Abstract
Very few cases of esophageal achalasia associated to severe dysautonomia have been reported. Among esophageal manometries performed from 1982 to 1987 (n=680), we found in 34 children (5%) a partial (insufficient relaxation of lower esophageal sphincter (LES)) or complete (increased LES pressure without relaxation) achalasia. Dysautonomia was associated in 11/34 cases (32%). Two groups appeared considering clinical and manometric patterns. In group 1 (n=5), complete achalasia was worsening, leading to surgery in 2 cases (modified Heller's myotomy). Dysautonomia was severe (Ondine's curse, Riley-Day syndrome) or mild (alacrima with glucocorticoids deficiency, pupillar dysmotricity). In group 2 (n=6) partial, asymptomatic achalasia was found in infants hospitalized for unexplained apneic episodes (“near-miss” sudden infant death syndrome), with gastro-esophageal reflux documented by intraesophageal PH-monitoring in 4/6. Oculo-cardiac reflex studies and computerized 24-hour ambulatory electrocardiographic recordings suggested an increase in cardiac vagal tone which was the only dysautonomic manifestation. Achalasia spontaneously disappeared in 4 to 6 months.
These data seem to bring interesting physiopathological light and justify systematic extensive evaluation of autonomic nervous system when esophageal achalasia is discovered (clinical assessment of vaso-motor, sweating and nociception abnormalities; pupilar motricity, lachrymal secretion and cardiac rhythm studies).
Article PDF
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Foucaud, P., Boige, N., Cargill, G. et al. 76 ESOPHAGEAL ACHALASIA AND DYSAUTONOMIA IN CHILDHOOD. Pediatr Res 24, 417 (1988). https://doi.org/10.1203/00006450-198809000-00099
Issue Date:
DOI: https://doi.org/10.1203/00006450-198809000-00099