Skeletal and Dental Habilitation of Residual Alveolar and Maxillary Clefts.

ABSTRACT: Management of residual clefts of the alveolus and maxilla requires the coordinated effort of multiple members of the craniofacial team including surgeon, orthodontist, and when teeth are hypoplastic or absent, the prosthodontist to achieve complete habilitation. Such cooperation among specialists begins early in the patient's life and continues through completion of care.Although numerous publications on this topic exist, few present definitive multidisciplinary reconstructive outcomes with longterm results. In this review paper, the authors present our comprehensive, multidisciplinary protocols, experience, and techniques as they have evolved with over 35 years of practice at our Craniofacial Center.Details of our updated protocols for each intervention and procedure, including our current thoughts on appropriate timing, follow up and advantages from the incorporation of current technologies are discussed. Close cooperation among specialists at all stages of care, the use of evolving technology, and adherence to, and modification where indicated, of time honored team protocols enables us to consistently achieve successful functional and esthetic outcomes, while minimizing complications.

The Median Mandibular Symphysis Tessier 30 Cleft:: 3D Imaging Analysis and Review of the Literature.

Atypical craniofacial clefts of the upper facial region have been well documented; however, the mandibular clefts remain rare and reported as isolated case reports. We report a case of a median mandibular cleft within the context of a Tessier 0-14 axis that we have followed over a 5-year period without surgical/orthodontic intervention. The mandibular symphysis cleft remained open without evidence of the fusion, in contrast to ossification of the metopic dysraphism. Within this context, we present a review of the median mandibular cleft cases from 1819 to 2015.

RUNX2 quadruplication: additional evidence toward a new form of syndromic craniosynostosis.

The RUNX2 transcription factor regulates osteoblast differentiation. Its absence, as with cleidocranial dysplasia, results in deficient bone formation. However, its excess seems to follow a dose response of over ossification. RUNX2 duplications (3 copies) are exceedingly rare but have been reported to cause craniosynostosis. There are no existing reports of quadruplications (4 copies). We present a case study of a boy with an atypical skull deformity with pan-craniosynostosis whose microarray analysis revealed 4 copies of a 1.24-Mb region from 6p12.3 to 6p21.1 containing the RUNX2 gene. Further characterization of this osteogenic pathway may aid in our understanding of the pathogenesis and subsequent prevention and treatment of syndromic craniosynostosis.