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Achalasia

Achalasia or acalasia is a failure of a ring of muscle (as a sphincter) to relax (completely). It refers most commonly to esophageal achalasia, which is a neuromuscular disorder of the esophagus characterized by the reduced ability to move food down the esophagus (peristalsis). In addition, the inability of the cardia ( also called lower esophageal sphincter)- to relax in response to swallowing (there is increased LES pressure- spasms). more...

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Synonyms are achalasia cardiae, cardiospasm, dyssynergia esophagus, esophageal aperistalsis.

Signs and symptoms

  • Dysphagia
  • Regurgitation of undigested food
  • Heartburn
  • Chest pains : increases after eating and may radiate to the back, neck, and arms.
  • Weight loss
  • Coughing, especially at night or when lying down

Diagnosis

Due to the similarity of symptoms, achalasia can be misdiagnosed as other disorders, such as gastroesophageal reflux disease (GERD) and Chagas disease

  • X-ray with a barium swallow or esophagography . Shows narrowing at the level of the gastroesophageal junction ("bird beak"), and various degrees of megaesophagus (esophageal dilation).
  • Endoscopy, which provides a view from within the esophagus.
  • Manometry, the key test for establishing the diagnosis. Measures the pressure induced in different parts of the esophagus and stomach during the act of swallowing.
  • CT scan, which provides further visual evidence.

Complications

  1. Gastroesophageal reflux disease-GERD or heartburn.
  2. Barrett's esophagus or Barrett's mucosa: in 10% of patients.
  3. There are two kinds of esophageal cancer: squamous cell carcinoma and adenocarcinoma. There are predisposing conditions that, if present for a long time, may lead to esophageal adenocarcinoma, like achalasia (in up to 5% of cases, Barrett's esophagus leads to esophageal adenocarcinoma).

Treatment and new expectations

  • Balloon (pneumatic) dilation. The muscle fibers will be stretched. Gastroesophageal reflux (GERD) occurs after dilatation in 25% to 35% of patients. Is a risk to later Heller myotomy.
  • Medication:
    • Intra-sphincteric injection of botulinum toxin (or botox), to paralyze cardia and prevent spasms. It is transitory and symptoms will return in the majority of patients within a year.
    • Drugs that reduce LES pressure such as nifedipine and nitroglycerin may be useful.
  • Heller myotomy laparoscopic surgery.
  • Transplant and artificial cardia.

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Diffuse aspiration bronchiolitis due to achalasia - communications to the editor - Letter to the Editor
From CHEST, 1/1/04

To the Editor:

Akritidis and coworkers (February 2003) (1) reported a 56-year-old woman who presented with fever and productive cough of 2 weeks in duration because of achalasia. They comprehensively assessed the signs and symptoms, and the patient examination was almost perfect; achalasia associated with aspiration Pneumonia was diagnosed. Although the symptoms could be explained by achalasia associated with aspiration pneumonia, they may have missed the diagnosis.

We believe that the case should be diagnosed as diffuse aspiration bronchiolitis (DAB) due to achalasia. DAB is a new term that we proposed to define a clinical entity that is characterized by a chronic inflammation of bronchioles caused by recurrent aspiration of foreign bodies. (2) Recurrent aspiration causes cough and waxing and waning pulmonary lesions. (2) Although DAB is originally recognized as a disease in the elderly, our survey revealed that DAB occurred in young patients with the same clinical manifestations as those observed in the elderly. (3-5) ht younger patients, dysphagia due to achalasia and gastroesophageal reflux disease (GERD) with associated recurrent aspiration are major risks for development of DAB. (3-5) DAB caused by achalasia was diagnosed in three patients aged 11, 12, and 56 years, respectively. (3-5)

Dysphagia and GERD are considerably associated with swallowing disorders and aspiration. (6-11) Both the impaired lower esophageal sphincter and the depressed upper airway reflex contribute dependently or independently to recurrent lung infiltrates, resulting in the manifestation of DAB.

In the current case, the patient had a long history of a chronic dry cough that was worse at night, and had dysphagia for 15 years for solids and liquids, with substernal chest discomfort and pain that was unrelated to meals or exercise. When aspiration pneumonia, as the authors diagnosed, is a primary disorder, the symptoms are of acute onset and severe. However, the symptoms are generally insidious and chronic. Recurrent aspiration causes cough and waxing and waning pulmonary lesions, but does not always cause aspiration pneumonia. The history and the manifestation of symptoms are quite similar to DAB in the elderly.

It may be interesting to know whether the lung pathology revealed chronic mural inflammation with multinucleated histiocytes, ie, foreign body reaction, in bronchioles, which is a features of DAB. Further, achalasia and GERD are closely associated with aspiration associated lung infiltrates, ie, aspiration pneumonia and DAB. (3-14) Thus, the case may be better diagnosed as DAB due to achalasia.

Shinji Teramoto, MD

Hiroshi Yamamota, MD

Yasuhiro Yamaguchi, MD

Tetsuji Tmoita, MD

Yasuyosht Ouchi, MD

Department of Geriatric Medicine

Tokyo University Hospital

Tokyo, Japan

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (e-mail: permissions@chestnet.org).

Correspondence to: Shinji Teramoto, MD, Department of Geriatric Medicine, Tokyo University Hospital, 7-3-1 Hongo Bunkyo-Ku, Tokyo, Japan 113-8652; e-mail: shinjit-tky@unlin.ac.jp

REFERENCES

(1) Akritidis N, Gousis C, Dimos G, et al. Fever, cough, and bilateral lung infiltrates: achalasia associated with aspiration pneumonia. Chest 2003; 123:608-612

(2) Matsuse T, Oka T, Kida K, et al. Importance of diffuse aspiration bronchiolitis caused by chronic occult aspiration in the elderly. Chest 1996; 110:1289-1293

(3) Matsuse T, Teramoto S, Matsui H, et al. Widespread occurrence of diffuse aspiration bronchiolitis, in patients with dysphagia irrespective of age [letter]. Chest 1998; 114:350-351

(4) Igarashi T, Hirasawa M, Shibuya Y, et al. A case of diffuse aspiration bronchiolitis secondary to achalasia of the esophagus [in Japanese]. Nihon Kyohu Shikkan Gakkai Zasshi 1991; 29:1059-1063

(5) Hirata M, Kohno K, Murakami S, et el. A case of recurrent aspiration pneumonia by achalasia. Nihon Kokyuki Gakkai Zasshi 2002; 40:149-153

(6) Olsen AM. The spectrum of aspiration pneumonitis. Ann Otol Rhinol Laryngol 1970; 79:875-888

(7) Travis KW, Saini VK, O'Sullivan PT. Upper-airway obstruction and achalasia of the esophagus. Anesthesiology 1981; 54:87-88

(8) Hughes RL, Freilich RA, Bytell DE, et al. Clinician conference in pulmonary disease: aspiration and occult esophagus disorders. Chest 1981; 80:489-495

(9) Teramoto S, Matsuse T, Oka T, et al. Investigation of effects of anesthesia and age on aspiration in mice through LacZ gene transfer by recombinant El-deleted adenovirus vectors. Am J Respir Crit Care Med 1998; 158:1914-1919

(10) Teramoto S, Matsuse T, Ouehi Y. Amantadine and pneumonia in elderly stroke patients. Lancet 1999; 353:2156-2157

(11) Teramoto S, Matsuse T, Fukuchi Y, et al. Simple two-step swallowing provocation test for elderly patients with aspiration pneumonia [letter]. Lancet 1999; 353:1243

(12) Teramoto S, Kume H, Ouchi Y. Altered swallowing physioIogy and aspiration in COPD. Chest 2002; 122:1104-1105

(13) Teramoto S, Kume H, Ouchi Y. Nocturnal gastroesophageal reflux: symptom of obstructive sleep apnea syndrome in association with impaired swallowing. Chest 2002; 122:2266-2267

(14) Teramoto S, Ouchi Y. A possible pathologic link between chronic cough and sleep apnea syndrome through gastroesophageal reflux disease in older people. Chest 2000; 117: 1215-1216

To the Editor:

We thank Dr. Teramoto et al for their comments on our article (February 2003), (1) and we appreciate the opportunity to respond. The diagnosis of aspiration pneumonia due to achalasia best explains the pulmonary lesions observed in the case that we described, and also the clinical and radiographic findings in our patient are consistent with this diagnosis and argue against the statement by Terareoto et al that our patient had "diffuse aspiration bronchiolitis" (DAB).

Aspiration occurs when the integrity of the neuromuscular system used for swallowing becomes altered or impaired anatomatically or physiologically. (2) Deglutition is a neurologieally controlled phenomenon that involves cognitive and motor control, integration of sensory, information, and multiple levels of central and peripheral reflex control. As we pointed out in our article, the long-standing esophageal achalasia can be presented with acute-onset aspiration pneumonia. Aspiration pnemnonia is an infectious process caused by the inhalation of oropharyngeal material that is colonized lay pathogenic, bacteria, and our patient had the clinical features of pneumonia. (3) In patients with achalasia, stasis of food in the esophagus and recurrent aspiration appears to be a primary cause of acute lung infection from nontuberculous mycobacteria. Also, the term aspiration pneumonia suggests the development of radiographically evident infiltrate(s) in patients who are at high risk for oropharyngeal aspiration. (4)

In our patient, we did not perform lung biopsy because there were no appropriate clinical and radiographic grounds to do so. In the English-Language literature, the term diffuse aspiration bronchiolitis was first proposed by Matsuse et al (5) to define a chronic inflammation of the bronchioles caused by recurrent aspiration of foreign particles. In the study by Matsuse et al, (5) the lesion was described at autopsy in 31 of 4,880 patients, many of whom had no clinical evidence of aspiration. The authors stated that patients with DAB had a relatively less severe and more insidious inflammatory response than aspiration pneumonia.

Our patient had prominent signs of systemic illness (14,090/[micro]L leukocytes, temperature of 38.4[degrees]C, etc), whereas Matsuse et al (5) noted that patients with DAB very often demonstrated peripheral leukocyte counts < 10,000/[micro]L, and also the majority of these patients were afebrile. (5) Even Oil radiographic grounds, our patient cannot be considered as having DAB: a chest radiograph in DAB shows diffuse small nodular shadows, (5) whereas in our patient chest radiography clearly demonstrated bilateral upper-lobe infiltrates. Consolidation is rare or not a major finding in patients with DAB, and also areas of hyperlucency are usually seen on the chest radiograph. More convincingly, CT scan of the chest in DAB patients reveled small, round opacities located around the end of the bronchovascular branchings in almost all of the lung fields, findings that resemble panbronchiolitis. (6) In our patient, chest CT did not detect this diffusely disseminated nodular shadows seen in DAB.

Nikolaos Akritidis, MD

Konstantinos Paparounas, MD, PhD

Hatzikosta General Hospital

Ioannina, Greece

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (e-mail permissions@ehestnet.org).

Correspondence to: Nikedaos Akritidis, MD, Department of Internal Medicine, Hatzikosta General Hospital of Ionnina, Maktygiatmi Ave 45001, Ioannina, Greece; e-mail: kostpap@ otenet.gr

REFERENCES

(1) Akritidis N, Gousis C, Dimes G, et al. Fever, cough, and bilateral lung infiltrates: achalasia associated with aspiration pneumonia. Chest 2003; 123:608-612

(2) Irwin RS. Aspiration. In Irwin RS, Cerra FB, Rippe JM, eds. Irwin and Rippe's intensive care medicine. 4th ed. Philadelphia, PA: Lippincott-Raven, 1999; 685-692

(3) Marik PE. Aspiration pneumonitis and aspiration pneumonia. N Engl J Med 2001; 344:665-671

(4) Erasmus JJ, McAdams HP, Farrell MA, et al. Pulmonary nontuberculous mycobacterial infection: radiologic manifestations. Radiographics 1999; 19:1487-1505

(5) Matsuse T, Oka T, Kida K, et al. Importance of diffuse aspiration bronchiolitis caused by chronic occult aspiration in the elderly. Chest 1996; 110:1289-1293

(6) Igarashi T. Teramoto S, Matsui H, et al. A case of diffuse aspiration bronchiolitis secondary to achalasia of the esophagus [in Japanese]. Nihon Kyobu Shikkan Cakkai Zasshi 1991; 29:1059-1063

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