Achalasia in childhood and adolescence

We analysed the charts of paediatric patients suffering from oesophageal disorders in 5 departments of paediatric surgery in Switzerland from 1972-1993 finding only 16 patients with proven achalasia (0.6%). The personal history showed a wide gap of several years from the first symptoms until diagnosis and treatment due to false interpretation of symptoms. Dilatations alone have never been definitely successful. In 16 patients, a Heller's procedure was done, 2 had an additional Thal, 12 a Nissen fundoplication, one a hiatoplasty and one no antireflux procedure. 3 patients needed further postoperative dilatations for several months up to 3 1/2 years. One patient suffering from an AAA syndrome (Achalasia/Alacrimia/ACTH deficiency) did not improve after Heller's procedure with Nissen fundoplication and dilatations. We finally had to resect the oesophagus and replace it by gastric transposition. Two children were lost for long-term follow-up. In 14 patients the follow-up is more than 10 years. 14 patients take normal food and are in the normal percentiles for length and height. Achalasia in childhood shows a wide spectrum of oesophageal dysmotility. In many cases, the propulsive force might be so weak that only a very lose antireflux procedure is tolerated. Occasionally, the whole oesophagus is without appropriate function and has finally to be resected as in our patient suffering from AAA syndrome. This child is now free of clinical pathological symptoms since several years.