Oral Manifestations in Rett Syndrome: a Study of 17 Cases
Scientific Article Oral manifestationsin Rett syndrome: a study of 17 cases RosangelaAlmeida Ribeiro, MSAna Regina Romano,MS Esther GoldenbergBirman, PhD. M;ircia P. Alves Mayer, MS, PhD Abstract holding spells, forced expulsion of air or saliva, elec- Seventeen patients with a meanage of 7.33 (range 2.7- troencephalogram abnormalities, seizures, peripheral 12.7) years with Rett syndrome(a progressive neurologi- vasomotor disturbances, constipation, abdominal cal disorder that occurs mainlyin females) were evaluated bloating, night laughing, hypotrophic small feet and hands, weight loss, growth retardation, and apparent for oral manifestationsand habits. The most frequent habits s were digit~hand sucking and~orbiting (17/17), bruxism insensitivity to pain. 1.3-7 In 1989, Rice noted that the (14/17), mouth breathing (7/17), drooling (5/17), Rett patient is extremely thin in spite of having a good tongue thrusting (5/17). Gingivitis (13/17) was the appetite, and that difficulty in gaining weightis a threat commonalteration of soft tissues. Only2.7% of tooth sur- to the child’s life. faces were decayed. Nonphysiologicaldental attrition was Oral findings present in 71%(12/17) of the children. Palatal shelving could be observedin 53 %(9/17) of the children, probably Although mentioned in some previous reports, oral related to the digit~hand sucking and~orbiting habits. A findings in Rett syndromehave received scant atten- high prevalenceof anterior open bite (9/17) wasobserved. tion in the literature. Bruxismis mentionedfrequently. No patients exhibited anomalies of tooth number, size, The RS bruxism is an episodic creaking sound similar form, structure, or eruption. (Pediatr Dent 19:349-52,1997) to that of a slowly uncorkedwine bottle.
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