CASE 4755 Published on 20.09.2006

Achalasia

Section

Paediatric radiology

Case Type

Clinical Cases

Authors

J.McKillen, A Paterson, J Turner, Department of Paediatric Radiology, Royal Belfast Hospital for Sick Children, 180 Falls Road, Belfast, BT12 6BE. Tel 02890632448. E-mail Jacqmckillen@doctors.org.uk

Patient

8 years, male

Clinical History
The patient presented with dysphagia, weight loss and cough.
Imaging Findings
A barium swallow revealed a food-filled dilated oesophagus with disordered motility. The oesophagus was tapered distally.
Discussion
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
Achalasia
Final Diagnosis
Achalasia
Case information
URL: https://www.eurorad.org/case/4755
DOI: 10.1594/EURORAD/CASE.4755
ISSN: 1563-4086