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[Achalasia in children]
1Servicio de Gastroenterología del Hospital de Niños J. M. de los Ríos, Caracas, Venezuela.
Insights
Childhood achalasia, a rare esophageal motility disorder, presents with progressive dysphagia and weight loss. Mechanical disruption of the lower esophageal sphincter improved symptoms and sphincter pressure.
Area of Science:
- Pediatric Gastroenterology
- Esophageal Motility Disorders
Background:
- Achalasia is a rare esophageal motility disorder with a long history, infrequently diagnosed in pediatric populations.
- This study examines four pediatric cases of achalasia diagnosed and treated at J. M. de los Ríos Hospital between 1971 and 1990.
Observation:
- Pediatric achalasia cases presented with progressive dysphagia (difficulty swallowing) for solids and liquids, regurgitation, weight loss, and nocturnal cough.
- Diagnostic evaluations included radiological, endoscopic, and manometric studies revealing esophageal dilation, altered motility, a narrow cardio-esophageal junction, elevated lower esophageal sphincter pressure, and non-peristaltic contractions.
Findings:
- The Starck dilator was employed for mechanical disruption of the lower esophageal sphincter muscle fibers.
- This intervention resulted in significant improvement of clinical symptoms and a reduction in elevated lower esophageal sphincter pressure.
Implications:
- Mechanical disruption of the lower esophageal sphincter offers a viable treatment for pediatric achalasia.
- The procedure facilitated adequate weight gain (pondostatural progress) and a satisfactory clinical outcome in the studied pediatric patients.
Abstract:
Achalasia is a pathology which was first described more than 300 years ago, it is relatively rare in children. Four cases of childhood achalasia were studied in children admitted to the J. M. de los Ríos Hospital during the period January, 1971 to June, 1990. The clinical presentation was characterized by progressive dysphagia to solids and liquids, regurgitations, weight loss and in certain cases, a moist cough, predominantly nocturnal. The diagnostic workup carried out included radiological, endoscopic and manometric studies. They were helpful to diagnose the following positive signs: esophageal dilation with alteration of motility and a small caliber cardio-esophageal junction, in addition to an elevation of the inferior esophageal sphincteric pressure with non-peristaltic esophageal contractions. The Starck dilator was used to create a mechanical disruption of the muscle fibers of the inferior esophageal sphincter. The procedure improved the clinical symptoms, lowered the inferior esophageal sphincteric pressure leading to adequate pondostatural progress and a satisfactory evolution.