title: Orofacial manifestations and dental considerations in association with Varadi-Papp syndrome: report of a rare case.
abstract: Varadi-Papp syndrome or oral-facial-digital syndrome type VI (OFDS VI) is a rare, autosomal recessive disorder characterised by a specific congenital malformation of the cerebellum and a broad spectrum of other phenotypic findings. It is distinguished from other OFDSs by metacarpal abnormalities with central polydactyly and by cerebellar abnormalities. Treatment for such patients is often considered challenging due to the presence of intellectual disability, hypotonia, and abnormal respiratory pattern in these patients. The present article reports the oral and systemic manifestations of a 5-year-old female patient having Varadi-Papp syndrome, considerations taken in her dental treatment and the successful management performed. The patient was followed up every 3 months for 2 years, to evaluate plaque control and to continue with the plaque control regimen. Periodic oral examinations and maintenance of good oral hygiene helped to improve the quality of life of the child. This case illustrates the favourable treatment outcomes in a Varadi-Papp syndrome patient. Furthermore, the need for periodic oral examinations and maintenance of good oral hygiene to prevent any complications in such patients has been highlighted.
authors.affiliation: Department of Pedodontics and Preventive Dentistry, Maulana Azad Institute of Dental Sciences, Delhi, India. nidhimahajandr84@gmail.com.
authors.forename: N
authors.initials: N
authors.lastname: Chhabra
authors.affiliation: Department of Dental Surgery, Safdarjang Hospital, Delhi, India.
authors.forename: A
authors.initials: A
authors.lastname: Chhabra
authors.affiliation: Department of Periodontics, Maulana Azad Institute of Dental Sciences, Delhi, India.
authors.forename: S
authors.initials: S
authors.lastname: Tandon
mesh_terms: D002675:Child, Preschool; D003729:Dental Care; D003773:Dental Plaque; D005260:Female; D006801:Humans; D009958:Orofaciodigital Syndromes / Q000473:pathology / Q000628:therapy*; D010641:Phenotype; D011788:Quality of Life