Achondroplasia: Orocraniofacial Features and Orthodontic-Surgical Management Guidelines Proposal

J Craniofac Surg. 2018 Nov;29(8):2186-2191. doi: 10.1097/SCS.0000000000004819.

Abstract

In this study, the authors aimed to describe orocraniofacial features and to suggest orthodontic-surgical managements in achondroplasia, based on a literature review. The authors focused on skeletal and dentoalveolar malocclusion in order to highlight the place of orthognathic surgery, based on our experience of 3 patients. Maxillary hypoplasia in achondroplasia typically results in an Angle class III malocclusion with an anterior open bite. The other orocraniofacial features include enlarged calvarium, prominent forehead and frontal bossing, midface hypoplasia, elongated lower face and saddle-shaped nose due to lack of development of the nasomaxillary complex.All our patients had a typical facial appearance but each of them had their own particularities regarding medical history and severity of the dentoskeletal dysmorphosis. Two of them were successfully treated by orthognathic surgery; the other declined surgical treatment and underwent orthopedic treatment only (anchorage plates). The treatment failure of this 3rd patient raises the question of the efficiency of anchorage plates in achondroplasia. In the light of the literature and our results, the authors conclude the need for personalized management based on age, medical history, severity of the dentoskeletal dysmorphosis, functional and/or esthetic disorders, and the patient's needs and requests. In any patient, orthodontic management should be initiated at an early age, and orthognathic surgery modalities should be personalized and adapted to each situation.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Achondroplasia / complications
  • Achondroplasia / pathology
  • Achondroplasia / surgery*
  • Adolescent
  • Bone Plates
  • Child
  • Female
  • Humans
  • Male
  • Malocclusion, Angle Class III / etiology
  • Malocclusion, Angle Class III / surgery*
  • Orthodontics, Corrective
  • Orthognathic Surgical Procedures
  • Orthopedic Procedures / instrumentation
  • Practice Guidelines as Topic
  • Skull / abnormalities