ABSTRACT
Feeding problems are common in Rett syndrome in which there are characteristic oropharyngeal abnormalities. This study investigated the ways in which individuals regulated their respiration accordingly, and how this affected their overall feeding ability. Respiration during feeding was studied in 28 individuals, recording nasal airflow, chest and abdominal movements, and swallow sounds. Time to first swallow was defined as that between introduction of liquid/solid on a spoon and the first swallow. Six individuals also had videofluoroscopy with simultaneous respiration monitoring. Results indicated different respiratory patterns according to the time to first swallow and neurological status; the amount of time spent in apnoea was particularly important. Videofluoroscopy showed that apnoeas occurred most often when liquid was delayed in the pharynx, but this could be overcome in subjects with a lower level of disability. The carer's estimate of the time for feeding was significantly related to the time spent in apnoea with liquids.
Subject(s)
Apnea/physiopathology , Feeding and Eating Disorders of Childhood/etiology , Rett Syndrome/physiopathology , Adolescent , Adult , Apnea/diagnosis , Apnea/etiology , Child , Child, Preschool , Deglutition , Feeding and Eating Disorders of Childhood/diagnosis , Female , Fluoroscopy , Humans , Monitoring, Physiologic , Respiration , Rett Syndrome/complications , Video RecordingABSTRACT
Feeding abilities in 20 individuals with Rett syndrome aged 1 1/2 to 33 years were investigated by history and clinical assessment during a meal, followed by videofluoroscopy of feeding. All were shown to have reduced movements of the mid and posterior tongue, with premature spillover of food and liquid from the mouth into the pharynx. They also showed delayed pharyngeal swallow, but otherwise pharyngeal problems were minimal. These findings were noted to be similar to those in Parkinson's disease. Those individuals with the most general neurological impairment tended to have the worst feeding problems and were smaller and malnourished.
Subject(s)
Child Nutrition Disorders/etiology , Deglutition Disorders/etiology , Growth Disorders/etiology , Rett Syndrome/complications , Activities of Daily Living , Adolescent , Adult , Child , Child Nutrition Disorders/diagnosis , Child, Preschool , Deglutition Disorders/diagnosis , Deglutition Disorders/prevention & control , Female , Fluoroscopy , Growth Disorders/diagnosis , Humans , Infant , Nutrition Assessment , Rett Syndrome/classification , Videotape RecordingABSTRACT
A multidisciplinary assessment, including videofluoroscopy, was carried out on 14 children with feeding difficulties associated with neurological problems. Recommendations were made on all aspects of feeding and the trunk position was changed for half of the patients. A later interview with the parents confirmed that the recommendations had been helpful. The optimum trunk position for feeding was determined during videofluoroscopy by positioning the patient in the erect or reclined position, and also by analysis of relative difficulties during the oral and pharyngeal phases of swallowing. Those with difficulties mainly in the oral phase fed best in the reclined position; those with difficulties mainly in the pharyngeal phase fed best in the erect position, particularly if they had upper-oesophageal sphincter spasm in association with a tonic labyrinthine reflex.
