Oral Manifestations of Rett Syndrome—A Systematic Review

Syed Sarosh Mahdi, Hafsa Abrar Jafri, Raheel Allana, Francesco Amenta, Mariam Khawaja and Syed Saad B. Qasim
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Syed Sarosh Mahdi: Department of Community Dentistry, Jinnah Medical and Dental College, Sohail University, Karachi 74800, Pakistan
Hafsa Abrar Jafri: Department of Community Dentistry, Jinnah Medical and Dental College, Sohail University, Karachi 74800, Pakistan
Raheel Allana: Department of Paediatrics & Child Health, Aga Khan University Hospital, Karachi 74800, Pakistan
Francesco Amenta: Centre of Clinical Research, Telemedicine and Telepharmacy, School of Medicinal and Health Products Sciences, University of Camerino, 62032 Camerino, Italy
Mariam Khawaja: Department of Community Dentistry, Jinnah Medical and Dental College, Sohail University, Karachi 74800, Pakistan
Syed Saad B. Qasim: Department of Bio Clinical Sciences, Faculty of Dentistry, Kuwait University, Kuwait City 12037, Kuwait

IJERPH, 2021, vol. 18, issue 3, 1-15

Abstract: Rett Syndrome is an x linked developmental disorder which becomes apparent in females after 6 to 18 months of age. It leads to severe impairments including loss of speech, loss of hand movements/manual dexterity, characteristic hand movements such as hang wringing and intellectual disability/learning problems. This systematic review was carried out to identify the dental manifestation of Rett syndrome and to shed light on treatment options available for oral health problems associated with Rett syndrome. A systematic literature search was conducted on the PubMed, Scopus, Biomed, Web of Science, Embase, Google Scholars, Cochrane and CINAHL using the following entries: Rett syndrome ( n = 3790), Oral health and Rett syndrome ( n = 17), dental health of Rett syndrome patients ( n = 13), and the MeSH terms listed below: Rett syndrome and Oral Health ( n = 17), Rett syndrome and dentistry ( n = 29). The final review included 22 search articles. The most common oral findings was bruxism. Masseteric hypertrophy was also reported. Anterior open bite and non-physiological tooth wear was observed. Other oral manifestations of Rett syndrome included mouth breathing, tongue thrusting, digit/thumb sucking, high arch palate. Increased awareness and dental education amongst dentists and assistants regarding the dental manifestations of Rett syndrome and similar neurodevelopmental disorders is required to improve the level of care and empathy they can provide to these differently able patients. Research on dental aspects of Rett is scarce and this remains a neglected topic.

Keywords: bruxism; rare disorders; dental education (search for similar items in EconPapers)
JEL-codes: I I1 I3 Q Q5 (search for similar items in EconPapers)
Date: 2021
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