Question
A 40 year old male presented with difficulty in swallowing solids and liquids intermittently.Barium Swallow showed the following features.What can be the most probable diagnosis?
A. Achalasia Cardia.
B. Esophageal Carcinoma.
C. Esophageal Stricture.
D. Plummer Vinson Syndrome.
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Correct Answer » A Explanation |
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Ans:A. Achalasia Cardia
Bird beak appearance is seen in the Barium swallow image suggestive to Achalasia Cardia.
Achalasia
- Achalasia (primary achalasia) refers to a failure of organised oesophageal peristalsis with an impaired relaxation of the lower oesophageal sphincter (LOS), resulting in often marked dilatation of the oesophagus and food stasis.
- Obstruction of the distal oesophagus (often due to tumour) has been termed “secondary achalasia” or “pseudoachalasia”.
Clinical presentation
- Typically patients present with dysphagia (which is for both solids and liquids, in comparison to dysphagia for solids only in cases of esophageal carcinoma ), chest pain/discomfort and eventual regurgitation. Initially, symptoms are intermittent.
Patients may also present with a complication of long-standing achalasia:
- oesophageal carcinoma
- the most dreaded complication and is seen in approximately 5%, and most often in the mid oesophagus
- thought to occur because of the chronic irritation of the mucosa by stasis of food and secretions
- aspiration pneumonia
- candida oesophagitis
- acute airway obstruction: this is a rare complication requiring immediate oesophageal decompression with a nasogastric tube
Pathology
- Peristalsis in the distal smooth muscle segment of the oesophagus may be lost due to an abnormality of Auerbach plexus (responsible for smooth muscle relaxation), resulting in weak contractions that are uncoordinated and therefore non-propulsive.
- The LOS eventually fails to relax, either partially or completel
Radiographic features
- Achalasia characteristically involves a short segment (less than 3.5 cm) of the distal oesophagus.
Chest x-ray findings include:
- right convex opacity behind right cardiac border; occasionally left convex opacity if thoracic aorta tortuous
- air-fluid level due to stasis in thoracic oesophagus filled with retained secretions and food
- small/absent gastric air bubble
- anterior displacement and bowing of trachea on the lateral view
Barium Swallow findings include:
- The Bird beak sign/rat-tail sign is used to refer to the tapering of the inferior oesophagus in achalasia
- oesophageal dilatation
- incomplete LOS relaxation that is not coordinated with oesophageal contraction
- pooling or stasis of barium in the oesophagus when the oesophagus has become atonic or non-contractile (a late feature in the disease)
- uncoordinated, non-propulsive, tertiary contractions
- failure of normal peristalsis to clear the oesophagus of barium when the patient is in the recumbent position, with no primary waves identified
Treatment :
Options include :
- lifestyle changes –
- eating slowly, increasing water intake with meals, avoiding eating near bed time
- avoiding foods that aggravate reflux
- calcium channel blockers
- pneumatic dilatation
- botulinum toxin injection
- surgical myotomy (e.g. Heller myotomy)
- 10-30% of patients develop gastro-oesophageal reflux, and thus it is often combined with a fundoplications (e.g. Dor, Toupet, Nissen)
- the use of per-oral oesophageal myotomy (POEM procedure) is increasing in select patient
