the esophageal mucosa.

CASE DESCRIPTION/METHODS:
76-year-old female with history of breast cancer status post mastectomy and radiation presented to her oncologist with one month of progressive dysphagia. She was referred to gastroenterology and scheduled for an outpatient EGD, but presented to the ED with weakness, fatigue and weight loss prior to completion of the EGD. CT scan in the ED showed two right lung masses, mediastinal adenopathy and esophageal wall thickening. Patient was admitted for further workup. Day 1 of admission, patient underwent EGD that was pertinent for severe Candida esophagitis, but was aborted after progressing only 1/3 of the way into the esophagus. Patient was started on fluconazole for esophagitis with improvement in dysphagia. On day 2, patient underwent bronchoscopy with biopsy and was discharged home with oncology follow up. Five days later, she was directly readmitted from the oncology office for worsened dysphagia and continued weight loss. Esophagram showed distal esophageal obstruction presumably secondary to post-inflammatory stricture. EGD the following day showed dilated and poorly contractile esophagus with a spastic LES concerning for achalasia. Manometry revealed absence of esophageal body contractility, but was unable to assess the LES due to intolerance of the procedure by the patient. Further workup including pathology showed that patient had metastatic non-small cell carcinoma (NSCC), lung primary. It was determined that patient had pseudoachalasia due to paraneoplastic syndrome in setting of non-small cell carcinoma of the lung without invasion of the tumor into the LES. Patient ultimately underwent palliative botox injection at the LES with improvement in symptoms.

DISCUSSION:
Paraneoplastic syndromes are considered rare disorders triggered by an altered immune response in the setting of malignancy. Paraneoplastic achalasia is a type of paraneoplastic syndrome that presents as a rapidly progressive dysphagia. Though it has been reported in the setting different malignancies, this is one of the very few case reports of paraneoplastic achalasia due to NSCC. This case also emphasizes the importance of considering other causes of achalasia when evaluating patients with abnormal presentations.
1803 Pseudoachalasia Following Nissen Fundoplication: A Progression Documented With High Resolution Manometry
Authors: Harbaugh Joshua W. , Clayton Steven 
Published: October 2019

INTRODUCTION:
Pseudoachalasia, indistinct from achalasia in symptoms and high-resolution manometry (HRM) findings, varies by a secondary etiology. Over half of the occurrences arise from malignancy and seldom following surgeries involving the esophagus. This case presents a rare development of pseudoachalasia following Nissen fundoplication, further unique by documenting complete progression on HRM.

CASE DESCRIPTION/METHODS:
A 68-year-old male with continued dysphagia and gastroesophageal reflux disease underwent repeat EGD showing progression to LA grade B (from grade A) esophagitis and a 5cm (from 3cm) hiatal hernia. Initial HRM had normal findings (Figure 1A). Laparoscopic Nissen fundoplication was performed. Recurrence of symptoms led to two stricture dilations in one year. Repeat HRM showed intact peristalsis and normal LES relaxation, but six of ten swallows had decreased distal latency < 4.5 seconds consistent with esophageal spasm (Figure 1B). He was referred to an esophageal disease clinic and reported dysphagia, poor pill swallow and 15lb unintentional weight loss. Timed barium esophagram revealed a high-grade obstruction in the distal esophagus (Figure 2). EGD had significant resistance to gastric intubation of the scope concerning for an extrinsic compression or achalasia. A CT chest, abdomen and pelvis showed no extrinsic masses or alternative malignancy. HRM had 100% incomplete liquid bolus transit, elevated LES basal (56.7mmHg) and median integrated relaxation (22.4 mmHg) pressures with complete aperistalsis validating Type I Achalasia by Chicago Classifications (Figure 1C). Diagnosis was mechanical esophagogastric junction outflow obstruction with pseudoachalasia secondary to a tight Nissen fundoplication. After Nissen fundoplication takedown, dysphagia resolved and results were maintained at follow up.

DISCUSSION:
The etiology of pseudoachalasia in this case was theorized as myenteric plexus injury during wrap construction or degeneration from sustained distal obstruction. Intermediary esophageal spasms on HRM may forewarn of developing pseudoachalasia. One should understand the risk, albeit rare, of post-Nissen fundoplication dysmotility. Being cognizant of a possible HRM progression, as identified in this patient, may spur earlier clinical follow up, repeat testing or intervention. Finally, those with rapid onset of achalasia symptoms over age 50 should raise concern and be evaluated with barium esophagram, EGD with biopsies, CT scan and HRM to decipher a secondary etiology.
1806 When the Cure Is the Cause: Checkpoint Inhibitor-Induced Lymphocytic Esophagitis
Authors: Pizzuti Jessica , Wander Praneet , Lech Diana , Ashamalla Michael , Rishi Arvind , Lee Calvin 
Published: October 2019

INTRODUCTION:
Immune checkpoint inhibitors are a group of novel treatment agents that are used for the treatment of multiple metastatic malignancies. Among these are programmed cell death 1 receptor (PD-1) antibodies that promote T cell proliferation, activating the immune response to tumor cells as well as non-tumor cells causing immune-related adverse events (irAEs). Diarrhea and colitis are common irAEs. Upper gastrointestinal involvement is rare with esophagitis limited to only case reports.

CASE DESCRIPTION/METHODS:
A 74-year-old female with metastatic renal cell carcinoma developed throat pain and dysphagia after 10 doses of Nivolumab. Proton pump inhibitor (PPI) and viscous lidocaine improved her symptoms. After 20 doses of therapy she developed odynophagia and dysphagia to both liquids and solids. She underwent an esophagogastroduodenoscopy which revealed esophagitis of 13 cm in length, manifest as circumferential erythema and exudate. Biopsy confirmed active esophagitis with dyskeratotic keratinocytes and lymphoplasmacytic infiltrate. Immunohistochemistry demonstrated markedly increased polyclonal plasma cells. This was consistent with nivolumab-induced esophagitis. Nivolumab was discontinued and she was started on methylprednisolone, with rapid improvement in symptoms and subsequent transition to prednisone. She was treated with a prolonged 3-month taper, but her dysphagia to solids recurred 2 months after completing the steroids. Repeat endoscopic biopsies were consistent with reflux esophagitis. She was treated with topical steroids for suspected residual Nivolumab-induced esophagitis as well as PPI with improvement in symptoms. Ten months later, the patient developed dyspepsia and early satiety. Repeat endoscopy showed discolored, linearly eroded, friable, sloughing mucosa. Biopsies taken revealed active and chronic esophagitis with lymphocytic infiltrate again consistent with Nivolumab-induced esophagitis. The patient symptomatically improved with sucralfate and PPI, and treatment was not further escalated.

DISCUSSION:
This is a rare case of Nivolumab-induced esophagitis demonstrating initial improvement with prolonged steroid taper followed by recurrent symptoms and persistent endoscopic disease treated with acid suppression, sucralfate and topical steroids. For refractory symptoms of esophagitis, immunosuppressants such as Infliximab could be considered. It is important to recognize the diversity and severity of immune-related adverse events to avoid incomplete treatment or a delay in therapy.
1811 Acute Esophageal Necrosis (Black Esophagus): A Rare Cause of Gastrointestinal Bleeding in Patients With Sepsis
Authors: Uy Pearl Princess , Coles Michael , Yap John Erikson , Vega Kenneth J. 
Published: October 2019

INTRODUCTION:
Acute esophageal necrosis (AEN) is extremely rare with less than one hundred cases reported in the literature. AEN is due to an ischemic insult causing decreased esophageal perfusion. We present two cases of AEN associated with sepsis.

CASE DESCRIPTION/METHODS:
Case 1: A 78-year-old male with coronary artery and cerebrovascular disease as well as atrial fibrillation on apixaban, was admitted for sepsis from cholangitis. Endoscopic retrograde cholangiography (ERCP) with common bile duct (CBD) stent placement was performed on day 4 which revealed a normal esophagus. He developed hematemesis on day 7; EGD discovered circumferential proximal to distal black esophagus and hematin in the stomach. Treatment included intravenous proton pump inhibitors, antibiotics, and fluid resuscitation. Apixaban was held. The patient did well post EGD and the remainder of his hospitalization was unremarkable. Case 2: A 47-year-old male with fistulizing Crohn’s disease, resulting in subtotal colectomy and diverting colostomy, as well as deep vein thrombosis post IVC filter was admitted for septic shock from gram negative bacteremia. He had hematemesis on day 8; EGD showed black mucosa throughout the entire esophagus and a large duodenal ulcer with visible vessel requiring embolization. Unfortunately, the patient expired from disseminated intravascular coagulation.

DISCUSSION:
AEN, also termed black esophagus, is rare and lethal, especially among patients with hemodynamic compromise. The distal esophagus is often involved due to blood supply and gastric acid exposure. The etiology is likely multifactorial resulting from entities causing tissue hypoperfusion along with mucosal barrier impairment/injury. Sepsis was present in both cases, with predisposing factors of atherosclerosis and thrombosis. AEN often presents as hematemesis or melena, with diagnosis made endoscopically. Treatment includes hemodynamic support, gastric acid suppression, bowel rest, and management of underlying causes including intravenous antibiotics for septic patients. Complications include esophageal stricture and perforation. Although rare, it is important to have a high index of suspicion for AEN as an etiology for upper gastrointestinal bleeding especially in septic patients given its high morbidity and mortality.
1814 Esophageal Actinomycetoma Masquerading as Cancer in an Immunocompetent Patient
Authors: Baig Sher Nazir , Rehman Sadia , Daniel Mina , Deshpande Vrushak , Abdelsayed George , Gonzalez Manuel O. 
Published: October 2019

INTRODUCTION:
All that looks like cancer is not always cancer. Esophageal Actinomycosis is a fine example that frequently mimics malignancy. Actinomyces is a bacteria acting like fungi which extremely rarely infects the esophagus in immunocompetent hosts. Risk factors are unknown due to paucity of data.

CASE DESCRIPTION/METHODS:
A 79-year-old African American woman was admitted with mild hematemesis. She had progressive, painless dysphagia for solids for 6 weeks and had lost 12 lb. She had curative lobectomy for lung cancer and quit smoking 10 years ago. She had COPD and GERD. Medications included Budesonide-Formoterol and Albuterol inhalers. She had no history of chemo/radiotherapy, tuberculosis, HIV, or diabetes. Physical exam was normal. CBC, renal function and LFTs were normal. CT chest with contrast showed air-fluid levels within the esophagus and wall thickening. EGD revealed a 1 cm friable mass in distal esophagus. Biopsy confirmed actinomycosis and candida esophagitis. She was discharged on Ceftriaxone 2 g/day for 4 weeks and oral Fluconazole 200 mg/day for 3 weeks. At 1-month follow up, dysphagia had improved and she was switched to oral Amoxicillin for 6 months.

DISCUSSION:
Actinomyces is an anaerobic, gram-positive, filamentous, branching rod. It grows as normal flora in the mouth and GI tract. Esophageal Actinomycosis is exceedingly rare in immunocompetent people with only 28 cases indexed in PubMed in English. 13 cases were reported in the US. 2/3 of patients were immunocompromised. It invades the esophagus following mucosal breach, and presents as a mass, esophagitis, ulcer, abscess, fistula, or stricture causing dysphagia/odynophagia. It is a great imitator, often misleading physicians, thereby triggering malignancy work-up. Diagnosis involves imaging and biopsy. Microscopy reveals yellow sulfur granules in 50% of cases. Actinomyces is difficult to isolate with culture yield as low as 24%. RNA sequencing can provide a quick, accurate diagnosis in future. Patient education and counseling is the cornerstone of successful prolonged antibiotic therapy. An initial course of IV penicillin G or Ceftriaxone for 4-6 weeks is followed by oral penicillin V or amoxicillin for 6-12 months. Necrotic ulcer, fistula, or abscess is managed surgically. We believe that inhaled corticosteroids may have created the milieu for the growth of Actinomyces and Candida in the esophagus by impairing local defenses. 43% of inhaled corticosteroid is deposited in the esophagus according to Gamma scintigraphic studies.
1815 Acute Esophageal Necrosis Masquerading as Acute Coronary Syndrome in an Uncontrolled Diabetic
Authors: Yuridullah Ruhin , Patel Varun , Melki Gabriel , Bollu Janardhan 
Published: October 2019

INTRODUCTION:
Acute esophageal necrosis (AEN) also known as “black esophagus,” “acute necrotizing esophagus,” or “Gurvits syndrome” is a rare syndrome with characteristic endoscopic finding of circumferential black appearance of the esophagus beginning at the gastroesophageal junction (GEJ) and extending proximally at variable lengths. Etiology is multifactorial with certain predisposing factors including diabetes mellitus. Most patients present with an acute upper gastrointestinal bleed, though other symptoms such as dysphagia, epigastric pain, and chest pain have been described. Herein, we present a case of isolated AEN in a patient with uncontrolled diabetes whose symptoms were mistaken for acute coronary syndrome.

CASE DESCRIPTION/METHODS:
An 80-year-old male with past medical history of hypertension, dyslipidemia, and uncontrolled diabetes mellitus was seen in the emergency department with a 1-hour complaint of epigastric and severe central chest pain accompanied by nausea and two episodes of coffee-ground emesis. A CT of abdomen/pelvis without contrast as well as cardiovascular work-up was unremarkable except for mildly elevated troponins of 0.062 ng/mL. Narcotics and nitroglycerine failed to resolve the chest pain prompting initiation of acute coronary syndrome (ACS) in consideration for non-ST-segment myocardial infarction (NSTEMI). Patient underwent an unremarkable cardiac catheterization. Due to persistent abdominal and chest pain, Gastroenterology were consulted. Subsequent esophagogastroduodenoscopy (EGD) showed circumferential, black-appearing, necrotic esophageal mucosa extending from the mid to distal esophagus consistent with AEN. Patient had an uneventful 5-day hospital course and discharged with close follow up.

DISCUSSION:
Acute esophageal necrosis (AEN) is a rare condition described by pathognomonic endoscopic findings of circumferential black appearance of the esophagus extending distally to the gastroesophageal junction (GEJ). It typically presents with an acute upper gastrointestinal bleeding, epigastric pain, and chest pain. In the case of our patient, severe and relentless chest pain along with episodes of hematemesis in the absence of significant EKG and laboratory findings, and failure of adequate narcotic analgesics to relieve symptoms should have prompted seeking alternative diagnoses. This case illustrates an example of AEN masquerading as ACS leading to delay in management.
1818 Acute Hematemesis From Kayexalate-Induced Esophageal Ulcer
Authors: Kumar Kishore , Essrani Rajesh Kumar , Tariq Hassan , Chime Chukwunonso U. , Dev Anil 
Published: October 2019

INTRODUCTION:
Hyperkalemia is one of the most common electrolyte abnormalities encountered in clinical practice. The treatment of hyperkalemia includes removal of the unwanted potassium from the body by cation exchange resins. e. g sodium polystyrene sulfonate (Kayexalate). The serious gastrointestinal side effects of sodium polystyrene sulfonate (SPS) have been reported with or without concomitant use of sorbitol including colonic perforation. The U.S food and drug administration (FDA) has issued a warning to avoid kayexalate administration with sorbitol. We present an individual with acute hematemesis due to bleeding esophageal ulcer immediately after treatment with kayexalate therapy.

CASE DESCRIPTION/METHODS:
63-year-old Hispanic man who was recalled to ED by renal team due to elevated potassium level of 6.6mg/dl. On arrival to ED his vitals were WNL. His physical examination includes distended non- tender abdomen and 2+ pitting edema of lower extremities. Rest of physical was within normal limits. His comorbid conditions include alcohol related decompensated cirrhosis, nephrotic syndrome and small nonbleeding esophageal varices seen on endoscopy performed 3 weeks before current illness. He was treated with oral kayexalate 30mg along with intravenous (I/V) dextrose, calcium gluconate and insulin. 45 minutes after oral SPS therapy patient developed large episode of hematemesis. He was intubated for airway protection. An emergent endoscopy was performed which showed multiple round, sharply demarcated, esophageal ulcers with mucosal oozing (Figure 1A) and small non bleeding esophageal varices were again seen. The two of the esophageal ulcers were actively oozing, to achieve the hemostasis, two hemostatic clips were deployed with cessation of bleeding (Figure 1B). The esophageal ulcer biopsies were not taken due to concern of causing iatrogenic bleeding in the presence of coagulopathy. He had no evidence recurrent gastrointestinal bleeding.

DISCUSSION:
Kayexalate induced colonic ulcer, colonic ischemia and colonic necrosis has been repeatedly described in the literature. The kayexalate induced esophageal ulcer with significant hematemesis is rarely reported. Although the exact mechanism by which kayexalate causes esophageal ulcer to be elucidated but it is worthwhile to be vigilant about its potential adverse effects. Our case highlights the rare but certainly the life threatening complication of kayexalate therapy.