ACHALASIA IN CHILDHOOD AND ADOLESCENCE

We analysed the charts of paediatric patients suffering from oesophage al disorders in 5 departments of paediatric surgery in Switzerland fro m 1972-1993 finding only 16 patients with proven achalasia (0.6 %). Th e personal history showed a wide gap of several years from the first s ymptoms until diagnosis and treatment due to false interpretation of s ymptoms. Dilatations alone have never been definitely successful. In 1 6 patients, a Heller's procedure was done, 2 had an additional Thal, 1 2 a Nissen fundoplication, one a hiatoplasty and one no antireflux pro cedure. 3 patients needed further postoperative dilatations for severa l months up to 31/2 years. One patient suffering from an AAA syndrome (Achalasia/Alacrimia/ACTH deficiency) did not improve after Heller's p rocedure with Nissen fundoplication and dilatations. We finally had to resect the oesophagus and replace it by gastric transposition. Two ch ildren were lost for long-term followup. In 14 patients the follow-up is more than 10 years. 14 patients take normal food and are in the nor mal percentiles for length and height. Achalasia in childhood shows a wide spectrum of oesophageal dysmotility. In many cases, the propulsiv e 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 fr om AAA syndrome. This child is now free of clinical pathological sympt oms since several years.