bluuebunny/crossref_metadata_2025_split huggingface dataset:
1780 Combined Esophageal Achalasia and Cricopharyngeal Achalasia in a Patient With Type 1 Myotonic Dystrophy
Authors: Ghazaleh Sami , Nehme Christian , Khader Yasmin , Ghanim Marcel , Hasan Syed , Nawras Ali 
Published: October 2019

INTRODUCTION:
Type 1 myotonic dystrophy (MD) is a rare inherited disease that presents with skeletal muscle weakness and myotonia. Involvement of smooth muscles is also common and mainly manifests in the gastrointestinal tract. We report a case of type 1 MD who presented with dysphagia and was found to have unique esophageal manometry findings.

CASE DESCRIPTION/METHODS:
A 57-year-old Caucasian male patient presented with progressive dysphagia to solids and liquids for the last few months. The patient had a history of type 1 myotonic muscular dystrophy diagnosed at the age of 34. Past medical history was also significant for gastroesophageal reflux disease, diaphragmatic paralysis, and obstructive sleep apnea. Last upper endoscopy showed a small hiatal hernia and a normal esophagus, stomach, and duodenum. His only home medication was omeprazole. Past surgical history was insignificant. Both his father and brother died in their 50s because of unclear respiratory problems. He was a former smoker and did not drink alcohol. Review of systems was unremarkable. On physical exam, the patient was alert, oriented, and in no acute distress. Vital signs were within normal limits. Cardiovascular, lung, abdominal, and skin exams were unremarkable. Neurological exam was significant for bilateral facial muscle weakness and mild ptosis. His other cranial nerves were intact. He had atrophy and weakness of the distal upper and lower extremities. Deep tendon reflexes were absent. Sensory, cerebellar, and gait exams were unremarkable. Upper endoscopy showed mild hyperemia of the stomach, but a normal esophagus and no hiatal hernia. Esophageal biopsies were normal and showed no evidence of eosinophilic esophagitis. After two weeks of being off omeprazole, the patient underwent a 24-hour esophageal pH testing which showed no significant acid reflux. Esophageal manometry revealed elevated lower esophageal sphincter (LES) pressure, elevated upper esophageal sphincter (UES) pressure, and very week peristalsis of the esophageal body.

DISCUSSION:
Esophageal involvement is common in type 1 MD manifesting with dysfunction of UES, esophageal body, and LES. Manometry usually describes a reduced resting tone of the UES and LES. Our patient had elevated LES pressure and week peristalsis of the esophageal body consistent with esophageal achalasia. He also had an elevated UES pressure consistent with cricopharyngeal achalasia. This is the opposite of what is expected in type 1 MD.
1781 Esophageal Squamous Cell Carcinoma Masquerading as Neuropathic Pain
Authors: Khare Anshika , Bhatt Amit , Cruise Michael , Mankaney Gautam 
Published: October 2019

INTRODUCTION:
The most common symptoms for esophageal carcinoma are dysphagia and odynophagia, though most are asymptomatic. We describe a case of upper esophageal squamous cell carcinoma masquerading as subphrenic neuropathic pain, not previously described in the literature.

CASE DESCRIPTION/METHODS:
A 67 year-old male with a past medical history of cholecystitis status post cholecystectomy, hepatic steatosis, acid reflux, hyperlipidemia, and 5-pack year tobacco history presented with 2 years of neuropathic subphrenic pain that radiated below the subcostal margins. The ingestion of solids and liquids triggered the pain, lasting for 1-2 seconds. The pain did not respond to viscous lidocaine or proton pump inhibitors. EGD two years prior revealed a nodular area of mucosa suspicious for esophageal squamous papilloma. His BMI was 29.6 and physical examination was otherwise unremarkable. He had normal peristalsis on esophageal manometry. EGD revealed a nodular mucosal patch in the upper third of the esophagus. Biopsies reproduced the patient’s sharp neuropathic pain. Pathology revealed ulcerated high-grade squamous dysplasia with a focus suspicious for stromal invasion. The stomach demonstrated segmental moderate mucosal changes characterized by atrophy, discoloration, and altered texture along the greater curvature of the gastric body with chronic inactive gastritis with extensive intestinal metaplasia on biopsy. H. pylori testing was negative.Endoscopic ultrasound demonstrated a confirmed a 4cm superficial lesion involving 1/3rd of the lumen. Endoscopic submucosal dissection was performed and pathology demonstrated a T1a squamous cell carcinoma with infiltration into the lamina propria and a focal tongue of tumor in the superficial muscularis mucosa. His pain resolved following resection.

DISCUSSION:
Esophageal squamous cell carcinoma masquerading as neuropathic pain is highly unusual. The mechanism behind this presentation remains unclear. The squamous papilloma visualized on prior EGD was a risk factor for the development of carcinoma. This case illustrates that squamous cell carcinoma can present with neuropathic pain, a symptom with swallowing that warrants further investigation.
1782 Cytomegalovirus-Induced Ulcerative Esophagitis Presenting as Near Food Bolus Impaction in an Immunocompromised Patient
Authors: Irizarry Karelys Burgos , Mulki Ramzi , Taborda Cesar , Sebastian Kavya 
Published: October 2019

INTRODUCTION:
Infectious esophagitis is a nuisance complication in the occurrence of systemic or esophageal immunodeficiency. Cytomegalovirus (CMV) is one of the most common opportunistic pathogens that cause infectious esophagitis, especially in post-transplant patients due to its potential to spread hemodynamically and increase viral load under immunosuppression. Odynophagia with or without dysphagia is the most common presentation and high index of suspicion must be attained in an immunocompromised patients with this complaint. To our best knowledge, limited data exists about infectious esophagitis presenting as a mechanical gastrointestinal emergency. We report a case of an immunocompromised patient with ulcerative CMV induced esophagitis which resulted in an impending near food bolus impaction.

CASE DESCRIPTION/METHODS:
The patient is a 63-year-old Caucasian male with End Stage Renal Disease who had a living unrelated donor renal transplant six months prior to his presentation with severe dysphagia and odynophagia. His immunosuppressive regimen consisted mycophenolate mofetil, tacrolimus and prednisone. His dysphagia and odynophagia were mostly to solids over the past month for which he was empirically treated with fluconazole for presumed candida esophagitis with no improvement in his symptoms. An esophagogastroduodenoscopy (EGD) was performed which revealed multiple, clean based, deeply cratered, esophageal ulcers in a circumferential manner involving the mid and distal esophagus (Figure 1). The ulcers had adherent food debris that prevented the passage of the endoscope and occupied the entirety of the esophageal lumen, that were removed with aggressive irrigation (Figure 2). Histopathology showed non-specific necro-inflammatory debris with no evidence of CMV or Herpes simplex (HSV). An inner lip mucosal ulcer was then identified and biopsied showing evidence of inclusion bodies, with confirmed CMV.

DISCUSSION:
This report contributes to a better understanding of atypical presentations of infectious esophagitis in immunocompromised patients. Despite the fact that is a common disease, it is often missed and complications like fistulas, strictures and/or esophageal perforation may result due to delays in treatment. Because gastrointestinal disease may be patchy, multiple biopsies may be needed to confirm the diagnosis of esophagitis, as in our case. A simple negative pathology report does not exclude the diagnosis and if high clinical suspicion, findings should not be attributed to other etiologies.
1785 Hematemesis in Valley Fever: A Case of Black Esophagus
Authors: Bader Nimrah 
Published: October 2019

INTRODUCTION:
Acute Esophageal Necrosis (AEN) is an uncommon clinical entity characterized by necrosis of the esophagus which is visualized as a circumferential black appearing esophagus during an endogastrodudoenoscope (EGD). Several factors are thought to be behind the tissue injury including hypoperfusion in low flow states, persistent exposure to gastric contents and impaired mucosal defense barriers. In this report, we present a case of black esophagus in a patient with disseminated coccidioidomycosis.

CASE DESCRIPTION/METHODS:
A 42 year-old-man with prior history of untreated HIV, HCV, cirrhosis and polysubstance abuse presented with abdominal pain, fatigue and weakness for four days. His exam was remarkable for a cachectic appearance and tender distended abdomen. He was initially treated for SBP. A diagnostic paracentesis was done which revealed hazy yellow fluid. The fluid analysis was remarkable for WBC 2580, 42% PMNs and 25% Eosinophils. The fluid culture had growth of coccidioides immitits. He was treated with Amphotericin B. He had a prolonged hospital course which was notable for HSV labialis and pneumonitis, Ogilve’s syndrome. On Day 5 his course was complicated by an iatrogenic pneumothorax following chest tube insertion, necessitating mechanical ventilation. One week after extubation, he developed hematemesis. The GI service was consulted and an EGD was done which revealed circumferential ulcerated esophagitis involving the whole length of the esophagus.

DISCUSSION:
AEN is an uncommon finding with prevalence of 0.001 to 0.2% as seen in a retrospective endoscopy review. Its etiological basis is multifactorial but has popularly been attributed to ischemic injury during low flow states and gastric outlet obstruction which overwhelm the mucosal protection of the esophagus. Other factors have also been associated with AEN such as antibiotics and concurrent infections (Candida albicans, Cytomegalovirus, Herpes virus, Klebsiella pneumoniae). On literature review there is no known association on coccidioidomycosis and AEN. After visualizing AEN on EGD, he was treated with high dose proton pump inhibitors, anti-reflux measures and kept NPO. IV fluids and packed red blood cell transfusions were done as supportive measures. Treatment is focused on correcting the underlying disease. Despite treatment of his fungal infection, the patient did poorly because of his infectious burden and multiple in-hospital complications. He elected to go home with hospice.
1786 Major Esophageal Bleeding Secondary to Mechanical Injury From Esophageal Deviation During Pulmonary Vein Isolation
Authors: Kassim Gassan , Bsrat Makda , Hershman Melissa , Mamun Rifat , Dong Ray , Smith Michael S. 
Published: October 2019

INTRODUCTION:
Radiofrequency catheter ablation and pulmonary vein isolation (PVI) are electrophysiologic interventions frequently performed for the management of atrial fibrillation (AF). Given the anatomic proximity of the esophagus and left atrium, the former is prone to thermal injury which can lead to peri-esophageal nerve injury, pain, dysphagia, and atrio-esophageal fistula formation. Mechanical deflection of the esophagus away from the ablation site is being used to minimize such complications. We present a case of major esophageal bleeding secondary to mechanical trauma in the setting of esophageal deviation during AF ablation.

CASE DESCRIPTION/METHODS:
60 yo female with past medical history significant for obstructive sleep apnea, pulmonary hypertension, AF with multiple direct current cardioversion attempts, underwent elective PVI using an esophageal deviation stylet (EsoSure, Northeast Scientific, Waterbury, CT). After PVI, a small amount of blood was noted as a result of suction using an orogastric tube. CT chest ruled out esophageal pathology including perforation. Subsequent upper endoscopy showed small mucosal tears in middle third of esophagus and at the esophago-gastric junction (EGJ) (Figure 1). She was discharged on daily proton pump inhibitor and remained on anticoagulation. Five days later, she presented to the emergency room with complaints of weakness, fatigue, melena and non-radiating substernal sharp chest pain. Blood pressure was 64/35. Hemoglobin was 6.4 g/dL compared to 10.3 g/dL upon discharge. Shortly after arrival, the patient had an episode of large volume hematemesis. She was urgently intubated for airway protection and volume resuscitation was initiated. Emergent upper endoscopy reveled an actively bleeding superficial mucosal tear at the EGJ which was treated successfully with epinephrine injection followed by hemostatic clip placement (Figures 2 and 3). After transfusion of 3 units of packed red blood cells, repeat Hgb improved to 8.8 g/dL and remained stable. Patient was extubated, remained clinically stable and was discharged.

DISCUSSION:
Mechanical displacement of the esophagus away from an atrial ablation site is being used more frequently in electrophysiologic interventions, as it reduces ablation-related esophageal thermal injury. Despite newer esophageal retractors which retain less heat and are less bulky, vigilance should be practiced to avoid mechanical esophageal trauma.
1789 Pseudotumor Esophagus: Acute Dysphagia Secondary to Mallory-Weiss Tear Associated Blood Clots
Authors: Snyder Patrick , Ebrahim Vivian , Kale Hemangi 
Published: October 2019

INTRODUCTION:
Acute onset dysphagia secondary to the mass effect from blood clots related to Mallory-Weiss tears (MWT) has not been reported in the literature. We present two such cases, both of which initially were thought to have a neoplastic etiology.

CASE DESCRIPTION/METHODS:
Case 1: A healthy 60-year-old woman developed acute dysphagia following impaction of a piece of steak. She forcefully regurgitated it with a coat of blood but continued to have substernal chest pressure and dysphagia with sticking at her suprasternal notch. A chest x-ray raised concern for pneumomediastinum, prompting an esophagram that showed a distal esophageal filling defect suggestive of obstructive neoplasm (Figure 1A). An upper endoscopy showed a long mucosal tear in the distal esophagus with a large adherent blood clot at the apex that could not be dislodged. The patient remained nil per os over the next couple of days with improvement in her discomfort. A repeat esophagram showed no filling defects (Figure 1B). Upon resumption of her diet, she experienced no further dysphagia. Case 2: An 80-year-old man with severe ischemic cardiomyopathy presented with volume overload. He was started on a heparin drip after an atrial thrombus was found on echocardiogram. One day later, he had sudden onset projectile vomiting and subsequent dysphagia with substernal sticking. An upper endoscopy showed a 3 cm fixed, partially obstructive mass-like lesion in the lower third of the esophagus with oozing at the base (Figure 2A). Biopsies showed non-specific findings including necrosis and foreign material. The following day, the patient tolerated solid food without dysphagia. A second upper endoscopy showed a 3 cm esophageal tear with a small adherent clot at the site of the previously noted mass-like lesion (Figure 2B). He continued to tolerate a regular diet without further symptoms.

DISCUSSION:
The presentation of MWT associated blood clots causing dysphagia and resembling distal esophageal neoplasms is novel. Both our cases experienced forceful regurgitation or emesis prior to the onset of dysphagia, suggesting a mucosal tear as the instigator with subsequent development of a large clot in the esophageal lumen. In each instance, resolution of symptoms correlated temporally with clearance of the clot. MWT are most likely to present with hematemesis but in rare cases may form a large blood clot mimicking a neoplastic process with resultant dysphagia. Awareness of such presentations may prevent unnecessary workups.
1791 Esophageal Nikolsky’s Sign: A Rare Finding in a Patient With Bullous Pemphigoid
Authors: Ghoneim Sara , Shah Aun , Calderon Amy 
Published: October 2019

INTRODUCTION:
Bullous pemphigoid (BP) is a chronic relapsing autoimmune blistering disease characterized by circulating antibodies directed against specific epitopes of hemidesmosomes involved in anchoring the epidermis to the dermal layer. We report a case of acute onset esophageal bullae identified in a patient with active skin BP seen only upon withdrawal of the upper endoscope, not present on insertion. This sign is analogous to Nikolsky’s sign where pressure or shearing results in formation of new bullae.

CASE DESCRIPTION/METHODS:
57-year-old Caucasian female with Type II DM, BP, CKD stage 3a presented to hospital for 5-6 episodes of melanotic stools for two days. She was diagnosed in September 2017 with BP by skin biopsy and direct immunofluorescence (Figure 1). At the time of presentation, she was normotensive, afebrile, heart rate 102 beats/min and respiratory rate of 14 breaths/min. Skin examination revealed tense bullae on upper extremities (Figure 2). Abdominal exam was notable for tenderness to palpate in the epigastric region. Digital rectal exam revealed melena. Laboratory findings showed hemoglobin 5.7 mg/dl, hematocrit 17.5 mg/dl. The patient received 1 unit of packed RBC, started on a proton pump inhibitor infusion and continued on mycophenolate with oral prednisone. She underwent an upper gastrointestinal endoscopy which revealed segments of sloughing mucosa in the esophagus. Additionally, a large bulla ranging from 20 to 25 cm was seen only upon withdrawal of the endoscope and not during initial insertion (Figure 3). She was discharged home on a high dose oral proton pump inhibitor, mycophenolate, prednisone, and topical steroid.

DISCUSSION:
Bullous Pemphigoid (BP) is the most common type of acute bullous dermatoses (ABD) affecting older adults without gender predilection. Esophageal manifestations of BP are rare and range from dysphagia, hematemesis or melena. Nikolsky sign is a specific skin finding of autoimmune blistering diseases but is only seen in 56% of the cases. The use of esophagogastroduodenoscopy can be challenging in these patients because of bullae formation after light contact between esophageal mucosa and the endoscope. Only gentle advancement and withdrawal of the endoscope is advised to prevent adverse events such as blood blisters, frank bleeding, or perforation. In cases of significant gastrointestinal bleeding and/ or hemodynamic instability, therapeutic endoscopy can be used to achieve hemostasis.
1793 A Rare Case of Pyogenic Granuloma in the Gastrointestinal Tract
Authors: Basilio Carlo , Baddour Nabil , Affronti John 
Published: October 2019

INTRODUCTION:
Pyogenic granuloma (PG), also known as lobular capillary hemangioma, is a common benign vascular cutaneous or mucosal lesion. This type of lesion rarely occurs in the gastrointestinal tract. This case describes an asymptomatic patient with PG at the gastroesophageal junction.

CASE DESCRIPTION/METHODS:
A 59-year-old woman with no family history of GI malignancy was referred for evaluation of a positive fecal immunochemical test (FIT). She had occasional dyschezia with blood-streaked stools, as well as alternating between occasional loose stools and constipation. She reported weight loss, but she was unable to quantify the amount or the time period. She has a 40 pack-year history of tobacco smoking. Her vital signs and physical exam were normal. Her labs included Hgb 13.8, MCV 86, and non-reactive for HCV. EGD revealed a 4 mm nodule at the gastroesophageal junction (GEJ), as well as prepyloric localized inflammation. The nodule at the GEJ was consistent with PG, and H. pylori testing was negative. As the patient was asymptomatic without any active bleeding, no further workup was necessary.

DISCUSSION:
Only 14 cases of esophageal and 10 cases of gastric PG have been reported. Half of esophageal granulomas were associated with symptoms, including dysphagia, retrosternal pain, hematemesis, and weight loss. Gastric PGs were commonly associated with pain, UGIB, or melena. These lesions can have pseudomalignant features, such as ulceration, as noted in this case. This case demonstrates the importance of understanding PG as part of the differential diagnosis for a positive FIT assay. PGs can be misdiagnosed as a malignant vascular neoplasm, including histopathologic features concerning for malignancy, but it should be considered in the differential diagnosis. The symptoms and features of this lesion can be highly variable, although rare in the GI tract, and gastroenterologists and pathologists must be aware of this to avoid overdiagnosis as a malignant neoplasm.
1794 Esophageal Adenocarcinoma in a Patient With Inclusion Body Myositis
Authors: Hornung Benjamin M. , Kathpalia Priya 
Published: October 2019

INTRODUCTION:
Inflammatory myopathies are rare autoimmune diseases characterized by chronic muscle inflammation and weakness. Dysphagia is often an early onset symptom in inflammatory myopathies. Here we present a case of esophageal adenocarcinoma diagnosed in a patient with inclusion body myositis (IBM) and chronic progressive dysphagia.

CASE DESCRIPTION/METHODS:
A 59 year old woman with an 8 year history of gradually progressive IBM presented to the ER with worsening dysphagia; she has had progressive dysphagia over the preceding 10 days to the point she is unable to tolerate solids. Despite longstanding, progressive symptoms, referral to a gastroenterologist was never made as it was assumed her symptoms were from her underlying IBM. On exam, she is a functional quadriplegic from her underlying IBM and is largely bedbound as a result. Her labs were notable for a macrocytic anemia with negative inflammatory myopathy antibody panel. Barium esophagram revealed a large filling defect in the mid to distal esophagus measuring approximately 3.6 × 2.4 cm and subsequent upper endoscopy revealed an 8cm nearly obstructive distal esophageal mass which appeared to arise in the setting of suspected Barrett’s esophagus. Biopsies demonstrated esophageal adenocarcinoma with signet cell features. Subsequent staging was consistent with cT3N0 disease though she was deemed not to be a surgical candidate due to her quadriplegia and severe deconditioning. An endoscopic esophageal bare metal stent was placed for symptom palliation and she was referred to hospice.

DISCUSSION:
The inflammatory myopathies are a group of autoimmune diseases characterized by chronic muscle inflammation accompanied by weakness. IBM, in contrast to polymyositis and dermatomyositis, has normal or minimally elevated creatine kinase, insidious progression, and is poorly responsive to immunosuppressive therapy. While IBM is generally not thought to be a paraneoplastic syndrome, there are case reports of them being paraneoplastic with a negative antibody panel (as seen in this case). Bulbar symptoms are common in IBM, and >50% of patients will develop oropharyngeal dysphagia usually due to cricoarytenoid weakness. In this case, the patient’s dysphagia was prematurely attributed to IBM, precluding a timely diagnosis of esophageal adenocarcinoma. In patients with inflammatory myopathy and dysphagia, endoscopy is still of utmost importance and symptoms should not be solely attributed to underlying IBM.