Case of the Week - Patient Summary 12559Peer Reviewed and Certified -
• Scleroderma
• Gastric/esophageal Carcinoma
• Peptic stricture with esophagitis
• Chagas disease
• Postvagotomy effect
Achalasia
Scleroderma is characterized by a dilated esophagus with patulous GE region; however, if complicated by peptic stricture may have this appearance.
Peptic strictures are commonly found with hiatal hernias, a rare occurrence with achalasia. Peptic strictures are also rarely associated with aperistalsis.
Esophageal findings in Chagas disease would be similar to achalasia. Evaluation of other characteristic features of Chagas disease and travel history to endemic regions would aid in differentiation.
• Clinical symptoms: coughing, dysphagia, foul breath, regurgitation, weight loss, aspiration, recurrent pneumonia or lung abscess. Variant form, denoted by vigorous achalasia, has high amplitude, simultaneous and repetitive contractions. These patients symptoms may differ with chest pain and less dilation.
• Associations: risk for carcinoma varies up to 9 x increase relative to general population
• Demographics: Primary achalasia range 30 to 50 whereas secondary achalasia is more often in older patients. There is an equal sex distribution.
• Gross Morphology: Massively dilated esophagus with smooth tapered distal segment at GE junction.
• Histology: Decreased number of ganglion cells in myenteric ganglia (Auerbach's Plexus) of the esophagus.
• Radiology:
Chest radiograph: mediastinal widening, anterior tracheal bowing, air-fluid level in mediastinum, decreased or absent gastric bubble.
• Barium swallow: markedly dilated tortuous esophagus, absent primary peristalsis, and (bird beak deformity) tapered narrowing of distal esophagus at GE junction.
• CT: Dilation of esophagus with diameter more than 4 cm, decreased or increased thickness, abrupt smooth narrowing at distal segment at GE junction, air fluid level or retained food debris and secretions.
• Manometrical: absence of primary peristalsis, elevated to normal resting lower esophageal pressures, and incompelete or absent relaxation.
• Prognosis and Treatment: There is no treatment for the abnormal motility or lower esophageal dysfunction. Therapy is directed at improving outflow by medical means such as calcium channel blockers or botulinum toxin injections. Surgical interventional therapy includes pneumatic dilation and Heller myotomy.
