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Air swallowing in Rett syndrome
R E Morton1, L Pinnington, R E Ellis
1Derbyshire Children's Hospital and Ronnie Mac Keith Child Development Centre, Derby City General Hospital, UK.
Developmental Medicine and Child Neurology
|May 5, 2000
Summary
Excessive air swallowing causes bloating in Rett syndrome (RS). This study found breath-holding during apnoea is a key cause, worsened by distress. An air-leaking dummy showed promise but had poor tolerance.
Area of Science:
- Neurology
- Gastroenterology
- Pediatrics
Background:
- Bloating due to excessive air swallowing is common in individuals with Rett syndrome (RS).
- Understanding the mechanisms of air swallowing is crucial for managing symptoms in RS.
Purpose of the Study:
- To investigate the causes of excessive air swallowing and subsequent bloating in individuals with Rett syndrome.
- To compare air swallowing patterns in RS with those in children with quadriplegic cerebral palsy.
Main Methods:
- Videofluoroscopy and respiration monitoring during feeding in seven individuals with RS.
- Resting monitoring of respiration, swallowing, and vocal cord position in 33 individuals with RS.
- Comparison with 11 individuals with quadriplegic cerebral palsy.
Main Results:
- Isolated pharyngeal swallows and mouth breathing were observed in both groups, but did not fully explain severe air bloat in RS.
- Twenty of 33 individuals with RS experienced air bloat, primarily linked to air swallowing during breath-holding (17/20) or hyperventilation (3/20).
- A dummy with an air leak was the most effective intervention for reducing air swallowing during apnoea, though poorly tolerated.
Conclusions:
- Air swallowing during breath-holding is a significant contributor to bloating in Rett syndrome.
- Distress exacerbates apnoeas and air bloat, suggesting potential benefits from anxiolytic medications.
- Further research into managing air swallowing and associated bloating in RS is warranted.