Diagnosis and management of esophageal achalasia in children: analysis of 13 cases

Abstract

Background

Esophageal achalasia is a rare disease and there have been very few reports about it, especially in children. We reviewed our experience in dealing with esophageal achalasia in 13 children.

Methods

Thirteen children (6 boys and 7 girls), who had been diagnosed with achalasia over a 12-year period between May 1993 and October 2005, were analysed with regard to clinical manifestations, esophageal manometry, endoscopic findings, and treatment. Their age ranged from 3 years to 14 years and 5 months (average 10.3 years) at the time of diagnosis.

Results

In the 13 children, 3 had a family history of esophageal achalasia, 2 of them were sisters. All the 3 children suffered from achalasia/alacrimia/ACTH deficiency. Dysphagia was the most common symptom in the affected children. Vomiting/regurgitation, retrosternal pain, retarded growth, and respiratory symptoms were also observed in some patients. Heller’s esophagocardiomyotomy was performed in 9 (69.23%) children, among whom 3 had an antireflux operation at the same time. In the remaining 4 children, 3 received a pneumatic dilatation and 1 received regular administration of nifedipine. Twelve patients were followed up: 8 patients by surgery were cured and have been in perfect condition until now, 3 patients recovered fairly, and 1 patient showed improvement.

Conclusions

Esophageal manometry combined with X-ray examination proves to be an effective diagnostic method for achalasia. It is also effective in evaluating the result of treatment. Heller’s esophagocardiomyotomy is a treatment of choice for children with achalasia because of its safety and long-term effective results after surgery.