Radiographic manifestations of the temporomandibular joint in a case of Proteus syndrome

Abstract

Proteus syndrome is a rare disorder with progressive asymmetrical and disproportionate overgrowth of various tissues of the body. The syndrome is characterized by a wide range of malformations, including craniofacial deformities. Extraoral examination revealed several of the classical craniofacial features of Proteus syndrome: pronounced hemifacial hypertrophy, macrodactyly and hyperostosis. Intraoral examination revealed a high arched palate and gingival hyperplasia. Other findings were unilateral enlargement of the tongue, alveolar growth and dilaceration of the roots of the teeth. There were severe degenerative changes and deformities in the left temporomandibular joint but the oversized condyle was asymptomatic; there was no pain, limitation and deviation at mouth opening. Treatment was not necessary owing to the asymptomatic situation but periodic follow-up with clinical and radiographic examination was considered. The aim of this article is to describe the radiographic manifestations of an asymptomatic condyle malformation and other craniofacial, oral and dental findings in a 33-year-old female patient with known Proteus syndrome.

Dental developmental alterations in patients with dilacerated teeth

“…According to the above mentioned, it was considered that RDAs are associated to disturbance and/or failure of the epithelial diaphragm during the early root development or to a deficient apical growth in length of the Hertwig’s epithelial root sheath (12,13). Previous studies communicated that root dilaceration can be found in patients with some genetic syndromes as Kabuki, Axenfeld-Rieger, Smith–Magenis Ehlers-Danlos and Proteus (14-18). These patients showed dilacerated teeth and other dental anomalies: tooth agenesis, taurodontism, enamel hypomaturation, microdontia, screwdriver central incisors, peg-shaped lateral incisors, enamel hypoplasia, short roots, atypical furcation of the roots, and supernumerary roots.…”

Phenotype and Surgical Treatment in a Case of Proteus Syndrome With Craniofacial and Oral Findings

“…Further observations refer to unusually high-set frenula, which can cause diastema mediale or develop bifid (47). There are also individual reports of unilateral hyperplasia of the tongue (21,51,52).…”

“…A systematic review of published data on maxillofacial manifestations of Proteus syndrome with special consideration of oral manifestations (32) (Table III) describes gingival hyperplasia in only two cases (49,50) based on 14 studies used for this evaluation. This overview can be supplemented by further two reports on gingival hyperplasia in Proteus syndrome (36,51). However, no histological findings are detailed in either report.…”

“…Hyperplasia of the jaws is usually unilateral in Proteus syndrome (3,12,51). Skelettal overgrowth can affect the entire side of one or both jaws or only sections of the respective bone, for example the condylar process (51,52,55,56). Functionally effective, symmetric underdevelopment of the mandibular corpus was also reported (57).…”

Thoracolumbar Scoliosis in a Patient With Proteus Syndrome

“… 3 , 6 About 200 cases of PS have been reported in the literature, and incidence of bone malformations is unknown. 7 , 8 The exact cause, pathogenesis, and embryologic origin of PS remain a subject of discussion. 9 , 10 There are limited reports regarding the diagnosis and management of PS with its possible resultant scoliosis.…”