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CT scan of chest showing dilatation of esophagus with air-fluid level. Dilated esophagus is compressing the trachea anteriorly.

CT scan of chest showing dilatation of esophagus with air-fluid level. Dilated esophagus is compressing the trachea anteriorly.

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Achalasia is a rare motility disorder of the esophagus which results from lack of enervation of the lower esophageal sphincter muscles and leads to dilatation of proximal esophagus. Patients with achalasia presents typically with dysphagia, vomiting of undigested food and failure to thrive. Cough can be present in achalasia patients due to aspirati...

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... However, respiratory symptoms such as chronic cough and wheezing may be seen [22]. In conditions such as achalasia, respiratory compromise may be caused by tracheal compression due to a dilated esophagus [24]. Non-specific esophageal motility disorders are characterized by abnormalities in esophageal contraction that are not well-defined and likely comprise a large proportion of the esophageal motility disorders in aerodigestive patients. ...
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Purpose of Review This review provides a comprehensive overview of the diagnosis and management of common gastrointestinal conditions found in pediatric patients with aerodigestive disorders. In particular, this review will address the impact of these gastrointestinal conditions on the airway and its contribution to respiratory disease. Recent Findings Multidisciplinary programs help to optimize management for medically complex aerodigestive pediatric patients with gastrointestinal disorders. Summary Aerodigestive patients are susceptible to multiple gastrointestinal conditions which may worsen pulmonary function. Timely diagnosis and treatment of these conditions have improved respiratory outcomes and decreased complications in this patient population.
... Mehdi et al 6 had reported two cases of achalasia which presented as chronic nocturnal cough and were treated for a long time as respiratory diseases before being diagnosed correctly. 6 Dabritz et al 7 had also found two cases of achalasia, where dysphagia, chest pain, and regurgitation were not predominant symptoms and patients were being treated as eating disorder. 7 Wani et al 8 had reported a achalasia patient who presented with massive haemoptysis and was initially treated as pulmonary tuberculosis but diagnosed as achalasia associated with Actinomycosis later on. ...
... Algunas causas inusuales de tos crónica observadas en nuestro Servicio son: acalasia: Alteración de la motilidad esofágica debido a la ausencia de inervación muscular del esfínter esofágico inferior determinando dilatación del esófago proximal. Observamos dos casos en quienes la tos crónica era consecuencia de la compresión traqueal secundaria a la dilatación del esófago (6) . ...
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La tos crónica es una causa frecuente de consulta a nivel ambulatorio. Es motivo de angustia para pacientes y padres, y en algunos casos se resuelve espontáneamente, sin lograr precisar su etiología. El Profesor Miles Weinberger destaca la importancia de realizar una acuciosa historia clínica para caracterizar la tos y su signología asociada, lo que permite identificar patrones típicos de algunas enfermedades.
... E sophageal achalasia is a primary esophageal motility disorder characterized by lack of esophageal peristalsis and partial or absent relaxation of the lower esophageal sphincter (LES) in response to swallowing. 1 As a result of the functional obstruction at the level of the gastroesophageal junction, the emptying of food from the esophagus into the stomach is hindered and patients experience dysphagia and regurgitation. 2 While in the past it was thought that achalasia rarely caused respiratory symptoms, today there is mounting evidence suggesting that cough, wheezing, hoarseness, and pneumonia are frequently present, and they are probably caused by episodes of micro-or macroaspiration of the retained food and secretions in the esophagus. [3][4][5][6][7][8][9][10][11][12][13][14] The goal of our study was to determine: (1) what percentage of achalasia patients experience respiratory symptoms; (2) what factors play a role in the genesis of these symptoms; and (3) the effect of Heller myotomy on dysphagia, regurgitation, and respiratory symptoms. ...
... The second mechanism is instead micro-or macroaspiration of esophageal contents into the tracheobronchial tree. [3][4][5][6][7][11][12][13][14] If this is indeed the main cause, either pneumatic dilatation or Heller myotomy should improve or resolve these symptoms by eliminating the functional obstruction at the level of the gastroesophageal junction and improving esophageal emptying. ...
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Background: Dysphagia and regurgitation are considered typical symptoms of achalasia. However, there is mounting evidence that some achalasia patients may also experience respiratory symptoms such as cough, wheezing, and hoarseness. Aims: The aims of this study were to determine: (1) what percentage of achalasia patients experience respiratory symptoms and (2) the effect of a laparoscopic Heller myotomy and Dor fundoplication on the typical and respiratory symptoms of achalasia. Patients and methods: Between May 2008 and December 2015, 165 patients with achalasia were referred for treatment to the Center for Esophageal Diseases of the University of Chicago. Patients had preoperatively a barium swallow, endoscopy, and esophageal manometry. All patients underwent a Heller myotomy and Dor fundoplication. Results: Based on the presence of respiratory symptoms, patients were divided into two groups: group A, 98 patients (59%) without respiratory symptoms and group B, 67 patients (41%) with respiratory symptoms. The preoperative Eckardt score was similar in the two groups (6.5 ± 2.1 versus 6.4 ± 2.0). The mean esophageal diameter was 27.7 ± 10.8 mm in group A and 42.6 ± 20.1 mm in group B (P < .05). The operation consisted of a myotomy that extended for 5 cm on the esophagus and 2.5 cm onto the gastric wall. At a median postoperative follow-up of 17 months, the Eckardt score improved significantly and similarly in the two groups (0.3 ± 0.8 versus 0.3 ± 1.0). Respiratory symptoms improved or resolved in 62 patients (92.5%). Conclusions: The results of this study showed that: (1) respiratory symptoms were present in 41% of patients; (2) patients with respiratory symptoms had a more dilated esophagus; and (3) surgical treatment resolved or improved respiratory symptoms in 92.5% of patients. This study underlines the importance of investigating the presence of respiratory symptoms along with the more common symptoms of achalasia and of early treatment before lung damage occurs.
... Achalasia is caused by loss of inhibitory innervation of lower esophageal sphincter and is characterized by failure of the sphincter to relax. This failure of relaxation causes poor emptying of the esophagus and subsequent dilatation and abnormal contractility of the proximal esophagus (Mehdi et al., 2008). It is a rare esophageal neurodegenerative disorder in the pediatric population. ...
... Tracheal obstruction due to compression from dilated esophagus may occur in achalasia and can be the only presentation. This can be a serious and potentially life threatening complication of achalasia (Mehdi et al., 2008;Kugelman et al., 2000;Akhter et al., 1988;Chapman et al., 1989;Lee et al., 2010). ...
... According to the study by Sinan et al. [7] which included 110 adult patients with achalasia, most frequent respiratory symptoms were cough (37%), aspiration (31%), and dysphonia (21%). In children with achalasia, cough is a common symptom [8]. ...
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Introduction: Achalasia is a rare esophageal disorder which, due to frequent presence of both respiratory and gastrointestinal symptoms, can initially be referred either to pulmonologist or gastroenterologist. Case outline: A 50-year-old patient was initially referred to gastroenterologist with the following symptoms: nausea, vomiting, occasional hiccups, weight loss, chest pain, dysphonia, and dry cough. After chest X-ray, the patient was referred to pulmonologist with differential diagnosis for pulmonary infiltration and thoracic aortic aneurysm. Pulmonologist interpreted chest X-ray as showing paratracheal mediastinal enlargement with air-fluid levels, thus suspecting achalasia. Computed tomography scan of the thorax with per os contrast showed extremely dilated esophagus with food stasis. The patient was then referred to thoracic surgeon, who ordered additional diagnostics (esophageal passage with contrast, esophagomanometry, esophagogastroduodenoscopy), and finally performed Heller myotomy. Postoperatively there were no complications, and the patient was symptom free during the follow-up. Conclusion: Although achalasia can also result in respiratory symptoms, fastidious anamnesis and accurate radiological interpretation are essential for the correct diagnosis.
... [4,5] An unusual case of achalasia in a child presenting with chronic cough due to compression and narrowing of the trachea has been described. [6] A postulated cause of achalasia is the degeneration of the ganglion cells in Auerbach's plexus of the oesophagus. The Oesophageal achalasia is a neuromuscular disorder of unknown aetiology, characterised by abnormal motility of the oesophagus and failure of the lower oesophageal sphincter to relax. ...
... Non-peristaltic tertiary contractions may also be seen. [6,8,9] At endoscopy, a dilated oesophagus with a tight LES that 'pops' open with gentle pressure is often observed, as well as retained food and saliva. Signs of oesophagitis may also be seen. ...
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Oesophageal achalasia is a neuromuscular disorder of unknown aetiology, characterised by abnormal motility of the oesophagus and failure of the lower oesophageal sphincter to relax. This causes an abnormal dilatation of the oesophagus and resultant symptoms of vomiting/regurgitation, dysphagia, chest pain and at times, signs of lung aspiration and infection. In children, it may present as a chronic cough. The condition usually presents in the 4th and 5th decades and has very rarely been described in children. We describe two cases of achalasia and their imaging findings in adolescents.
... 5,6 Cough as a primary presenting symptom of achalasia has rarely been reported and only so in the pediatric population. 7,8 Furthermore, a few cases of achalasia misdiagnosed as uncontrolled asthma or as an aggravating factor for existing asthma have been reported, again predominantly in pediatric patients. [11][12][13] Although cases of acute airway obstruction due to achalasia have been reported in the elderly, 14 achalasia has not been reported as the primary cause of cough in adult patients who presented with chronic cough but did not have gastrointestinal symptoms. ...
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Cough is one of the most common symptoms that causes patients to seek outpatient medical care. If cough persists longer than 8 weeks, common causes of chronic cough, such as upper airway cough syndrome, asthma, and gastroesophageal reflux disease (GERD), should be considered. Although not a common cause of chronic cough, achalasia may cause symptoms very similar to reflux that can lead to its misdiagnosis as GERD. In this report, a 40-year-old woman presenting with chronic cough was initially diagnosed with GERD; however, her symptoms were refractory to conventional GERD treatment. Finally, she was diagnosed with achalasia. Her cough improved completely after pneumatic dilatation. Achalasia is a rare disease accompanied by dysphagia or regurgitation. If cough presumably due to GERD does not respond to treatment, or if the cause of chronic cough is uncertain, physicians should suspect achalasia.
... 1-3 It leads to incomplete emptying of the esophagus, with a gradually esophageal dilatation and its consequences. 4 The disease was first described by Thomas Willis in 1674 and, in 1953, King described the first case of achalasia in childhood, presenting the story of a 6-month-old infant. 2,4-6 This is a rare condition, especially in the pediatric age range, 2 being more frequent in adults between 25 and 60 years old. ...
... Cough may still occur, either due to aspiration of food or by compression of the airway by a dilated esophagus. 4 The predominant symptoms in infantile achalasia are respiratory symptoms and regurgitations which can easily be confused with gastroesophageal reflux disease. 2 Thus, the diagnosis of achalasia should be considered in children with persistent vomiting or growth failure, particularly when associated with symptoms of food impact, dysphagia and weight loss. 6 Secondary pulmonary disease can occur due to regurgitation and aspiration of the retained material in the esophagus, manifested by nocturnal cough, choking, frequent pulmonary infections, wheezing, atelectasis, and less likely, pulmonary empyema. ...
... 6 Secondary pulmonary disease can occur due to regurgitation and aspiration of the retained material in the esophagus, manifested by nocturnal cough, choking, frequent pulmonary infections, wheezing, atelectasis, and less likely, pulmonary empyema. 4 Some patients may develop hoarseness caused by direct compression of the recurrent laryngeal nerve by a distended esophagus. 4 A serious and potentially fatal complication of achalasia is tracheal obstruction by compression of a dilated esophagus, which may present as the only clinical manifestation of the disease. ...
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Introdução A acalásia é uma doença rara em idade pediátrica, de etiologia desconhecida. Pela sua raridade e dificuldade diagnóstica, os autores relatam o caso de um adolescente com acalásia. Caso clínico Adolescente de 15 anos, sexo masculino, com antecedentes pessoais irrelevantes. Por queixas de regurgitação e disfagia com 3 meses de evolução, efetuou Endoscopia Digestiva Alta (EDA) que foi normal. Após agravamento das queixas, com perda ponderal (9%), foi colocada a hipótese diagnóstica de perturbação do comportamento alimentar, pelo que foi internado para esclarecimento do quadro. Analiticamente não apresentava alterações. As características da disfagia durante o internamento (inicialmente para líquidos e posteriormente também para sólidos) sugeriram alteração da motilidade esofágica, tendo sido realizado trânsito esofágico baritado, cujo resultado foi compatível com a hipótese de acalásia. A manometria esofágica de alta resolução confirmou este diagnóstico. Foi submetido a miotomia laparoscópica de Heller com fundoplicatura de Dor, sem recorrência das queixas. Comentários A acalásia é uma doença rara, associada a dificuldade e atraso no diagnóstico. A normalidade da EDA e a hipótese de perturbação do comportamento alimentar, contribuíram para atrasar o diagnóstico, pelo que, na presença de queixas persistentes de regurgitação ou disfagia é importante caracterizar exaustivamente os sintomas, pensar na doença e prosseguir com a investigação.
... We have seen two cases where chronic cough was the result of the dilated esophagus compressing the trachea (Fig. 6). 33 Another uncommon cause of cough seen jointly with our pediatric otolaryngologists occurred from large palatine tonsils making contact with and compressing the epiglottis (Fig. 7). 34 Another example of irritating the epiglottis as a cause of chronic cough was a 4-yearold boy who stated he coughed because he felt something in the back of his throat (Fig. 8). ...
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A cough is considered chronic when it lasts >4 weeks. Chronic cough can be from a variety of causes. This article provides a structured approach to evaluating the child with chronic cough. Beginning with the disturbing cough that is absent once asleep, consistent with the habit cough syndrome, the diagnostic criteria for 10 causes of chronic cough are discussed. Using a structured approach to the differential diagnosis, common and uncommon causes can be identified. Well-established causes of chronic cough, such as asthma, are likely to be well known to the reader, whereas more recently identified etiologies, such as protracted bacterial bronchitis, are presented in more detail. The differential value of flexible and rigid bronchoscopy and bronchoalveolar lavage for aiding in the differential diagnosis is included for those entities where their use is essential.