Progressive Dysphagia Research Articles

P.89 Immune-mediated necrotizing myopathy associated with anti-SRP Antibodies: Three cases in Korea

Immune-mediated necrotizing myopathy associated with anti-SRP antibodies (anti-SRP myopathy) is characterized by progressive proximal muscle weakness and markedly elevated creatinine kinase (CK. Here, we report the clinical features of three Korean patient with anti-SRP myopathy. A 63-year-old man presented with progressive proximal muscle weakness and dysphagia. He showed symmetrical proximal weakness. The serum CK was 5,560U/L. The muscle biopsy of the biceps brachii showed mild muscle fiber size variation and a few degenerating muscle fibers. Muscle weakness and dysphagia improved after using steroids and azathioprine, but gradually worsen. At the last follow-up study, serum CK was 1,252 IU/L and the serum anti-SRP antibody was positive. A 46-year-old man was referred to our clinic due to progressive proximal muscle weakness. The serum CK was 6,114 U/L. The muscle biopsy of the vastus lateralis muscle showed marked size variation in muscle fibers and many necrotic fibers. Steroid pulse therapy lowered serum CK level, but muscle weakness did not improve. The serum anti- SRP antibody was positive. After the diagnosis, steroid, methotrexate, and intravenous immunoglobulin were used, but his muscle strength did not improve significantly. 43-year-old woman presented with proximal muscle weakness and elevated serum CK level. The serum CK was greater than 13,000 U/L. Needle EMG showed active generalized myopathy. The muscle biopsy of vastus lateralis muscle revealed a few necrotic fibers without endomysial inflammatory cell infiltration. The serum anti-SRP antibody was positive. After steroid pulse therapy, her muscle weakness did not improve and dysphagia was newly developed. She was currently treated with steroid and azathioprine. Anti-SRP myopathy is one of the most disabling and less responsiveness to treatment and we would like to report cases have rarely been reported in Korea.

S2379 Squamous Cell Carcinoma of the Gastroesophageal Junction as an Unusual Underlying Cause of Pseudoachalasia

Introduction: Achalasia is a rare esophageal motility disorder characterized by progressive dysphagia to solids and eventually liquids[1-2]. Pseudoachalasia refers to an achalasia secondary to an underlying etiology, such as masses, post-operative complications, or paraneoplastic syndromes[1-2]. The most common cause of pseudoachalasia is adenocarcinoma of the cardia or esophagus [1] however, we discuss a rare case that deviates from the typical etiology of esophageal cancer. Case Description/Methods: This is a 71-year-old male presenting with intermittent dysphagia for years with rapid progression for a month including intolerance of oral intake and 6-pound weight loss. On examination he was hemodynamically stable with a soft, nontender abdomen. His initial barium esophagram revealed decreased esophageal peristalsis, esophageal distention, and narrowing at the gastroesophageal junction (GEJ) with “bird beak” appearance consistent with possible achalasia. Further imaging studies were performed with computed tomography (CT), esophagogastroduodenoscopy (EGD) with dilation, and endoscopic ultrasound with biopsies revealing an invasive squamous cell carcinoma of the GEJ with submucosal involvement extending into the gastric cardia (Figure). We present our patient as the first reported case of pseudoachalasia due to SCC of the GEJ. The patient underwent esophageal stenting and mediport placement with plans for neoadjuvant chemotherapy with concurrent radiotherapy followed by definitive resection. Discussion: Pseudoachalasia is often misdiagnosed for primary achalasia because of its rarity, indistinguishable symptomatology, and normal findings on CT, EGD, and biopsy[1-2]. Nearly 75% of pseudoachalasia cases are due to adenocarcinoma of the gastric cardia[1]. Only one case of pseudoachalasia has been known to be caused by SCC in the distal esophagus[3]. SCC of the GEJ is an extremely rare esophageal malignancy and has never previously been reported as an underlying cause of pseudoachalasia.Figure 1.: Esophagogastroduodenoscopy revealed severe stricture at the GEJ with a 4 cm submucosal mass at the GEJ with extension to the gastric cardia.REFERENCES 1. Campo SM, Zullo A, Scandavini CM, Frezza B, Cerro P, Balducci G. Pseudoachalasia: A peculiar case report and review of the literature. World J Gastrointest Endosc. 2013;5(9):450-454. 2. Roushan N, Zolfaghari A, Asadi M, Taslimi R. Pseudoachalasia: a diagnostic challenge. Med J Islam Repub Iran. 2014;28:54. 3. Kahrilas, P., Kishk, S., Helm, J., Dodds, W., Harig, J. and Hogan, W., 1987. Comparison of pseudoachalasia and achalasia. The American Journal of Medicine. 1987;82(3):439-446.

S2269 Malignant Dysphagia in Latent Triple Negative Metastatic Breast Cancer

Introduction: Luminal gastrointestinal metastasis of breast cancer is rare and esophageal metastasis from breast cancer is reported in less than 100 cases. We present a unique case of metastatic esophageal cancer from a primary breast malignancy, 20 years post initial diagnosis. Case Description/Methods: A 79-year-old African American female with past medical history of triple negative metastatic breast cancer, COPD, HTN, & T2DM presented to the hospital with 3 weeks of progressive dysphagia to liquids & solids, regurgitation and 10kg weight loss. No heartburn, nausea/vomiting or change in bowel habits. No prior EGD and colonoscopy many years ago showed 2 benign polyps. She was diagnosed with breast cancer in early 2000s and underwent right mastectomy; recurrence was discovered in 2014, which led to lumpectomy, chemotherapy/radiation. Vital signs stable on arrival. Physical examination notable for cachectic female; no lymphadenopathy, abdominal distension/tenderness or edema. Labs showed: WBC 7.9, Hb 11, MCV 82, Plt 291. EGD, showed severe stenosis in the mid-esophagus – dilation was attempted, but unsuccessful; biopsies were negative. Esophogram revealed severe stenosis of the mid-esophagus. CT thorax demonstrated a focal density in the mid-esophagus & multiple hepatic lesions concerning for metastasis. Endoscopic ultrasound (EUS) revealed severe stenosis of mid-esophagus; dilation, fine needle aspiration (FNA) and stent placement were performed. Pathology demonstrated metastatic triple negative breast cancer. The patient planned to start paclitaxel but was placed on hospice (Figure). Discussion: Esophageal metastasis of breast cancer has a prevalence of 0.59-5.9%. The most common presenting symptom is dysphagia. Studies describe a latency period between the diagnosis of breast cancer and symptoms of esophageal metastasis. Diagnosis is difficult as mucosal involvement is rare and most cases will present with stricture and normal mucosa. EUS with FNA is necessary to confirm diagnosis. Some success has been shown with expandable stents for symptomatic relief as dilation is limited by high risk of perforation. As in our case, esophageal metastasis is usually part of multi-organ metastasis, so treatment aimed at the primary malignancy, but palliative in most cases. Concern for malignant dysphagia must be included in the differential for breast cancer patients presenting with dysphagia. Due to difficult diagnosis and latency period, more routine screening with EUS may be beneficial.Figure 1.: Repeat EGD with EUS & Fluoroscopic guided stent placement.

WEST NILE-INDUCED MYASTHENIA GRAVIS CRISIS: AN UNEXPECTED CASE OF RESPIRATORY FAILURE

WEST NILE-INDUCED MYASTHENIA GRAVIS CRISIS: AN UNEXPECTED CASE OF RESPIRATORY FAILURE

S2298 Dysphagia Lusoria Secondary to an Aberrant Left Subclavian Artery

Introduction: Dysphagia lusoria, also known as Bayford-Autenrieth dysphagia, is a rare condition with prevalence of approximately 0.5%. First reported by David Bayford in 1790, it is defined as dysphagia secondary to extrinsic compression of the esophagus as a result of congenital abnormality of the aortic arch and its branches. We report a case of Dysphagia lusoria in a patient with congenital heart syndrome. Case Description/Methods: A 36-year-old woman with history of Dysphagia lusoria secondary to an aberrant left subclavian artery (SCA), congenital heart syndrome with known right sided thoracic aorta, tricuspid atresia and stenosis, hypoplastic right ventricle, and aberrant left SCA presented to the hospital with worsening dysphagia. She reported vomiting with any solid foods and only tolerating pureed foods or liquids. She reported a history of progressive dysphagia since 2018, when she underwent an esophagogastroduodenoscopy (EGD) that showed subtle pallor in the upper esophagus concerning for partial obstruction due to aberrant subclavian artery. She also underwent a manometry that was normal. On hospital admission, she underwent an x-ray upper gastrointestinal series that showed unchanged vascular impression of the upper esophagus with mild esophageal dysmotility, consistent with her diagnosis of Dysphagia lusoria (Figure). Cardiology and vascular surgery were consulted and deemed the patient a poor surgical candidate due to her prior operations for congenital disease and fontan physiology. Management was deferred to gastroenterology, who recommended small meals and chewing well and proton pump inhibitor therapy for associated heart burn. The patient reported symptomatic improvement with dietary modification. Discussion: The most common alteration in Dysphagia lusoria is an aberrant right subclavian artery. Most patients are asymptomatic; symptoms usually include dysphagia, regurgitation, chest discomfort, weight loss, and Horner syndrome. Diagnosis is best achieved by barium esophagram, which often shows extrinsic compression above the aortic arch. EGD can further show extrinsic compression of the posterior wall of the esophagus. Mildly symptomatic patients are recommended to chew thoroughly, sip liquids, and avoid exacerbating foods. Surgical intervention with the goal of removing the aberrant vessel and reconstructing a more functional vascular system is rarely performed but reserved for severe cases. The significant risk of morbidity and mortality with surgery needs to be weighed in these patients.Figure 1.: X-ray upper gastrointestinal series showing vascular impression of the upper esophagus secondary to aberrant left subclavian artery.

S2406 A Case of Cervical Esophageal Adenocarcinoma Arising From Gastric Inlet Patch: A Benign Lesion With Malignant Potential

Introduction: Esophageal adenocarcinoma is typically localized to the distal third of the esophagus. It is associated with long-standing acid reflux and resultant Barrett’s esophagus. In contrast, adenocarcinoma in the proximal esophagus without Barrett’s metaplasia is extremely rare. A gastric inlet patch (GIP) is a lesion of ectopic gastric mucosa usually found in the cervical esophagus and is considered a benign lesion. However, albeit rare, malignant transformation of GIP can occur. Here, we present a case of cervical esophageal adenocarcinoma arising from gastric inlet patch. Case Description/Methods: A 50-year-old male with GERD presented with a 6-month history of progressive dysphagia to solids and liquids and 20-pound weight loss. He reported a 1 pack-year smoking history but quit 30 years prior and consumed alcohol socially. Family history was negative for esophageal cancer. Upper endoscopy (HQ190F) showed a malignant stricture (2 cm long, 6mm diameter) at 18 cm from the incisors and a gastric inlet patch adjacent to the stricture. The endoscope was downsized (XP190N), and the stricture was dilated (24F) and traversed. The Z-line was at 40 cm with Barrett’s esophagus extending from 36 cm to 40 cm. Pathology of biopsies from the stricture showed acute and chronic inflammation with increased intraepithelial eosinophils suggestive of acid reflux. CT scan of the neck with contrast showed a heterogeneously enhancing ill-defined mass involving the cervical esophagus below the cricoid cartilage indenting the posterior margin of the trachea. Bronchoscopy was negative for bronchogenic cancer. Endoscopic ultrasound showed a non-circumferential hypoechoic mass in the cervical esophagus 18 cm from the incisors extending to 20 cm. The mass was predominantly extrinsic but was also noted to have a luminal component and poorly defined endosonographic borders. Pathology of FNAB showed moderately differentiated adenocarcinoma (Figure). Discussion: Proximal esophageal adenocarcinoma is extremely rare, making up less than 1% of esophageal cancers. Case studies have been published associating GIP and esophageal adenocarcinoma, but the pathogenesis of malignant transformation remains unclear. There are no established screening guidelines for ectopic gastric mucosa and routine biopsies are not recommended as dysplasia within GIP is rare. GIP may be overlooked during EGD and NBI exam may improve detection rates. Our case re-emphasizes careful examination of GIP and to strongly consider biopsy if abnormal.Figure 1.: A) Endoscopic view of malignant stricture 18 cm from incisors. Gastric inlet patch (arrow) adjacent to stricture. B) Endoscopic ultrasound image at the level of esophageal stricture showing irregular hypodensity suggestive of malignancy. C) Pathology of biopsy performed through fine needle aspiration (EUS) of upper esophageal mass demonstrating moderately differentiated adenocarcinoma, cribriform pattern, with mild nuclear pleomorphism, abundant mitoses, and apoptosis (H&E, 10x).

NON-HODGKIN FOLLICULAR LYMPHOMA COMPLICATED BY HISTOPLASMOSIS-INDUCED CHYLOTHORAX AND PNEUMOHYDROPERICARDITIS SECONDARY TO AN ESOPHAGOPERICARDIAL FISTULA

NON-HODGKIN FOLLICULAR LYMPHOMA COMPLICATED BY HISTOPLASMOSIS-INDUCED CHYLOTHORAX AND PNEUMOHYDROPERICARDITIS SECONDARY TO AN ESOPHAGOPERICARDIAL FISTULA

S2419 A Motility Study in a Patient With Isolated Diaphragmatic Agenesis: An Interesting Study in a Very Rare Entity!

Introduction: Diaphragmatic agenesis is a very rare congenital defect. Diagnosis is typically made in infancy and it is commonly associated with other congenital malformations and high mortality. However, it has also been described in the literature as a distinct clinical entity with potentially later in life diagnosis, as in the case reported here. Case Description/Methods: Our subject is a 57-year-old male who was diagnosed to have diaphragmatic agenesis at the age of 45 during a thoracotomy for a suspected diaphragmatic hernia. He presented to the gastroenterology clinic with a chief complaint of life-long intermittent but progressive dysphagia to both solids and liquids. Chest x-ray showed bowel loops and air-fluid level in the left-side of the chest with a cardiac and a mediastinal shift to the right side. Barium swallow was done and was negative for any abnormalities. A motility study was also done and with details showing in Tables 1-4 (Figure). Findings of the motility study are summarized as the following: 1) Normal resting pressure at lower esophageal sphincter with incomplete relaxation during swallows (high IRP), 2) Normal amplitude peristaltic contractions in esophageal body (Normal DCI), 3) Complete bolus transition, 4) Esophagogastric morphology I, and 5) Normal upper esophageal sphincter resting pressure. A chest X-ray obtained and attached in figure A shows the position of the probe used in the esophageal manometry with the gastroesophageal junction in the thoracic cavity. Figure B shows our patient's manometric study evaluating pressure changes that occurred during liquid swallowing. The diagnosis of esophagogastric junction (EGJ) outflow obstruction was made based on study results. Discussion: Esophagogastric junction outflow obstruction is a major motility disorder based on the Chicago Classification of esophageal motility disorders. This entity involves a heterogenous group of underlying etiologies with diaphragmatic agenesis being one potential but rare cause evidenced by our reported case. Management of (EGJ) outflow obstruction is dependent on the underlying etiology.Figure 1.: (Top) Motility study details. A) Chest Xray showing the position of the probe used in the esophageal manometry. B) Patient's manometric study evaluating pressure changes that occurred during liquid swallowing.

S2266 Achalasia as a Sequelae of COVID-19

Introduction: Achalasia is a neurodegenerative motility disorder of distal esophagus and lower esophageal sphincter characterized by loss of myenteric neurons in esophagus. It therefore results in deranged peristalsis and characteristic manifestations of regurgitation, dysphagia and retrosternal chest pressure sensation. Recent studies have suggested inflammatory neurodegenerative insult from viral infection as a potential etiology. Sars-Cov-2 causes multiple gastrointestinal (GI) manifestations like ageusia, nausea, vomiting, pain and diarrhea. We present a rare case of achalasia that presented soon after COVID infection in an otherwise healthy male. Case Description/Methods: A 63-year-old man was referred for further evaluation of dysphagia that started a few months after a non-severe COVID infection with associated ageusia. He reported progressive dysphagia to both solids and liquids with concomitant regurgitation and chest pain. By the time he presented, he had lost 45 lbs over the course of 6 months and had an Eckardt score of 8. High resolution manometry was suggestive of evolving type II achalasia. A follow up Endoflip exam with a 16 cm catheter inflated to 60 cc revealed a esophagogastric diameter of 7.3 mm and a distensibility index (DI) of 1.9. Timed barium esophagogram showed a 5 cm contrast column in distal esophagus at 5 minutes. The patient has since been referred for per oral endoscopic myotomy (Figure). Discussion: Sars-Cov-2 enters the GI epithelial cells through ACE2 receptors and exerts its effects through an aberrant immune response involving T- lymphocytes rather than direct epithelial damage. An anomalous immune reaction resulting in degeneration of inhibitory neurons in the myenteric plexus may be the cause of achalasia in COVID-19. On literature review, we came across at least 1 case report of a patient developing achalasia after COVID-19. A Venezuelan study noted that the frequency of patients with achalasia during the year 2020 and 2021 was far greater than those of previous years and at least 2/3rd of these patients had presented with COVID-19 infection. It will be interesting to note if coronavirus can be isolated from muscle biopsies of these subjects as has been the case with VZV previously. Further investigation especially into patients presenting with GI symptoms with COVID infection is warranted.Figure 1.: Endoflip ; Barium Esophageogram.

S2303 Endoscopic Balloon Dilation of Recurrent Peptic Stricture in a Patient With Portal Hypertension

Introduction: Variceal hemorrhage is a devastating complication and a major cause of mortality in patients with portal hypertension. Unfortunately, one third of patients with varices develop bleeding in their lifetime. This case depicts a rarely reported intervention that physicians may be hesitant to pursue. Case Description/Methods: A 61-year-old male with history significant for atrial fibrillation (afib), alcoholic cirrhosis complicated by gastric and esophageal varices, and GERD presented with dysphagia. He previously underwent endoscopic variceal band ligation (EVL) in 2009 for primary prevention of esophageal varices and placed on daily nadolol. His GERD was controlled with twice daily PPI and as-needed H2 antagonist. Despite this, he developed strictures secondary to severe erosive esophagitis. He was then started on apixaban for afib and transitioned from nadolol to carvedilol. His MELD-Na is 8 and had a reported distant history of hepatic encephalopathy. He presented to the esophageal motility clinic with progressive dysphagia to both solids and liquids. An EGD was scheduled which revealed a partially obstructive ulcerative stricture spanning 29 - 34 cm from incisors. The stricture was injected with 4 ml of triamcinolone acetonide distributed in 1 cm increments across 4 quadrants. Then the stricture was dilated with a 10-12 mm cre balloon under fluoroscopic guidance. Dilation caused shallow mucosal tears with scant blood but otherwise no significant bleeding or complication. The scope was traversed into the stomach with retroflexion revealing moderate gastric varices in the cardia. After the procedure was completed, the patient had complete resolvent of symptoms and tolerated oral nutrition (Figure). Discussion: This case describes a cirrhotic patient with portal hypertension requiring frequent endoscopic balloon dilations for severe dysphagia secondary to recurrent peptic strictures. Extensive literature review did not reveal any documented cases describing this scenario. The presence of gastric varices in this patient is indicative of active portal hypertension. This in combination with the patient’s anticoagulation places a theoretical high risk of bleeding during the procedure. Although this case demonstrates that it is safe to perform serial dilations in patients with portal hypertension if they have undergone EVL and do not have active esophageal varices.Figure 1.: A) EGD showed ulcerative peptic stricture located in the mid-esophagus. (B) There was scant bleeding after dilation, otherwise no significant bleeding or complication. (C) Retroflexion was performed in the stomach and revealed moderate gastric varies in the cardia.

S2490 Discovery of Gastric Adenocarcinoma During PEG Tube Placement in Patient With Epiglottic Squamous Cell Carcinoma

Introduction: Multiple primary neoplasms constitute up to 2-17% of cancer diagnoses. Etiological factors such as genetics, lifestyle choices, and immune status have a significant impact on the likelihood of patients developing synchronous multiple primary tumors. The timing of diagnosis for the different cancers has a major impact on disease management, treatment plans, and patient outcomes. We report a patient who was diagnosed with gastric adenocarcinoma at the time of percutaneous endoscopic gastrostomy (PEG) tube placement for dysphagia secondary to squamous cell carcinoma of the anterior epiglottis. Case Description/Methods: A 77-year-old male presented to the outpatient surgery center for upper endoscopy with PEG tube placement. Two months prior, the patient was diagnosed with p16 negative invasive squamous cell carcinoma of the anterior epiglottis. He was referred for PEG tube placement for nutrition supplementation due to 5 months of progressive dysphagia and protein calorie malnutrition with an unintentional weight loss of 50 lbs. Past social history was significant for tobacco dependence with 52 pack years and alcohol dependence. At the time of PEG tube placement, endoscopy revealed a 1.5 cm excavated lesion at the gastric incisura. Biopsy was performed to rule out malignancy. PEG tube was successfully placed. The gastric biopsy was consistent with diffuse type signet ring gastric adenocarcinoma. PET scan 1 month prior to PEG tube placement did not show any foci of abnormal FDG uptake outside of the primary lesion in the epiglottis. Patient is currently undergoing treatment for laryngeal carcinoma with chemotherapy and radiation. Assessment and treatment for gastric cancer diagnosis will be deferred until completion of treatment for laryngeal carcinoma (Figure). Discussion: Concurrent laryngeal and gastric cancer is a unique diagnosis that has not been well reported in the literature. However, given the strong association for both malignancies with chronic alcohol and tobacco use, it is not unreasonable for both to occur in the same patient in an independent manner. The literature has shown that signet ring cell carcinomas have significantly lower 18F-FDG uptake than other forms of gastric cancer. These findings highlight the importance of completing a full endoscopic evaluation in all patients undergoing endoscopy even for procedures as straightforward as PEG tube placement.Figure 1.: Esophagogastroduodenoscopy showing gastric adenocarcinoma at the gastric incisura.

S2417 A Diagnosis That Is Difficult to Swallow: Digging Deeper for Answers

Introduction: Esophageal parakeratosis (EP) is an uncommon finding with largely unknown clinical implications and malignant potential. Here, we report a case of severe esophageal parakeratosis. Case Description/Methods: Patient is a 69-year-old African American male with history significant alcohol and cocaine abuse, prior tobacco abuse, keloids, gastroesophageal reflux, food impaction, and progressive dysphagia for 20 years with 40-pound weight loss. Patient’s first esophagogastroduodenoscopy (EGD) in 2000 revealed moderate esophagitis and a distal esophageal stricture with 9 mm lumen bougie dilated to 12 mm, but dysphagia persisted. Repeat EGDs between 2006 and 2016 showed whitish plaques with no evidence of fungal organisms. Given classic appearance of EOE, he was trialed on empiric fluticasone and budesonide with no improvement in dysphagia. In September 2021, he was hospitalized with severe dysphagia. EGD (Figure) at this time showed gray, yellow frondlike lesion replacing esophageal lumen from mid to distal esophagus with mucosal friability. His dysphagia transiently responded to repeat dilations, but continued to recur and worsen. Unfortunately, patient continued to have weight loss and dysphagia unresponsive to dilations. The patient was then started on gastric tube feedings. He was offered surgery due to the severity of his symptoms, but he declined. Most recently, the patient underwent endoscopic mucosal resection with deeper tissue samples revealing superficial fragments of laterally spreading squamous cell carcinoma of the esophagus. Discussion: In this case, there was a high clinical suspicion for malignancy, leading the endoscopist to obtain deeper tissue samples via endoscopic mucosal resection as superficial fragments of the esophagus only revealed parakeratosis. It is important to trust clinical judgment, especially if there is a high suspicion for malignancy. Tylosis also has a known association with EP and often presents with areas of thickened skin plaques on palms and feet. Tylosis has a strong association with head, neck, and esophageal squamous cell carcinoma. The patient had no family history of tylosis and no cutaneous features. Despite this, a careful inspection of the entire EP area with targeted biopsies should be obtained to evaluate for dysplasia. This case represents EP with years of no evidence of squamous cell cancer despite aggressively searching for it, which at least anecdotally suggests a precancerous potential for EP.Figure 1.: A. Initial EGD B. Recent EGD.

478. ENDOSCOPIC RESECTION OF A HUGE ESOPHAGEAL LIPOSARCOMA WITH LIGASURE INSTRUMENT

Abstract Esophageal liposarcoma is a very rare pedunculated tumor of esophagus. It may cause progressive dysphagia due to mass effect and sudden death due to tumor suffocation. Surgical resection is challenging due to peduncle usually located at the superior esophageal sphincter region. We introduce a novel approach method that can transect the tumor peduncle safe and easy. Review of the chart of Chimei Medical Center. One 70 year old man with progressive dysphagia for 5 months and body weight loss over 8 KGs. A huge tip necrotic pedunculated tumor was found with endoscopy. Cut off the tumor peduncle with ligasure instrument under endoscopic guide was performed and the tumor was removed successfully. No tumor recurrence was found in 2 years. Esophageal liposarcoma is a rare low grade malignancy of esophagus. Ligasure cut off the tumor peduncle can make a clear cut margin and avoid perforation or stenosis of esophagus.

A novel technique for the treatment of radiation-induced acquired esophageal atresia in patients with head and neck cancer

Dysphagia is a common complaint for patients after radiation therapy for head and neck cancer. Chronic dysphagia ensues when the radiation-induced injury matures into a fibrotic stricture, with the severity of symptoms paralleling the degree of stenosis. Most patients experience progressive dysphagia that prompts medical attention before complete esophageal obliteration. Rarely, patients present late with inability to clear their secretions because of complete obstruction, also termed acquired atresia. These patients represent a challenge and require aggressive and unconventional interventions to reestablish lumenal patency. Using a case series, we hereby describe a novel yet simple technique to treat patients with acquired esophageal atresia. Five patients with head and neck cancer in various stages who all underwent nonsurgical treatment with definitive chemotherapy and radiation along with enteral feeding tube placement prior to/during treatment presented with acquired esophageal atresia. All patients underwent an EGD to reestablish lumenal patency. This was accomplished with gentle pressure that was applied in a to-and-fro semi-circular rotational manner as if pushing a corkscrew and twisting it in alternating clockwise and counterclockwise manner. In all cases we were able to reestablish esophageal lumenal patency with a single procedure. There were no adverse events. Four of the 5 patients required additional dilations for symptomatic management. However, all patients' esophageal lumens remained patent. We describe a novel yet simple technique to treat acquired esophageal atresia after radiation for head and neck cancer. This technique allows for generous dilation yielding complete resolution of the stenosis in a single session. When our approach is used, the patient can be discharged home the same day and resume immediate oral intake.

An Introduction to Home Care for Esophageal Cancer

Esophageal cancer is a common tumor of the digestive tract and is a malignant tumor which affect one’s health and is potentially life threatening. The incidence and mortality rate of esophageal cancer in China is relatively high, with the incidence rate being the sixth highest and the mortality rate being the fourth highest among all malignant tumors. The lesion often occurs in the middle segment of the epithelium of the esophagus, often showing a family tendency, and is more likely to occur in middle-aged and elderly men. The typical symptom of esophageal cancer is progressive dysphagia, first with dry food, then with semi-liquid food, and finally with water and saliva. Treatment is divided into surgical treatment, radiotherapy, chemotherapy, and combination therapy. Apart from malnutrition and pain, the treatment can also cause great psychological stress to patients with esophageal cancer. Therefore, diet, pain, and psychological care for patients with esophageal cancer are crucial. Therefore, it more care is given to the patients by medical and nursing staff during hospitalization. However, not much have been discussed about home care for patients with esophageal cancer. In this article, we will talk about the home care of esophageal cancer patients from the following aspects: dietary, pain management, and psychological care.

A case study of combined neoadjuvant chemotherapy and neoadjuvant immunotherapy in resectable locally advanced esophageal cancer

BackgroundThe prognosis of patients under existing neoadjuvant chemotherapy or neoadjuvant chemoradiotherapy requires improvement. Whereas programmed cell death 1 (PD-1) inhibitors have shown promising response in advanced esophageal cancer, they have not been used in the perioperative treatment of resectable locally advanced esophageal cancer. Whether immunotherapy can be incorporated into neoadjuvant therapy has became a challenging question for researchers.Case presentationWe present a case of a 65-year-old male who had a history of progressive dysphagia for approximately 1 month. He underwent pertinent studies including computed tomography (CT)，gastroscopy，and pathological biopsy resulting in a diagnosis of medium-low differentiated squamous carcinoma of the thoracic segment of the esophagus (cT2N2M0 stage III). After 4 cycles of neoadjuvant chemotherapy combined with immunotherapy, gastroscopy showed the lesion in the esophagus was no longer present. Subsequently, the patient received thoracoscopic radical resection of esophageal cancer and achieved a pathological complete response (pCR) in postoperative pathological evaluation. During the whole treatment, no adverse effect was recorded and to date no evidence of recurrence has been recorded.ConclusionOur report suggest that neoadjuvant chemotherapy combined with immunotherapy not only improve the R0 resection and pCR rate in patients with resectable locally advanced esophageal cancer, but also the adverse effects are within the control range. However, the selection of therapeutic strategy, predictors of response to treatment, and interval time between neoadjuvant treatment and surgery still await more reliable evidence-based studies with large prospective samples.

Inclusion body myositis in an older patient

Inclusion body myositis (IBM) is a condition also referred to as sporadic IBM. It is a rare variant of a broader group of diseases described under the banner of inflammatory myositis. In general, myositis/myositides tend to present with a varying cluster of symptoms such as muscular weakness, aches and/or pain. Myositis has been linked to multiple causes or triggers, but generally share the common feature (present to a variable extent) of inflammatory changes within skeletal muscle tissue. In addition, some associations (e.g. Sjogren’s disease and systemic lupus erythematosus – SLE) have been reported for IBM. Some medication classes (e.g. statins and fibrates) may present a risk to certain individuals developing drug-associated myopathies. IBM can be challenging to diagnose, and may be mistaken for many other causes of muscle weakness e.g. polymyositis. Furthermore, IBM tends to affect muscles asymmetrically, and runs a typically progressive and chronic course. Consequently, over time IBM may result in significant functional impairment, activity limitation, and participation restriction. This case report describes an older woman with a clinical diagnosis of probable IBM complicated with oesophageal dysmotility. She presented to hospital with progressive dysphagia, breathlessness, and a productive cough for which treatment was started for aspiration pneumonia. The report considers some broader ‘principles of management’ of a chronic myositis. In the current absence of a definitive curative treatment, we also discuss some realistic and practical pharmacological treatment options that may be used for the selective care of patients presenting with an incurable chronic myositis such as IBM.

DIFFUSE LEIOMYOMATOSIS OF THE ESOPHAGUS – A RARE CAUSE OF PEDIATRIC PSEUDOACHALASIA.

Achalasia is an esophageal motility disorder with progressive dysphagia for liquids and solids. It occurs due to absence or degeneration of myenteric plexus of nerves in the distal esophagus. The term pseudoachalasia refers to achalasia like symptoms and imaging manifestations caused by diseases other than aganglionosis [1]. It can be due to stricture, extrinsic compression, malignant neoplasm or proliferation of smooth muscle cells as in leiomyomatosis. Diffuse leiomyomatosis of the esophagus is a very rare cause of pseudoachalasia and requires total esophagectomy with gastric pull through surgery. We present a rare case of a seven year old boy with diffuse leiomyomatosis of esophagus presenting with pseudoachalasia, aspiration pneumonitis and bronchiectasis secondary to recurrent aspirations.

A Rare Cause of Dysphagia: Diagnosis and Treatment

Question: A 55-year-old woman presented to our hospital with intermittent dysphagia for 2 months and progressive dysphagia for 44 days. A preliminary computed tomography scan had been performed at an outside hospital and revealed circumferential thickening of the middle and distal esophageal wall, and no thoracic neoplasm outside of the esophagus.

Progressive dysphagia caused by IgG4-related sclerosing disease involving the esophagogastric junction

A 46-year-old man presented at our clinic because of intermittent dysphagia sensations for 1 year, and this symptom had progressed in the recent 2 months. EGD showed a protruding mass with a lobulated surface and firm density, causing a stenotic lumen at the esophagogastric junction (A). The initial pathologic examination of the biopsy specimen showed ulcerated mucosa with granulation tissue and leukocytic infiltration. Repeated EGD was performed to obtain multiple biopsy specimens, and no evidence of malignancy or specific pathogens such as viral and fungal infection was identified by serial stains. Because of the suspicion of esophageal cancer, 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET)/CT was arranged and showed increased FDG uptake at the cardiac portion of the stomach and distal end of the lower esophagus (B). Because of progressive dysphagia and a concern regarding malignancy, esophagectomy was performed, and multiple nodular lesions with fibrotic changes at the esophagogastric junction were found (C). Immunostaining showed increased IgG4-positive plasma cells, finding ≤225 per high-power field, with a 50.2% IgG4+/IgG+ plasma cell ratio (IgG4 stain, orig. mag. ×200) (D). These findings were consistent with IgG4-related sclerosing disease involving the esophagogastric junction.