Proximal Esophagus Research Articles

S2161 It's All Downhill From Here: A Case of Proximal Esophageal Varices in a Patient With SARS-CoV2/COVID-19 Infection

INTRODUCTION: We present a case of proximal esophageal varices in a patient with COVID-19 infection. CASE DESCRIPTION/METHODS: A 79-year-old male presented with right neck pain after recent hospitalization for COVID-19 complicated by acute renal failure requiring hemodialysis and melena which was medically managed. On arrival, he was febrile (100.4F) and tachycardic (HR 112). Physical exam noted tenderness along the right sternocleidomastoid muscle. Labs were notable for hemoglobin 8.2 g/dL, platelets 244 (103/µL), D-dimer 2,613 (ng/mL-previously 24,780), fibrinogen 490 (mg/dL-previously 1,190). Ultrasound of the neck revealed a thrombus in the right internal jugular vein in the location of his dialysis catheter. Upper endoscopy was pursued given history of melena and need for anticoagulation. Endoscopy revealed columns of non-bleeding, grade II esophageal varices with no high-risk stigmata in the proximal esophagus. A subsequent MRI of the neck showed occlusive thrombus involving the entirety of the right internal jugular vein with likely extension into superior vena cava (SVC). The dialysis catheter was removed and anticoagulation was initiated. DISCUSSION: Proximal or “downhill” esophageal varices are rare and associated with SVC occlusion. Obstruction at the SVC increases pressure in collaterals that drain the proximal and mid esophagus, resulting in development of proximal esophageal varices. SVC obstruction is most commonly caused by mediastinal malignancies but cases of catheter-induced thromboses have been reported.1 Treating proximal esophageal varices requires relieving the underlying SVC obstruction. Bleeding from proximal esophageal varices is rare, but could result in significant consequences.1 In the case presented, thrombosis was likely caused by hypercoagulability from COVID-19 in the presence of a dialysis catheter. Emerging literature has described COVID-19 hypercoagulability, and some have recommended escalating inpatient thromboprophylaxis regimens and considering continuation after discharge.2 Though a rare phenomenon, downhill varices may be encountered more frequently as the COVID-19 pandemic continues and provider awareness could lead to improved patient outcomes.Figure 1.: Image 1: MRI of the neck in coronal view revealing occlusion of the entire right internal jugular vein.Figure 2.: Image 2: Upper gastrointestinal endoscopy view of non-bleeding, grade II varices in the proximal esophagus.

S2013 A Rare Cause of Dysphagia – Epiphrenic Esophageal Diverticulum Complicated by Complete Esophageal Obstruction

INTRODUCTION: Epiphrenic esophageal diverticulum (EED) are a rare protrusion of the distal esophagus arising a few centimeters above the lower esophageal sphincter and is estimated to occur in 1:500,000 individuals per year. It results in motility dysfunction and typically presents as dysphagia and weight loss. Complications include upper GI bleed, esophageal carcinoma, and formation of atypical esophagorespiratory fistulas. CASE DESCRIPTION/METHODS: 66-year-old male with known history of Barrett’s esophagus and daily alcohol ingestion presented for 3 months of dysphagia with 22 kg weight loss. The initial esophagogastroduodenoscopy (EGD) demonstrated a large obstructive distal esophageal stricture for which Bougie French #60 dilation was performed at an outlying facility. Biopsy showed normal squamous mucosa. Two weeks later, dysphagia remained in which patient underwent CT chest that showed distended proximal thoracic esophagus and wall thickening with soft tissue mass involving the distal esophageal lumen consistent with obstruction; no extrinsic mass was noted (Figure 1). Laboratory workup including a complete blood count and comprehensive metabolic panel were within normal limits. Subsequent esophagram showed patulous and dilated esophagus with a large obstructive epiphrenic esophageal diverticulum (Figure 2). Esophageal ultrasound demonstrated food content within the esophageal false lumen (Figure 3). The patient subsequently had a PEG tube placed with plan to undergo traditional open transthoracic surgery with diverticulectomy and esophageal myotomy in the future. DISCUSSION: Most cases of epiphrenic esophageal diverticula are asymptomatic and do not warrant treatment though some may present with dysphagia, dyspepsia, and aspiration pneumonia. It is important to have a broad differential diagnosis including EED in patients with dysphagia. Delayed diagnosis and treatment could put patient at risk for life-threatening complications. Esophageal endoscopy is needed to rule out other dysphagia etiologies. Our case demonstrates complete esophageal obstruction leading to significant weight loss because of EED. In contrast to Zenker’s diverticulum, which occur in the proximal esophagus, EED are much rarer and occur in the distal esophagus. To contrast their rarity, EED occur at a ratio of 1:5 with that of Zenker’s. Traditionally, surgical resection is the treatment of choice. However, endoscopic techniques are being investigated.Figure 1.: CT chest shows dilated, obstructed, and irregular-shaped distal esophagus (red arrow) posterior to the pulmonary artery (PA). There is no extrinsic paraesophageal mass compression.Figure 2.: Esophagram shows epiphrenic esophageal diverticulum (red arrow) with complete distal esophageal obstruction indicates by the absence of contrast in the distal esophagus (black arrow); only an occasional trickle of contrast can be seen to transgress the distal esophagus into the fundus of the stomach. Note the proximity of the diverticulum to the cardiac silhouette.Figure 3.: Esophageal ultrasound showing food content within the esophageal false lumen at the 6 o’clock position.

S1922 Rapidly Extensive Recurrence of Esophageal Neuroendocrine Carcinoma Following Complete Pathologic Response to Definitive Chemoradiation

INTRODUCTION: Esophageal neuroendocrine carcinoma (eNEC) is a rare (<3% of US esophageal cancers) and aggressive malignancy (<5% 5-year overall survival [OS]) that lacks treatment guidelines. The timing and nature of relapse after successful treatment is not well characterized. We report a case of rapidly extensive recurrence after complete pathologic response (cPR) to definitive chemoradiation (dCRT) and maintenance therapy (MT). CASE DESCRIPTION/METHODS: A 51 year old woman with no medical history presented with 3 weeks of epigastric pain, mild acid reflux, and dysphagia. She denied smoking or alcohol use. Pantoprazole, previously prescribed by her primary care doctor, did not help. Endoscopy revealed a 4 cm, necrotic, mid-esophageal ulcer (Figure 1) with NEC histology (Figure 2). Staging with endoscopic ultrasound, brain MRI, and PET-CT determined T3N2M0 disease (Figure 3). dCRT (cisplatin/etoposide) was completed in 6 months with symptom resolution. PET-CT revealed resolution of the initial FDG-avid ulcer and lymph nodes (LNs) (Figure 3). Endoscopy with esophageal biopsies was normal, confirming cPR (Figure 1). After 3 months of atezolizumab for subsequent MT, chest pain occurred. PET-CT showed FDG-avid masses in the liver, bones, thoracic LNs, and subcutaneous tissue (Figure 3). Biopsy of the hepatic lesions confirmed metastatic NEC despite a normal repeat endoscopy (Figure 1). DISCUSSION: This case is unusual given our patient’s atypical demographic for esophageal carcinoma. eNEC’s median age of diagnosis is 62 years with male preference (73%). About 50% and 25% of patients have a smoking and alcohol abuse history, respectively. Selecting dCRT over radical esophagectomy maximized our patient’s quality of life while accepting a potentially suboptimal OS. Recent data on stage III eNEC patients show a 43 month median OS and 50% 3-year OS for those receiving surgery with neoadjuvant chemotherapy, versus 15 months and 15% for those receiving non-surgical treatment. Innovative MT was essential given eNEC’s risk for relapse and lack of established protocols. Our trial of atezolizumab is the first reported off-label use of immune checkpoint inhibition in eNEC. Ongoing phase II trials are studying the efficacy of anti-PD-1 monotherapy and dual anti-PD-1/anti-CTLA-4 therapy in eNEC. Relapse only 4 months after cPR emphasizes eNEC’s insidious and early metastatic course. Given eNEC’s nature, it is imperative that empathic, shared decision-making be an essential component of patient-centered care for this rare malignancy.Figure 1.: Comparison of endoscopy findings at initial presentation (July 2019), cPR after dCRT (December 2019), and recurrence (April 2020). In July, there was a 4 cm, necrotic, half-circumferential, cratered ulcer spanning between 28-34 cm from the incisors. Esophagus was otherwise unremarkable, aside from mild reflux esophagitis at the GE junction. Stomach and duodenum (to the second portion) were unremarkable. In December, there was near complete resolution of the original ulcerated lesion. Biopsies returned as normal squamous mucosa, without intestinal metaplasia, dysplasia, or malignancy. In April, the appearance of the esophagus was generally similar to that in December, aside from mild re-ulceration of the lesion now measuring 1 cm. Biopsies still revealed normal squamous mucosa, with some necrotic debris. A: Necrotic ulcer, approached from the normal, proximal esophagus; B: Direct view of the ulcer in the mid to distal esophagus; C: Former lesion site, approached from the proximal esophagus; D: Former lesion site, exiting toward the distal esophagus; E: Former lesion site, with mild raising and ulceration, F: Unremarkable distal esophagus.Figure 2.: Biopsy histopathology (from July endoscopy) confirming primary esophageal neuroendocrine carcinoma. Homogeneous, small, round, purple/blue cells on H&E staining are characteristic of this high grade, poorly differentiated neuroendocrine malignancy. Immunohistochemistry was positive for specific neuroendocrine markers: synaptophysin, chromogranin, and CD56. The neoplasm was strongly Ki-67 positive (80-90% of cells), reflecting its highly proliferative nature. Staining was negative for adenocarcinoma markers, including mucicarmine and CDX2. Negative staining for CD45, SOX10, and CD30 ruled out hematopoietic, neural crest, or lymphatic tissue origin, respectively.Figure 3.: Comparison of PET-CT findings at initial staging (August 2019), cPR after dCRT (December 2019), and recurrence (April 2020). In August, there were no distant metastases (including a negative brain MRI). Increased FDG activity was confined to the mid-esophageal mass (A, B) and lymph nodes within the gastrohepatic ligament (C). CT demonstrated a 2.6 × 2.2 cm, right lower esophageal, eccentric mass with central necrosis and positive gastrohepatic LNs up to 1.3 cm. Imaging was negative for intrathoracic or cervical lymphadenopathy. In December, there was near complete resolution of abnormal FDG uptake at the esophageal site (D, E), with decreased size and reduced FDG uptake at the gastrohepatic LNs (F). In April, there was interval development of FDG-avid masses within the liver (G), bilateral iliac bones (G), L2/L3 vertebral bodies (H), left scapula, right posterior sacrum, thoracic lymph nodes (I), and right chest wall subcutaneous tissue (J). White arrows indicate areas of abnormal FDG-avidity corresponding to malignant lesions.

S1889 Esophageal Lichen Planus – An Additional Diagnosis in the Differential Requiring Additional Stains

INTRODUCTION: Lichen planus (LP) is a chronic inflammatory condition that commonly affects the skin and mucous membranes with an estimated incidence of 0.5%–2%. While the most frequent sites of involvement are the mouth, genitalia, scalp, and nails, reports of lichen planus with esophageal involvement (ELP) in the literature remain scant. CASE DESCRIPTION/METHODS: An 86-year old female with a past medical history of hypothyroidism, sarcoidosis, and oral ulcers was evaluated for a four year history of dysphagia to solids and liquids and 30 pound weight loss. Gastrointestinal review of systems was otherwise negative except for recent recurrence of oral ulcers. The patient underwent a barium esophagram, which revealed a tortuous stricture involving 3 cm of the cervical esophagus. A subsequent esophagogastroduodenoscopy (EGD) was performed, revealing several rings and few plaques along the proximal esophagus (Figure 1). The stricture (Figure 2) was difficult to traverse but was successfully dilated to 12 mm. Biopsies of the proximal esophagus were initially unremarkable with histology revealing benign esophageal squamous mucosa with mild reactive changes. With the history of oral ulcers and sarcoidosis, additional immunohistochemical stains were added. Further analysis revealed positive fibrin deposition consistent with lichenoid inflammation, concerning for ELP. The patient was started on oral budesonide and serial dilations up to 16 mm were performed, which resulted in some improvement of symptoms. DISCUSSION: While cutaneous and oral findings of LP are easily detectable, ELP remains a diagnostic challenge. Given its rarity and resemblance to other esophageal pathologies such as eosinophilic esophagitis, reflux esophagitis, and candida esophagitis, accurate diagnosis requires additional stains to make a diagnosis. The mean time to diagnosis is around five years, and often after multiple endoscopies and dilations. Given the scarcity of ELP, treatment is based on anecdotal reports, and usually consists of oral steroids with dilation. Furthermore, given the risk of malignant transformation to squamous cell carcinoma, patients should be carefully followed though there are no surveillance guidelines as the diagnosis is rare. This case highlights the importance of accurate and timely diagnosis with additional stains in patients presenting with progressive dysphagia to provide adequate treatment and malignancy surveillance.Figure 1.: Endoscopic view of the proximal esophagus revealing several proximal rings and few plaques.Figure 2.: Endoscopic view of the severe tortuous stricture in the proximal esophagus.

S1920 Pruritis &amp; Dysphagia: Gastrointestinal Manifestations of Lichen Planus

INTRODUCTION: Lichen planus is an inflammatory disorder of the mucocutaneous tissues. Esophageal involvement is rare and is often asymptomatic early in disease course. Therefore, delay in diagnosis is common and may predispose patients to both failed symptom treatment and development of oral squamous cell carcinoma. Esophageal lichen planus (ELP) is a rare and underrecognized cause of dysphagia that has been reported almost exclusively in middle-aged to elderly women. We present a male patient with ELP and the uncommon endoscopic finding of esophageal pseudodiverticulosis and histology notable for Civatte bodies. CASE DESCRIPTION/METHODS: 36-year-old male presents with 4 years of dysphagia to solids that worsened following his fourth esophageal balloon dilatation for esophageal stenosis. He endorsed a pruritic rash on his torso, localized to his hands, arms, and abdomen (Figure A). EGD revealed multiple pseudodiverticula and a benign appearing stricture in the proximal esophagus at 15–18 cm from the incisors (Figure B). Random esophageal biopsies demonstrated increased intraepithelial lymphocytes with scattered Civatte bodies and rare eosinophils (Figure C). Patient’s hospital course was complicated by concurrent diarrhea; stool culture positive for Campylobacter and Clostridium difficile. Therefore, patient was started on topical steroids and oral antihistamine instead of the standard treatment of systemic steroids. DISCUSSION: ELP is a rare condition that often occurs in conjunction with other manifestations of lichen planus. Yet, ELP continues to be under-recognized due to misinterpretation of the endoscopic features. Predominant endoscopic finding of ELP include stricturing of the proximal esophagus as seen in our patient. The most suggestive evidence of ELP is the presence of atrophic epithelial changes, lymphohistiocytic inflammatory infiltrate, and scattered, degenerated and dyskeratotic cells also known as Civatte bodies. Mucosal changes from ELP varies, but in our patient, we found an uncommon endoscopic finding of esophageal pseudodiverticulosis. Based on previous case series and case reports, easy sloughing of the esophageal mucosa seems to be another characteristic sign of ELP. In conclusion, ELP is a rare condition that is commonly missed; the presence of esophageal pseudodiverticulosis may hint at ELP. It is critical to consider ELP in patients with dysphagia and dermatologic manifestations of lichen planus, as delayed diagnosis of ELP may result in oral squamous cell carcinoma.Figure 1.: Pruritic rash on torso, hands, arms, and abdomen with oral lesion on physical examination.Figure 2.: Image 1 & 2: EGD revealed multiple pseudodiverticula in the esophagus marked by arrows Image 3:Benign appearing stricture in the proximal esophagus at 15-18cm from the incisors.Figure 3.: Esophageal biopsy concerning for increased intraepithelial lymphocytes with scattered Civatte bodies and rare eosinophils. Arrows marking Civatte bodies.

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.