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Maxillofacial manifestations of Proteus syndrome: a systematic review with a case report

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Abstract

Objectives

Proteus syndrome (PS) is an extremely rare disorder with asymmetric and disproportionate bone overgrowth. Craniofacial abnormalities in PS are less frequent than skeletal abnormalities. Although there are recognized oral and maxillofacial manifestations of PS, few case reports describing these manifestations are available. Thus, the objective of this systematic review and case report is to describe oral and maxillofacial manifestations of PS and to report a PS case.

Methods

A 31-year-old male presented with restricted mouth opening and pain during mastication. A panoramic radiograph and an occlusal radiograph were obtained. Reports with relevant keywords were assessed. Data were summarized and demonstrated using a critical appraisal checklist for case reports.

Results

The panoramic radiograph demonstrated unilateral overgrowth of the mandible, impacted teeth, and deciduous prolonged retention. Thirteen PS case reports were identified.

Conclusions

Proteus syndrome oral and maxillofacial manifestations may include dental agenesis, impacted teeth, malocclusion, asymmetric dental growth and maturation, frontal line displacement, asymmetric tongue enlargement, mandibular hemihypertrophy and asymmetry, presence of exostoses/hyperostosis, degenerative changes in the temporomandibular joint, alterations of maxillary and mandibular vertical and/or horizontal growth, and enlargement of mandibular canal and foramen. The PROSPERO systematic review registration number is CRD42019140942.

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Correspondence to Luciana Munhoz.

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Luciana Munhoz, Danielle Ayumi Nishimura, Plauto Christopher Aranha Watanabe, and Emiko Saito Arita declare no conflicts of interest.

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All procedures followed were in accordance with the ethical standards of the responsible on human experimentation (institutional and national) and with the Helsinki Declaration of 1964 and later versions. As the product presented in the text is not tested in humans, as specified in the manuscript text, informed consent was not already obtained from all the patients.

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Munhoz, L., Arita, E.S., Nishimura, D.A. et al. Maxillofacial manifestations of Proteus syndrome: a systematic review with a case report. Oral Radiol 37, 2–12 (2021). https://doi.org/10.1007/s11282-019-00416-y

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