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Achalasia of the cardia is rare in children. We report our experience in the management of 20 children with achalasia.Twenty children (seven girls including two sisters) with achalasia were studied, seven of them had a morbid association. Age at diagnosis ranged from eight months to 18 years (med: 6.4 yrs). Duration of symptoms prior to diagnosis ranged from one to 62 months (med: 8 months). Regurgitations, weight loss and recurrent pneumonias were the most common presenting symptoms. Diagnosis was established using esophageal manometry, which showed aperistalsis throughout the esophageal body with impaired relaxation of lower esophageal sphincter in all children, and chest x-ray and barium esophagram, which were abnormal in 11 and 18 children respectively.Nifedipine used in nine children was ineffective. Two balloon dilatations were performed in one child with a poor result. Surgery with Heller's myotomy combined with an antireflux procedure was performed in 19 children. One child died six months later due to an hypoglycemic coma. During the follow-up (6 months-21 years) symptoms reappeared in five children. Seventy-two percent of the children had no symptoms one year after the surgery and 45%, five years after. Esophageal manometry performed after surgery in seven children showed a normal lower esophageal sphincter pressure but with impaired relaxation, and an aperistalsis throughout the esophageal body. These results justify prolonged follow-up of children with achalasia of the esophagus.

Depuis 5 ans, nous avons etudie en manometrie œsophagienne, 7 cas de megaœsophage idiopathique infantile. Dans toutes les observations, meme s’il n’existait pas de distension œsophagienne radiologique, nous avons note des anomalies specifiques: hypertonie et achalasie du sphincter inferieur de l’œsophage (SIO) et dyskinesie caracteristique du corps de l’œsophage. La manometrie nous a permis d’eliminer les autres causes de megaœsophage infantile. Une intervention de Heller modifiee, suivie d’un procede anti-reflux, a ete pratiquee de premiere intention chez 3 patients avec de bons resultats dans 2 cas. La persistance des symptomes dans le troisieme, a motive une nouvelle cardiomyotomie. Les 4 derniers malades ont ete traites medicalement par Nifedipine. Le premier a ete perdu de vue. Les resultats ont ete excellents dans 2 cas, mais de facon transitoire (5 mois) chez l’un d’eux âge d’un an. Dans le troisieme, les signes cliniques et manometriques se sont aggraves, malgre l’augmentation des doses de Nifedipine. Ces 3 patients ont ete finalement operes. Les etudes manometriques post-operatoires montraient un effrondrement du tonus du SIO, mais une persistance de l’achalasie et une regression moderee de la dyskinesie œsophagienne. Lorsqu’il est efficace, le traitement medical permet aux patients d’affronter l’acte chirurgical dans d’excellentes conditions nutritionnelles.

Constant rate enteral feeding in the patient's home considerably shortens the stay of children in a specialized pediatric unit. A study of 111 children treated by this method for the past 5 years shows that, provided close co-operation with the family is obtained, obvious psychological benefits can be expected with little risk of technical complications. From an economic point of view, the method provides an effective means of reducing costs.