Long-term outcomes of per-oral endoscopic myotomy in a patient with distal esophageal spasm followed up for 2.5 years: two sides of the same coin - A Case Report and Literature Review.

INTRODUCTION: Distal esophageal spasm (DES), a relatively rare condition, is characterized by simultaneous contractions of the distal esophagus and manifested by dysphagia and chest pain. Several treatment options are recommended, such as pharmacological therapy, endoscopic interventions, and surgical myotomy. Recently, per-oral endoscopic myotomy (POEM) has been adopted as an effective and less-invasive treatment due to its excellent short-term clinical outcomes. Nevertheless, few reports describe its long-term effects. CASE PRESENTATION: A 65-year-old woman complained of chest pain accompanied with dysphagia and weight loss for 4 months. A series of examinations suggested that she was suffering from DES and then POEM was performed. During the 2.5-year follow-up, we observed an exciting long-term outcome. Interestingly, hematoxylin and eosin staining verified a large number of eosinophils in the muscularis externa, which was absent in the mucosa of the esophagus of the patient. CONCLUSIONS: We herein report a case of DES who underwent POEM to eliminate persistent esophageal contractions. Eckardt score, esophageal emptying test, and high-resolution manometry were assumed to monitor the efficacy of POEM. During treatment and 2.5 years after operation, esophageal muscle biopsies and/or mucosal tissues were obtained. This case has been presented to describe that POEM could be a strategy for DES with a long-term curative effect and that eosinophils in the muscle layer of the esophagus might be involved in the pathogenesis of DES. What is more, we reviewed literature to find similar cases reported in the past.

Challenges of peroral endoscopic myotomy in the treatment of distal esophageal spasm.

OBJECTIVE: Distal esophageal spasm (DES) is a rare motility disorder characterized by premature and rapidly propagated contractions of the distal esophagus. Treatment options are limited and often poorly effective. Peroral endoscopic myotomy (POEM) seems an effective and attractive new treatment option for DES. In this case report we describe some of the difficulties that could arise. MATERIALS AND METHODS: A 84-year old man with therapy-refractory DES and complaints of severe dysphagia and chest pain underwent a POEM procedure under general anesthesia. A longer myotomy was performed to cleave the circular muscle layer from start till end of the spastic contractions. RESULTS: The length of the myotomy was 16 cm. Hyperactive spastic contractions during the procedure complicated the creation of the submucosal tunnel, extended the duration (134 vs. 60-90 min for achalasia), increased postoperative pain and prolonged hospital admission. Intravenously nitroglycerin peroperative diminished spastic contractions. Postoperative a remnant of spastic contractions was present, proximal to the myotomy, causing persistent symptoms. CONCLUSION: Performing POEM for DES is challenging due to reactive hyperactive spastic contractions during the procedure causing technical difficulties and an extended procedure. A long myotomy, several centimeters above the proximal border of the spastic region, is essential to prevent remnants of spasticity.

Diffuse esophageal spasm: CT findings in seven patients.

OBJECTIVE: The purpose of this study was to determine the frequency and appearance of esophageal wall thickening on CT scans in a series of patients with findings of diffuse esophageal spasm on barium studies. CONCLUSION: CT revealed marked esophageal wall thickening in seven (21%) of 33 patients who had findings of diffuse esophageal spasm on barium studies. CT showed significantly greater esophageal wall thickening in the lower thoracic esophagus 5 cm above the gastroesophageal junction than in the upper thoracic esophagus at the level of the aortic arch or in the midthoracic esophagus at the level of the carina (p < 0.01). This esophageal wall thickening corresponded to the presence of multiple strong nonperistaltic contractions in the lower thoracic esophagus on barium studies. Our findings suggest that diffuse esophageal spasm should be included in the differential diagnosis when CT shows smooth circumferential wall thickening in the lower half of the thoracic esophagus, particularly in elderly patients with dysphagia or chest pain.

[Diffuse esophageal spasm (DES). Practical concepts of diagnosis and treatment]. / El espasmo esofágico difuso (EED). Conceptos prácticos sobre diagnóstico y tratamiento.

Diffuse esophageal spasm (DES) is a motility disorder of undetermined etiology, typically presenting with chest pain, dysphagia or both. The aim of this paper is to provide a critical review of the prevalence, pathogenesis, diagnosis and therapy of DES. Data from referral centers indicates that this is a rare disorder with a prevalence of 4-7%. The diagnosis is based on the combination of typical symptoms, radiological findings and manometry (simultaneous contractions (SC) in the distal esophagus in > or = 20% of wet swallows mixed with normal peristalsis). The pathogenesis remains poorly understood. Recent evidence suggests that nitric oxide deficiency may explain the SC that characterizes this condition at manometry. Gastroesophageal reflux (GER) can coexist in DES and GER has also been implied in the pathogenesis of DES. Whether patients with DES and GER represent a subtype of DES with a different prognosis or outcome is unknown. We present a critical appraisal regarding different therapeutic approaches available for DES and conclude suggesting a management algorithm based on current available literature.

Corkscrew esophagus.

Corkscrew esophagus (also referred as rosary bead esophagus) is a classic finding of diffuse esophageal spasm (DES) in barium studies reflecting abnormal contractions, leading to compartmentalization and curling of the esophagus, ultimately giving an appearance similar to a corkscrew or rosary beads. We review the pathophysiology of this finding, correlating it to corkscrew and rosary images that originated this classic description.

A case of seropositive autoimmune autonomic ganglionopathy with diffuse esophageal spasm.

Autoimmune autonomic ganglionopathy (AAG) is an immune-mediated disorder that leads to various autonomic failures associated with anti-ganglionic acetylcholine receptor antibodies (anti-gAChR-Abs). Diffuse esophageal spasm (DES) is an uncommon esophageal motility disorder. We herein report the case of a 68-year-old woman with DES as a partial symptom of AAG. She presented with chronic esophageal transit failure, constipation, and numbness of the hands and feet, Adie's pupil, thermal hypoalgesia, and decreased deep tendon reflexes. Right sural nerve biopsy showed significantly decreased numbers of small myelinated fibers. Barium swallowing X-ray showed repetitive simultaneous contractions indicating DES in the esophagus. Gastrointestinal endoscopy and CT image showed a dilated esophageal lumen and liquid effusion. Simultaneously, serum anti-gAChR-α3-Ab indicating AAG was detected. After pulse intravenous methylprednisolone (IVMP) and intravenous immunoglobulin therapy (IVIg), the bolus progression and liquid effusion improved, suggesting that DES is an important gastrointestinal symptom of AAG.

Manometría de alta resolución en pacientes con trastornos motores esofágicos / High resolution manometry in patients with esophageal motor disorders

Introducción: La manometría de alta resolución es la prueba de referencia para el estudio de los trastornos motores esofágicos. Objetivo: Determinar la factibilidad de la manometría de alta resolución para el estudio de las características de los trastornos motores esofágicos. Métodos: Se realizó una investigación descriptiva, transversal, en el Centro Nacional de Cirugía de Mínimo Acceso, entre septiembre de 2018 y diciembre de 2019, en 56 pacientes cubanos con diagnóstico de trastorno motor esofágico por manometría de alta resolución, con edades entre 18 y 80 años, que dieron su consentimiento para participar en el estudio. Se excluyeron pacientes con acalasia esofágica y los trastornos menores de la peristalsis. Las variables incluidas fueron: edad, sexo, diagnósticos manométricos y sus características, síntomas, tipos de unión esofagogástrica, diagnósticos imagenológicos o endoscópicos. Para el análisis de los resultados se empleó el porcentaje, medidas de tendencia central y ji cuadrado de Pearson de homogeneidad, con un nivel de significación p 8804; 0,05 y 95 por ciento de confiabilidad. Resultados: Predominó la contractilidad ausente (39,28 por ciento), el sexo femenino (58,9 por ciento) y la disfagia (66,07 por ciento). A la obstrucción al flujo de la unión esofagogástrica correspondió la media de presión de reposo del esfínter esofágico inferior más alta (43,28 mmHg) y la media de la presión de relación integrada por encima de 15 mmHg (38,88 mmHg). El esófago hipercontráctil presentó media de contractilidad distal integrada elevada (5564,25 mmHg/s/cm). Se comprobó la existencia de contracciones rápidas en el espasmo esofágico distal (media de 21,4 cm/ s). Conclusiones: La manometría de alta resolución es factible de ser empleada para el diagnóstico de los trastornos motores esofágicos(AU)

Introduction: High-resolution manometry is the gold standard for the study of esophageal motor disorders. Objective: A descriptive, cross-sectional research was carried out in el Centro Nacional de Cirugía de Mínimo Acceso, between September 2018 and December 2019, in 56 patients, diagnosed with esophageal motor disorder by high-resolution manometry, aged between 18 and 20 years, who gave their consent to participate in the study. Esophageal achalasia and minor peristalsis disorders were excluded. The variables included were: age, sex, manometric diagnoses and their characteristics, symptoms, types of esophagogastric junction, imaging or endoscopic diagnoses. For the analysis of the results, the percentage, measures of central tendency and Pearson's chi square of homogeneity were used, with a level of statistical significance 8804; 0.05 and 95 percent reliability. Development: Absent contractility (39,28 percent), female sex (58,9 percent) and dysphagia (66,07 percent) predominated. The obstruction to the flow of the esophagogastric junction corresponded to the highest mean resting pressure of the lower esophageal sphincter (43,28 mmHg) and the mean integrated pressure ratio above 15 mmHg (38,88 mmHg). The hypercontractile esophagus presented mean high integrated distal contractility (5564,25 mmHg/s/cm). Rapid contractions were found in distal esophageal spasm (mean 21,4 cm/s). Conclusions: High resolution manometry was feasible to be used for the diagnosis of major esophageal motor disorders(AU)

Transient failure of opening of the lower oesophageal sphincter on upright oesophagrams: radiographic and clinical findings.

The purpose of our study was to determine the frequency, radiographic features, and clinical importance of transient failure of opening of the lower oesophageal sphincter (LOS) on upright double-contrast views of the oesophagus. A computerized search of radiology records identified 16 patients who had transient failure of opening of the LOS on upright views from biphasic oesophagrams or upper gastrointestinal tract examinations using high-density barium but normal opening of the LOS on prone views using low-density barium. The radiographic findings were reviewed and correlated with the clinical and manometric findings. In all cases, barium studies revealed tapered, beaklike narrowing of the distal oesophagus on upright double-contrast views, with a normal-appearing distal oesophagus, normal opening of the LOS, and intact peristalsis on prone single-contrast views. Only seven patients (44%) had dysphagia. Five of these patients had clinical follow-up, and the dysphagia improved or resolved without specific treatment for LOS dysfunction in four. The remaining patient had persistent dysphagia, but this individual had polymyositis as the likely cause for his dysphagia. Manometry revealed incomplete relaxation of the LOS in two patients and normal relaxation in one. Our experience suggests that failure of opening of the LOS may be observed as a transient finding of little clinical importance on upright double-contrast views of the oesophagus using high-density barium, with normal opening of the LOS on prone single-contrast views using low-density barium. It is important to be aware of this finding, so that it is not mistaken for achalasia or other abnormalities of the distal oesophagus.

Using botulinum toxin A to improve speech and swallowing function following total laryngectomy.

OBJECTIVE: To evaluate a technique to reduce dysfunctional spasm in the pharyngoesophageal segment (PES) in patients after laryngectomy. DESIGN: Pharyngoesophageal segment function related to voice and/or swallowing in patients who had undergone a laryngectomy was evaluated before and after the injection of botulinum toxin A. SETTING: Academic referral medical center. PATIENTS: Eight outpatients with voice and/or swallowing complaints after undergoing a total laryngectomy. INTERVENTIONS: Videofluoroscopic contrast examination was completed to identify stricture vs spasm in the PES in patients with voice and/or swallowing complaints after undergoing a laryngectomy. Lidocaine hydrochloride injection under fluoroscopic guidance was completed to facilitate immediate relaxation of spasm. After positive results with lidocaine, botulinum toxin was injected into the same area to facilitate longer-lasting benefit. MAIN OUTCOME MEASURE: Patient report of benefit and videofluoroscopic evaluation of PES function. RESULTS: Six of 8 patients demonstrated improved function within the PES after lidocaine injection. Five of these 6 received transcutaneous injection of botulinum toxin. Four of the 5 patients demonstrated improved swallowing and/or voice function, and 3 of these 4 received subsequent injections of botulinum. No serious complications were encountered. CONCLUSIONS: Transcutaneous injection of botulinum toxin in the PES under videofluoroscopic guidance provides improvement in voice and/or swallowing function without significant complications. Additional clinical study will be required to evaluate dose and technique influences on degree and duration of benefit and complications.

Cricopharyngeal dysfunction.

The aim of the study was to determine the manometric patterns in dysphagic patients with radiologic evidence of upper esophageal sphincter (UES) dysfunction. Nineteen patients with radiographic abnormalities of the UES underwent measurement of several parameters of UES tonic pressure and pharyngoesophageal water swallow dynamics. At least two UES tonic pressures were elevated in six subjects, compared with a control group of 67 healthy volunteers. No patients had UES achalasia. The cricopharyngeal impression in the remaining patients may represent muscular hypertrophy or deficiency of UES opening, despite manometric relaxation, but its relationship to the patient's symptoms remains unknown. Cricopharyngeal myotomy appears to be a reasonable treatment for patients with manometric UES hypertonicity.

Cineradiography of the pharyngoesophageal segment in postlaryngectomy patients.

The use of tracheoesophageal voice prostheses has gained wide acceptance in the field of vocal rehabilitation after total laryngectomy. In a randomized study with 3 arms, alaryngeal speech proficiency was assessed in 60 postlaryngectomy patients: 20 patients underwent primary unilateral pharyngeal myotomy, 21 patients underwent neurectomy of the pharyngeal plexus in addition to pharyngeal myotomy, and 19 patients did not undergo an additional surgical procedure. Pharyngoesophageal (PE) dynamics were examined during esophageal and tracheoesophageal speech. A single vibrating PE segment was seen in good alaryngeal speakers. Hypertonicity, spasm, strictures, and hypotonicity of the PE segment were correlated significantly with poor or moderate alaryngeal speech. Unilateral myotomy with or without unilateral neurectomy prevented hypertonicity or spasm of the PE segment. The acquisition of alaryngeal speech did not differ significantly between the 2 groups who had undergone an additional surgical procedure. Evaluation of anatomic and physiological factors may be helpful in subsequent clinical management to achieve effective alaryngeal speech.

[Use of botulin toxin in the treatment of diffuse esophageal spasm. A case report]. / Emprego da toxina botulínica no tratamento do espasmo difuso esofagiano. Relato de caso.

The diffuse esophageal spasm distinguishes from others esophageal disturbs by the symptoms severity and bad results to conservative treatment. This situation requires formal indication to surgical treatment or dilation. In high risk patients this situation becomes a big problem, due to the lack of another therapeutic option. In a patient with this problem we use an endoscopic injection of botulin toxin in cardia with good results in three months follow-up. Despite this short period of observation we believe that the infiltration with botulin toxin in cardia constitutes a valid therapeutic option in treatment of diffuse esophageal spasm.

[Dynamic scintigraphy of the esophagus--a method for esophageal dysfunction]. / Cintilografia dinâmica do esôfago--um método para disfunção esofagiana.

The authors present their experience with radionuclide transit in the study of esophageal motility using a very simple and easy technique. They have established a normal pattern and analyse their findings in achalasia and probable diffuse esophageal spasm. They review the literature and submit the method as a very important tool for the diagnosis of esophageal motor disorders.

Symptomatic improvement of diffuse esophageal spasm after botulinum toxin injection.

Diffuse esophageal spasm, an uncommon esophageal motility disorder, has recently been defined using high-resolution manometry. Patients with distal esophageal spasm usually complain of chest pain or dysphagia. The etiology and pathophysiology of this disorder are poorly known, and treatment options are limited. However, some options to improve symptoms are available, including endoscopic injection of botulinum toxin. Nevertheless, few reports have described the effects of endoscopic injection of botulinum toxin in patients with symptomatic diffuse esophageal spasm with clear endoscopic and high-resolution manometry images. Here, we report a case of diffuse esophageal spasm diagnosed with high-resolution manometry and treated by endoscopic injection of botulinum toxin with good results at the 7-month follow-up.