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A cephalometric study in patients with Wilson's disease
1Department of Neurology and Research Center of Neurology in Second Affiliated Hospital, and Key Laboratory of Medical Neurobiology of Zhejiang Province, Zhejiang University School of Medicine, Hangzhou, China; Department of Neurology, Huashan Hospital, Shanghai Medical College, Fudan University, Shanghai, China.
Summary
Patients with Wilson's disease (WD) exhibit distinct craniofacial features, including maxillary protrusion and vertical mandibular growth patterns. Early-onset neurological symptoms in female WD patients are associated with more pronounced vertical mandibular growth and retrusion.
Area of Science:
- Dentistry
- Genetics
- Neurology
Background:
- Wilson's disease (WD) is a genetic disorder affecting copper metabolism.
- Previous observations suggest potential unique dento-maxillo-facial structures in WD patients.
Purpose of the Study:
- To investigate and characterize craniofacial structural differences between adult patients with Wilson's disease and healthy controls.
- To identify specific cephalometric parameters associated with WD.
Main Methods:
- A case-control study involving 44 adult WD patients and 67 healthy controls.
- Lateral cephalometric radiography was used to analyze 13 angular parameters of craniofacial features.
- Cephalometric evaluations were performed blindly by trained professionals.
Main Results:
- Both male and female WD patients showed a tendency towards maxillary protrusion (higher SNA angle) and vertical mandibular growth patterns.
- Specific findings included higher SNA, ANB, and angle of convexity, and a lower AB angle in WD groups compared to controls.
- Female patients with early-onset neurological symptoms (<20 years) exhibited significantly greater vertical mandibular growth (higher SNtoGoGn, Y-axis, and mandibular plane angles) and mandibular retrusion (lower facial angle) compared to late-onset patients.
Conclusions:
- Wilson's disease patients present with characteristic craniofacial features, notably maxillary protrusion and a vertical growth pattern of the mandible.
- Early neurological onset in female WD patients correlates with more pronounced vertical mandibular development and retrusion, suggesting a potential link between disease progression and facial morphology.