bluuebunny/crossref_metadata_2025 huggingface dataset:
1711 An Esophageal Mass Masquerading as Asthma Exacerbation: An Unfortunate Outcome
Authors: Chan Matthew , Patel Akash , Bowers Jacqueline
Published: October 2019

INTRODUCTION:

Esophageal masses can present in a variety of ways, such as a result of mediastinal mass effect leading to cough, hoarseness, and/or dysphagia, or they can be incidental findings on imaging as part of the evaluation of an unrelated condition. A definitive diagnosis generally requires tissue sampling. We present a case of a woman with a recent diagnosis of asthma unresponsive to standard therapies who was found to have an esophageal mass that unfortunately led to respiratory arrest.

CASE DESCRIPTION/METHODS:

A 32-year-old African American female with history of G6PD deficiency, alpha thalassemia, GERD, morbid obesity, and recent diagnosis of asthma presented with dyspnea. A month prior, she was seen in the ER on two separate occasions for similar symptoms and treated for asthma exacerbations and subsequently discharged home. Chest x-rays performed then were unremarkable. Upon further history, the patient also had progressive dysphagia from solids to liquids and globus pharyngis. There was no history of tobacco or alcohol use, and no family history of malignancy. On admission, vitals were unremarkable. Exam revealed hoarseness, biphasic stridor greater upon inspiration, and expiratory wheezing throughout lung fields. Labs showed a chronic microcytic anemia, and chest x-ray was again unremarkable. She was treated for presumed asthma exacerbation and started on IV PPI and IV steroids for possible airway edema due to GERD. ENT was consulted to assess for any airway obstruction. Flexible nasopharyngolaryngoscopy showed paralysis of the left true vocal cord, and a CT with IV contrast of the neck and chest was performed that showed a large mass involving the mid-thoracic esophagus compressing the trachea. Gastroenterology and Cardiothoracic Surgery were consulted, and the patient was transferred to an outside hospital for emergent tracheal stenting. Unfortunately, the patient had respiratory arrest prior to intervention. Family declined autopsy studies to determine the etiology of the mass.

DISCUSSION:

This case illustrates that careful history and physical examination clues be obtained in cases of asthma unresponsive to standard therapy. A low threshold for obtaining further dedicated imaging such as chest CT can help with prompt identification and management of life-threatening processes such as respiratory compromise. Some differentials for esophageal masses include squamous cell cancer, adenocarcinoma, and lymphoma among others, and this case highlights that everything that wheezes is not asthma.
1720 A Case of Sunitinib-Induced Necrotizing Esophagitis
Authors: Bertran-Rodriguez Carlos E. , Mony Shruti , Slone Samuel , Arroyo Yadis , Pagan Andrea , Rabbanifard Roshanak
Published: October 2019

INTRODUCTION:

Acute esophageal necrosis (AEN), also known as “black esophagus” or “acute necrotizing esophagitis”, is a rare entity thought to be caused by a known ischemic insult from a low perfusion state. Etiologies include multi-organ dysfunction, hypoperfusion, vasculopathy, sepsis, among others. Sunitinib, a chemotherapeutic agent for renal cell cancer and gastrointestinal stromal tumors, has common gastrointestinal (GI) side effects of diarrhea and vomiting but severe GI side effects are rare. We present a unique case of Sunitinib-induced AEN.

CASE DESCRIPTION/METHODS:

A 72-year-old man presented to the hospital for a two days history of epigastric abdominal pain, bilious vomiting, and multiple episodes of non-bloody diarrhea. His medical history was significant for metastatic papillary renal cell carcinoma on Sunitinib. On presentation, his blood pressure was 74/52 mm Hg and heart rate 107 beats per minute. He exhibited generalized abdominal tenderness without rebound or guarding. His hemoglobin was 15 g/dL. He was admitted to the ICU for hypovolemic shock thought to be secondary to severe gastroenteritis requiring the use of vasopressors. He later developed ongoing melena and his hemoglobin decreased to 10.8 g/dl requiring urgent Endoscopy. The examination revealed circumferential, black, necrotic mucosa in the lower one-half of the esophagus with a sharp demarcation of healthy mucosa at the level of gastroesophageal junction without any active bleeding. Findings were consistent with AEN. The patient remained hypotensive despite aggressive resuscitation and the use of vasopressors. Unfortunately, the patient died several days later.

DISCUSSION:

AEN is characterized by the classic endoscopic image of diffuse, circumferential, black-appearing necrotic mucosa. The exact etiology leading to the hypoperfusion state in our case is unclear and could be attributed to Sunitinib. The most common adverse GI reactions to Sunitinib include diarrhea (53%), nausea (44%), and vomiting (24%) which were intractable in the above case. This medication has also been linked to severe GI hemorrhage. Sunitinib inhibits platelet-derived growth factors and vascular endothelial growth factors which play a major role in angiogenesis and apoptosis. We believe that by inhibiting these cellular processes, the patient was predisposed to development of AEN which was potentiated by the patient’s hypovolemic state.
1746 A Surprising Case of Boerhaave's Syndrome Successfully Treated With a Single Hemostatic Clip
Authors: Collins Kelsey , Herman Michael , Blank Andrew
Published: October 2019

INTRODUCTION:

Boerhaave’s Syndrome (BS), defined as transmural esophageal perforation, is rare and associated with 35% mortality. Patients with BS can develop associated mediastinitis, sepsis, and septic shock. Patients require urgent management, usually with volume resuscitation, antibiotics, surgical intervention, or occasional endoscopic placement of a metallic stent. There are few reported cases demonstrating successful repair of esophageal perforation with hemostatic clips alone. We present a case of direct repair of BS with a single hemostatic clip.

CASE DESCRIPTION/METHODS:

A 44-year-old male presented to the ED for food impaction with subsequent development of large volume hematemesis. He was stable, with no sign of respiratory distress. However, given his persistent hematemesis, he was intubated for airway protection. Initial labs were remarkable for leukocytosis to 14.8 and hemoglobin of 18.4. A Mallory Weiss tear was expected, but his CT chest showed inflammation in the mediastinum associated with debris in the esophagus and esophageal wall thickening. His clinical picture and imaging were consistent with BS complicated by mediastinitis and esophagitis with superimposed esophageal perforation. He was given one dose of both Zosyn and a PPI and was taken for emergent EGD. Red blood and clots were seen throughout his esophagus, and a large non-bleeding tear with stigmata of recent bleeding was seen in the distal esophagus. One hemostatic clip was placed with successful resolution of the defect. He was then admitted to the medical ICU for further management. Over the next 24 hours, he developed melena and became tachycardic. His leukocytosis peaked at 21, and his hemoglobin gradually decreased to 8.9. Repeat CT chest was obtained due to worsening symptoms, but imaging showed that the esophageal clip was in place, and there was improvement in prior esophageal wall thickening, but there was a new small left pleural effusion. Cardiothoracic Surgery evaluated the patient and recommended an esophagram, which showed no evidence of esophageal contrast extravasation. He was successfully extubated and was discharged home the following day.

DISCUSSION:

BS is often a medical emergency requiring urgent intervention. Most cases are treated surgically with direct repair of the rupture via thoracotomy or laparotomy. Although there is a known role for endoscopic management using a metallic stent, there is an evolving role for treatment of more mild cases of BS with clip placement alone.
1750 Bevacizumab-Induced Esophageal Perforation: A Case Report
Authors: Abidian Mohamed Modar , Nath Anand , Shafa Shervin
Published: October 2019

INTRODUCTION:

Esophageal perforation can range from micro perforation to full wall thickness rupture. Patient symptoms can vary based on the injury extent. Common causes of perforation include iatrogenic (endoscopy), traumatic, peptic ulceration and/or foreign body ingestion. Rarely, spontaneous perforation (Boerhaave’s syndrome) occur as a result of sudden increase in the intraesophageal pressure from severe vomiting. The management depends on the severity of the rupture and includes conservative therapy, endoscopy and/or surgery. We present a rare incidence of spontaneous esophageal perforation in the setting of bevacizumab therapy.

CASE DESCRIPTION/METHODS:

Our patient is a 57 year-old female with past medical history of colorectal cancer diagnosed in 2002. Since then, patient had undergone partial resection of the colon, radiotherapy and multiple courses of chemotherapy. However, her colorectal cancer had metastasized to retroperitoneal and pelvic lymph node, skeletal bones and lungs. Her last chemotherapy regimen included FOLFIRI (Fluorouracil, Leucovorin, Irinotecan) plus Bevacizumab, which was started 8 days prior to admission. The patient had also complained of persistent nausea and recurrent vomiting prior to admission. She had also reported lower abdominal pain, prompting a CT of the abdomen. The CT showed small pneumomediastinum in the posterior mediastinum located between the distal esophagus and the descending thoracic aorta (Figures 1 and 2). The patient had remained hemodynamically stable without any other clinical features of active perforation. The decision was made to forego endoscopic diversion and to continue with conservative management. She remained nothing per mouth and received antibiotics and intravenous fluids. A follow-up esophagram did now show evidence of contrast leak at the site of perforation (Figure 3).

DISCUSSION:

Bevacizumab has shown improved outcomes in patients with metastatic colorectal However, gastrointestinal perforation is an adverse event reported in 0.3–3.2% of patients. The mechanism by which bevacizumab induces perforation has not been determined, but may be associated with delayed wound healing. Moreover, Bevacizumab is frequently combined with cytotoxic regimens, including taxanes, which may be associated with gastrointestinal wall inflammation and necrosis. It is possible that her episodes of emesis may have contributed to this microperforation. As more cases are reported, clinicians can gain better understanding of related adverse effects of this medication.
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.
1841 An Unusual Case of Persistent Globus Due to Granular Cell Tumor of the Distal Esophagus
Authors: Makar Ranjit , Maklad Muthena , Aziz Hamza , Lankarani Daisy S. , Fayad Joseph M.
Published: October 2019

INTRODUCTION:

Granular cell tumors (GCT) are rare soft tissue tumors arising from the Schwann cells. Most common sites involved are head and neck, although the gastrointestinal tract can be rarely involved. GCT of the esophagus are rare; their incidence in endoscopy series has been estimated to be approximately 0.033 percent, representing approximately 1 percent of benign esophageal tumors. GCT are mostly benign but rarely can turn malignant in 2% cases1. Patient are usually asymptomatic, although when symptomatic, patients can experience GERD, dysphagia and rarely Globus. We present an unusual case of persistent Globus due to granular cell tumor of the distal esophagus.

CASE DESCRIPTION/METHODS:

46 yo male with past history of asthma referred to the Gastroenterology clinic for symptoms of intermittent dysphagia to solids along with a feeling of persistent Globus sensation for 3 months. He was also experiencing symptoms of intermittent dysphagia to solids but none to liquids. Barium Esophagogram failed to reveal any pathology. Patient subsequently underwent EGD which revealed a 5mm sub mucosal lesion in the esophagus at 32 cms from the incisors (Figure 1). EUS demonstrated the lesion limited to the submucosa. Biopsy from the lesion revealed Granular cell tumor confirmed with immunostain positive for S 100 (Figure 2). The esophageal lesion was subsequently removed en bloc using band assisted EMR (Figure 3). A follow up clinic visit revealed complete resolution of globus and dysphagia.

DISCUSSION:

GCT are rare soft tissue tumors most commonly arising from the tongue, skin and subcutaneous tissue. Intestinal involvement is rare. Esophagus is the most commonly involved site in the GI tract and these lesions can present as subtle submucosal lesions. Although asymptomatic, GCT have been reported to cause dysphagia and rarely globus. Immunostain is usually positive for S-100 protein. Early recognition followed by en bloc endoscopic mucosal resection has been associated with complete resolution of symptoms.
1846 Mammalian Meat Allergy Masquerading as IBS-D
Authors: Bensinger Andrew , Green Patrick
Published: October 2019

INTRODUCTION:

Mammalian meat allergy, often referred to as alpha gal allergy, is a recently discovered and increasingly prevalent condition with a wide range of clinical manifestations resulting from an allergic reaction mediated by IgE antibodies directed against galactose-alpha-1,3-galactose. Patients can frequently experience nausea, diarrhea, indigestion, urticaria, angioedema or anaphylaxis typically arising three to six hours after ingestion of mammalian protein. Interestingly, patients have longstanding tolerance to mammalian meats years before symptom onset and were bitten by the lone star tick, Amblyomma americanum, prior to symptom onset. We present a case of mammalian meat allergy diagnosed after the discovery of a tick on a patient’s lower extremity during a colonoscopy.

CASE DESCRIPTION/METHODS:

A 64-year-old female with a history of IBS-D presented to our clinic several years of chronic urticaria, diarrhea, nausea and abdominal pain, typically occurring after food intake. An extensive workup of chronic diarrhea was largely unrevealing. However, during a colonoscopy a tick was discovered on the patient’s inner thigh and was removed. Given her symptom complex, additional blood work was pursued and revealed elevated levels of serum alpha gal IgE. Further history revealed multiple prior tick bites with severe localized skin reactions in the past. At a follow-up visit after avoidance of mammalian meat and byproducts, the patient reported a 90% improvement in her symptoms, with just one formed bowel movement per day. She continued to have some bloating and abdominal pressure, but overall her symptoms were drastically improved with a modified diet.

DISCUSSION:

This case illustrates the challenges in identifying alpha gal allergy, a unique and frequently misdiagnosed allergic condition that is increasing in prevalence. The clinical presentation of alpha gal is unique when compared to other food allergies as there are a lack of immediate oral symptoms and episodes only occur after lone star tick exposure. Additionally, onset of symptoms occurs three to six hours after mammalian meat consumption, making its association difficult to uncover. The case also highlights the importance of taking a thorough history, with higher suspicion in patients with recurrent episodes of urticaria, angioedema, gastroenteritis, or anaphylaxis without an obvious cause. Thus, improved awareness of alpha gal allergy is essential, particularly for providers located in endemic areas like the southeastern United States.
1847 Buspirone for the Management of Functional Dyspepsia With Rapid Gastric Emptying (RGE)
Authors: Radetic Mark , Gabbard Scott
Published: October 2019

INTRODUCTION:

Functional Dyspepsia is characterized by epigastric pain/burning, early satiety, and post-prandial fullness in the absence of any gastrointestinal pathology. It affects 5-10% of patients world-wide and is responsible for up to 75% of all cases of chronic dyspepsia. Although the pathophysiology is not fully understood, several mechanisms have been proposed: abnormalities in gastric compliance, visceral hypersensitivity, and post-infectious complications. A subset of patients with Functional Dyspepsia have rapid gastric emptying (RGE) – defined as <30% gastric retention at 1-hour on Gastric Emptying Scintigraphy (GES). A case is reported of Functional Dyspepsia with RGE responsive to treatment with buspirone – a 5-HT1A agonist that has been shown to improve post-prandial symptoms in Functional Dyspepsia.

CASE DESCRIPTION/METHODS:

The patient is a 60-year-old male with advanced COPD s/p bilateral lung transplant who initially presented with heartburn and acid regurgitation. Esophageal pH testing demonstrated increased esophageal acid exposure consistent with GERD. He was treated with a proton pump inhibitor (PPI) with improvement in his symptoms. Over the next year, he started experiencing persistent epigastric discomfort, nausea/vomiting, early satiety, and loose stools culminating in 5 hospitalizations over a 1-year period. An EGD and colonoscopy were unremarkable with negative biopsies for H pylori. A GES revealed 7% gastric retention at the 1-hour mark – consistent with rapid gastric emptying (RGE). The patient was diagnosed with Functional Dyspepsia with RGE and started on buspirone. Within 1 week, he reported complete resolution of his symptoms.

DISCUSSION:

The natural history of Functional Dyspepsia results in symptom relapse and/or persistence in up to 50% of patients. Guidelines recommend a step-wise approach to therapy: a 4-8 week trial of a PPI followed by a tricyclic anti-depressant (TCA) for persistent symptoms. While treatment with PPIs and/or TCAs provides relief for some, a significant number of individuals continue to experience refractory symptoms. A fraction of patients with Functional Dyspepsia are found to have RGE, which may provide us with an additional therapeutic target. Buspirone has been shown to promote fundic relaxation/increase gastric accommodation which may help normalize the rate of gastric emptying by preventing early redistribution of a meal to the distal stomach. Thus, buspirone may be of benefit in patients with Functional Dyspepsia found to have RGE.
1849 Chronic Periumbilical Abdominal Pain: Abdominal Migraine in an Adult
Authors: Peng Frederick B. , Kaur Manreet
Published: October 2019

INTRODUCTION:

Chronic abdominal pain is a challenging, but frequently encountered clinical complaint. For adults, the symptoms are often debilitating and have significant implications on their daily lives. Often, these patients have undergone extensive work-up with laboratory testing and imaging that have either been unrevealing or non-specific. The history therefore becomes a key element in delineating potential etiologies. Here, we present a case of recurrent periumbilical abdominal pain in an adult with historical elements consistent with abdominal migraine – a clinical entity more commonly described in pediatric patients.

CASE DESCRIPTION/METHODS:

A 52-year-old man with diabetes and hypertension requiring renal transplant presented with 48 hours of periumbilical abdominal pain, increased eructation, and bilious emesis. For years, his symptoms had been minimally managed with IV fluids, analgesia, and anti-emetics during emergency room visits or hospital admissions. His last pain episode was a year ago. Work-up has been extensive and largely unrevealing, including multiple endoscopies, several CT scans, and a gastric emptying study. These episodes are intense, triggered by a lack of sleep, and often last a day or more with intermittent periods of relief (weeks to months). The pain is always associated with nausea, vomiting, and photophobia. He has a family history of migraines, but no personal history of headaches. On exam, he is hemodynamically stable, but purposely keeps the room dark. He has normoactive bowel sounds and is mildly tender to palpation in the periumbilical region. There is no rebound tenderness or guarding. Laboratory and imaging findings are unremarkable. Of note, his symptoms were previously managed with amitriptyline. A single dose of sumatriptan relieves his pain and he is discharged with outpatient GI follow-up.

DISCUSSION:

An abdominal migraine is a diagnosis of exclusion and its diagnostic criteria is delineated in Rome IV (Table 1). The described prodrome often resembles that of a typical migraine and management is similar. Prophylactic therapies include amitriptyline, propranolol, and topiramate, while abortive therapies involve triptans. Thus far, the disease entity is more commonly seen in pediatrics, but there is an increasing number of cases reported in adults. Like other functional GI disorders, a thorough history after comprehensive evaluation of all other etiologies differentiates abdominal migraine as a distinct clinical entity.
1853 Impaired Gastric Accommodation: A Key Player in Functional Dyspepsia
Authors: Figueredo Carlos , Boroda Konstantin , Hertan Hilary
Published: October 2019

INTRODUCTION:

Poor gastric accommodation (GA) causes inadequate relaxation of the stomach during a meal. This leads to early satiety, fullness sensation, nausea, and vomiting. Poor GA is frequently seen in functional GI disorders and often coincided with anxiety and stress. It should be considered in the absence of metabolic disorders, eating disorders, and self-induced vomiting. Poor GA is quantified by the average increase of gastric volume after a meal over 1 hour. Poor GA is thought to be caused by altered neuro-modulatory pathways that affect accommodation time after a meal.

CASE DESCRIPTION/METHODS:

A 53-year-old woman with history of Type II Diabetes with Hba1c: 9.4%, complicated by diabetic neuropathy was admitted for treatment of UTI and Herpes Zoster. She also complained of new onset nausea, vomiting, and early satiety for 1 week. She could tolerate only thin liquids but no solids, due to persistent emesis within 10-20 min of food ingestion. An EGD was done and ruled out gastric outlet obstruction, but revealed candida esophagitis, which was treated with Fluconazole. The patient was then started empirically on Metoclopramide for presumptive Gastroparesis in the setting of poorly controlled Diabetes. The dose was gradually titrated up without favorable response. Her therapy was then escalated to Erythromycin along with Zofran and Benadryl, with no success. The patient was unable to complete a Gastric Emptying (GE) Study twice due to emesis following radiotracer ingestion. However, images from incomplete GE studies showed rapid movement of radiotracer from the fundus to the antrum. The patient was then switched to Buspirone 5 mg 30 minutes before lunch and dinner with remarkable improvement of her symptoms in 2 days.

DISCUSSION:

Early redistribution of radiotracer seen on scintigraphy is suggestive of impaired accommodation and is usually associated with early satiety, dyspepsia, nausea and vomiting. Rapid clinical improvement after 5-HT1a receptor agonist therapy not only implies anxiety and stress as contributing factors in this disorder, but also the role of presynaptic inhibitory 5-HT1a receptor activation in the gastric accommodation reflex. This allows for better stomach relaxation both in fasting and postprandial states. This therapy should be considered in patients with gastroparesis-like symptoms, but rapid gastric emptying seen on scintigraphy.