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Dentomaxillofacial imaging in Proteus syndrome
H Korbmacher1, M Tietke, U Rother
1Department of Orthodontics, University Hospital Hamburg-Eppendorf, Martinistr. 52, 20246 Hamburg, Germany. korbmacher@uke.uni-hamburg.de
Dento Maxillo Facial Radiology
|June 18, 2005
Summary
Proteus syndrome can cause unusual bone and facial growth. This case highlights unilateral overgrowth and dental asymmetry in a child, emphasizing the need for detailed craniofacial evaluation.
Area of Science:
- Medical Genetics
- Craniofacial Surgery
- Pediatric Dentistry
Background:
- Proteus syndrome is a rare congenital disorder characterized by mosaic overgrowth of multiple tissues.
- Craniofacial manifestations are reported infrequently, necessitating further investigation into characteristic symptoms.
Observation:
- A 7-year-old girl with Proteus syndrome presented with significant unilateral overgrowth of the facial skeleton.
- Radiological evaluation revealed bialveolar prognathism, skeletal class III, dolicocephalic pattern, and left facial scoliosis.
Findings:
- Specific findings included an elevated left lesser sphenoid wing, hypertrophic ethmoidal complex, and enlarged left mandibular ramus and body.
- Asymmetric dental development with a precocious dental age on the affected side was a prominent feature on panoramic imaging.
Implications:
- Early diagnosis and effective therapy for Proteus syndrome rely on thorough clinical and imaging evaluations.
- Establishing a comprehensive list of characteristic craniofacial symptoms is crucial for improved patient management.