Author Affiliations: Department of Pediatrics, Shanghai Ruijin Hospital Affiliated to School of Medicine, Shanghai Jiaotong University, Shanghai 200025, China (Zhang Y, Xu CD); Digestive Functional Exploration Department, Necker Children's Hospital, Paris, France (Zaouche A); Department of Pediatric Surgery, Shanghai Xinhua Hospital Affiliated to School of Medicine, Shanghai Jiaotong University, Shanghai, China (Cai W)

Corresponding Author: Chun-Di Xu, Department of Pediatrics, Shanghai Ruijin Hospital Affiliated to School of Medicine, Shanghai Jiaotong University, Shanghai 200025, China (Tel: +86-21-64370045; Email: chundixu@hotmail.com)

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.

Key words: dysphagia; esophageal achalasia; esophageal manometry;  Heller's esophagocardiomyotomy; pneumatic dilatation

                   World J Pediatr 2009;5(1):56-59