^T^^B^D: A motor disorder of the oesophagus with aperistalsis and failure of lower oesophageal sphincter relaxation while swallowing.
Degeneration of ganglionic cells of myenteric plexus of the oesophageal
O sphincter disrupts the peristaltic coordination. Cause of the degeneration is unknown. Infection with Trypanosome cruzi may produce a similar syndrome, but this is only common in South America.
^^ |a/R: Rare association with alacrimation and Addison's disease (Triple A syndrome).
Annual UK incidence is 1/100000. All age groups but rare in childhood.
^IH Intermittent dysphagia involving solids and liquids, food may be regurgitated ^^ (particularly at night), atypical/cramping retrosternal chest pain, weight loss.
^IP Micro: Degeneration of intramural ganglionic cells of the myenteric plexus at the oesophageal sphincter. Degeneration of dorsal vagal nucleus in the brainstem medulla may also be seen.
Macro: Oesophagus can become severely dilated and elongated (see Figs 1a & 1b).
I: CXR: May show dilated oesophagus (double right heart border) and fluid level behind heart shadow.
Barium swallow: Dilated body of oesophagus, which smoothly tapers down to the sphincter (beak-shaped), lack of peristalsis. Oesophagoscopy: Excludes malignancy.
Manometry: Oesophageal and sphincter pressures. Abnormal sphincter-resting pressure is > 30 mmHg.
Bloods: Exclude Chagas' disease (serology for antibodies against Trypanosome cruzi), blood film might detect parasites.
M: Medical: Nifedipine or verapamil (calcium channel antagonists) or isosorbide mononitrate as needed (for short-term relief). Endoscopic balloon dilatation of lower oesophageal sphincter (80% success rate, but small risk of perforation). Endoscopic injection of botulinum toxin may be promising. Surgery: Heller's cardiomyotomy of lower oesophageal sphincter via an abdominal or thoracic approach to relieve obstruction. This can cause future reflux oesophagitis, so it may be combined with a fundoplication procedure.
C: If untreated, aspiration pneumonia, malnutrition and weight loss may result. 5% risk of oesophageal malignancy regardless of treatment (on average ~25 years after diagnosis).
P: Good if treated. If untreated, oesophageal dilation worsens, causing pressure on mediastinal structures.
Achalasia continued 5
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