The patient presented with dysphagia, weight loss and cough.
A barium swallow revealed a food-filled dilated oesophagus with disordered motility. The oesophagus was tapered distally.
Achalasia is a progressive motor disorder of the distal oesophagus caused by absence of Auer Bach plexus ganglion cells. It rarely presents before the age of six and is an uncommon cause of childhood
dysphagia. Adrenocortical insufficiency and alacrima have been reported in association with achalasia in an autosomal recessive pattern of inheritance(1). Due to its rarity there can be a delay in
diagnosis with consequent significant morbidity, including developmental disorders. Presenting symptoms include dysphagia, weight loss, chest pain, coughing, halitosis, recurrent respiratory
infections and noisy respiration. Younger children may present with failure to thrive. The primary pathology is failure of relaxation of the distal oesophagus during swallowing. On plain radiography
a dilated oesophagus containing a fluid level or food debris is seen. The gastric bubble may be absent. If barium studies are equivocal, oesophageal manometry reveals a raised resting pressure of the
lower sphincter. Treatment is surgical, by modified Heller’s myotomy. Other options include injection of botulinum toxin (2) or hydrostatic balloon dilatation (3).
Differential Diagnosis List