bluuebunny/crossref_metadata_2025 huggingface dataset:
1780 Combined Esophageal Achalasia and Cricopharyngeal Achalasia in a Patient With Type 1 Myotonic Dystrophy
Authors: Ghazaleh Sami , Nehme Christian , Khader Yasmin , Ghanim Marcel , Hasan Syed , Nawras Ali
Published: October 2019

INTRODUCTION:

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

CASE DESCRIPTION/METHODS:

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

DISCUSSION:

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

INTRODUCTION:

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

CASE DESCRIPTION/METHODS:

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

DISCUSSION:

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

INTRODUCTION:

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

CASE DESCRIPTION/METHODS:

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

DISCUSSION:

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

INTRODUCTION:

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

CASE DESCRIPTION/METHODS:

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

DISCUSSION:

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

INTRODUCTION:

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

CASE DESCRIPTION/METHODS:

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

DISCUSSION:

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

INTRODUCTION:

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

CASE DESCRIPTION/METHODS:

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

DISCUSSION:

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

INTRODUCTION:

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

CASE DESCRIPTION/METHODS:

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

DISCUSSION:

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

INTRODUCTION:

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

CASE DESCRIPTION/METHODS:

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

DISCUSSION:

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

INTRODUCTION:

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

CASE DESCRIPTION/METHODS:

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

DISCUSSION:

The inflammatory myopathies are a group of autoimmune diseases characterized by chronic muscle inflammation accompanied by weakness. IBM, in contrast to polymyositis and dermatomyositis, has normal or minimally elevated creatine kinase, insidious progression, and is poorly responsive to immunosuppressive therapy. While IBM is generally not thought to be a paraneoplastic syndrome, there are case reports of them being paraneoplastic with a negative antibody panel (as seen in this case). Bulbar symptoms are common in IBM, and >50% of patients will develop oropharyngeal dysphagia usually due to cricoarytenoid weakness. In this case, the patient’s dysphagia was prematurely attributed to IBM, precluding a timely diagnosis of esophageal adenocarcinoma. In patients with inflammatory myopathy and dysphagia, endoscopy is still of utmost importance and symptoms should not be solely attributed to underlying IBM.
1797 Intramural Esophageal Dissection: A Rare Cause of Hematemesis
Authors: Al-Jashaami Layth , Rezaie Aida , Abidali Hussein , Alishahi Yasmin
Published: October 2019

INTRODUCTION:

Intramural esophageal dissection (IED) was first described by Williams in 1957. It is a rare form of acute esophageal trauma defined as an injury in the esophageal submucosal layer extending between the mucosa and muscular layer. Patients may present with acute chest pain, epigastric pain, dysphagia, retching or hematemesis. It most cases, IED is associated with trauma during food ingestion.

CASE DESCRIPTION/METHODS:

A 91-year-old Veteran presented to the Phoenix VA Health Care System’s Emergency Department ED with a 1-day history of retching followed by dysphagia after pill ingestion. He subsequently developed massive hematemesis described as a ‘basin full of bright red blood’ and lightheadedness. His medications were notable for aspirin 81 mg daily, but no anticoagulant. A computed tomography scan of the chest showed severely thickened esophageal wall without gas or fluid in the mediastinum. Urgent esophagogastroduodenoscopy (EGD) was performed revealing a blood clot in the oropharynx and around the Endotracheal tube, and a large organized hematoma without active bleeding in the mid to distal esophagus adherent to the esophageal wall. Multiple attempts to remove the blood clot were made by snare and endoscopic basket without success. A repeat follow-up EGD was performed demonstrating the same hematoma with successful removal via distal attachment cap. Beneath the hematoma, a 10-cm, non-circumferential, deep submucosal tear was seen from mid- to distal esophagus associated with small spots of bleeding (Figure 1). Nine endoscopic clips were placed (Resolution clip, Boston Scientific) (Figure 2). The patient did not develop any further hematemesis, and his dysphagia improved throughout admission.

DISCUSSION:

We present a case of spontaneous IED involving 10cm of the esophagus in a 91-year-old Veteran treated with endoscopic zipper clipping to achieve successful hemostasis. While conservative management is reported in most cases in the form of bowel rest, this case demonstrates the significance of endoscopic evaluation when hemodynamic instability is present. For this Veteran, the hematoma provided a natural form of hemostasis until endoscopic clips could be placed to stop bleeding.
1798 An Endoscopic Vacuum-Assisted Closure Technique Modified Through the Overtubes for Patients With Esophageal Anastomotic Leaks
Authors: Kwon Hyeonwoo , Kim Seongmin , Lee Jun Su , Cho Byungha , Choi Jongwook , Kim Ki Bae , Kim Si-Wook , Kim Dohun , Kim Hyoung Woo , Han Joung-Ho , Yoon Soon Man , Youn Sei Jin , Chae Hee Bok , Park Seon Mee
Published: October 2019

INTRODUCTION:

Endoscopic vacuum-assisted closure (E-VAC) therapy is safe and effective for esophageal anastomotic leaks. However, the repetitive exchange of sponge tubes is technically demanding.

CASE DESCRIPTION/METHODS:

We designed an E-VAC technique, modified through the use of overtube, to make E-VAC therapy safer and more comfortable for both endoscopists and patients. Intraluminal E-VAC therapies were used for the treatment of post-operative thoracic esophageal leaks in five cases of esophageal cancer. After overtube insertion, sponge tube was inserted through the overtube, while keeping biopsy forcep inside the nasogastric tube (NGT), and was positioned at intraluminal leakage site under endoscopic guidance. NGT was repositioned from the mouth to the nostrils using carrier tube and a controlled negative pressure was applied. Sponge tube was regularly changed twice a week until the complete closure of leaks. Technical and clinical success rates were analyzed to evaluate the safety and efficacy of this technique. The modified E-VAC therapy was applied in five selected cases for a mean of 13.8 days (range, 7-28 days) with a mean of 3.2 sponge tube changes (2-6 changes) for anastomotic leaks that were, on average, 1.0 cm (0.5-2 cm). The mean procedural time was 14.9 min (12-30 min) and no E-VAC therapy-related complications occurred. All patients were discharged, on average, after 44.4 days (range, 14-95 days) of hospitalization and two cases were treated with additional esophageal metal stents after six and two sponge tube changes, respectively.

DISCUSSION:

Our E-VAC therapy technique, modified via inserting sponge tubes through overtube, is effective and safe for patients with thoracic esophageal anastomotic leaks.
1805 AFP Value of 2400 With No HCC; Metastatic Esophageal Cancer With Neuroendocrine Differentiation With Rare Increase of AFP
Authors: Pacha Faris , Skrove Jamie L. , Sobrado Javier , Rana Zaid
Published: October 2019

INTRODUCTION:

Adenocarcinoma (AC) accounts for 60 percent of all esophageal cancers in the USA. Dysphagia and weight loss are among the most common symptoms. In the US, an estimated 17,290 cases of esophageal cancer will be diagnosed each year, and 15,850 deaths are expected from the disease. In the US, smoking and excessive alcohol consumption, account for approximately 90 percent of the total cases of esophageal CA. The majority of cases are located near the GEJ and are associated with endoscopic evidence of Barrett's Esophagus (BE). AC arising in BE may present as an ulcer, a nodule, an altered mucosal pattern, or no visible endoscopic abnormality. Early AC not associated with BE arises from an ulcer, plaque, or nodule near the GEJ.

CASE DESCRIPTION/METHODS:

Patient is a 67-year-old male who presented to the E.D. for worsening epigastric pain and dysphagia to solids with no history of EGD. The patient is a 40 pack year smoker and non-drinker. The patient denies other symptoms other than epigastric dull pain. During his hospital stay imaging demonstrated severe lymphadenopathy near the aorta along with multiple liver lesions with the largest being 3.5cm. The patient received a CT Chest that demonstrated pulmonary nodules with speculated appearance suggested of metastatic disease, mediastinal lymphadenopathy, and multiple liver lesions greatest of which being 3.5cm. The patient then had an EGD that demonstrated friable, mottled, irregular mass extending from 40cm in the esophagus to 45cm. The patient also had an elevated CEA at greater than 949, CA 19-9 at 221 and an AFP of 2400. The patient’s esophageal mass biopsy resulted in AC with neuroendocrine differentiation. The patient then received a liver biopsy that showed metastatic Adenocarcinoma not HCC. A repeat liver biopsy was done and the pathology was read again as AC.

DISCUSSION:

This is a rare case of esophageal cancer resulting in a marked increase of AFP without HCC or testicular involvement. Also the patient had esophageal cancer with neuroendocrine differentiation with liver masses. The patient is without active hepatitis, liver disease or testicular involvement. Very elevated AFP level and esophageal cancer with no relation has only been described in few cases and if there is elevation it is moderate. A lesson to be learned from this case is the patient should have had EGD sooner as most adenocarcinomas arise from Barrett’s esophagus. Had the patient had and EGD the diagnosis of and treatment of Barrett’s could have been initiated sooner.
1807 Esophageal Melanocytosis: An Uncommon Finding
Authors: Patel Milan , Shyu Irene , Mochel Mark , Smallfield George B.
Published: October 2019

INTRODUCTION:

Esophageal melanocytosis is a benign, rare melanocytic proliferation in the basal layer of the squamous epithelium. The etiology of the melanocytosis is not clear; however, it has been suggested that aberrant migration during embryogenesis and chronic irritation such as GERD could lead to its occurrence. While largely asymptomatic, these proliferations raise concern for mucosal melanoma.

CASE DESCRIPTION/METHODS:

A 69-year-old Trinidadian female with past medical history of gastroesophageal reflux disease (GERD) presented with a two week history of dysphagia. Physical examination did not reveal any cutaneous or mucocutaneous lesions. Neurological exam also was normal. A computerized tomography (CT) scan showed circumferential wall thickening and moderate patulous proximal esophagus. A barium esophagram revealed severe esophageal dysmotility and GERD. Given dysphagia and CT findings, an esophagogastroduodenoscopy (EGD) was performed revealing hyperpigmented mucosa in the lower third of the esophagus (Figure 1A). Biopsies revealed melanin within the basal squamous epithelium with evenly spaced melanocytes and pigmented dendritic processes (Figure 1B). Immunohistochemistry for Melan-A and SOX10, a nuclear marker of melanocytes, highlighted these melanocytes without nesting or pagetoid scatter (Figure 1C). The patient was scheduled for a follow-up EGD for monitoring in 12 months.

DISCUSSION:

Endocscopically, anal and colonic lesions have been discovered along with esophageal lesions. Esophageal lesions largely present in the middle and lower third of the esophagus. Initial visualization of these lesions should be managed with a biopsy to determine if melanoma is present. Should esophagael melanocytosis be discovered on biopsy, repeat EGD with biopsy is recommended as early melanoma-in-situ may closely mimic esophageal melanocytosis.
1808 Dysphagia: A Rare Case of Neuroendocrine Carcinoma of the Esophagus
Authors: Zaver Himesh B. , Devani Kalpit H. , McKinney Jason
Published: October 2019

INTRODUCTION:

Neuroendocrine carcinoma (NEC) of the esophagus is a rare, poorly understood disease with high mortality rates. We present a de novo case of NEC of the esophagus responsive to multi-modality therapy facilitated by early endoscopic evaluation and clinical suspicion. Our objective is to raise awareness on rarer causes of dysphagia in order to improve time to treatment and patient outcomes.

CASE DESCRIPTION/METHODS:

A 63-year-old male with a past medical history significant for GERD and tobacco use, presented with a complaint of a one month history of progressive dysphagia to solids. He denied any history of presenting symptoms and had no prior endoscopic examination in the past. Vitals, laboratory workup, and physical exam including chest and abdominal exam were normal. In the setting of progressive dysphagia the patient underwent Esophagogastroduodenoscopy (EGD) revealing a distal esophageal mass (Figure 1). Subsequent Computed Tomography (CT) and Positron Emission Tomography (PET) scan, showed an esophageal mass approximately 4.7 cm x 8.5 cm without any distant metastasis (Figure 2). Biopsy revealed poorly differentiated, discohesive cells indicative of malignant, high-grade neoplasm with neuroendocrine features. Immunohistochemical testing was positive for CD56, synaptophysin, AE1/AE3, and CK8/18. Our patient was referred to a multidisciplinary team and underwent local radiation and chemotherapy of Cis-platinum and Etoposide with excellent response. Surveillance EGD with random biopsies and PET-CT following treatment at 1 year from diagnosis showed no evidence of residual malignancy (Figure 3). However, surveillance EGD at year 2, showed irregular Z lines and biopsy revealed small nests of atypical cells with neuroendocrine features. PET-CT and EUS were negative for any metabolically active neoplasm, and the patient declined further treatment.

DISCUSSION:

Esophageal NEC is rare with only 0.4 and 2% of all diagnosed NETs being found in the esophagus. Our case presents a patient with limited disease surviving past the median survival time of 17-23 months. Our patient was responsive to local radiation and Cis-platinum and Etoposide management. However, surveillance EGD two years after initial diagnosis showed recurrent malignancy. Poor patient outcomes and the limited number of reported cases have made it difficult to define first-line therapeutic strategies. Albeit rare, increased clinical suspicion and early endoscopic evaluation can hasten time to treatment and improve patient outcomes.
1809 Gurvits Syndrome: A Rare Cause of Upper Gastrointestinal Bleed
Authors: Ali Sara I. , Tariq Saad , Patel Jigar , Cherian Sujith , Larson Scott
Published: October 2019

INTRODUCTION:

Acute Esophageal Necrosis (AEN), also known as Black Esophagus or Gurvit’s syndrome, is a rare disorder that can present as an acute gastrointestinal (GI) bleed. Given the high risk of perforation and mortality, a GI bleed in the correct clinical context should put AEN on the differential. We discuss clinical, endoscopic and management of AEN.

CASE DESCRIPTION/METHODS:

57-year-old female with hypertension and gastroesophageal reflux disease presented after a pre-syncopal event. She was admitted for septic shock secondary to urinary tract infection. She was hypothermic with a blood pressure of 76/48 mm Hg on arrival to the ICU. She was started on broad spectrum antimicrobials, in addition to, norepinephrine and vasopressin to maintain mean atrial pressures above 60 mmHg. On ICU day two, she had multiple episodes of coffee-ground emesis and was intubated for airway protection. Her hemoglobin acutely dropped from 8 g/dl to 6.8 g/dl over 12 hours. EGD revealed circumferential ulceration of the lower esophagus with areas of necrosis. Patient was started on high-dose intravenous proton pump inhibitor and supportive therapy with treatment of septic shock. Three days later an endoscopic nasojejunal tube was placed for enteral feeding which revealed increasing amounts of viable mucosa with a decrease in the area of necrosis. There were no further GI bleed and patient was discharged 30 days later.

DISCUSSION:

AEN is multifactorial with a combination of an ischemic insult, corrosive injury to the mucosa from gastric contents and impaired reparative mechanism in setting of acute illness. Risk factors include male sex, older age and multiple chronic medical conditions. Men are four times more commonly affected than women. Diagnosis is made via direct visualization with findings of circumferential black discoloration of the distal esophagus that abruptly ends at the gastroesophageal junction. With comparatively less dense vasculature, the distal esophagus is the water-shed area of the esophagus. Treatment of AEN focuses on aggressive treatment of underlying disease, supportive therapy, and acid suppression. Complications of AEN include esophageal perforation, mediastinitis, and abscess formation. Long term complications include stricture formation and stenosis. Insertion of nasogastric tube should be avoided given the high risk of perforation secondary to the friability of the esophageal mucosa. In our patient, endoscopically placed nasojejunal tube was a safer alternative to restoring enteral feeding.
1813 A Killer in the Dark: The Black Esophagus
Authors: Zahir Fathima Z. , Buffie Charlie , Wan David , Betesh Andrea
Published: October 2019

INTRODUCTION:

Acute esophageal necrosis, also known as “black esophagus” is a rare condition of unclear etiology. Only 88 patients were diagnosed over a period of 40 years. It is diagnosed by endoscopy showing darkly pigmented necrotic esophageal mucosa. Prognosis is poor, with a mortality rate of 31.8-50%. We report a case of this condition in an elderly woman who presented with hematemesis and sepsis.

CASE DESCRIPTION/METHODS:

A woman aged 72 presented after a fall with head strike without loss of consciousness. CT head was unremarkable. That evening, she had an episode of coffee-ground emesis associated with hypotension, tachycardia, and worsening mental status. Hemoglobin was 6.1, raising suspicion of GI bleed. However, she developed leukocytosis without abdominal pain or melena, suggesting septic shock. She received fluids and antibiotics with no improvement. CTPA showed bilateral pulmonary embolism. Anticoagulation was held given concern for GI bleed. Later a massive transfusion protocol was activated when hemoglobin dropped to 4.8; 400 cc of blood was aspirated from the stomach. She received 3U pRBCs, 1U FFP, 1U platelets, 500 cc albumin and vasopressors with improvement of hemoglobin to 11. She then became increasingly acidotic and hypothermic for which a bair hugger was placed. An EGD revealed a severely inflamed esophagus with black material extending from the mid esophagus to the gastroesophageal junction, consistent with esophageal necrosis, the likely source of her GI bleed. The patient stopped following commands, moving extremities or responding to pain. Despite maximal measures, the patient’s hemodynamic instability worsened and she died.

DISCUSSION:

Acute necrotizing esophagitis is a fatal condition that commonly presents with hematemesis or melena. It is associated with multiple factors including diabetes, hemodynamic instability, trauma, hypercoagulable state, gastric volvulus, and some infections. While the pathophysiology is not well known, it is hypothesized that the condition results from an ischemic insult. Esophageal perforation, stricture, infection and death are the main complications. As with our patient, the most likely cause of death are underlying comorbidities such as infections, sepsis, hypovolemic shock or pulmonary embolism, while underlying esophageal disease as the cause of death have been reported in only less than 10% of patients. Elderly patients with comorbidities like diabetes and vascular disease, presenting with upper GI bleed must always get an upper GI endoscopy.
1817 Blastic Mantle Cell Lymphoma of the Gastroesophageal Junction: A Unique Presentation
Authors: Ahmed Ahmed , Vossough Sima
Published: October 2019

INTRODUCTION:

Though vast, the medical literature is deficient on reports of gastroesophageal junction (GEJ) involvement of mantle cell lymphoma (MCL). We present a unique case of a 76-year-old male who was found to have a blastic variant of MCL in the GEJ. This case provides additional distinct features to the various clinical presentations associated with this rare proliferative disorder, thereby enhancing the medical literature on MCL.

CASE DESCRIPTION/METHODS:

A 76-year-old male with a history of prostate and bladder cancer presented with worsening dysphagia, weight loss, and heartburn for over 6 months. He had extensive workup in the past for an ulcerative, gastric/GEJ lesion, found on 4 separate esophagogastroduodenoscopies (EGD) and 2 esophageal ultrasounds (EUS), whose biopsies ranged from mild gastritis to intestinal metaplasia, but were repeatedly negative for malignancy. Physical exam and labs were unremarkable except for anemia. Computerized tomography (CT) scan showed an irregular mass extending from the proximal fundus to the GEJ, and diffuse lymphadenopathy (LAD). EGD on admission showed a large friable, ulcerated lesion with heaped up margins involving the cardia, with extension into the GEJ. GEJ biopsies showed histological and immunohistochemical (IHC) findings consistent with blastic MCL. Further workup of a positron emission tomography (PET) scan showed a fluorodeoxyglucose (FDG) avid mass involving the GEJ and stomach as well as FDG avid regional LAD. A bone marrow biopsy showed minimal involvement (<5%) of CD5+/CD23+ B cells and was negative for both Cyclin D1 and t (11;14). He was started on a chemoimmunotherapy regimen (CITR) with a good response and is currently progression free after 2 years.

DISCUSSION:

The EGD biopsy was diffusely infiltrated by atypical lymphocytes with prominent nucleoli and IHC stains positive for CD-20, Cyclin D1, BCL-2, and BCL-6, and a Ki-67 proliferative index >90%; all consistent with blastic MCL; a rare and aggressive subtype of MCL. He was not a candidate for stem cell transplant due to his age/comorbidities, so he was started on guideline-based CITR of R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone) followed by rituximab maintenance therapy. PET/EGD done 1 year later showed improvement of the LAD and GEJ mass, with negative biopsies for lymphoma. To the best of our knowledge, there have been no reported cases of GEJ involvement of blastic MCL appearing as a large friable lesion, illustrating the unique aspect of this case.
1822 Esophageal Neuroendocrine Tumor: A Rare Case of High-Grade Carcinoma of the Esophagus
Authors: Parsa Nour , Mansoor Muhammad Sohail , Tageldin Omar , Zelman Sara , Batool Asra
Published: October 2019

INTRODUCTION:

Neuroendocrine tumors (NET) of the esophagus are extremely uncommon. The rarity of esophageal NETs is thought to be due to an underdeveloped neuroendocrine system in the esophagus. When diagnosed, esophageal NETs are usually found in the distal esophagus where neuroendocrine cells are primarily distributed in associated mucosal glands.

CASE DESCRIPTION/METHODS:

We describe a unique case of a 62-year-old man diagnosed with an esophageal NET. His PMHx is notable for psoriasis, chronic tobacco use (45 pack years), cirrhosis from heavy alcohol use and illicit drug use including cocaine. He first presented to the hospital with complaints of intermittent episodes of chest pain. He underwent full cardiac workup which was unremarkable. Several months later, he returned to clinic with symptoms of progressive dysphagia to solids. Esophagram revealed a 7cm narrowed segment along the distal esophagus with mucosal irregularity concerning for esophageal neoplasm. A follow up CT abdomen/pelvis showed circumferential wall thickening of the distal esophagus, a 5.2cm necrotic lymph node within the gastrohepatic ligament, as well as a smaller necrotic lymph node at the level of the gastroesophageal junction concerning for malignancy. Follow up EGD revealed a fungating mass in the distal esophagus with biopsies confirming tumor cells positive for MNF116, CD56 and synaptophysin; the overall findings were consistent with high grade neuroendocrine carcinoma of the esophagus. The patient started chemotherapy with Carboplatin and Etoposide; he was subsequently treated with single agent Paclitaxel. The metastatic burden continued to worsen with increased involvement of the liver, lymph nodes and most recently the brain. At his most recent clinic visit, he continues to lose weight and still experiences dysphagia to solids though he is tolerating liquids. He will be starting radiation treatments to the brain and also palliative radiation to the GE junction to help alleviate his dysphagia.

DISCUSSION:

Prompt diagnosis of esophageal NET is critical as these tumors are known to be rapidly progressive and often have already metastasized by the time of diagnosis. Favorable prognostic factors include no lymphatic metastasis, tumor size of <1.0 cm and histology that reveals well-differentiated tumor cells. For unclear reasons, men are disproportionately more likely to develop NET of the esophagus. The most frequent clinical complaint is dysphagia followed by unintentional weight loss, melena and then typical carcinoid symptoms.