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Disorders of upper esophageal sphincter motility in children
A Staiano1, S Cucchiara, B De Vizia
1Department of Clinical Pediatrics, 2nd School of Medicine, Naples, Italy.
Insights
Upper esophageal sphincter dysfunction in children with GERD can cause swallowing and breathing issues. Surgical intervention improved symptoms in one case, suggesting a role for UES dysfunction in pediatric GERD complications.
Area of Science:
- Pediatric Gastroenterology
- Neurology
- Otolaryngology
Background:
- Gastroesophageal reflux (GERD) is common in children.
- Upper esophageal sphincter (UES) dysfunction can impact swallowing and respiration.
- Associated conditions like neuromuscular disorders may influence UES function.
Purpose of the Study:
- To investigate the prevalence and impact of upper esophageal sphincter (UES) dysfunction in pediatric patients with gastroesophageal reflux (GERD).
- To explore the relationship between UES dysfunction, swallowing disorders, and respiratory symptoms in this population.
Main Methods:
- Retrospective analysis of 44 pediatric patients with GERD.
- Esophageal manometry to assess UES relaxation patterns.
- Clinical evaluation of swallowing, vomiting, failure to thrive, and pulmonary aspiration.
Main Results:
- Five out of 44 children (11.4%) exhibited UES dysfunction.
- Three of these five children had co-occurring intellectual disability and genetic syndromes (Silver Russell, 5p) or myopathy.
- UES dysfunction manifested as incomplete relaxation, incoordination with pharyngeal contractions, or both.
- GERD treatment did not resolve symptoms in four children; one underwent successful cricopharyngeal myotomy.
Conclusions:
- UES dysfunction may be a significant factor in swallowing and respiratory issues in pediatric GERD patients.
- This dysfunction can be primary or secondary to underlying neuromuscular conditions.
- Cricopharyngeal myotomy may be an effective treatment for refractory cases of UES dysfunction-related symptoms.
Abstract:
Dysfunction of the upper esophageal sphincter was found in five out of 44 children with gastroesophageal reflux. Three of the five children had mental retardation associated with Silver Russell syndrome, 5p syndrome, or minimal change myopathy. The five patients had swallowing disorders, vomiting, and failure to thrive; four also had pulmonary aspiration. Esophageal manometry showed incomplete upper esophageal sphincter relaxation in two patients, upper esophageal sphincter relaxation incoordinated with pharyngeal contractions in two other patients, and both incomplete and incoordinated upper esophageal sphincter relaxation in the last patient. Intensive and successful treatment of gastroesophageal reflux did not improve swallowing or symptoms of pulmonary aspiration in four children. The fifth patient underwent cricopharyngeal myotomy, with complete resolution of respiratory and swallowing symptoms. It is suggested that a dysfunction of the upper esophageal sphincter, either primary or secondary to neuromuscular disorders, may play a role in the swallowing disorders and respiratory symptoms of pediatric patients.