[Primary esophageal motor disorders in childhood, genuine achalasia excluded]

Esophageal manometry has been increasingly used in children allowing better description of esophageal primary disorders as partial achalasia. The aim of this retrospective study was to describe clinical manifestations of partial achalasia, to look for their specificities and to follow clinical and manometric evolution in response to treatment.

Eighteen patients (mean age four years: range one month-13.5 years) presenting with partial achalasia were examined from 1990 to 1998. The symptomatology leading to esophageal manometry was: dysphagia (n = 9), gastroesophageal reflux (n = 6), swallowing disorder (n = 3). pH-metry (n = 8), esophageal endoscopy (n = 11) and barium transit (n = 12) were also performed.

Twelve children were treated with nifedipine, (dysphagia n = 6, gastroesophageal reflux n = 5, swallowing disorder n = 1). At follow-up, a good clinical response was observed in six children while no effect or transient improvement were observed in two and four children respectively. Two of them presented spontaneous clinical resolution of symptoms after nifedipine was stopped but four children needed Heller procedure. Six of 18 patients (dysphagia n = 3, gastroesophageal reflux n = 2, swallowing disorder n = 1) developed achalasia or recurrent symptoms which required Heller surgery. At the first examination, no clinical or manometric features could differentiate these six patients from the remainders who presented a favorable outcome.

Esophageal primary disorders as partial achalasia in children are observed in various clinical conditions. The possible development of achalasia and persistence of symptoms in some children justify both attentive clinical and manometric follow-up.