Dentomaxillofacial imaging in Proteus syndrome

Abstract

Proteus syndrome is a rare condition that involves atypical growth of the bones, skin and head and a variety of other symptoms. Only a few authors have reported on the craniofacial manifestations so far. The authors present a case of a 7-year-old girl with Proteus syndrome in which the facial skeleton showed unilateral overgrowth. The analysis of the radiological evaluation revealed a bialveolar prognathism, a skeletal class III, a dolicocephalic growth pattern and a left convex face scoliosis. On the left side, the lesser wing of the sphenoid was elevated and the ethmoidal cell complex was hypertrophic. The left ramus and body of the mandible were enlarged. The asymmetric dental development with a precocious dental age on the affected side was the most striking feature on the panoramic view. Early diagnosis and therapy depend on clinical evaluation and imaging. Therefore, further evaluations on the craniofacial features of patients with Proteus syndrome are necessary in order to establish a list of characteristic symptoms.

Orthopedic Treatment Outcomes in Class III Malocclusion

“…[10][11][12][13] Class III malocclusions associated with craniofacial disharmonies are much more difficult to treat and tend to relapse. [14][15][16] Early treatment of Class III malocclusion has been advocated to reduce the need of treatment in the permanent dentition, when camouflage orthodontic treatment or surgery become the only options. 1 A series of treatment approaches can be found in the literature regarding orthopedic treatment in Class III malocclusion.…”

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

“…8,[10][11][12][13][15][16][17]19 A high arched palate and unilateral enlargement of the tongue on the affected side were found in our patient. Most of the teeth on the left side were extracted owing to periodontal disease.…”

“…9 The diagnosis depends heavily on clinical evaluation and imaging. 7 Diagnostic criteria for PS have been published by Biesecker et al 9 and Turner et al 5 Although several cases about the general clinical and genetic findings of PS have been reported in the literature, few reports have been published describing the dentomaxillofacial manifestations, 8,[10][11][12][13][14][15][16][17] and the radiographic findings of the temporomandibular joint (TMJ) in PS have not been described in detail. Our case report presents the radiographic manifestations of an asymptomatic condyle malformation and also other craniofacial, oral and dental findings in a female patient with known PS.…”

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

“…These cutaneous signs were called CCTN because of the similarity between the affected skin and the surface of the exposed brain. Until recently, the "gyrated (skin) surfaces" (35) or "gyriform masses" (36) were so characteristic for Proteus syndrome that evidence of CCTN -in conjunction with the general category -was conclusive for clinical diagnosis of Proteus syndrome (8,37). Current studies show this specificity of the finding is not valid (38).…”