bluuebunny/crossref_metadata_2025_split huggingface dataset:
1728 A Spastic Spiral: Paraneoplastic Pseudoachalasia in an Elderly Male With Multiple Malignancies
Authors: Dalsania Raj , Cassani Lisa S. , Raja Shreya M. 
Published: October 2019

INTRODUCTION:
Pseudoachalasia is an uncommon condition that is difficult to distinguish from primary achalasia. Pseudoachalasia is usually caused by malignancy involving the gastroesophageal junction (GEJ); however, a paraneoplastic etiology has been reported in rare cases. Our aim is to describe the diagnosis and management of a case of paraneoplastic pseudoachalasia resulting from acetylcholine receptor (AchR) ganglionic (a3) antibodies.

CASE DESCRIPTION/METHODS:
A 72-year-old male with a history of lung adenocarcinoma, bladder transitional cell carcinoma and prostate adenocarcinoma presented with acute chest pain, regurgitation, and dysphagia. Positron emission tomography scan showed increased distal esophageal uptake prompting a referral for endoscopy. Endoscopy showed a tortuous, spiraling esophageal body, distal esophageal ulcer, and narrowed GEJ (Figure 1). Ulcer biopsies returned benign, and endoscopic ultrasound (EUS) was performed. EUS of the GEJ showed diffuse thickening of the muscularis mucosa with no discrete mass. Motility evaluation was performed. Barium esophagram demonstrated a corkscrew esophagus and persistently narrowed GEJ (Figure 2). Esophageal manometry was attempted, but the catheter was unable to traverse the lower esophageal sphincter. Tracings of the esophageal body revealed spastic contractions with occasional pan-esophageal pressurization suggestive of possible mixed Type II/III achalasia (Figure 3). A paraneoplastic serum autoantibody panel was obtained and returned positive with AChR a3 antibody level of 220 pmol/L (normal < 53). Evaluation for recurrent malignancy was negative. Trial of a cholinesterase inhibitor and calcium channel blocker were attempted without improvement. Intravenous immunoglobulin therapy was declined by the patient. Endoscopic botulinum toxin injection to the esophageal body and LES was performed and resulted in sustained symptom relief.

DISCUSSION:
Paraneoplastic pseudoachalasia is rare, and requires a high index of suspicion for diagnosis. It may be the initial clinical sign of malignancy, or as in our case, a finding concerning for recurrent malignancy prompting re-evaluation. Prior reports suggest AChR antibodies are associated with adenocarcinomas. In patients with known malignancy who present with achalasia-type symptoms, a paraneoplastic autoantibody panel can assist with diagnosis. Treatment of the malignancy is the primary therapy, while medications and endoscopic interventions remain anecdotal.
1729 An Unusual Case of Ventilatory Compromise and Mega-Esophagus
Authors: Pinto Jonathan , Asif Ammar , Thomas Kafi , Culpepper-Morgan Joan 
Published: October 2019

INTRODUCTION:
Mega-esophagus, usually due to achalasia or pseudoachalasia, can cause bronchial compression when filled with phytobezoars. We present a case of mega-esophagus not due to achalasia but with profound ventilatory consequences.

CASE DESCRIPTION/METHODS:
A 67 year old alcoholic man presented with 24 hours of increasing dyspnea at rest. He denied fevers, chills, cough, chest pain, heartburn, dysphagia, abdominal pain or weight loss. On physical exam he was somnolent but arousable. He was able to speak in full sentences. Lungs revealed right sided rhonchi. He was afebrile, BP 211/100, pulse 68, respiratory rate 18, and O2 saturation 80-85% on room air which improved to 94% after 4L nasal O2. Urine toxicology was positive for opiates and cocaine. Chest CT showed a 6.8 cm esophagus filled with debris. It was herniated into the right upper thorax and terminated in a narrow taper at the aortic arch. The trachea deviated rightward and narrowed to 4 mm. The proximal left main-stem bronchus was narrowed to 2 mm. The right upper lobe showed scarring, cystic bronchiectasis, and pleural thickening. On day 2 the patient developed severe dyspnea and orthopnea. O2 saturation varied with position. After intubation and transfer to the ICU for treatment of acute ventilator failure, GI was consulted to decompress the mega-esophagus presumed due to end stage achalasia. EGD initially revealed a dilated esophagus with a phytobezoar. Upon partial clearing two lumens were noted: one containing more food and one which lead to a normal distal esophagus, stomach, and duodenum. The first opening was a large diverticulum located about 20 cm from the incisors and was filled with recently ingested food. Transfer to a tertiary care center was recommended to manage possible total airway collapse. There, repeat chest CT showed a distended esophagus to the level of the aortic arch with no compression of the airways. Esophagram was not consistent with achalasia but demonstrated a large bezoar filled diverticulum. Repeat EGD was done to complete bezoar removal. Patient’s respiratory status normalized. Elective esophagectomy was planned, but the patient was lost to follow up.

DISCUSSION:
Our patient likely had a traction diverticulum as he gave no antecedent history of heartburn or dysphagia and the food in the bezoar was not fermented. In addition, his distal esophagus appeared normal on barium swallow. This is may be the first reported case of airway compression due to mega-esophagus due to a traction diverticulum.
1730 Esophageal Actinomycosis in a Patient With Long-Standing Esophageal Stent
Authors: Kumar Vikas , Lam Steven , Deivert Duane , Obuch Joshua , Baidoo Cletus , Khara Harshit S. , Khan Yakub 
Published: October 2019

INTRODUCTION:
Actinomyces is a commensal in aero-digestive tracts which rarely causes infection. The commonest site is cervicofacial region. Risk factors are low immunity and pre-existing tissue damage. Esophageal involvement is rare. We report a case of esophageal actinomycosis in an immunocompetent patient.

CASE DESCRIPTION/METHODS:
A 71-year-old male presented with recurrent dysphagia. The patient had multiple prior EGDs for management of refractory peptic stricture due to gastrin secreting pancreatic neuroendocrine tumor. He undewent multiple prior procedures with placement of fully-covered self-expanding metal stents (SEMS) for therapy of refractory stricture, as surgical esophagectomy was not possible due to co-morbidities and patient preference. On previous EGD, one of the stents could not be removed due to tissue ingrowth at the distal end, and another SEMS was placed overlapping the imbedded stent and extending to the gastric cardia. The patient was lost to follow up for 7 months until presenting with recurrence of dysphagia. EGD now showed proximal migration of the distal stent with severe stenosis, inflammation, and nodularity distal to the esophageal stents. The distal stent was imbedded in the abnormal mucosa and could not be removed. Following balloon dilation another SEMS was placed in the distal esophagus. Pathology revealed ulcerated acute esophagitis with actinomycetes colonies. The patient was placed on a long course of IV penicillin-G. At subsequent EGD a month later, the tissue ingrowth improved allowing removal of the esophageal stents. Repeat biopsies did not reveal actinomyces.

DISCUSSION:
Actinomyces, a gram positive anaerobic rod, is unable to penetrate healthy tissue. It requires mucosal compromise to invade tissue, leading to abscess and sinus formation across tissue planes. Our patient had multiple risk factors - mucosal damage, endoscopic dilations and stenting. Esophageal stent in situ for longer than 3 months increases the risk of embedment. Discovery of esophageal ulceration or tissue ingrowth in this setting should raise suspicion for possible actinomycosis (among other diagnoses) and biopsies should be obtained. Gram stain and histology would reveal necrosis with yellow sulfur granules and gram-positive filamentous pathogen. Early diagnosis is necessary to initiate treatment in a timely manner to prevent the potentially debilitating sequelae of chronic infection. Treatment includes penicillin G or amoxicillin for 3-12 months.
1732 Atypical Presentation of Herpes Simplex Virus Esophagitis in an Immunocompetent Patient
Authors: Patel Pooja R. , Wehbeh Antonios , Emerson Robert E. , Fatima Hala , Kim Hak Nam 
Published: October 2019

INTRODUCTION:
Herpes Simplex Virus esophagitis (HSVE) is a well-documented opportunistic infection affecting immunocompromised patients. It can rarely manifest in the immunocompetent patient, with the most recent review in 2010 demonstrating 56 documented cases. Such patients commonly present with acute odynophagia, fever, and retrosternal pain. Here, we report a case of HSVE affecting an immunocompetent patient with a more subacute presentation.

CASE DESCRIPTION/METHODS:
A 37-year-old Nigerian female presented with generalized weakness, weight loss, and new onset dysphagia of two months duration. The dysphagia had progressed from solids to liquids with intermittent odynophagia. She denied fevers, chills, hematemesis, and melena. On exam, she was afebrile and exhibited no oropharyngeal lesions. Her abdomen was nontender to palpation and she demonstrated no hepatosplenomegaly or palpable masses. Labs showed severe iron deficiency anemia requiring transfusion, a normal white cell count, and a negative HIV screening test. An upper endoscopy (EGD) showed multiple small non-bleeding erosions throughout the middle and distal esophagus, as seen in Figures 1A and 1B respectively. Esophageal biopsy demonstrated squamous epithelium with viral inclusions and multinucleated cells. As demonstrated in Figure 2, immunohistochemical stain was focally positive for HSV supporting the diagnosis of herpes esophagitis. She was subsequently started on 7-day treatment of oral acyclovir 400 mg three times a day and had resolving symptoms one week later at her primary care visit.

DISCUSSION:
This case illustrates the importance of considering a diagnosis of HSVE in immunocompetent individuals, who can present with a subacute course of worsening dysphagia without systemic symptoms. Moreover, as HSVE endoscopic lesions can vary in appearance and location, gastroenterologists should have a low threshold for biopsying atypical lesions in the immunocompetent. In a recent study comparing endoscopic features of HSVE in immunocompromised and immunocompetent patients, endoscopists were more likely to mention the possibility of viral esophagitis on the endoscopy report in patients that were immunocompromised. Although HSVE in the immunocompetent is typically a self -limited disease, treatment with oral acyclovir can result in resolution of symptoms within 1-2 weeks. The etiology of HSVE in the immunocompetent may represent a primary infection of traumatized esophageal mucosa or reactivation after a previous infection.
1735 A Rare Case of Coexisting Achalasia and Pseudoachalasia
Authors: Dayah Rawan , Clark August , Reep Gabriel , Okereke Ikenna , Qiu Suimin , Nahmood Karen 
Published: October 2019

INTRODUCTION:
Achalasia is an esophageal motility disorder in which the lower esophageal sphincter fails to relax and there is a loss of esophageal peristalsis. Pseudoachalasia closely mimics achalasia. We present a rare case of a patient who was initially diagnosed with pseudoachalasia but was later found to have achalasia after symptoms persisted following the resection of an esophageal leiomyoma.

CASE DESCRIPTION/METHODS:
A 39 year-old male was evaluated for weight loss and progressively worsening dysphagia to solids and eventually liquids with a 100 pound weight loss over 3 years. Esophageal manometry was consistent with achalasia with esophageal compression, subtype II (Figure 1). He underwent laparoscopic exploration and planned Heller myotomy and Dor fundoplication. During surgery, a very large submucosal mass was found in the distal esophagus extending to the gastroesophageal junction. The mass was enucleated and a Dor fundoplication was performed. Pathologic analysis showed the mass to be a leiomyoma measuring 6.7 centimeters in maximum diameter (Figure 2). Postoperatively, his symptoms of dysphagia persisted andrepeat esophageal manometry remained consistent with achalasia. He underwent Heller myotomy and redo Dor fundoplication 5 months after the initial surgery. His symptoms completely resolved after the second surgery and he has remained asymptomatic on follow-up.

DISCUSSION:
Patients with achalasia typically present with progressive dysphagia, initially to solids and then to liquids, due to the degeneration of ganglion cells within the myenteric plexus in the distal esophagus resulting in the loss of peristalsis. Pseudoachalasia closely mimics the presentation of idiopathic achalasia and is the diagnosis in approximately 4% of patients with achalasia-like symptoms. Intraluminal evaluation with endoscopic ultrasound may be a valuable tool to evaluate muscular wall thickness in motility disorders and could result in earlier diagnosis of pseudoachalasia. Intraoperative esophageal pressure monitoring is not used frequently but may have also been beneficial in this case. This case is unique since the patient was discovered to have an esophageal leiomyoma after he was diagnosed with primary achalasia. There are no documented cases of a patient with both primary achalasia and pseudoachalasia based on a literature review.
1737 Successful Endoscopic Repair of Boerhaave’s Syndrome: Changing the Narrative
Authors: Then Eric O. , Lopez Michell , Ofosu Andrew , Gayam Vijay , Are Vijay S. , Gaduputi Vinaya 
Published: October 2019

INTRODUCTION:
Boerhaave’s syndrome (BS) is a rare, life-threatening entity that is defined as spontaneous esophageal rupture occurring after forceful vomiting. Classically treatment of BS has consisted of a surgical approach. Here we present a rare case of BS that was successfully treated through endoscopic measures.

CASE DESCRIPTION/METHODS:
Patient is a 65 year old male with a medical history of chronic obstructive pulmonary disease who presented the emergency department with chest pain, shortness of breath and productive cough after an episode of retching 2 days prior. Notable workup in the emergency department included a chest x-ray which showed left lower lobe pneumonia, pneumothorax, and a left pleural effusion. A chest tube was then placed and a computed tomography angiography of the chest was also done. This showed pneumomediastinum with air around the mid to distal esophagus. A barium esophagram was subsequently done which showed contrast leak at the distal esophagus. An upper endoscopy was then performed and showed a large linear perforation near the gastroesophageal junction. This was closed with 2 over the scope clips in addition to placement of an overlapping partially covered stent. In order to ensure complete closure an esophagram was done with radioisotope. This showed complete closure of the perforation, with no evidence of leakage of the radiotracer. After the procedure the patient was able to tolerate oral feedings with no clinical sequelae. During his hospital stay the patient completed treatment with antibiotics, his chest tube was successfully removed and he was subsequently discharged home.

DISCUSSION:
Boerhaave’s syndrome is a lethal pathology that carries a high mortality rate. It mostly presents on the left posterolateral wall of the esophagus and occurs after forceful vomiting. Symptoms are non-specific and include vomiting, chest pain, dyspnea, and subcutaneous emphysema. Diagnosis of BS can be achieved through endoscopy, which allows direct visualization of the esophageal tear. Imaging studies such as barium swallow and CT scan may also be useful. These will show extravasation of contrast and pneumomediastinum respectively. Surgical repair is currently the treatment of choice in BS. Present day, endoscopic measures are challenging this consensus. More recently cases of BS have been successfully treated with the use of endoscopic suturing with esophageal stent placement. Our case was successfully treated by using 2 over the scope (OTS) clips and placement of an esophageal stent.
1738 Refractory Candida Esophagitis in an Immunocompetent Patient: A Rare Presentation
Authors: Then Eric O. , Hernandez Kimberly , Ofosu Andrew , Lopez Michell , Culliford Andrea , Gaduputi Vinaya 
Published: October 2019

INTRODUCTION:
Candida esophagitis is an invasive fungal infection of the esophagus classically caused by Candida albicans. It is usually considered an opportunistic fungal infection affecting those who are immunocompromised. Recently however, there has been an increased incidence of the infection in immunocompetent patients. Here present a case of refractory candida esophagitis in an immunocompetent female.

CASE DESCRIPTION/METHODS:
The patient is an 83-year-old immunocompetent female with a past medical history of congestive heart failure, hypertension, hyperlipidemia, diabetes mellitus, venous insufficiency, obstructive sleep apnea, fibromyalgia, and degenerative joint who presented to the clinic with a 5-month history of dysphagia and odynophagia. She was recently diagnosed with candida esophagitis through esophagogastroduodenoscopy (EGD) and biopsy and was treated with fluconazole with temporary resolution of symptoms. We performed a repeat EGD which showed diffuse whitish mucosal patches consistent with severe candida esophagitis of the entire esophagus (Figure 1). Biopsies stained with GMS special stain confirmed the diagnosis. Due to the patient’s previous resistance to fluconazole, the patient was referred to an infectious disease specialist who placed the patient on a Nystatin swish and swallow for 14 days with a subsequent course of fluconazole. One-month post-treatment, a follow-up EGD was performed and showed significant improvement with few scattered specks of whitish mucosal exudates in the entire esophagus consistent with mild candidiasis (Figure 2). One month later at follow-up, the patient reported complete resolution of symptoms.

DISCUSSION:
Candida esophagitis was thought to be a disease of the immunocompromised (i.e. AIDS patients) but, has shown to be on the rise in immunocompetent patients. Several studies showed that diabetes, old age, corticosteroid use, and antibiotic therapy are all major risk factors for development of the infection in this population. It is usually an easily treatable condition through the use of fluconazole, an antifungal agent that is usually seen with great success. However, there have been reports of resistance to the drug and a possible explanation of this is its widespread use leading to growth of fluconazole-resistant strains such as non-albicans candida strains. Species identification and fluconazole susceptibility testing may aid in adjustment of treatment protocols for those with recurrence of candida esophagitis.
1740 Syphilitic Lichenoid Esophagitis: A Rare Finding
Authors: Khurana Shruti , Siddiqui Shaheer , Larson Scott 
Published: October 2019

INTRODUCTION:
Extra-genital manifestations involving gastro-intestinal tract is extremely rare. We describe a case of biopsy-proven esophageal syphilis causing extremely rare pathological diagnosis of lichenoid esophagitis.

CASE DESCRIPTION/METHODS:
A 50 year-old woman with HIV-AIDS, HCV cirrhosis and recent anterior syphilitic uveitis presented with 4 days of melena. Esophagoduodenoscopy showed severe inflammation with LA grade D esophagitis. Mid esophagus had severe ulceration with crater formation and a stricture was noted at 35 cm (Figure 1). Histopathology of the biopsies showed marked intraepithelial lymphocytes, neutrophils, dyskeratotic keratinocytes (Civatte bodies) and dense band-like lymphoplasmocytic infiltrates in the lamina propria, consistent with lichenoid injury pattern (Figure 2), and visualization of spirochetes in the mucosa (Figure 3). No fungal elements or viral inclusion bodies were noted. Immunochemical staining was negative for CMV and HSV I/II. She was treated with a 14-day course of Penicillin G and reported complete resolution of symptoms on follow-up visit.

DISCUSSION:

Treponema pallidum rapidly spreads via the blood stream and lymphatics after the initial inoculation and can disseminate multiple foci to visceral organs including the gastrointestinal tract. Most common sites reported are oral cavity, stomach, rectum and rarely esophagus (1). T. pallidum incites an immune response with proliferative endarteritis affecting small vessels surrounded by T-lymphocytes, plasma cells and macrophages. This leads to extensive mucosal injury seen as diffuse esophagitis along with focal ulceration, which is prone to bleeding (2). The lichenoid pattern of injury with band-like infiltrate of lymphocytes in lamina propria and scattered degenerated keratinocytes (Civatte bodies) is atypical of syphilitic injury. This may cause fibrosis of the esophageal mucosa leading to stenosis and stricture (3). Salaria et. al. found that patients with lichenoid esophagitis were more likely to have concurrent HIV or viral hepatitis and were taking >3 medications (4).
1742 A Rare Case of Colorectal Cancer of the Neoesophagus
Authors: Shah Maulik , Truscello David , DeRosa Justin , Al-Annouf Nabil , Shmuts Robert 
Published: October 2019

INTRODUCTION:
In the case that follows, we present a case and literature review of oligometastatic intestinal adenocarcinoma originating in a colonic interposition repair of esophageal atresia. Uniquely, the pathology of the tumor was consistent with colonic mucosa which only a few such instances have been reported in literature.

CASE DESCRIPTION/METHODS:
A 58-year-old female with a past medical history of tetralogy of Fallot (repaired at age 14), esophageal atresia status post esophageal colonic interposition, esophageal and pyloric stenosis, neo-esophageal polyp (found 2 years prior with biopsy showing reactive epithelial atypia), and atrial fibrillation on Coumadin presented to the hospital complaining of fatigue and dyspnea of one week duration. Her family history was significant for colon cancer in her father. Initial lab workup revealed a hemoglobin of 5. She was transfused 3 units of packed red blood cells. Due to severe symptomatic anemia, an upper endoscopy and colonoscopy were performed revealing an ulcerated mass, approximately 50% in circumference in the lower third of the esophagus, as well as moderate pyloric stenosis that prevented passage of the endoscope. The esophageal masswas not amenable to endoscopic excision due to undulations of the cardiac beat, however biopsy demonstrated invasive, moderately differentiated, intestinal-type adenocarcinoma (Figure 1). Given these findings a PET/CT scan was obtained which revealed a 2cm mass in the distal aspect of the interposed colonic segment and a 1.7 × 1.2cm hypodense mass in the medial segment of the left hepatic lobe, consistent with metastasis. She was referred to Cardiothoracic Surgery for tumor resection and Oncology for neoadjuvent chemotherapy.

DISCUSSION:
Oligiometastatic colorectal cancer or the esophagus is an extremely rare medical condition. Our patient had an initial polyp removed in 2016, that was read as reactive epithelial atypia. More diligent surveillance was likely warranted for her case. This was shown by Lindahl et al in long-term endoscopic follow-ups in patients with this condition. Of the 12 patients observed in the study, a variety of colonic pathology was foundranging from dysplasia, colonic polyp, and aneuploid cells. Given these findings, albeit rare, with one case report citing less than 15 instances, special considerations should be made for close surveillance of these patients. Although no specific treatment guidelines exist for this anomaly, there has been documented success with a curative resection.