iDentistry The Journal September-December 2017

The Journal Orthodontic treatment in some important syndromes Treacher Collins Syndrome It is also called as ‘mandibulofacial dysostosis’ and is autosomal dominant. Oral features includes fish like mouth, macrostomia, high arched palate, malocclusion, pronounced concavity of the undersurface of the mandible and an obtuse mandibular angle. In orthodontic treatment of an 11-year-old boy with Treacher Collins syndrome expansion of the maxillary arch to correct the transverse discrepancy, and extraction of permanent teeth to correct the arch length tooth-size discrepancy were done. Surgical treatment was needed to correct the open bite and improve the soft-tissue profile. Mandibular distraction osteogenesis was considered to address the micrognathia. 32 Ehler-Danlos Syndrome It is also called as ‘rubber man’. Oral features include enamel hypoplasia and severe periodontal destruction. There is also hypermobility of TMJ resulting in repeated 33 dislocation. Catharina Hagberg et al found that persons with EDS are naturally predisposed to TMJ problems. The self- registration of maximal mandibular opening capacity was a useful diagnostic tool to provide an objective clinical measure of movement capacity of the TMJ. Apert syndrome It is also called as ‘Acrocephalosyndactyly. Oral features includes high palatal vault and V- shaped maxillary alveolar ridge, trapezoid shaped appearance of lip when lip are relaxed, posterior palatal cleft and bifid uvula, retarded eruption and dental malocclusion, Class II malocclusion. David Rynearson 34 did a multisegmental LeFort I maxillary osteotomy to address the transverse, sagittal and coronal skeletal discrepancies. Following orthognathic surgery; continued orthodontics to harmonize the occlusion. 36 Turner syndrome Oral features include micrognathia, high arched palate and corners of mouth appear pulled down. There is premature eruption of teeth. 35 Russell treated a case with turner syndrome with Orthognathic surgery involving a 3- piece segmental and differential maxillary LeFort 1 osteotomy with concomitant anterior bone grafting and an asymmetric bilateral sagittal split ramus osteotomy with a midline 6-mm mandibular advancement and a 3 mm genioplasty advancement. Marfan Syndrome Oral findings are temporomandibular joint dysarthrosis, multiple odontogenic cysts of maxilla and mandible, high arched palate and bifid uvula. In a case report, the authors present the records of three patients with Marfan syndrome who were treated at a dental school. Two patients had severe periodontal disease in the absence of significant contributing local factors. The presentation of systemic symptoms and typical physical characteristics varied. The syndrome thus went unnoticed in one patient for many years 36 . Cleidocranial dysplasia Underdeveloped maxilla, multiple impacted or unerupted, permanent or supernumerary teeth 37 are some important oral findings. Rocha et al describes the treatment and long-term follow- up care of a patient diagnosed with cleidocranial dysplasia who had multiple impacted permanent and supernumerary teeth. The patient was treated with a multidisciplinary therapeutic protocol including orthodontic and surgical procedures, and traction of 11 permanent teeth. Chondroectodermal Dysplasia Oral findings mainly include deficient teeth number; and those which develop are small, rudimentary, conical, spaced and irregular in position. The permanent dentition is more likely to be defective than the deciduous one. Vol. 13 No. 3 Sep-Dec 2017

iDentistry The Journal September-December 2017 | Page 37