bluuebunny/crossref_metadata_2025_split huggingface dataset:
1706 Achalasia With Megaesophagus Presenting as Septic Shock
Authors: Suchartlikitwong Sakolwan , Shroff Roshan , Tikue Alay M. , Mingbunjerdsuk Thammasak , Laoveeravat Passisd , Attaya Mohamed , Islam Sameer , Islam Ebtesam 
Published: October 2019

INTRODUCTION:
Achalasia has wide range presentations including gastrointestinal and respiratory symptoms. Septic shock is less common.

CASE DESCRIPTION/METHODS:
We report a case of a 59-year-old Caucasian woman who presented with 1-month history of dyspnea and cough worsening for one week. She reported intermittent vomiting, dysphagia and unintentional weight loss for the past 2 months. Upon admission, she was hypoxemic and developed septic shock. Computer tomography showed multifocal pneumonia and severe distension of the esophagus filled with debris (Figure 1) measuring 9.5 × 6 cm at the largest diameter. She was intubated and given board spectrum antibiotics and then transferred to the medical intensive care unit. Upper endoscopy demonstrated dilation in the entire esophagus filled with food and scattered whitish esophageal plaques, consistent with esophageal candidiasis. Four weeks of oral fluconazole was initiated to treat esophageal candidiasis. Propionibacterium species grew in one out of two blood cultures but the respiratory culture was negative. She required vasopressors for two days and then successfully extubated two days later. She underwent a barium esophagography showing dilated esophagus with distal narrowing “bird beak’s” appearance (Figure 2). Esophageal manometry confirmed type I achalasia demonstrating apersistalsis of the entire esophagus and hypertensive lower esophageal sphincter (Figure 3). She recovered from aspiration pneumonia and weaned off oxygen. She was scheduled to come back in 2 weeks for perioral endoscopic myotomy evaluation and was discharged home with oral fluconazole.

DISCUSSION:
Achalasia is an esophageal disease caused by loss of relaxation in the distal esophagus and lower esophageal sphincter. Most common presentations are dysphagia (90%), heartburn (75%) and regurgitation or vomiting (45%). Respiratory symptoms including cough or chronic aspiration account for 20-40%. However, septic shock as initial presentation is not common. Our patient had chronic aspiration to the lungs and developed aspiration pneumonia progressing to septic shock. Patients presenting with respiratory symptoms are reported to have a more dilated esophagus with an average mean diameter of 4.3 ± 2 cm. Our case has an esophagus double the average size. Perioral endoscopic myotomy has gained universal acceptance to treat achalasia and shows safety and efficacy.
1709 A Rare Case of Muscular Dystrophy in an Adult Diagnosed by Suspicion on High Resolution Esophageal Manometry
Authors: Jarvis Mackenzie , Thompson Eric , Moshiree Baha 
Published: October 2019

INTRODUCTION:
Oropharyngeal dysphagia with severe upper esophageal sphincter (UES) dysfunction may be seen due to neuromuscular, neurodegenerative and structural diseases. However, the diagnosis of Myotonic Dystrophy based on interpretation of High Resolution Esophageal Manometry (HRM) is rare.

CASE DESCRIPTION/METHODS:
A 28-year-old Caucasian man presented with a history of dysphagia since childhood with choking and gasping during sleep. During meals he regurgitated frequently and drank large amounts of water to swallow food. He had dysphagia to both solids and liquids. Patient also noted coughs after most meals. He noted a weight loss of 25 pounds with a normal appetite. Patient wanted to eat but was worried about choking. No prior history of food allergies, asthma or food intolerances. No seasonal allergies. Past medical history notable for a normal upper endoscopy. On physical examination the patient was noted to have an elongated face with drooping of his eyelids bilaterally. He had thinning of his temporal area, jaw and anterior neck musculature and thenar wasting bilaterally. Weakness of both extremities was noted with normal reflexes. Review of prior testing was done. A Modified Barium Swallow study showed no evidence of aspiration or penetration and decreased hyolaryngeal excursion with impaired epiglottic inversion with decreased duration of the cricopharyngeal. Given the conflicting findings above, a HRM was performed in our motility lab. (See Figures 1 and 2). Solid swallows were unable to be performed due to the severe extent of the patient’s dysphagia and risk of aspiration. Based on our manometry results, we performed the following testing to rule out neuromuscular diseases prior to his referral to a neurologist. Several serologic testing was performed. (see Table 1) Based on testing, he had an elevated Aldolase and CPK with suspicion of a muscular disorder. For his absent contractility based on his HRM, he was started on Bethanechol, a cholinesterase inhibitor and muscarinic agonist at a dose of 25 mg prior to meals. Patient’s nutrition was supported with protein supplementation and a Gastrostomy-Jejunostomy tube was suggested due to significant oropharyngeal dysphagia. He was referred to the Myotonic Dystrophy Clinic.

DISCUSSION:
Patient’s presenting with oropharyngeal dysphagia who are found to have a hypotensive UES on HRM should spark concern for a myopathy or neurological etiology.
1715 Esophageal Stent Causing Osteomyelitis: An Emerging Complication
Authors: Stone Bryan , Odah Tarek , Shafa Shervin 
Published: October 2019

INTRODUCTION:
Esophageal stents are used to treat a wide variety of problems, most commonly malignant dysphagia as a form of palliation. Delayed complications include tumor ingrowth/overgrowth, stent migration, hemorrhage, and delayed perforation. We present a rare case of osteomyelitis secondary to a stent that was placed for the closure of a tracheoesophageal fistula.

CASE DESCRIPTION/METHODS:
A 55-year-old female presented with stage IV non-small-cell lung cancer with distant metastases. She was started on chemotherapy (gemcitabine and carboplatin) as well as immunotherapy (pembrolizumab). A bronchoscopy had revealed extensive cancer in the bronchial tree and detected a tracheoesophageal fistula, which required placement of an esophageal stent for closure. The stent had been repositioned twice for better location and patient comfort. Four months after the last stent, she presented with fevers, which was initially attributed to her malignancy. However, her symptoms eventually progressed to dysphagia, cough, chest and back pain. Serologic evaluation was notable for an elevated ESR at 112 and an elevated CRP of 105. Subsequent CT of the chest revealed erosive endplate changes at the T1-T2 vertebral level with soft tissue density adjacent to the proximal aspect of the stent. These findings were concerning for osteomyelitis in the setting of an esophageal stent erosion. The decision was made to reposition the stent endoscopically. EGD revealed that the proximal end of the stent was embedded into the esophageal wall without clear erosions or evidence of perforation. Attempt to reposition the esophageal stent was performed, but follow up CT revealed minimal change from endoscopic repositioning. No further endoscopic intervention was performed and she was treated for osteomyelitis with intravenous antibiotics.

DISCUSSION:
Few cases have reported osteomyelitis related to esophageal stent migration. Whether the stent-related osteomyelitis is potentiated by the effects of chemotherapy on the esophageal wall or related to pressure erosion caused by the stent is not entirely clear. With newer therapies for malignancies and increased life expectancies, new complications may emerge. The decision to perform endoscopic manipulation would largely depend on patients’ clinical status and a discussion of risks versus benefits in a case-based scenario. This case illustrates the importance of having a high index of clinical suspicion for osteomyelitis in a patient with previous esophageal intervention.
1718 Acute Esophageal Necrosis in Patient With Decompensated Cirrhosis
Authors: Tosounian Shannon L. , Miick Ronald , Walter James 
Published: October 2019

INTRODUCTION:
Acute Esophageal Necrosis (AEN) is a rare condition characterized by diffuse circumferential necrosis of esophagus. The majority of cases have no identified etiology, but risk factors that may lead to hypoperfusion (thromboembolic events, sepsis, trauma, vasculopathy) are typically present. We present the case of a woman with decompensated cirrhosis presenting with hematemesis and endoscopic findings of AEN.

CASE DESCRIPTION/METHODS:
A 66-year-old female with cirrhosis secondary to alcohol abuse complicated by ascites presented from home due to witnessed hematemesis. History was limited due to patient’s mental status. Upon initial examination her blood pressure was 128/53 mmHg, heart rate of 118 bpm, Sp02 of 84%. On examination she appeared lethargic and in mild distress with dried blood in her oropharynx. Her abdomen was soft but distended. Laboratory results included: WBC 24.0 103/mcL, Hemoglobin 4.9 g/dL, Platelets 234 103/mcL, INR 2.7, and lactic acid 4.18 mmol/L. A chest X-ray showed bilateral pleural effusions. She was started on pantoprazole and octreotide drips, ceftriaxone, and two units of packed red blood cells. She was taken emergently for upper endoscopy due to concerns for variceal hemorrhage. The entire lumen of the esophagus extending from the incisors to top of gastric folds was black and necrotic-appearing circumferentially (Figures 1 and 2). Biopsies were obtained (Figure 3). Type 2 gastroesophageal varices, medium in diameter with no bleeding were found in the gastric fundus. She was placed on broad spectrum antibiotics and admitted to medical ICU. The following day she had increased O2 requirements secondary to acute respiratory distress syndrome and required intubation. She became hemodynamically unstable requiring vasopressors, then developed an acute kidney injury requiring continuous renal replacement therapy. Due to declining clinical status, she was made comfort care and ultimately expired on Day 4 of admission. Esophageal pathology demonstrated necrotic esophageal mucosa with acute and chronic inflammation. The etiology of her necrotic esophagus remains unclear.

DISCUSSION:
AEN is a rare diagnosis associated with low perfusion state with an estimated 32% mortality rate. It is associated with liver dysfunction in approximately 15% of cases, as in our patient. Early recognition and supportive care is crucial for survival, and further studies are needed to better understand etiology and management of AEN.
1722 Dysphagia Lusoria Associated With Imperforate Anus
Authors: Andersen Karl , Hoff Ryan T. , Silas Dean N. 
Published: October 2019

INTRODUCTION:
Dysphagia lusoria is a rare intrathoracic vascular abnormality that results in the compression of the esophagus, causing dysphagia. The most common cause of dysphagia lusoria is an aberrant right subclavian artery of the aortic arch, though nearly all major arteries within the thorax have been reported to cause cause this condition.

CASE DESCRIPTION/METHODS:
68-year-old male with a history of developmental delay and imperforate anus s/p colostomy and colostomy revision presented with decreased appetite, decreased ostomy output, and regurgitation of food and water. Past medical history was notable for GERD and prior small bowel obstructions. Abdominal obstructive x-ray series showed multiple nondilated gas-filled loops of small bowel. Chest x-ray showed tortuosity of the thoracic aorta. Fluoroscopic swallow evaluation showed no evidence of aspiration, and barium esophagram showed the calcified aortic arch trapping the proximal esophagus anteriorly and posteriorly, resulting in a narrowed channel with a normal appearing distal esophagus (Figure 1). CTA chest confirmed compression of the esophagus by the anomalous aortic arch (Figure 2). Multiple treatment options were considered. However, due to the patient's poor performance status and ability to tolerate small frequent sips of liquids, no surgery was pursued. The patient was discharged home in satisfactory condition.

DISCUSSION:
The majority of patients with dysphagia lusoria are asymptomatic and the discovery of the anomaly is often incidental. This patient had a tortuous thoracic aorta, which was known from previous imaging studies, but remained without dysphagia until this admission. The median onset of symptoms is 48-50 years; the late-onset of this condition may be due to the increased rigidity of the esophagus or calcification and atherosclerosis of the major vessels, decreasing their compliance. The presenting symptoms of dysphagia lusoria is usually progressive dysphagia, usually for solids, accompanied by regurgitation of unchewed foods, chest pain, or cough. The diagnosis is usually achieved with an initial barium esophagram, followed by CT or MRI scan. Endoscopic ultrasound with doppler is also used for diagnosis. Surgery is the preferred treatment option for symptomatic patients that can tolerate it. To our knowledge, this is the first reported case of dysphagia lusoria associated with development delay and isolated imperforate anus.
1733 Wonder Neutralization: Esophageal Stents in Caustic Injury
Authors: Leon-Jarrin Daniela , Kumar Manoj , Fahmawi Yazan , Mizrahi Meir 
Published: October 2019

INTRODUCTION:
Introduction Common causes for benign esophageal strictures (ES) are post -surgery, caustic ingestions and radiation therapy. Caustic strictures of the gastrointestinal tract can be a challenge to treat even after multiple endoscopic treatments due to its inherent relapsing nature. We report here our experience with self-expandable metallic stents (SEMS) in a patient with corrosive stricture of the esophagus.

CASE DESCRIPTION/METHODS:
Case A 25-year-old female with 2-year history of refractory ES due to caustic injury presented for endoscopic treatment. Patient had multiple endoscopic dilation attempts in past. Pediatric gastroscope showed esophageal stricture starting at 20 cm from the incisors however it could not traverse through the stricture. Under fluoroscopic guidance a 0.035 in x 260 cm guidewire was placed in the stomach. The proximal end of stricture was occluded by biliary 8.5 mm stone extraction balloon and contrast was injected through balloon catheter under fluoroscopy guidance showing 12 cm long significantly narrowed ES. A 18 mm × 103 mm fully covered esophageal metal stent (FCEMS) was placed under fluoroscopic guidance keeping the distal end of the stent above the gastroesophageal junction. Four days later, follow up EGD showed previously placed FCEMS was patent and widely open. The distal end of the stent was at 27cm and GEJ was at 35cm from entry point. Under fluoroscopic imaging and wire guidance another FCEMS (18 mm × 153 mm) was placed successfully to treat the left-over portion of the stricture below the previously placed stent. The second stent was deployed with in the first stent with its distal end at GEJ. Post stent placement, endoscopic and fluoroscopic imaging’s were satisfactory. At 2 weeks follow up, patient revealed significant improvement in her symptoms and ability to tolerate soft diet.

DISCUSSION:
Discussion ES secondary to caustic injury tend to require multiple dilation procedures, multiple hospitalizations with associated risks and cost, affecting patient’s quality of life. There is a need for novel therapeutic strategies that will provide a long-term solution. Our case highlights the growing interest and utility of multiple FCEMS in the treatment of severe and long ES. The deployment of multiple FCEMSs in our patient with long history of ES related dysphagia dramatically improved her symptoms and patency of esophagus.
1734 An “Off the Wall” Case of Sloughing Esophagitis
Authors: Johnson Alyson M. , Koutlas Nicholas J. , Guy Cynthia D. , Wilson Joanne A. 
Published: October 2019

INTRODUCTION:
Esophagitis dissecans superficialis (EDS), or sloughing esophagitis, is a rare, benign disease characterized by large, longitudinal sheets of sloughing mucosa visualized on upper endoscopy. Symptoms typically vary and include dysphagia, odynophagia, abdominal pain, and dyspepsia. EDS has been associated with medications, chemical and thermal irritants, autoimmune bullous dermatoses, and celiac disease. However, its pathogenesis is still poorly understood. We present a case of EDS in a patient with multiple comorbid autoimmune disorders including type 1 diabetes mellitus, Hashimoto’s thyroiditis, and celiac disease.

CASE DESCRIPTION/METHODS:
A 22 year old female was admitted with diabetic ketoacidosis (DKA), dysphagia, abdominal discomfort and weight loss. Her medical history included poorly controlled type 1 diabetes mellitus, celiac disease, and Hashimoto’s thyroiditis. Her upper gastrointestinal symptoms led to anorexia and unintentional weight loss. After resolution of DKA, Gastroenterology was consulted for evaluation of her symptoms. Upper endoscopy was performed which revealed mild esophagitis, mild gastritis, and scalloping in the duodenum. Esophageal biopsies demonstrated fragments of squamous mucosa with features consistent with "sloughing esophagitis". Her appetite eventually improved with initiation of proton pump inhibitor therapy and management of her poorly controlled diabetes. On outpatient follow up, her upper GI symptoms continued to improve and her weight stabilized.

DISCUSSION:
EDS is a rare endoscopic finding of white plaques in vertical columns that represent sloughing of large fragments of squamous tissue. Histologically, the most common features of EDS are parakeratosis, pauci-immune intraepithelial sloughing, and intraepithelial splitting. The pathogenesis of EDS is not fully understood though has been associated with autoimmune skin disorders and celiac disease. There are no specific treatments for EDS as it is usually self-limited. We present a case of sloughing esophagitis in a patient with multiple pre-existing autoimmune diseases including type 1 diabetes mellitus, Hashimoto’s thyroiditis, and celiac disease. This case is unique in that she has a polyglandular like autoimmune disorder. This suggests that autoimmunity may play a role in the pathogenesis of EDS. Interestingly, she lacked all of the dermatologic manifestations that are most frequently associated with EDS.
1736 Mega Esophageal Diverticulum Complicated by Post-Operative Esophageal Stricture
Authors: Then Eric O. , Aloreidi Khalil , Gayam Vijay , Paniagua-Morales Julian , Culliford Andrea , Gaduputi Vinaya 
Published: October 2019

INTRODUCTION:
Esophageal diverticulae are rare outpouchings of the esophagus that occur secondary to underlying motility disorders. On average they measure approximately 7 cm. Here we present a case of mega diverticulum measuring 8.5 cm, complicated by post-operative esophageal stricture.

CASE DESCRIPTION/METHODS:
Patient is a 58-year old female with a medical history of gastroesophageal reflux disease who presented to our gastroenterology clinic complaining of progressively worsening reflux and weight loss. Given these symptoms, an esophagogastroduodenoscopy (EGD) was scheduled. This was done and showed a large esophageal diverticulum with luminal distortion and retained food in the lower third of the esophagus. Subsequently a barium swallow was ordered to better evaluate the esophagus. This was done and showed an 8.5 × 6.0 cm diverticulum from the right side of the distal esophagus with slight irregularity of the mucosa superior to the diverticulum (Figure 1). The patient was then referred the surgical clinic for possible intervention. After discussing the risks and benefits of treatment the patient agreed to undergo surgical repair consisting of a diverticulectomy. This was done successfully with no postoperative complications. Five days after the procedure a repeat barium swallow was done and showed resolution of the diverticulum with no leakage of barium, but did reveal lower esophageal narrowing with a dilated thoracic esophagus (Figure 2). Following these findings a repeat endoscopy was done which showed a lower esophageal stricture that was treated with balloon dilation (Figure 3). After the procedure the patient was able to tolerate oral intake and was safely discharge home.

DISCUSSION:
Management of esophageal diverticula depends on the severity of symptoms. In asymptomatic patients a conservative approach may be employed. In symptomatic patients who present with dysphagia, regurgitation, vomiting or chest pain, surgery is the mainstay of treatment. Surgical management consists of diverticulectomy with long myotomy and subsequent fundoplication. Postoperative complications include pulmonary edema, pneumonia, and leakage of barium swallow into the thoracic cavity. Rarely will esophageal narrowing resulting in persistent dysphagia occur. In these cases an endoscopic treatment with balloon dilation should be pursued. Our case highlights a rare presentation of a mega esophageal diverticulum complicated by a post-operative esophageal stricture that was successfully treated with balloon dilation.
1744 Not an Ordinary Ulcer: A Case of an Aorto-Esophageal Fistula
Authors: Poola Shiva , Jampala Nannaya , Naseer Maliha , Ali Eslam 
Published: October 2019

INTRODUCTION:
Hematemesis is a common manifestation of variceal and non-variceal upper gastrointestinal (GI) bleed with a broad differential. Aortoesophageal fistula (AEF) is a life-threatening and catastrophic cause of gastrointestinal bleeding, which is caused by the abnormal communication between the aorta and the esophagus. We are presenting a rare case aorto-esophageal fistula in a young patient who was found to have a benign appearing esophageal ulcer on endocscopy that subsequently was found to be an (AEF).

CASE DESCRIPTION/METHODS:
A 33-year-old Caucasian male presented to the hospital for fatigue, non-blood emesis and progressively worsening leg swelling. His history is significant for hypertension, seizure disorder, chronic kidney disease (due to pauci-immune glomerulonephritis), heart failure and a descending aortic transection due to an accident 12 years ago which was repair endovascularly. The patient reported usage of NSAIDs prior to admission for pain control but otherwise denied alcohol or other substance abuse. The patient was admitted for his progressive renal failure and was started on hemodialysis complicated with a DVT for which was started on warfarin. During his hospitalization, the patient had an episode of hematemesis with hemodynamic instability which prompted cessation of anticoagulation. An EGD revealed a 2-3 cm non-bleeding ulcer in the proximal esophagus. The patient had stabilized hematocrit and hemodynamics and was restarted on anticoagulation. A week later, the patient had another episode of massive hematemesis with hypotension. A chest x-ray showed widening of the aortic knob, suggested change in location of the thoracic stent. CT angiogram showed aortoesophageal fistula with a leaking pseudoaneurysm at the proximal aspect of the thoracic aortic endovascular stent. Surgery proceeded with endovascular aortic repair of thoracic pseudoaneurysm. Patient had multi-disciplinary management strategy for staged repair.

DISCUSSION:
AEF are classified as primary i.e. spontaneous erosion of the aortic wall into the esophagus or secondary caused by either esophageal malignancy, surgeries or foreign body. Exact incidence of the AEF is unknown. Early recognition is important to decrease mortality. Chiari et al described the triad of midthoracic pain, herald hemorrhage, and fatal hematemesis as usual presenting features. Endoscopy plays a significant role in the evaluation however it has a poor rate of detection for AEFs prompting the need of CT imaging with high clinical suspicion.