Standard orthodontic treatment after condylectomy for patients with active unilateral condylar hyperplasia.

INTRODUCTION: Unilateral condylar hyperplasia (UCH) is a progressive, nonneoplastic overgrowth of the condyle of the temporomandibular joint. For treating active UCH, a popular method combines orthognathic surgery with high condylectomy and orthodontic treatment. The goal of this study was to introduce a new method to correct asymmetry for active UCH. METHODS: Retrospectively, 47 patients with active UCH were divided into horizontal-type, vertical-type, and combined-type. All patients were treated with condylectomy plus postsurgery standard orthodontics (CPSO) with applied miniscrews implanted in infrazygomatic crest and hard palate to intrude affected side of maxillary molars and apply intermaxillary traction for contralateral molars. Cone-beam computed tomography was taken at presurgery, postsurgery, and the end of orthodontics (T3). RESULTS: In the vertical (n = 10) and combined (n = 28) types, deviation of the chin and the canting of the mandible and maxillary occlusal plane were significantly reduced at T3. A difference in the torque of bilateral maxillary first molar (U6) and bilateral mandibular first molar (L6) was significantly reduced at T3. The anterior, superior, and posterior joint spaces in the vertical-type and combined-type were significantly decreased at T3 compared with postsurgery. In contrast, in the horizontal-type group (n = 9), the deviation of the chin was corrected; however, the canting of the mandible and maxillary occlusal plane was significantly increased at T3 compared with presurgery. CONCLUSIONS: CPSO restored facial and occlusal symmetry for vertical-type and combined-type active UCH and returned affected-side condyle to the glenoid fossa. However, CPSO was not suitable for treating the horizontal-type UCH.

Generalized bullae in a young girl with KRT6A-related pachyonychia congenita.

Pachyonychia congenita (PC) is a rare genodermatosis showing heterogeneity with five causative keratin genes (KRT6A, KRT6B, KRT6C, KRT16, or KRT17). Clinically, PC is characterized by hypertrophic onychodystrophy, painful palmoplantar keratoderma, oral leukokeratosis, and follicular hyperkeratosis. We describe an atypical case of PC in a young Chinese girl presenting with generalized bullae and identified a recurrent heterozygous missense mutation c.1406T > C (p.Leu469Pro) in KRT6A. This suggests that bullae may represent an important feature of KRT6A-related PC.