Proximal Esophagus Research Articles

S1956 An Unusual Case of Proximal Esophageal Stricture​: Esophageal Lichen Planus

INTRODUCTION: Lichen planus (LP) is an inflammatory disease of unknown etiology that most commonly affects the skin and mucous membranes. Roughly 0.5-2% of the population is affected and it usually affects middle-aged females. A rare manifestation of this disease is esophageal lichen planus (ELP). Often correct identification and diagnosis of ELP is difficult and delayed, as presented below. CASE DESCRIPTION/METHODS: A 58-year-old male with a history of DM2, HTN, HLD, tobacco use and obesity presented with complaints of progressively worsening dysphagia and emesis. He denied odynophagia, weight loss or difficulty swallowing liquids. His physical exam was unremarkable. An esophagram demonstrated a 2.5 cm long area of narrowing within the upper esophagus at the level of the sternoclavicular joint. A CT chest showed no evidence of a mediastinal mass. An EGD showed a smooth circumferential luminal narrowing at 25 cm insertion in the proximal esophagus. There were no focal lesions; however, the mucosa did show scattered white exudates. This area could not be traversed with the endoscope and the lumen was ∼ 8 mm in diameter. The stricture was dilated and biopsies were obtained. Of note, both the stomach and duodenum appeared normal. Pathology showed a basilar lymphocytic infiltrate with scattered apoptotic squamous cells (Civatte bodies); no intraepithelial eosinophils were identified. These findings were consistent with a diagnosis of ELP and the patient was started on swallowed fluticasone. Repeat EGD, again demonstrated a proximal, smooth, non-focal, circumferential, luminal stricture. Multiple small white plaques with new punctate circular erosions were appreciated throughout the proximal and mid-esophagus; biopsies were again obtained. Pathology resulted in similar findings as prior with the addition of concurrent fungal yeast and hyphae identified on H&E stain. He was advised to continue current treatment and start fluconazole with close follow-up. DISCUSSION: ELP is a rare disease with variable presentations. In our case specifically, the patient did not fit the usual demographic for a patient with ELP and he had no other manifestations of LP. Delays in diagnosis are likely related to a lack of both suspicion for the disease and strict diagnostic criteria. ELP is difficult to treat and often requires trials of multiple different medications. Added awareness, timely diagnosis and a better understanding of its underlying pathogenesis will likely result in more effective treatments in the future.Figure A.: Esophageal surgical pathology specimen showing basilar lymphocytic infiltrate with scattered apoptotic squamous cells known as civatte bodies (black arrows).Figure B.: EGD demonstrating proximal esophageal stricture with white exudates.

S2008 A Case of Esophageal Adenocarcinoma in a Young Male With Longstanding Untreated Eosinophilic Esophagitis

INTRODUCTION: Eosinophilic Esophagitis (EoE) has been increasing in incidence over the last decade due to the increasing number of EGD with biopsies preformed. Up to 6.5% patients undergoing EGD for any indication are diagnosed with EoE and 22% in patients for dysphagia. EoE is present in all age groups but peak incidence is bimodal, in first and third decades of life. Here we describe a case of esophageal adenocarcinoma in a patient with history of EoE. CASE DESCRIPTION/METHODS: A 42 year-old male (BMI 27 kg/m2) with PMHx of untreated EoE for 10 years presented to the ED for food bolus impaction a few hours after eating pork. No history of alcohol or tobacco use. Emergent EGD was performed with successful removal of solid food bolus in middle third of the esophagus. There were endoscopic mucosal changes consistent with EoE (EoE-EREFS: Edema 1, Rings 2, Exudates 1, Furrows 1 and Stricture 0) within the entire esophagus and a nodularity appreciated at the GEJ. Biopsies obtained from proximal and distal esophagus showed more than 50 and 100 eosinophils/per HPF respectively. The GEJ lesion showed high grade dysplasia in the settings of goblet cell metaplasia. Repeat EGD 3 weeks later revealed a medium sized fungating and ulcerated mass at the GEJ which was circumferential and partially obstructing. The biopsies showed esophageal adenocarcinoma with indeterminate HER2 oncogene expression, staged as T3N1 based on mini-probe EUS. Oncology classified the patient as cT3N1Mx. The patient received chemo-radiation (5-fluorouracil/oxaliplatin) over 37 days followed by robotic assisted laparoscopic Ivor Lewis esophagogastrectomy with left gastric hepatic artery and celiac lymphadenectomy with successful remission, T0N0. Patient remained in clinical remission at 6 months follow up. DISCUSSION: An estimated 17,500 new cases of esophageal cancer were reported in 2016. Incidence was 4 in 100,000 and increases with age starting from 0.2 in 100,000 at age 30 increasing to 26 in 100,000 at age 80. Mortality was 3.9 in 100,000 deaths and has a 5 year survival of 18.9%. Current risk factors for development of esophageal cancer are alcohol, smoking, and obesity. No clear genetic factors have been associated with development and it is unknown if EoE is a risk factor. However, chronic inflammation and structural changes may be a contributor to malignancy and further research should focus on this area given diseases mortality rates.Figure 1.: A. Food Bolus at Middle Third of the Esophagus B. Abnormal Mucosa at the Gastro-Esophageal Junction C. Gastric Cardia, retroflex view: Mass.Figure 2.: Proximal esophagus shows increased intraepithelial eosinophils, consistent with eosinophilic esophagitis.Figure 3.: GE Junction biopsy shows adenocarcinoma arising in the background of barrett’s esophagus.

S2087 Endoscopic Cap-Assisted Retrieval of a Fishbone in the Esophagus

INTRODUCTION: Foreign body ingestion is a common cause of dysphagia and odynophagia. Common presenting symptoms can include dysphagia and odynophagia as previously mentioned, as well as chest pain/pressure, globus sensation, nausea, vomiting, or neck pain. While the majority of foreign bodies will pass spontaneously, a subset can become stuck and require retrieval. Cap-assisted endoscopic retrieval can aid in removal of the foreign body. We present a case of a patient who presented with odynophagia and globus sensation found to have a fishbone in his esophagus after eating fish for dinner. CASE DESCRIPTION/METHODS: The patient is a 62 year old male without significant past medical history who presented to the emergency department with odynophagia and a globus sensation. He was eating fish for dinner and felt as though a piece became stuck in his throat and presented to the ED for evaluation. CT scan of the neck showed a fishbone horizontally stuck in the esophagus at the level of C6 with penetration of the esophageal wall and extraluminal air. The patient went for upper endoscopy where a 2.5 cm fishbone was found in the proximal esophagus, lodged into both walls. A transparent cap was employed during the endoscopic retrieval. The foreign body was then removed with a Raptor grasping device and hemostatic clips were places along the perforated esophageal edges to repair the tear. He subsequently had a barium esophagram, which showed no esophageal leak. His diet was advanced successfully and he was discharged on a 7-day course of augmentin. DISCUSSION: Obtaining a reliable patient history is paramount in determining the correct diagnosis. If the clinical history prompts a high degree of suspicion, obtaining CT scan is the preferred imaging modality. Once the presence of a foreign body is confirmed on imaging, it can be retrieved via endoscopy. Utilizing endoscopic caps can aid in the visualization and retrieval of foreign bodies in the gastrointestinal tract. As seen with our patient, his clinical history created a high suspicion, prompting CT scan, which confirmed the presence of a fishbone lodged in the esophagus. The foreign body was retrieved via upper endoscopy the following morning with improvement in symptoms. His course was notable for esophageal wall penetration, which was successfully repaired endoscopically.Figure 1.: Foreign body in the esophagus with a clear cap in view.

S1937 Development of Eosinophilic Esophagitis in a Patient With Radiation-Induced Esophageal Strictures: A Case Report

INTRODUCTION: Radiation therapy for head, neck, and esophageal cancer often results in difficult to manage esophageal strictures. These strictures often require repeat dilation to improve dysphagia symptoms. Eosinophilic esophagitis (EOE) can present with similar symptoms of malnutrition and dysphagia and can lead to strictures. We present the unique case of a female with suspected radiation strictures found to have associated eosinophilic esophagitis with improvement in dysphagia with anti-reflux therapy. CASE DESCRIPTION/METHODS: A 49 year old female with history of tongue cancer status post hemiglossectomy and multiple radiation treatments developed worsening chronic oropharyngeal dysphagia and regurgitation. Patient underwent a barium esophagram which showed esophageal narrowing. She then had an upper endoscopy which revealed a cervical esophageal stricture and underwent bougie dilation. Patient was diagnosed with a post-radiation stricture. On repeat endoscopy one month later, patient was found to have a similar esophageal stricture but noted to have associated linear furrows with upper esophageal biopsies showing 110 eosinophils per high power field. She was dilated at that time and also started on anti-reflux therapy daily. On repeat endoscopy, patient was noted to have significant improvement in her symptoms with no subsequent dilations required and improved nutritional performance. DISCUSSION: Upper esophageal strictures can be challenging to treat. Strictures in patients with EOE can often escape detection during endoscopy. Our patient demonstrates a unique case with simultaneous radiation and eosinophilic stricture causing dysphagia. Her symptoms responded significantly better after initiation of proton pump therapy, highlighting the importance of considering dual etiology for esophageal strictures. In patients who do not respond to multiple dilations for radiation strictures, co-existing pathology such as EOE should be considered. Patients with refractory radiation esophagitis warrant careful endoscopic evaluation for associated EOE. Treatment for patients with radiation strictures and dysphagia often requires dilation, which may place patients at higher risks for complications with underlying EOE. Patients with radiation esophagitis with suspicion for EOE should undergo cautious dilation. This case propagates the role for empiric anti-reflux therapy in patients with a high pre-test probability of EOE complicating radiation and other benign esophageal strictures.Figure 1.: Proximal Esophagus Stricture.Figure 2.: Linear Furrows in Upper Esophagus.

S1910 A Case Series of Esophageal Granular Cell Tumor in Eosinophilic Esophagitis

INTRODUCTION: Granular cell tumors (GCTs) are rare, soft tissue neoplasms arising from Schwann cells and can be found anywhere in the body. Gastrointestinal GCTs represent 6–10% of all cases with the majority arising in the esophagus. On endoscopy, GCTs appear as white-grey or a yellow subepithelial lesion. Most GCTs are benign, but approximately 0.5–2% of cases progress to malignancy and carry a poor prognosis. Eosinophilic esophagitis (EoE) is a chronic, immune-mediated condition characterized by eosinophil-predominant inflammation of the esophagus. We present three cases of esophageal GCTs diagnosed endoscopically in the setting of EoE. CASE DESCRIPTION/METHODS: Case 1: A 25 year-old female with asthma and environmental allergies presented with chronic dysphagia and intermittent solid food impaction never requiring endoscopy. She underwent EGD which showed mild inflammation. There was also a 5 mm small nodular area with adherent exudate (Figure 1). Biopsies of the proximal and distal esophagus revealed squamous mucosa containing innumerable intraepithelial eosinophils, consistent with EoE. Additionally, the nodule was found to be a GCT. Case 2 :A 38 year-old male with a history of biopsy-proven EoE presented for a follow up EGD after receiving PPI therapy for twelve weeks. EGD revealed a normal esophagus (Figure 2); however, random biopsies revealed incidental GCT in the mid-esophagus. Case 3: A 33 year-old male with a history of childhood environmental allergies presented with chronic dysphagia and intermittent solid food impaction. On EGD, the esophagus appeared normal. However, random biopsies from the mid esophagus showed eosinophilia consistent with EoE as well as a GCT. Therefore, repeat EGD was performed with white light along with endoscopic ultrasound to further evaluate for GCT and revealed a 2 mm submucosal lesion (Figure 3). DISCUSSION: The relationship between GCT and EoE remains unclear. Previously, case reports noted the simultaneous finding of GCT in patients with EoE on EGD. Here, we present three different endoscopic findings of esophageal GCT. Most notably, in two cases GCT was not diagnosed visually, but rather histologically. These cases add to the growing body of literature associating EoE and GCT, though a pathophysiologic relationship has not yet been drawn. Whether the presence of GCT in these cases is completely incidental or immune-mediated is unknown. Thus, as the prevalence of EoE continues to increase, clinicians should be aware of the association between GCT and EoE.Figure 1.: EGD of the esophagus revealing a 5 mm granulosa cell tumor (GCT).Figure 2.: Normal endoscopic appearance of the esophagus without endoscopic appearance of a GCT.Figure 3.: Submucosal GCT seen on white light endoscopy.

S3543 Esophageal Parakeratosis? You GERD Be Kidding!

INTRODUCTION: Esophageal parakeratosis is an atypical finding on histology with limited data available. On microscopy it is characterized by superficial, compact squamous epithelium with retained pyknotic nuclei. References describe parakeratosis in various clinical settings such as candidiasis, vitamin B12 and zinc deficiencies, esophageal lichen planus and ethanol exposure in adults. To our knowledge, based on a PubMed Search, this condition has never been reported in children. We describe a child with esophageal parakeratosis with history of reflux symptoms and recent coin ingestion. CASE DESCRIPTION/METHODS: An 8-year-old boy with a history of regurgitation presented to the emergency room with dysphagia after swallowing a penny earlier that day. Chest X-ray revealed a discoid metallic foreign body lodged in the mid esophagus. During upper endoscopy the coin was located in the mid esophagus and retrieved easily. There was an erosion at the site of the penny lodgment and furrowing of the distal esophagus. Biopsies were obtained from the distal esophagus and mid esophagus, distal to the site of coin lodgment. On histological examination of the mid esophageal biopsies, there was mild reactive esophageal squamous mucosa with focal superficial erosion, focal parakeratosis, mild mixed inflammation and vascular congestion without evidence of eosinophilic esophagitis. Based on the pathology, he was diagnosed with gastroesophageal reflux disease (GERD) esophagitis. He was treated with a proton pump inhibitor for 3 months with clinical resolution of reflux symptoms. A 3-month follow-up endoscopy revealed mild edematous mucosa in the distal esophagus without furrowing, ulcers or erythema. Histopathology of the proximal and distal esophagus revealed persistence of minimal reactive esophageal mucosa, with vascular congestion and minimal chronic inflammation but with no parakeratosis. DISCUSSION: Esophageal parakeratosis is often a non-specific histological finding. Based on the clinical history, the resolution of esophageal parakeratosis on repeat biopsy in an area distal to the coin lodgment, along with subtle findings of esophagitis after PPI therapy, led us to believe that the parakeratosis was a manifestation of GERD. It is unlikely that these changes are secondary to a blunt foreign body (coin) reaction in such a short window of time. Clinicians should be aware that esophageal parakeratosis may be associated with GERD.

S3002 Dysphagia Leading to Endoscopic Diagnosis of Primary Amyloidosis

INTRODUCTION: Amyloidosis is a rare disorder that is caused by the extracellular deposition of abnormal protein fibrils in organs and tissues. Gastrointestinal (GI) involvement in systemic amyloidosis is rare with a reported prevalence of about 3%. We report a case of a 66-year-old female with complaint of dysphagia diagnosed with primary (AL) amyloidosis based on esophagogastroduodenoscopy (EGD) and biopsy. CASE DESCRIPTION/METHODS: A 66-year-old woman with a past medical history of hypertension, diastolic heart failure, history of stroke, and gastroesophageal reflux presented with symptoms of dysphagia accompanied by dysgeusia, anorexia, and unintentional 20 lb weight loss. On physical examination, the patient exhibited mild epigastric tenderness. Laboratory results notable for alkaline phosphatase 440 IU/L, total bilirubin 1.4 mg/dL, hemoglobin 11.7 g/dL, and INR 1.2 with normal aspartate aminotransferase and alanine aminotransferase, and negative hepatitis serologies. The patient underwent EGD with multiple large non-bleeding angioectasias found in the stomach, which were treated with argon plasma coagulation (APC) prophylactically. Patchy, moderately erythematous mucosa without bleeding was found in the stomach and was biopsied. No gross endoscopic esophageal abnormality was found to explain the patient's dysphagia. Four biopsies were obtained in the proximal and distal esophagus. Random gastric biopsies stained with Congo red demonstrated amyloidosis involving oxyntic mucosa with mild chronic inactive gastritis. Distal esophageal biopsies stained with Congo red demonstrated deposits suspicious for amyloid. DISCUSSION: GI-related symptoms in AL amyloidosis is reported to be 8–60%. However, the prevalence of gastrointestinal amyloidosis with both symptoms and biopsy-proven involvement of tissue is about 3%. Common symptoms of GI amyloidosis include weight loss, diarrhea, abdominal pain, and varying degrees of bleeding. The above patient presented with dysphagia, which is a rarely reported symptom of GI amyloidosis. The gold standard for diagnosing amyloidosis is tissue biopsy with Congo red stain demonstrating green birefringence under polarized light. Both the esophageal and stomach tissue samples demonstrated amyloid deposition. The dysphagia experienced by the patient was likely due to neuromuscular infiltration causing dysmotility. This case demonstrates the unusual presentation of dysphagia leading to the diagnosis of AL amyloidosis with rare biopsy proven infiltration of the esophagus and stomach.Figure 1.: Stomach biopsy sample stained with Congo red demonstrating apple green birefringence under polarized light.Figure 2.: Esophagus biopsy sample stained with Congo red demonstrating apple green birefringence under polarized light.Figure 3.: Stomach biopsy sample stained with Congo red demonstrating congofilic deposits.

S1992 Immune Checkpoint Inhibitor-Mediated Eosinophilic Esophagitis and Gastroenteritis

INTRODUCTION: Immune checkpoint inhibitors (ICPI) are immunomodulatory antibodies that enhance the immune system in the management of malignancies resulting in significantly improved prognosis especially in melanoma treatment. They are often associated with immune mediated side effects affecting multiple organ systems. The most common gastrointestinal side effect is colitis resulting in diarrhea, however eosinophilic esophagitis (EoE) and gastroenteritis is rarely reported. CASE DESCRIPTION/METHODS: A 50 year old female with history of stage IIIB melanoma managed with resection and 12 months of adjuvant nivolumab presented after immunotherapy completion with severe pruritis, dysphagia, and epigastric pain. Laboratory workup was notable for 19.9% eosinophilia with an absolute count of 1,700 cells/µL. Esophagogastroduodenoscopy (EGD) revealed ringed esophagus, white plaques and edema in the entire esophagus as well as edema and friability in the stomach and duodenal bulb. Biopsies showed increased intraepithelial eosinophils (eos), greater than 50 per high power field (hpf) in the proximal and distal esophagus. Gastric and duodenal biopsies revealed increased eos in the lamina propria with villous blunting. Her abdominal pain improved with a prednisone taper but she then developed severe heartburn and worsening dysphagia. Repeat EGD showed longitudinal furrows, edema, rings, and exudates consistent with EoE and normal stomach and duodenum. Pathology showed up to 80 eos/hpf in the esophagus, with resolution of the gastric and duodenal eosinophilia. Her symptoms resolved with a course of omeprazole and viscous budesonide slurry. DISCUSSION: Nivolumab is associated with several immune mediated toxicities including pneumonitis, colitis, endocrine and dermatological side effects. Eosinophilia may be seen following treatment with ICPIs and is potentially associated with improved prognosis. Eosinophilic tissue inflammation, particularly involving the GI tract is uncommon. A prior case report described eosinophilic enteritis following combination ICPIs ipilumab and nivolumab. A French case series reported 37 patients with hypereosinophilia following ICPI therapy; 57% had organ involvement, none with esophagitis or gastroenteritis. Most patients responded well to ICPI discontinuation and/or corticosteroids. This is the first report of eosinophilic esophagitis and gastroduodenitis following completion of 1 year of nivolumab therapy. There appears to be resolution with systemic and topical corticosteroid therapy.

S0435 Determination of the Eosinophilic Esophagitis Endoscopic Reference Score Associated With Histologic Response to Therapy: Analysis From a Phase 3 Placebo-Controlled Trial of Budesonide Oral Suspension

INTRODUCTION: The validated Eosinophilic Esophagitis Endoscopic Reference Score (EREFS) quantifies five key endoscopic features associated with eosinophilic esophagitis (EoE). We aimed to determine the post-treatment EREFS threshold that corresponds best with histologic response in patients with EoE after 12 weeks of therapy with budesonide oral suspension (BOS). METHODS: This post hoc analysis used data from a phase 3, 12-week, placebo-controlled trial in patients 11–55 years old with EoE and dysphagia (NCT02605837). Patients were randomized (2:1) to receive BOS 2.0 mg twice daily or placebo. Changes in EREFS from baseline to week 12 were measured as a secondary efficacy endpoint; total scores ranged from 0 to 20 (proximal and distal esophagus). We used a receiver operating characteristic (ROC) curve analysis to assess the accuracy of EREFS to distinguish between histologic responders (≤ 6 eosinophils/high-power field [eos/hpf]) and non-responders (>6 eos/hpf) after 12 weeks of BOS therapy, for patients who received ≥ 1 dose. The ROC curve was generated using the true-positive rate (sensitivity) versus the false-positive rate (1−specificity) for different cut-off thresholds of EREFS. The optimal threshold for EREFS was determined using the Youden Index (sensitivity+[specificity−1]). RESULTS: Overall, 318 patients received ≥ 1 dose of study drug. Only BOS-treated patients were included in this analysis (n = 213); of these, 113 (53.1%) were histologic responders. Greater improvements in least-squares mean EREFS were observed for BOS- versus placebo-treated patients (−4.0 vs −2.2, respectively; P < 0.001) from baseline to week 12. Histologic responders had significantly lower post-treatment EREFS and greater improvements in EREFS from baseline to week 12 of therapy than non-responders (Table 1). The area under the ROC curve was 0.705 for EREFS and histologic response (Figure 1). An EREFS of 4 was the optimal threshold for distinguishing histologic response to BOS therapy. Specifically, there was a 68% probability that a patient with a post-treatment EREFS of ≤ 4 was a true positive (responder). CONCLUSION: Endoscopic scoring of EoE with EREFS is responsive to BOS therapy and represents a parameter which has moderate accuracy for determining histologic response (≤ 6 eos/hpf). Analyses that evaluate EREFS compared with additional disease parameters will further support its use as an independent assessment of disease activity in patients with EoE.Table 1.: EREFS at baseline and after 12 weeks of therapy with BOS 2.0 mg b.i.d., stratified by histologic response statusFigure 1.: ROC curve analysis of EREFS to predict histologic response (≤ 6 eos/hpf) to 12 weeks of therapy with BOS 2.0 mg b.i.d. in patients with EoE.

S1970 Utility of Covered Metal Stents in Esophageal Penetrating Injuries

INTRODUCTION: Management of esophageal perforations and leaks is traditionally managed via a surgical approach, however, here we show that self-expanding metal stents (SEMS) can be used to successfully treat esophageal injuries. CASE DESCRIPTION/METHODS: Case 1: A 27-yo man presented for GSW to the chest and went for surgical repair of the proximal esophagus. The esophageal defect was oversewn, but on day 7 an esophagram revealed an anterior leak. On EGD, there were 2 medium-sized perforations 30 cm from the incisors that were covered with a 12 cm × 20 mm fully covered stent under fluoroscopic guidance and anchored with two hemostatic clips. One week later the stent was exchanged because one perforation was still present. At two-weeks after the first EGD, the perforations had completely healed that was confirmed with esophagram. Case 2: A 21 year-old-woman presented for GSW to the neck who was found to have a T1 spinal cord injury with severe damage to the soft tissue. An esophageal perforation was found on a later esophagram and surgical repair failed to close the leak. EGD showed a perforation at 20 cm from the incisors that was treated with a 12 cm × 20 mm fully covered stent under fluoroscopic guidance and anchored with two hemostatic clips. She had a repeat EGD with stent exchange, but at week-two the tract had epithelialized that was further treated with APC and closure with 3 clips. DISCUSSION: Esophageal perforations are a life-threatening complication. If not corrected within the first 24 hours, morbidity and mortality increases significantly due to increased risk for fulminant mediastinitis and pleural contamination.1 Successful closure of benign esophageal perforations with stents is approximately 82%-100%.1 In a study of 15 patients, early (∼45 minutes) versus late (∼123 hours) esophageal stenting decreased hospital stay to 5 days compared to 44 days in the late group related to complications of sepsis and multiorgan failure.4 Serious complications from esophageal stenting are stent perforation, stent-related bleeding, and strictures which occurred at 2%, 0.8%, and 3.2%, respectively. In that same study, 73/340 experienced stent migration.5 A study of 44 patients comparing rates of esophageal migration with and without anchoring clips, migration occurred in 3/23 patients with hemostatic clips versus 12/21 without hemostatic clips.6 Here are two cases of esophageal perforation where fully covered esophageal SEMS were used without complication of stent migration due to anchoring clips.Figure 1.: Esophageal perforation.Figure 2.: SEMS covering perforation.Figure 3.: Healed perforation after SEMS.

S2003 Cytomegalovirus (CMV)-Induced Acute Esophageal Necrosis (Black Esophagus) in the Lung Transplant Recipient: A Rarest Combination

INTRODUCTION: Acute esophageal necrosis (AEN) also known as black esophagus is a rare clinical entity with characteristic endoscopic appearance of diffuse circumferential necrosis of distal esophagus with relative sparing of proximal esophagus. We report a 60-year-old man with a double lung transplant a year ago, who developed cytomegalovirus induced AEN. To the best of our knowledge and literature review, this is the first and only case of CMV related AEN with resistant stricture in double lung transplant recipient. CASE DESCRIPTION/METHODS: A 60-year-old male with advanced COPD status post double lung transplant a year ago presented with acute onset of odynophagia with solids/liquids for two days. CT scan of the chest showed diffuse thickening of distal esophageal wall without evidence of perforation. An upper endoscopy revealed severe diffuse necrotic mucosa extending from 26 cm to 39 cm from the incisors (Figure A). Biopsies showed CMV related acute and chronic inflammation with granulation tissue (Figure B). Patient was kept NPO and managed with intravenous hydration, total parenteral nutrition, ganciclovir along with proton pump inhibitors for aggressive acid suppression. Repeat endoscopic evaluation two weeks later showed complete resolution of necrosis but moderately severe stenosis in the lower third of the esophagus (Figure C) that required serial dilations over the course of 1 year without significant improvement. DISCUSSION: Acute esophageal necrosis is a rare condition with an incidence of 0.01-0.28%. Infection is a major contributor in post-transplant patients with immunosuppressed state. Proposed pathophysiology contributing to AEN includes impaired esophageal mucosal reparative mechanisms seen in chronically debilitated patients along with esophageal ischemia seen in low flow states. Most common presentation is upper gastrointestinal bleeding in up to 90% of cases. EGD is diagnostic with distal esophageal involvement in 97% of cases. Esophageal biopsy aids in exclude/confirm infectious etiology and typically not associated with increased risk of perforation. Treatment is mainly supportive with management of underling etiology. Potential complication includes stricture formation and perforation. AEN should be considered in the differential diagnosis of UGI bleed and dysphagia especially in post-transplant patients. In conclusion, isolated CMV related AEN is extremely rare in lung transplant recipients and this is the first ever case reported in the literature.Figure A.: Initial EGD showing black necrotic esophagus and follow up EGD two weeks later with healing of necrosis along with distal esophageal stricture.Figure B.: Giant cell with inclusion body characteristic of CMV esophagitis.Figure C.: Barium Esophagram showing long tapering distal esophageal stricture.

S2012 Esophageal Lichen Planus: Making the Diagnosis with History and Endoscopy With Histopathologic Evaluation

INTRODUCTION: Esophageal lichen planus (ELP) is a rare manifestation of lichen planus, an inflammatory disease of unknown etiology that commonly affects mucocutaneous membranes. Diagnosing ELP can be challenging because of nonspecific endoscopic or histologic findings, variable symptoms and lack of clear diagnostic criteria. CASE DESCRIPTION/METHODS: A 67-year-old female with a past medical history of gastroesophageal reflux disease and hypertension presented to clinic with two years history of dysphagia to solids, odynophagia, and 30lbs weight loss. Symptoms began with oral ulcers, bleeding gums, and hypogeusia. Her medications included a thiazide diuretic. Physical examination was negative for skin or oropharyngeal lesions and abdominal exam was benign. Laboratory results were negative for antinuclear antibody, anti-Ro/La, and hepatitis C antibody. Patient was started on a proton pump inhibitor and proceeded to esophagogastroduodenoscopy, which revealed an upper esophageal web that was dilated. The mucosa was diffusely inflamed throughout the length of the esophageal body, making the identification of the boundary between squamous and columnar epithelium difficult. A short distal stricture 2 cm above the top of the hiatal hernia was also dilated. Biopsy of proximal esophagus revealed squamous mucosa with basilar lymphocytic infiltrate and scattered apoptotic squamous cells (Civatte bodies) (Figure 1), consistent with a diagnosis of ELP. Distal esophageal biopsy revealed intestinal metaplasia without dysplasia and squamous mucosa with predominantly lymphocytic mixed inflammatory infiltrate and rare apoptotic squamous cells. Patient tolerated endoscopic dilation without complications and patient’s thiazide diuretic was held. Patient re-presented to clinic 6 months after EGD without dysphagia and 9lbs weight gain since previous visit. DISCUSSION: Lichen planus is an inflammatory disease of unknown etiology that commonly affects mucocutaneous membranes but can also involve the esophagus. ELP should be suspected in any patient who has history of lichen planus. Dysphagia and odynophagia are two primary symptoms associated with ELP. A comprehensive endoscopic, radiologic and histopathologic evaluation aid in the diagnosis. ELP is treated with systemic steroids and endoscopic dilation. Endoscopic dilation with discontinuation of potential culprit medications (i.e. thiazides) is a reasonable first step management, as in our case.Figure 1.: Biopsy specimens showing inflammatory infiltration of basal mucosa and scattered apoptotic squamous cells, also known as Civatte bodies (arrow).

A RARE CASE OF CERVICAL HARDWARE MIGRATION CAUSING ESOPHAGEAL PERFORATION

SESSION TITLE: Medical Student/Resident Procedures Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Esophageal perforation caused by migration of cervical hardware in the delayed setting is a rare but deadly complication of anterior cervical spine surgery (ACSS). Esophageal perforation is a known complication of ACSS, which accounts for more than 40% of documented cases. However only 0.2-1.5% of those cases happened in the delayed setting and the initial presentation can be atypical. The purpose of this report is to highlight the importance of promptly diagnosing these patients and moving forward with the appropriate management. CASE PRESENTATION: 32-year-old man with a PMH of C6 spinal cord injury 3 years prior with residual quadriplegia requiring anterior cervical discectomy and fusion (ACDF) of C6-T1 with C7 corpectomy and a tracheostomy. He presented with worsening abdominal pain. Imaging and colonoscopy were both normal so an EGD was performed which found an eroded foreign body in the lumen of the proximal esophagus. Neurosurgery was consulted and ordered CT imaging which confirmed our suspicion. A bronchoscopy was then done to evaluate his tracheostomy site which did not appear to have any effect on his airway nor were any fistulas observed. Despite the aforementioned findings the patient was clinically stable and denied any dysphagia, odynophagia, poor appetite, or irregular bowel movements. Patient is currently scheduled for cervical spine hardware revision. DISCUSSION: ACDF has been around since the 1950s, is well documented, and has an overall complication rate of 5%. That being said, surgical complications including perforation can be further subdivided into acute or delayed (>30 days post-op) and can range from local infection to mediastinitis and death. While most acute perforations during or after ACSS are iatrogenic, the majority of delayed perforations are from hardware failure (41%) which come from loosened or migrated screws/plates. Chronic erosion of hardware makes up 31% of cases and is thought to be caused by tissue ischemia from the prolonged pressure of cervical hardware, thus weakening the posterior esophageal wall leading to deadly complications. Esophageal perforation in this subset of patients can happen during ACSS or up to 11 years post-operatively in some studies. Obtaining a pertinent history is paramount in the settings of acute versus chronic esophageal perforations. An acute perforation would present with dysphagia and odynophagia (54%), fever (21%), and/or neck swelling (21%). Our case is atypical in that our patient was being worked up for something entirely different and denied any of the aforementioned signs and symptoms. CONCLUSIONS: Delayed esophageal erosions require a high level of suspicion as presenting signs and symptoms may be nonspecific. Operative interventions with revision of cervical spine surgery and myofascial repair are the standard of care for this condition with considerations being made for comorbidities and clinical circumstances. Reference #1: S. H. Halani, G. R. Baum, J. P. Riley et al., “Esophageal perforation after anterior cervical spine surgery: a systematic review of the literature,” Journal of Neurosurgery: Spine, vol. 25, no. 3, pp. 285–291, 2016. Reference #2: A. Marquez-Lara, S. V. Nandyala, H. Hassanzadeh, M. Noureldin, S. Sankaranarayanan, and K. Singh, “Sentinel events in cervical spine surgery,” The Spine Journal, vol. 39, no. 9, pp. 715–720, 2014. Reference #3: D. Yin, X. Yang, Q. Huang et al., “Pharyngoesophageal perforation 3 years after anterior cervical spine surgery: a rare case report and literature review,” European Archives of Oto-Rhino-Laryngology, vol. 272, no. 8, pp. 2077–2082, 2015. DISCLOSURES: No relevant relationships by Zaid Ansari, source=Web Response No relevant relationships by MAYKEL IRANDOST, source=Web Response No relevant relationships by Tyler Putnam, source=Web Response No relevant relationships by Ali Saeed, source=Web Response

The Usefulness of Esophageal Baseline Impedance Levels for the Diagnosis of Nonerosive Reflux Disease and the Proper Time for Measurement in Endoscopy-negative Korean Patients With Esophageal or Supraesophageal Symptoms.

Background/AimsLow baseline impedance levels (BILs) have been suggested to be evidence of GERD. The aim of this study is to investigate the usefulness of esophageal BILs for the diagnosis of nonerosive reflux disease (NERD) and the proper time for measurement in endoscopy-negative Korean patients with esophageal or supraesophageal symptoms.MethodsEndoscopy-negative patients with esophageal or supraesophageal symptoms who underwent esophageal multichannel intraluminal impedance-pH monitoring were included. BILs were measured in the proximal and distal esophagus around 10 minutes before meals, 10 minutes and 30 minutes after meals, 30 minutes before the start of nighttime sleep, and 30 minutes and 60 minutes after the start of nighttime sleep.ResultsA total of 104 patients were included in the study. Distal and proximal esophageal BILs were decreased after meal ingestion. The BILs of the distal esophagus were significantly lower at all time points in the NERD group, but not in the reflux hypersensitivity (RH) group, compared with the functional group. The area under the receiver operating characteristic curve for the diagnosis of NERD was significant at all time points, but that for the diagnosis of RH was not. The cut-off value of 2375 Ω or 2125 Ω measured around 30 minutes before or 60 minutes after the start of nighttime sleep, respectively, were appropriate for the diagnosis of NERD.ConclusionThe BILs of the distal esophagus measured at time points before or after the start of nighttime sleep appear to be useful for the diagnosis of NERD, but not for the diagnosis of RH, in endoscopy-negative Korean patients with esophageal or supraesophageal symptoms.

Plasminogen Activator Inhibitor-1 as a Marker of Esophageal Functional Changes in Pediatric Eosinophilic Esophagitis

Esophageal remodeling in eosinophilic esophagitis (EoE) can lead to esophageal rigidity with eventual luminal compromise and stenoses. Gauging esophageal functional alterations in EoE is challenging. An epithelial marker of functional remodeling would impact EoE management. Esophageal biopsy specimens from children with and without EoE and primary human esophageal epithelial cells were used for PAI-1 immunohistochemistry, and cell proliferation experiments. PAI-1 immunostaining and basal cell hyperplasia were assessed in the context of concurrently obtained esophageal compliance measures on endoscopic functional lumen imaging probe (EndoFLIP). EndoFLIPs were performed in 45 children (32 with and 13 without EoE). Epithelial PAI-1 was increased in patients with active EoE versus inactive or control patients (P < .01). Esophageal compliance was lower in EoE patients versus controls, particularly in the proximal esophagus (P < .001). Proximal compliance was the strongest predictor of EoE (AUROC 0.88, 95% CI 0.77, 0.98) with esophageal compliance of less than 2.6%mL/mmHg demonstrating 82% sensitivity and 84% specificity for EoE. PAI-1 inhibition significantly diminished esophageal epithelial cell proliferation, suggesting PAI-1 could trigger basal cell hyperplasia. A composite mid-esophageal BZH+ PAI-1 score was the strongest predictor of altered compliance (P = .02, AUROC 0.89 (95% CI 0.80, 0.99). PAI-1 is significantly elevated in pediatric EoE and distinguishes altered compliance in children. PAI-1 may be a novel disease marker and therapeutic target.

Çocuklarda Inlet Patch’in Klinik Önemi

Inlet patch (IP) is an area of heterotopic gastric mucosa located in proximal esophagus. Although the majority of IP are asymptomatic, they may be associated with digestive and respiratory symptoms. We aimed to assess prevalence, endoscopic and histopathological findings, clinical significance and outcome of inlet patch in children.The patients with histopathologically proven IP and aged between 0-18 years old were enrolled. Demographic data, clinical symptoms, endoscopic and histopathological findings, treatment modality, and outcomes were collected from medical records. Retrospective review of 2674 esophagogastroduodenoscopy records revealed 11 (0.41%) children. Eight of our patients had a solitary patch whereas others had two (n=1) or three (n=2). Histopathological evaluation revealed that 9 patients had fundic and 2 patients had antral type gastric mucosa. One patient with hematemesis and other with dysphagia had hyperemic patchy areas of which were colonized by H. pylori. Inlet patch was the only pathological endoscopic finding in 4 patients with a single symptom each: heartburn, dysphagia, hematemesis and hoarseness. Symptoms were completely resolved with PPI treatment in 8 children. Helicobacter pylori eradication was achieved in all infected patients. No respiratory symptom was recorded except hoarseness in one patient. No complications like perforation, stenosis or dysplasia that might be related to IP were recorded at follow-up. We suggest that an IP may accompany or may be responsible for digestive symptoms in children and PPI treatment is effective. Endoscopist should be aware of this condition, especially if the patient has dyspeptic symptoms and normal endoscopic findings.

Intestinal metaplasia around the gastroesophageal junction is frequently associated with antral reactive gastropathy: implications for carcinoma at the gastroesophageal junction

Increasing evidence suggests that bile reflux (BR) plays a major role in mucosal injury, leading to adenocarcinoma of the proximal stomach and distal esophagus. However, gastric BR is difficult to diagnose and investigate. Reactive gastropathy (RG), in the absence of nonsteroidal anti-inflammatory drugs (NSAIDs) and other known causes, likely represents bile-mediated injury to the gastric mucosa. The goal of this study is to explore the association between antral RG and gastroesophageal junction (GEJ) mucosal inflammation and intestinal metaplasia (IM). The pathology database was searched for patients who had gastric biopsies with a diagnosis of antral RG and concurrent gastric cardia/GEJ/distal esophagus biopsies from 2013 to 2015. Age- and sex-matched patients with normal gastric antral biopsies served as controls. Biopsies from the GEJ region were evaluated for histological changes, including inflammation, antral and pancreatic metaplasia, RG, the type of gastric glands, proton pump inhibitor (PPI) changes, and IM. Detailed clinical history and medication use (including PPIs and NSAIDs) were recorded. IM in the GEJ region was more frequent in patients with antral RG than in controls (33.0% vs. 5.2%, 95% confidence interval [18.3-37.3%]). In addition, inflammation, other mucosal changes around the GEJ (RG and foveolar hyperplasia), antral IM, and PPI-associated mucosal changes were also more frequently seen in patients with antral RG. Our results show that antral RG is associated with mucosal injury and IM around GEJ, suggesting a role of BR. Further studies are needed to study duodenogastric-esophageal BR and its role in development of proximal gastric and distal esophageal adenocarcinoma.

430 BALLOON TAMPONADE UTILIZATION FOR SEVERE ESOPHAGITIS CAUSING HEMORRHAGIC SHOCK

Abstract Esophagitis as a cause of upper gastrointestinal bleeding (UGIB) is a difficult entity to treat and is associated with significant morbidity. Epidemiologic studies note a 7-18% incidence of significant hemorrhage due to esophagitis. Current existing treatment options for UGIB complicated by hemorrhagic shock secondary to severe esophagitis are limited when there is a lack of response to proton pump inhibitor (PPI) medical therapy. Methods We present a case series of two patients who developed hemorrhagic shock from esophagitis, underwent endoscopic evaluation revealing limited treatment options, and ultimately required balloon tamponade resulting in successful resolution of bleeding and improvement in hemodynamics. A 55-year-old male with history of alcoholism complicated chronic pancreatitis and esophagitis presented with seizures. On admission he experienced hemorrhagic nasogastric tube output, anemia unresponsive to transfusions and hemodynamic instability. A 44-year-old patient with decompensated alcoholic cirrhosis was initially admitted for renal failure, and during the hospitalization developed melena and hemorrhagic shock requiring transfusions and vasopressors. The patients were promptly started on PPI infusion. Results Both patients underwent esophagogastroduodenoscopy revealing severe bleeding esophagitis extending from the gastroesophageal junction to the proximal esophagus and no varices. Active bleeding was not amenable to endoscopic therapy and both patients underwent balloon tamponade placement. The first patient required inflation of both the esophageal and gastric balloons for control of hemorrhage for 6 hours whereas the second patient required just gastric balloon inflation for 12 hours. Bleeding, transfusion requirement, and hemodynamics improved in both patients. Second look endoscopy demonstrated resolution of bleeding and hemostatic spray was applied to the esophagus prophylactically. Neither patient had recurrence of bleeding during the hospitalization. Conclusion UGIB secondary to esophagitis is difficult to control due to the highly vascularized nature of the esophagus. Endoscopic therapies are limited in extensive mucosal injury and risks of complications such as perforation are high. Other therapeutic options such as angiography are restricted due to vascular collateralization and surgical interventions have high morbidity and mortality. Balloon tamponade provides a viable treatment option for severe UGIB secondary to esophagitis that is not responsive to medical therapy.

Robot-assisted minimally invasive esophagectomy for esophageal cancer: Meticulous surgery minimizing postoperative complications.

Minimally invasive esophagectomy (MIE) has been reported to reduce postoperative complications especially pulmonary complications and have equivalent long‐term survival outcomes as compared to open esophagectomy. Robot‐assisted minimally invasive esophagectomy (RAMIE) using da Vinci surgical system (Intuitive Surgical, Sunnyvale, USA) is rapidly gaining attention because it helps surgeons to perform meticulous surgical procedures. McKeown RAMIE has been preferably performed in East Asia where squamous cell carcinoma which lies in more proximal esophagus than adenocarcinoma is a predominant histological type of esophageal cancer. On the other hand, Ivor Lewis RAMIE has been preferably performed in the Western countries where adenocarcinoma including Barrett esophageal cancer is the most frequent histology. Average rates of postoperative complications have been reported to be lower in Ivor Lewis RAMIE than those in McKeown RAMIE. Ivor Lewis RAMIE may get more attention for thoracic esophageal cancer. The studies comparing RAMIE and MIE where recurrent nerve lymphadenectomy was thoroughly performed reported that the rate of recurrent nerve injury is lower in RAMIE than in MIE. Recurrent nerve injury leads to serious complications such as aspiration pneumonia. It seems highly probable that RAMIE is beneficial in performing recurrent nerve lymphadenectomy. Surgery for esophageal cancer will probably be more centralized in hospitals with surgical robots, which enable accurate lymph node dissection with less complications, leading to improved outcomes for patients with esophageal cancer. RAMIE might occupy an important position in surgery for esophageal cancer.

Impaired Mucosal Integrity in Proximal Esophagus Is Involved in Development of Proton Pump Inhibitor-Refractory Nonerosive Reflux Disease

Background and Objective: Weakly acidic reflux reaching to the proximal esophagus is closely related to the perception of gastroesophageal reflux in patients with nonerosive reflux disease despite treatment with a proton pump inhibitor (PPI). However, little is known about the involvement of the patients’ mucosal integrity of the proximal esophagus. Methods: We recruited 15 symptomatic nonerosive gastroesophageal reflux disease (GERD) patients with a positive symptom index despite PPI treatment and 11 healthy asymptomatic volunteers as controls. The biopsy specimens obtained from the proximal and distal esophagus were applied to a mini-Ussing chamber system to measure transepithelial electrical resistance (TEER) against a pH 4 weak acid. The esophageal biopsy samples were subjected to quantitative real-time PCR and immunohistochemical analysis. Results: In the proximal esophagus, the weak acid exposure reduced the TEER in the PPI-refractory patients compared to that in the controls. The frequency of the reflux extending to the proximal esophagus had a significant correlation with the reduction in the proximal esophageal TEER in the patients. The reduced TEER in the proximal esophagus was accompanied by an increase in IL-8 and IL-1β mRNA and a decrease in occludin mRNA levels. The proximal esophageal mucosa in the patients presented infiltration of CD3-positive lymphocytes and an increased expression of solute carrier organic anion transporter family member 2A1 (SLCO2A1), a passage gate of reflux symptom-evoking molecules. Conclusions: The reflux perception is related to an impairment of the proximal esophageal mucosal integrity in patients with nonerosive reflux disease despite PPI.