Diagnosis and Management of Giant Esophageal Fibrovascular Polyp With Hypopharyngeal Pedicle

Background: Fibrovascular polyps (FVPs) with hypopharyngeal pedicles (hFVPs) are the rare intraluminal benign tumours of the upper aerodigestive tract, and their accurate diagnosis and optimal management are challenging. Purpose: The present retrospective study attempted to explore the optimal diagnosis and treatment of hFVPs. Research Design: The clinical records of 2 patients with giant, irregularly shaped hFVPs, who underwent several failed surgical procedures after inaccurate diagnosis, were reviewed. Finally, the patients were correctly diagnosed and successfully treated at Capital Medical University Beijing Friendship Hospital in different years, 2018 and 2020. Results: Case 1 was of a 43-year-old woman with 2 months of progressive dysphagia. Gastroenterologists overlooked the origin of her FVP, and decided to sever its narrowest point in the oesophagus through endoscopy. However, upon unsuccessful removal of the mass, a gastrotomy procedure was performed to extract the mass 7 days later. Symptoms recurred 3 months after the treatment, and a fibreoptic laryngoscopy confirmed hFVP in the patient at our department. A transcervical approach was used to sever the hypopharyngeal pedicle, achieve haemostasis and remove the oesophageal tumour. No recurrence was detected during the 2-year follow-up period after the treatment. Case 2 was of a 32-year-old man with dysphagia who had previously undergone transthoracic and transcervical oesophagotomy procedures within a gap of 3 months for the removal of FVP causing dysphagia. The hypopharyngeal pedicle was not diagnosed in the patient. The symptoms of dysphagia recurred 4 years after the treatment, and a fibreoptic laryngoscope confirmed hFVP at our department. The tumour was removed successfully through the transcervical approach. No recurrence was detected during the 6-months follow-up after surgery. Conclusion: In conclusion, the transcervical approach is suitable for achieving haemostasis and removing giant, irregularly shaped hFVPs.

Multiple Esophageal Leiomyoma Presenting with Clinical Dysphagia from Mechanical Obstruction and Motility Disorder

Esophageal leiomyoma is uncommon. However, this tumor is the most common subepithelial tumor affecting the esophagus, comprising approximately two-thirds of benign esophageal tumors. Leiomyomas of the esophagus rarely cause symptoms when they are single and <5 cm. The mainstay of treatment is esophagectomy for symptomatic patients. A 68-year-old male patient presented with progressive dysphagia for 4 months. The degree of dysphagia and chest discomfort was more severe on solid rather than liquid diet. The CT scan of the chest showed multiple well-defined, submucosal nodules, up to 1.9 cm in diameter located at the middle esophagus. The barium swallow study illustrated multiple, well-defined, smooth, semilunar filling defects along the mid to distal esophagus. Meanwhile, esophagogastroduodenoscopy revealed 8 smooth subepithelial masses. Moreover, the radial EUS showed multiple hypoechoic masses arising from the 4th layer, with some of the tumors connected to others as a horseshoe-like shape causing narrowed lumen. Last, high-resolution esophageal manometry revealed ineffective esophageal motility. We report a rare case of numerous esophageal leiomyomas which caused dysphagia as a result of both mechanical obstruction and hypomotility disorder. The histopathology confirmed the diagnosis of esophageal leiomyoma. Symptoms improved significantly after lifestyle modifications and adherence to dietary advice on the part of the patient.

Elderly-Onset Multiple System Atrophy with Lewy Body Pathology: A Case Report

An 81-year-old woman presented with a 2-year history of progressive dysarthria and gait disturbance. Subsequently, she developed orthostatic hypotension, obstructive sleep apnea, right-sided resting tremor, and rigidity. Together with characteristic findings of imaging studies, she was diagnosed with multiple system atrophy (MSA). Despite progressive dysphagia and repeated choking episodes, the patient elected not to use artificial feeding or tracheostomy. She died suddenly at age 91 after 12 years of illness. The autopsy revealed neuropathological features of both MSA and of Parkinson’s disease. The peripheral autonomic ganglia revealed both pre- and postganglionic involvement by synucleinopathy, which may have underscored the sudden death of the patient. The patient survived 10 years after onset, despite the presence of multiple poor prognostic factors in MSA including the onset of old age and early appearance of orthostatic hypotension and falls, in addition to the complication of PD pathology found by autopsy. Multidisciplinary team approach and her preserved cognitive function may have been contributory to the long-term survival.

753 SCARLESS APPROACH FOR KILLIAN JAMIESON DIVERTICULUM: TRANSORAL ENDOSCOPIC ASSISTED ESOPHAGODIVERTICULOSTOMY

Killian-Jamieson Diverticulum (KJD) is a rare variant of proximal esophageal diverticulum that arises from the Killian-Jamieson area (antero-lateral). It differs from the more common Zenkers diverticulum which usually arise from the posterior aspect of the proximal esophagus. The choice of surgical intervention for symptomatic KJD is still unclear due its rare incidence and surgeons still prefer the transcutaneous cervical approach. Here we present the transoral endoscopic assisted esophagodiverticulostomy approach and the relevant literature. Methods 26 year old male presented with progressive dysphagia for the past two years. A Killian-Jamieson Diverticulum was diagnosed after an Oesophagogastroduodenoscopy and Barium swallow. Patient underwent a transoral endoscopic assisted esophagodiverticulostomy. Results The surgery was uneventful and no complications were observed post-operatively. A year after the surgery patient is asymptomatic and no sign of recurrence. Conclusion Transoral endoscopic assisted esophagodiverticulostomy is a safe approach for treatment of symptomatic KJD. In this era of minimally invasive surgery it should be considered when formulating a treatment approach based on its obvious advantages.

Megaesôfago idiopático: relato de caso

o objetivo foi relatar um caso de megaesôfago idiopático que foi tratado por cardiomiotomia videolaparoscópica a Heller-Pinotti em um paciente masculino, de 29 anos. Foi realizada triagem inicial com anamnese e exame físico e o paciente alegou disfagia progressiva sem alterações dos hábitos intestinais associadas; exame físico abdominal sem alterações. A investigação diagnóstica laboratorial incluiu tireoideopatia, doença de Chagas e doença do refluxo gastroesofágico. Após extensa investigação foi detectada acalasia (confirmada por esofagomanometria) idiopática, sendo assim realizada a cardiomiotomia videolaparoscópica a Heller-Pinotti. Posteriormente ao tratamento cirúrgico, o paciente apresentou boa evolução pós-operatória, não havendo queixas de pirose ou regurgitação e desaparecendo a queixa de disfagia. Declara-se boa experiência com o tratamento cirúrgico proposto, no entanto, novos estudos se tornam necessários para aprimoramento da técnica cirúrgica e consequente maior benefício para o paciente com acalasia idiopática. the goal was to report a case of idiopathic achalasia that was treated by Heller-Pinotti’s videolaparoscopic cardiomyotomy in a male patient of 29 years. The initial assessment was done with clinical history and physical examination and the patient claimed progressive dysphagia without changes in bowel habits associated; abdominal physical examination without abnormalities. Laboratory diagnostic investigation included thyroidopathy, Chagas’ disease and gastroesophageal reflux disease. After extensive research, it was detected idiopathic achalasia (confirmed by esophageal manometry) and Heller- Pinotti’s videolaparoscopic cardiomyotomy was realized. After surgery, the patient presented good postoperative evolution, with no complaints of heartburn or regurgitation and the dysphagia complaint had gone. We can declare good experience with the proposed surgical treatment, however, further studies are needed to make improvements in surgical technique and consequent greater benefit to the patient with idiopathic achalasia.

Facial-onset sensory-motor neuronopathy, a rare variant of Huntington’s disease or chance association?

Objectives: To describe a patient with facial-onset sensory-motor neuronopathy (FOSMN) that later developed Huntington’s disease (HD). Case report: A 62-year-old woman complained of progressive dysphagia 8 years before referral. At initial evaluation, there was excessive salivation, dysphagia, and sensory-motor trigeminal impairment. Denervation was noted on the upper limbs and the tongue. Blink reflexes were abolished. Genetic study of amyotrophic lateral sclerosis (ALS)-related genes was normal. She was diagnosed with FOSMN syndrome. Her clinical state progressively worsened with corneal anesthesia, severe denutrition, right arm and axial weakness. Seven years after referral, she was unable walk and developed generalized chorea. Abnormal huntingtin gene repeat expansion confirmed the diagnosis of HD. She died 16 years after onset of dysphagia. Conclusion: Cases with both HD and ALS have already been reported but not FOSMN and HD, to our knowledge. Some FOSMN cases have been linked to ALS-related gene mutations and HD phenocopies have been associated with C9ORF72 repeat expansions. Recently, huntingtin repeat expansions were described in the ALS population. Although a chance association cannot be excluded, data from the literature are in favor of a pathogenic relationship between FOSMN and HD in this particular case. We suggest that huntingtin gene be more systematically studied in patients with FOSMN.

Use of Supraclavicular Flap by End to Side Technique in Pharyngeal SCC: A Case Report and Review of Literature

Objectives. In recent years, conservation laryngeal surgeries, including partial pharyngectomy, have been introduced as an alternative procedure for selected cases of hypopharyngeal squamous cell carcinoma (HSCC). Reconstruction of these defects presents a considerable challenge for the surgeon after partial pharyngectomy due to its circumferential nature. In this case report, we represent the innovative “End to side” technique to reconstruct hypopharyngeal defect using the rolled supraclavicular flap after laryngeal-preserving partial pharyngectomy. Methods and Results. A 70-year-old female presented with a history of progressive dysphagia and odynophagia. The evaluations revealed a T3N0M0 SCC of pyriform sinus. The mass was successfully resected through partial pharyngectomy, and the hypopharyngeal defect reconstruction was achieved using the rolled supraclavicular flap via the “End to side” technique. The patient was discharged after decannulation on day 10. The 3-week barium swallow was performed with no evidence of anastomotic leakage, and the oral feeding was started after NG tube removal. At week 5, complete movement of the true vocal cord on the one side and good phonation and deglutition was observed. There was no evidence of recurrence after 1 year. Conclusions. Laryngeal-preserving partial pharyngectomy and hypopharyngeal reconstruction with the rolled supraclavicular flap via the “End to side” technique could lead to good oncological and functional outcomes in selected cases of pyriform sinus.