bluuebunny/crossref_metadata_2025_split huggingface dataset:
1754 Plummer-Vinson Syndrome: A Time for a Redefinition and New Perspective
Authors: Badhiwala Vismay , Herlihy John D. , McCallum Richard , Hernandez Cecilio M. 
Published: October 2019

INTRODUCTION:
Plummer-Vinson syndrome (PVS), or Kelly-Patterson syndrome, is a rare condition defined by the classic triad of dysphagia, iron-deficiency anemia, and esophageal webs, and dominated by Caucasian females. The pathophysiology remains unknown although iron deficiency is one theory as well as part of an autoimmune spectrum. We present two patients seen recently meeting criteria for PVS in whom new observations were made.

CASE DESCRIPTION/METHODS:
A 46-year-old Hispanic female with history of hypothyroidism presented with a Hgb of 3 and a ferritin of 16. She reported 2 weeks of fatigue, dyspnea, and constipation without overt bleeding, preceded by several months of dysphagia to solids. She was severely hypothyroid and placed on IV levothyroxine. Barium swallow suggested an esophageal web in the upper esophagus. At esophagogastroduodenoscopy (EGD) both adult and neonatal scopes could not be passed through a tight stenosis at the cricopharyngeus (Figure 1). This was dilated with Savary dilators to 8 mm. She was discharged home on iron supplements and follow up EGD with dilations scheduled. A 55-year-old Hispanic female with history of lupus, a positive ANA, Raynaud's, prior partial thyroidectomy, and chronic anemia was referred to GI clinic for evaluation of chronic dysphagia which had required multiple dilations at an outside facility over the past 18 months without improvement. She had a hgb of 6.7 with iron deficiency without any overt GI bleeding. EGD was performed and showed two benign appearing areas of intrinsic stenosis 15 and 20 cm from the incisors that were smooth and not ulcerated and could not be passed with the adult or pediatric endoscopes (Figure 2). Savary dilators were used to dilate to 11 mm diameter. Patient was placed on iron replacement and a fo llow up EGD was scheduled.

DISCUSSION:
Why PVS is only in females with accompanying iron deficiency anemia and a personal/family history of autoimmune phenomena and now is decreasing in recognition are not clear. The resolution of dysphagia with iron supplementation, could also be explained by the accompanying esophageal dilations restoring oral intake and hence improving iron absorption. Our cases highlight that PVS: 1) is not a true “esophageal web” but rather a tight fibrotic stricture at the level of the upper esophageal sphincter requiring aggressive dilation approach 2) occurs also in Hispanic females presenting with iron deficiency, dysphagia, and an autoimmune picture.
1761 Heterotopic Pancreatic, Sebaceous, and Gastric Tissue: Report of Case and Review of the Literature
Authors: Cheung Maggie , Elfanagely Yousef , Skole Kevin 
Published: October 2019

INTRODUCTION:
Heterotopic mucosa refers to the presence of normal tissue at a foreign site, and is thought to be either congenital from an error during organogenesis or metaplastic from an error during differentiation of pluripotent cells. We present a rare case of pancreatic and sebaceous gland heterotopia in the esophagus and gastric mucosal heterotopia in the rectum. To our knowledge, this is the first case with three types of heterotopia found in a single patient.

CASE DESCRIPTION/METHODS:
A 61-year-old man with GERD presented with periodic dysphagia to solids. He denied regurgitation, heartburn, melena, hematochezia, constipation, and diarrhea. Physical exam was unremarkable. Barium swallow showed a small hiatal hernia, and mild concentric narrowing at the GE junction. EGD showed a low grade Schatzki ring and diffuse beige 2-3 mm specks in middle third of esophagus. A colonoscopy was also scheduled for screening purposes and showed a 20 mm semi-sessile polyp in the rectum. Biopsies identified focal pancreatic and sebaceous gland heterotopia in the esophagus and gastric fundus heterotopia in the rectum.

DISCUSSION:
Heterotopic pancreatic, sebaceous, and gastric mucosa have been described along the entire GI tract. Our literature review identified 11 case reports of pancreatic heterotopia in the esophagus, 23 case reports of heterotopic sebaceous glands in the esophagus, and 73 case reports of gastric heterotopia in the rectum. However, no case reports found a single patient with all three types of heterotopia. In all three types, there was a male predominance, and ages ranged from birth to 6th decade of life. The origin of the heterotopic tissues is hypothesized to be either congenital vs metaplastic. As the combination of organ heterotopia described here includes components of mesoderm and endoderm, there was presumably incomplete cellular differentiation in weeks 2-3 of embryonic development. Patients can be asymptomatic or present with dysphagia, epigastric pain, or reflux with heterotopic pancreatic mucosa and sebaceous glands in the esophagus, and hematochezia with heterotopic gastric mucosa in the rectum. Treatment for the heterotopic tissues vary but surgical or endoscopic resection is typically pursued. Given that the tissue is often removed, it is unclear the malignant potential of the lesions and close follow up is necessary. Ultimately, presentations such as hematochezia and dysphagia may have rare etiologies and necessitates sufficient biopsies.
1762 A Rare Case of an Alpha-Fetoprotein-Producing Esophageal Adenocarcinoma
Authors: Dasu Neethi R. , Khalid Yaser , Gordon Richard , Itidiare Michael , Walters Richard 
Published: October 2019

INTRODUCTION:
AFP-producing esophageal adenocarcinoma (EAC) is a very rare occurrence. Elevation of serum AFP is commonly associated with hepatocellular carcinoma and yolk sac tumors, but rarely with esophageal carcinoma. This is a case of a 75-yr-old female who presented with progressive weakness and decrease in appetite, she was diagnosed with EAC despite high suspicion for HCC due to the extremely elevated AFP level. Prognosis is poor given the advanced presenting stage and high metastatic potential when finally diagnosed.

CASE DESCRIPTION/METHODS:
A 75-yr-old female with a past medical history of gastroesophageal reflux disease and Barrett’s esophagus status-post multiple upper endoscopies and a colonoscopy presented with progressive weakness, decrease in appetite, and abdominal pain over a few weeks. She also complained of dysphagia for solid food, and recent weight loss. On physical exam, she had mild epigastric tenderness. Her labs revealed markedly elevated CEA of 742, alpha-fetoprotein 46,135, and CA 19-9 of 6842. In the hospital, she had tachycardia so a CTA of her lungs was done to rule out a pulmonary embolism but instead revealed gastric thickening, celiac adenopathy, and multiple large liver masses. These findings were confirmed by a CT of her abdomen and pelvis with and without IV and oral contrast. Upper endoscopy revealed a large non-obstructing mass in the distal esophagus that extended into the stomach. A needle aspiration biopsy of a liver lesion revealed esophageal adenocarcinoma with metastasis.

DISCUSSION:
There have been only fifteen reported cases of AFP-producing esophageal adenocarcinoma in the literature. Our patient presented not only with EAC but also extremely high levels of AFP >40,000, with 320 ng/mL as the highest reported AFP level previously. Most EACs were located over the lower third of the esophagus and were related to Barrett’s esophagus as seen in our patient. The serum AFP level can be serially measured for monitoring clinical status, evaluating cure, and assessing recurrence or metastases. Most cases were treated with adjuvant chemotherapy with cisplatin plus 5-fluorouracil (5-FU) as first-line and combination of paclitaxel and cisplatin as second-line. Our patient was treated with oxaplatin, folinic acid, and 5-FU. She had temporary reduction of metastasis, but unfortunately there was recurrence. Clinicians should consider AFP-producing EAC in their differential diagnosis when working up a liver mass in the setting of elevated AFP or liver function impairment.
1765 Delayed Esophageal Clearance Plays an Important Role in Reflux Symptomology in Scleroderma Patient
Authors: Averbukh Leon D. , Tran Victoria , Tadros Micheal 
Published: October 2019

INTRODUCTION:
We present the case of a patient with advanced scleroderma who experienced symptoms of refractory reflux despite maximum acid suppressing therapy. The use of 24-hour ambulatory esophageal pH impedance measurement led to the diagnosis of esophageal stasis, with subsequent management leading to the improvement of symptoms even with decreased proton pump inhibitor (PPI) dose.

CASE DESCRIPTION/METHODS:
70-year old female with history of advanced scleroderma presented with severe retrosternal burning which she described as worst at night. She was taking 60mg Dexlansoprazole daily, an aluminum-magnesium-antacid, as well as an H2 blocker. An outside CT of the chest and abdomen showed a fluid-filled dilated esophagus and upper endoscopy was significant for a small hiatal hernia. The patient initially underwent high resolution manometry (HRM) which showed absent peristalsis. Subsequent pH impedance study identified low impedance in the esophagus with good gastric acid control (gastric acid exposure of 36%) but markedly abnormal gastric acid exposure in the distal esophagus: recumbent time acid exposure 94%, upright acid exposure 3%, and total acid exposure time of 40% despite high dose PPI. Recumbent position acid exposure at the distal esophagus was more prolonged than nocturnal gastric acidity, indicating esophageal stasis was a major factor in symptomatology. The patient was instructed to take her PPI and antacids close to bedtime, sleep with head elevation, and avoid late meals. The patient experienced significant symptom improvement and her PPI dose was successfully decreased.

DISCUSSION:
Scleroderma is a multiorgan connective tissue disorder affecting the gastrointestinal tract. The most commonly affected portion of the GI tract is the esophagus, with destruction of the smooth muscles of the lower esophageal sphincter and distal esophageal body. Abnormal reflux has been identified in patients with scleroderma on 24-hour pH monitoring in as many as 80% of patients. However, these study findings do not account for delayed esophageal clearance from failed or ineffective esophageal contractions. Patients with systemic sclerosis frequently have large dilated esophagi (pseudoachalasia) with subsequent stasis a major contributing factor for their symptoms. Thus, while pH studies are a useful diagnostic tool in patients with advanced scleroderma, therapeutic measures addressing esophageal stasis, such as nighttime head elevation, should be encouraged.
1769 Esophageal Candidiasis as an Unusual Cause of Upper Esophageal Stricture
Authors: Higley Cory , Shahbazi Shandiz , Choden Tenzin , Malhotra Nidhi 
Published: October 2019

INTRODUCTION:
The location of an esophageal stricture often provides clues for its etiology. Upper esophageal strictures are most commonly associated with previous mediastinal radiation, caustic ingestion, or bullous dermatologic disorders. They are rarely attributed to infectious etiology. We present a case of an upper esophageal stricture that we believe is secondary to candida infection.

CASE DESCRIPTION/METHODS:
A 52-year-old female with past medical history significant for diabetes mellitus type 2 and invasive ductal carcinoma of right breast status post mastectomy and chemotherapy, now in remission and only on hormonal therapy, had presented to our gastroenterology clinic complaining of dysphagia with associated unintentional weight loss. Dysphagia had been present for several months prior and was progressive, with near inability to tolerate solids, and not associated with regurgitation or acid reflux symptoms. She had also reported a 15-pound weight loss during this time. Initial esophagogastroduodenoscopy revealed an upper esophageal stricture approximately 15cm from the incisors, preventing initial passage of standard upper endoscope. Neonatal scope was used which both traversed and dilated to 9mm. Past the stenosis, friable and desquamative- appearing mucosa was found. Upper esophageal biopsies were consistent with Candida esophagitis. Gastric cardia biopsy incidentally noted Helicobacter pylori. Patient was subsequently treated with oral fluconazole, triple therapy, and proton-pump inhibitor. Repeat follow-up esophagogastroduodenoscopy again demonstrated the initial upper esophageal stenosis, which could now be traversed with a standard upper endoscope. A Savary dilator was used to dilate the stricture to 39 Fr. Repeat esophageal and stomach biopsies were negative for Candida and H. Pylori, respectively. Subsequently, the patient reported improved symptoms without any dysphagia to solids or liquids.

DISCUSSION:
Candidal esophagitis is fairly common, however it is not typically known to cause upper esophageal strictures. This case highlights the need for physicians to maintain a broader differential and consider candida as a risk factor for developing stenotic lesions, even in immunocompetent patients and especially in the absence of other more well-known etiologies. Correct identification of etiology is necessary for appropriate treatment and preventing recurrence.
1775 Tracheoesophageal Fistula Managed With Double Stenting of Bronchus and Esophagus
Authors: Abdehou Samuel , Massey Jonathan , Jiwani Sania , Nguyen Vinh , Jordan Paul 
Published: October 2019

INTRODUCTION:
Malignancy is responsible for 5-15% of acquired tracheoesophageal fistulas (TEF). Such patients have a short survival and management of these fistulas is a challenge. We present a case of TEF secondary to esophageal malignancy that was managed with double stenting of the bronchus and esophagus.

CASE DESCRIPTION/METHODS:
The patient is a 56 year old black female with no known past medical history who presented to our emergency department with complaints of chest pain, shortness of breath and a productive blood tinged cough. She had been recently discharged from another facility for a diagnosis of bronchitis, however symptoms had not abated. She reported a fifty-pound weight loss with poor oral intake. Her vital signs and labs on arrival were stable. Initial imaging in the ER was consistent with pneumonia of the right middle and lower lobes. She underwent a CT Neck, which showed esophageal wall thickening and a mass suspicious for an esophageal neoplasm. Due to concern of her recurrence of pneumonia and esophageal mass, she underwent an esophagram, which showed a stricture of the middle third of the esophagus with a fistula from the esophageal tumor to the right lower lobe bronchus. Due to concern of airway collapse with upper endoscopy, Pulmonology was consulted. The patient first underwent a bronchoscopy in which a Novatech Y-stent was deployed stabilizing the airway. The patient then underwent an endoscopy in which an esophageal stent was placed. Both procedures were successful and she tolerated oral intake well thereafter. She was discharged in stable condition to palliative hospice services.

DISCUSSION:
The case has relevance from both an interesting initial clinical presentation as well as management of this patient from both a technical and procedural aspect. This patient had continual infections from continuous overspill of esophageal contents into the respiratory tree. Although her prognosis to begin with was quite poor, these interventions allowed the patient some quality of life. This case also commands procedural respect. The patient’s left mainstem bronchus was extremely friable and actually ruptured during the initial course of balloon dilation. The stent in position did not completely cover the TEF however it did keep the central airway open. A long esophageal stent was placed by gastroenterology, which did completely cover the fistula. This patient’s case highlights potential difficulties in double stenting and anticipatory management by utilization of other services.
1799 A Rare Cause of Dysphagia-Mucosal Leishmaniasis
Authors: Adu-Gyamfi Kwabena , Hunt Scott , Morales Edward , Adeniji Olaitan 
Published: October 2019

INTRODUCTION:
Leishmaniasis is a parasitic disease spread by the bite of infected sand flies. Depending on the location of tissue or organ involvement, it may be described as cutaneous, mucosal or visceral. We present a case of mucosal leishmaniasis, exclusively involving the upper aerodigestive tract with primary symptom of dysphagia. This pattern of anatomical involvement is uncommon, and tissue biopsies may be non-specific, leading to delays in diagnosis and treatment. In our case immunohistochemical staining, molecular studies and clinical suspicion were required for the diagnosis.

CASE DESCRIPTION/METHODS:
A 42-year-old Hispanic man presented to the clinic with a 4-month history of progressive dysphagia. He denied sinonasal or respiratory symptoms. He had normal vital signs. Oral examination revealed a large painless exophytic mass replacing the soft and hard palates, extending to the hypopharynx (Figure 1). A CT scan of the neck with contrast showed a severe infiltrative process throughout the upper aerodigestive tract extending from the oral cavity to the subglottic airway (Figure 2). Repeat biopsies were non-diagnostic initially, showing pseudoepitheliomatous hyperplasia without evidence of dysplasia or neoplasia. Work up for endemic fungal, tuberculous and non-infectious granulomatous etiologies were negative. Further histopathologic review led to immunohistochemical staining of biopsies and assays which were positive for Leishmania panamensis. He was treated with oral miltefosine. At a 3-month follow up, oropharyngeal lesions had substantially regressed (Figure 3) and his dysphagia resolved. Sustained improvement was noted at a 1-year follow up.

DISCUSSION:
Mucosal leishmaniasis may be caused by different species, including Leishmania braziliensis and panamensis. It results from lymphangitic or hematogenous spread of leishmania parasites, usually from a pre-existing skin lesion. Symptoms of dysphagia or odynophagia may be the primary manifestation. Lesions are mostly exophytic and commonly involve the tongue, palate, oropharynx or larynx. Diagnosis requires demonstration of leishmania amastigotes or positive immunohistochemical staining in biopsy samples. Different treatment modalities may be used. Miltefosine is the latest drug treatment approved by the FDA for mucosal leishmaniasis. A high index of suspicion is required for the diagnosis of mucosal leishmaniasis as it potentially mimics other granulomatous, neoplastic and infectious etiologies of the upper aerodigestive tract.
1810 Esophageal Cancer-Associated Myositis
Authors: Bofill Aliana , Rodriguez Ramos Roberto J. , Rodriguez-Berrios Frances , Santiago Loscar 
Published: October 2019

INTRODUCTION:
The inflammatory myopathies are a group of acquired diseases characterized by a proximal myopathy due to skeletal muscle inflammation. Myositis association with malignancy has been well described, particularly in dermatomyositis (DM).Although dysphagia may be the presenting symptoms for DM given striated muscles at upper esophagus;it has also been associated with increased risk of malignancy.Cancer-associated myositis (CAM) is thought to be a cross-reaction to regenerating muscle tissue as seen in tumor antigen. An estimated 20-30% of DM patients will develop cancer.

CASE DESCRIPTION/METHODS:
Case of a 70-year-old male who arrived to the emergency department due to three-week history of progressive dysphagia with a 45-pound weight loss in the past 3 months. Also reported skin rash and generalized weakness. Physical examination with lower proximal limb weakness and generalized erythematous patches more prominent over the trunk with scaly violaceous raised papules in the knuckles and in the interphalangeal joints. Laboratories showed elevated muscle enzymes including aldolase, creatinine kinase, transaminases and lactate dehydrogenase.Negative myositis specific antibodies including ANTI-JO1, ANTI-MI2 and ANTI-SRP. MRI showed inflammatory muscle disease. Gastroenterology proceed with esophagogastroduodenoscopy for evaluation of dysphagia with findings of a nodular lesion inside a hiatal hernia. Biopsies were consistent with adenocarcinoma with positive stains for HER2 and AE1/AE3 suggesting advance poorly differentiated adenocarcinoma. Metastatic workup with evidence of retroperitoneal adenopathy. Hematology Oncology started chemotherapy with FOLFOX-6 and radiotherapy. He showed some initial improvement; however, later he succumbed to respiratory tract infection.

DISCUSSION:
In this case, the patient was found with an underlying esophageal malignancy along with DM which are findings consistent with CAM. The absence of myositis specific antibodies supports the diagnosis. Dysphagia can be the presenting symptom of both DM and esophageal cancer. In DM is due to the involvement of the oropharyngeal striated muscles and upper esophagus.In the setting of an underlying inflammatory myopathy symptoms can be misleading, causing misdiagnosis. It is important that all patients diagnosed with a myopathy along with alarming symptoms undergo evaluation for the possibility of an underlying malignancy since prognosis and life expectancy in CM patients is determined by the underlying malignant disease.
1834 Is Achalasia Part of Pure Autonomic Failure?
Authors: Adusumilli Radha Kishan , Patel Manishkumar , Gonzalez Victoria , Kulairi Zain I. 
Published: October 2019

INTRODUCTION:
Pure autonomic failure is a sporadic neurodegenerative disorder which is characterized by idiopathic autonomic system failure without any focal neurological deficits. It causes orthostatic hypotension, urinary symptoms and gastrointestinal symptoms like constipation. Being a fairly uncommon disease, little is known about the extent of organ system involvement of the disease.

CASE DESCRIPTION/METHODS:
A 79-year-old male with 5 years history of pure autonomic failure presented with acute hypoxic respiratory failure. He was found to have aspiration pneumonia secondary to achalasia & was treated with antibiotics. Organic causes for achalasia were ruled out with history, endoscopy and manometry studies. Patient continued to have multiple recurrence which required multiple dilations and botulinum injections and eventually he had a percutaneous endoscopic gastrostomy tube to decrease the risk of aspiration.

DISCUSSION:
Although the gastrointestinal system is under the control of the enteric plexus predominantly, it is innervated by the sympathetic and parasympathetic system. The decreased activity of the sympathetic system causes overactivity of the parasympathetic system causing the overactivity of the smooth muscles causing the urinary symptoms and lower gastrointestinal symptoms like constipation. Achalasia by definition is impaired relaxation of the lower esophageal sphincter which is a predominantly smooth muscle. Cases with achalasia in the past which have been studied showed that these patients have some kind autonomic dysfunction. In conclusion, patients with pure autonomic failure are prone to developing achalasia and it is not an independent disorder by itself and could be the sign of progression to multiple system atrophy.