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ARS Home » Plains Area » Houston, Texas » Children's Nutrition Research Center » Research » Publications at this Location » Publication #92966
Title: OROPHARYNGEAL DYSFUNCTION AND GASTROESOPHAGEAL DYSMOTILITY ARE COMMON AMONGGIRLS AND WOMEN WITH RETT SYNDROME

Author
item Motil, Kathleen
item SCHULTZ, REBECCA - BAYLOR COLLEGE OF MEDICIN
item BROWNING, KERRIE - TEXAS CHILDREN'S HOSPITAL
item TRAUTWEIN, LYNN - BAYLOR COLLEGE OF MEDICIN
item GLAZE, DANIEL - BAYLOR COLLEGE OF MEDICIN

Submitted to: Journal of Pediatrics
Publication Type: Peer Reviewed Journal
Publication Acceptance Date: 3/1/1999
Publication Date: N/A
Citation: N/A

Interpretive Summary: Feeding children with neurological disorders, including Rett syndrome (RS), is often difficult. Feeding difficulties lead to nutritional problems for these patients. We wanted to define the clinical characteristics of oral, pharyngeal and upper gastrointestinal problems and evaluate their impact on the dietary intake and nutritional status of girls and women with RS. Swallowing dysfunction was found in all our subjects, while nearly 70 percent had upper gastrointestinal problems. The scope and severity of these abnormalities were apparent only by videofluoroscopy. The extent of these eating-related problems in girls with Rett syndrome warrants early diagnostic evaluation and strategies to lessen the adverse impact in order to improve the nutritional status of these patients. This study is significant because this is the first time the extent of these problems and their effects on nutrition have been delineated in girls with Rett syndrome.

Technical Abstract: Objective: To characterize the clinical features of oropharyngeal and gastroesophageal dysfunction and their impact on the dietary intake and nutritional status of female subjects with Rett syndrome (RS). Study design: The clinical features of dysfunction in 13 female RS subjects, age 3.7 to 25.7 y, were characterized by an oral feeding assessment, swallowing gfunction study, upper gastrointestinal series, and parental questionnaire. Growth, nutritional status, and body composition were determined by stadiometry and anthropometry. Dietary intakes were determined from 3-day food records. Results: Oropharyngeal dysfunction and gastroesophageal dysmotility were present in 100% and 69%, respectively, of RS subjects. The scope and severity of these abnormalities were apparent only by videofluoroscopy. Abnormalities of oropharyngeal function included poor tongue mobility, reduced oropharyngeal clearance, and laryngeal penetration nof liquids and solid food during swallowing. Esophageal dysmotility included absent primary or secondary waves, delayed emptying, atony, the presence of tertiary waves, spasm, and gastroesophageal reflux. Gastric dysmotility included diminished peristalsis or atony. Lower dietary energy intakes were associated with persistence of residue in the valleculae and pyriform sinuses and less body fat. Conclusion: The prevalence of oropharyngeal dysfunction and gastroesophageal dysmotility warrants early diagnostic evaluation and intervention strategies to improve the nutritional status of girls and women with RS.