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Alternative Method for Full Oral Rehabilitation in Patients with Pycnodysostosis Syndrome: A Case Report

Authors Costa MM, Pennisi PRC, Oliveira ACZM, Oliveira JEC, Oliveira GV, Moffa EB, Paranhos LR

Received 29 January 2020

Accepted for publication 17 March 2020

Published 15 April 2020 Volume 2020:12 Pages 131—139


Checked for plagiarism Yes

Review by Single anonymous peer review

Peer reviewer comments 2

Editor who approved publication: Professor Christopher E. Okunseri

Márcio Magno Costa,1 Pedro Rogério Camargos Pennisi,1 Andréa Cristina Zacarias Martins de Oliveira,2 João Edson Carmo de Oliveira,1 Gustavo Vilela Oliveira,3 Eduardo Buozi Moffa,4 Luiz Renato Paranhos1

1Federal University of Uberlândia, School of Dentistry, Uberlândia, MG, Brazil; 2Private Clinic, Uberlândia, Brazil; 3Federal University of Uberlândia, School of Mechanical Engineering, Uberlândia, MG, Brazil; 4University Center - UNIFAE, School of Dentistry, São João da Boa Vista, SP, Brazil

Correspondence: Luiz Renato Paranhos
Federal University of Uberlândia, Uberlândia, MG, Brazil
Tel +55 34 32258145

Abstract: Pycnodysostosis (PYCD) is a rare autosomal recessive skeletal dysplasia arising from a change in the production of the cathepsin K enzyme and it is also known as Maroteaux-Lamy Syndrome. The main changes in the buccal and maxillofacial region are modifications of the facial bones (leading to the loss of the mandibular angle due to micrognathia), deep palate, premature and/or delayed tooth eruption, hypopneumatization of the maxillary sinuses, chin hypoplasia, hypercementosis, enamel hypoplasia, hyperdontia, incomplete dentin calcification, root hypoplasia, pulp chamber obliteration, and increased free functional space. This paper aims to report a case of a patient with PYCD, in which are highlighted the oral manifestations of this syndrome and present an option of oral rehabilitative treatment through a removable overdenture made on natural tooth of the patient. The technique of dental coating through overdenture is a simple, viable and conservative alternative for oral rehabilitation of patients with large maxillomandibular discrepancies, as in cases of pycnodysostosis, especially in the presence of increased free space – characteristic of patients with this syndrome. The result was quite satisfactory both aesthetically and functionally and provided a better life quality for the patient.

Keywords: malocclusion, pycnodysostosis, oral rehabilitation

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