Achalasia

A 56-year-old woman presented with fever and productive cough of 2 weeks in duration. Clinical examination revealed a thin, slightly tachypneic patient with temperature of 38.4[degrees]C, heart rate of 100 beats/min, and respiratory rate of 22 breaths/ min. Her BP and arterial blood gas measurements were normal. There was no finger clubbing or lymphadenopathy. Chest examination revealed crackles at the upper third of both lung fields. She did not have a history of arrhythmias or heart failure, and cardiovascular examination was normal. The rest of the physical examination was unremarkable. Laboratory investigation revealed only leukocytosis (14,000/[micro]L) and a raised C-reactive protein (35 mg/L). A tuberculin skin test result was negative, and sputum and blood culture findings were unremarkable.

She 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. She received inhaled anti-inflammatory agents and bronchodilators for many years for a diagnosis of chronic inflammatory disease of the airways, ie, asthma. Regurgitation of food occurred occasionally at night, waking her from sleep because of coughing and choking. She said that she had learned to live with her complaints. As her symptomatology was gradually worsening over the past 2 years, her family physician prescribed amitryptiline, possibly because he suspected a psychogenic disorder. She had no history of previous pulmonary infection. A chest radiograph obtained at the time of hospital admission demonstrated bilateral pulmonary infiltrates (Fig 1).

[FIGURE 1 OMITTED]

What is the diagnosis?

Diagnosis: Achalasia associated with aspiration pneumonia

Chest CT scan demonstrated the left lung infiltrate and a large esophageal elongation with a gas-fluid level and alimentary residue (Fig 2, 3). Based on the CT scan, barium esophagography was performed, which disclosed marked esophageal dilation with "bird's beak" distal esophagus (Fig 4). After esophagography, the endoscopic examination excluded a tumor at the gastroesophageal junction. Finally, esophageal manometry confirmed the diagnosis of achalasia by demonstrating esophageal aperistalsis and severe impairment of lower esophageal sphincter relaxation with swallowing.

[FIGURES 2-3 OMITTED]

DISCUSSION

Achalasia, a Greek term that means "failure to relax," is an idiopathic esophageal dysmotility characterized by loss of peristalsis in the distal two thirds of the esophagus (smooth muscle), and impaired relaxation of the lower esophageal sphincter. (1) Although the etiology of achalasia is unknown, hereditary, autoimmune, degenerative, and infectious factors may be implicated in the pathogenesis of this disorder. (2) In particular, pathologic changes found in the esophageal myenteric plexus of Auerbach's may play a primary role in causing this disorder. These changes include loss or marked reduction of myenteric ganglion cells, inflammatory responses within myenteric nerves consisted of a mixture of lymphocytes and eosinophils, and focal or complete replacement of myenteric nerves by collagen. (3) The result of these inflammatory changes is the selective loss or dysfunction of postganglionic inhibitory neurons containing vasoactive intestinal polypeptide and nitric oxide in the myenteric plexus of the esophagus. (4) The preservation of postganglionic excitatory neurons of the myenteric plexus and their unopposed cholinergic activity results in insufficient lower esophageal sphincter relaxation and loss of esophageal peristalsis. (5)

Patients with achalasia usually complain of the gradual onset of dysphagia for solids, and in most cases, for liquids as well, with regurgitation of food and saliva. Regurgitation becomes a major problem as the disease progresses and, especially, when the esophagus begins to dilate. Waking from sleep because of coughing and choking is common in patients with regurgitation of undigested food or accumulated saliva. Also, chest pain in patients with achalasia is a frequently misinterpreted symptom with unknown cause. It occurs in up to half of all patients with this disease and it has been described as angina-like retrosternal pain that occasionally overshadows more typical symptoms as dysphagia and regurgitation. (6) In these patients, aspiration of esophageal contents into the respiratory tract leads to chronic and/or acute pulmonary infections. The majority of reports described cases of patchy bilateral alveolar opacities that resembled aspiration pneumonia.

The long-standing esophageal achalasia in our patient presented with acute-onset aspiration pneumonia. Although in this patient culture findings of sputum samples were negative, nontuberculous mycobacteria are increasingly recognized as causes of pulmonary infection in patients with achalasia. Stasis of food in the esophagus and recurrent aspiration appear to play a primary role in the nontuberculous mycobacteria infection in these patients. Mycobacterium fortuitum-chelonei complex appears to be the predominant infective agent rather than other atypical mycobacteria. (7-9)

Diagnosis of primary achalasia in our patient has confirmed by radiographic, manometric, and endoscopic studies. Barium esophagography reveals characteristic findings, including esophageal dilation, loss of esophageal peristalsis, poor esophageal emptying, and a symmetric bird's beak tapering of the distal esophagus. The diagnosis is established by esophageal manometry. Aperistalsis of the esophagus and abnormal lower esophageal sphincter relaxation are always present in all patients. All patients with suspected achalasia should undergo endoscopy, since some primary or metastatic tumors that invade the gastroesophageal junction can produce pseudoachalasia. CT can help in the diagnosis of pseudoachalasia. In addition, the differential diagnosis must include other primary esophageal motility disorders, such as diffuse esophageal spasm, hypocontracting or hypercontracting esophagus, and some secondary disorders of esophageal mobility due to a multisystem disease, especially scleroderma. (2,10) Chaga disease or trypanosomiasis, a parasitic infection caused by Trypanosoma cruzi, can also produce an achalasia-like syndrome in the esophagus. (11)

Endoscopically guided injection of botulinum toxin into the lower esophageal sphincter blocks the unopposed cholinergic activity and results in a reduction in lower esophageal sphincter pressure with initial improvement in symptomatology that usually lasts several months in the majority of cases. (12,13) Some patients exhibit good relief of dysphagia after pneumatic dilatation of the lower esophageal sphincter. (14) Our patient underwent surgical laparoscopic myotomy and she demonstrated an excellent symptomatic improvement. Her dysphagia and cough disappeared, and she could enjoy her sleep.

REFERENCES

(1) Vaezi MF, Richter JE. Diagnosis and management of achalasia. Am J Gastroenterol 1999; 94:3406-3412

(2) Richter JE. Oesophageal motility disorders. Lancet 2001; 358:823-828

(3) Goldblum JR, Whyte RI, Orringer MB, et al. Achalasia: a morphologic study of 42 resected specimens. Am J Surg Pathol 1994; 18:327-337

(4) Goyal RK, Hirano I. The enteric nervous system. N Engl J Med 1996; 334:1106-1115

(5) Holloway RH, Dodds WJ, Helm JF, et al. Integrity of cholinergic innervation to the lower esophageal sphincter in achalasia. Gastroenterology 1986; 90:954-929

(6) Eckardt VF, Stauf B, Bernhard G. Chest pain in achalasia: patient characteristics and clinical course. Gastroenterology 1999; 116:1300-1304

(7) Miller WT. Spectrum of pulmonary nontuberculous mycobacterial infection. Radiology 1994; 191:343-350

(8) Aronchick JM, Miller WT, Epstein DM, et al. Association of achalasia and pulmonary Mycobacterium fortuitum infection. Radiology 1986; 160:85-86

(9) Hadjiliadis D, Adlakha A, Prakash UB. Rapidly growing mycobacterial lung infection in association with esophageal disorders. Mayo Clin Proc 1999; 74:45-51

(10) Bassotti G, Battaglia E, Debernardi V, et al. Esophageal dysfunction in scleroderma: relationship with disease subsets. Arthritis Rheum 1997; 40:2252-2259

(11) Koberle F. Chagas' disease and Chagas' syndromes: the pathology of American trypanosomiasis. Adv Parasitol 1968; 6:63-116

(12) Pasricha PJ, Ravich WJ, Hendrix TR, et al. Intrasphincteric botulinum toxin for the treatment of achalasia. N Engl J Med 1995; 332:774-778

(13) Prakash C, Freedland KE, Chan MF, et al. Botulinum toxin injections for achalasia symptoms can approximate the short term efficacy of a single pneumatic dilation: a survival analysis approach. Am J Gastroenterol 1999; 94:328-333

(14) Vaezi MF, Richter JE. Current therapies for achalasia: comparison and efficacy. J Clin Gastroenterol 1998; 27:21-35

* From the Department of Internal Medicine, "Hatzikosta" General Hospital of Ioannina, Ioannina, Greece.

Manuscript received November 7, 2001; revision accepted December 6, 2001.

Correspondence to: Nikolaos Akritidis, MD, Department of Internal Medicine, "Hatzikosta" General Hospital of Ioannina, Makrygianni Ave 45442, Ioannina, Greece, e-mail: kostpap@otenet.gr

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