title: Management of nasal deformity in osteogenesis imperfecta.
abstract: Osteogenesis imperfecta (OI) is a rare hereditary disorder of collagen synthesis that results in weak bones that are easily fractured resulting in deformities. Osteogenesis imperfecta refers to a wide array of genetic disorders with variable phenotypic presentation. In general, type I is the mildest form of disease; types IV, III, and II, respectively, increase in severity. There is lack of knowledge about the feasibility of nasal surgery in patients with bony disorders in the literature. Although rhinoplasty is one of the most frequent procedures all over the world, there are only very few case reports about OI patients undergoing rhinoplasty. The authors could find 2 old case reports, published in 1965 and 1977. The only recent publication that could be found was published in 2000, but it was about a follow-up case that had been operated 15 years ago. A 24-year-old woman with OI who has been operated on because of severe airway obstruction and cosmetic reasons is presented. Open rhinoplasty was performed, keeping in mind that bones and cartilages are more fragile, and they heal late in OI. Therefore, greenstick fractures were preferred instead of median osteotomies, and cartilages were fixed to each other at multiple points with nonabsorbable sutures. There were no complications in the postoperative period, and the patient was very satisfied with her breathing and appearance. Current literature is reviewed in accordance with our experience with this unique case. In conclusion, good cosmetic and functional rhinoplasty results may be obtained in OI cases with careful case selection, limited mobilization of the nasal bones, and careful fixation of the nasal cartilages with nonabsorbable sutures.
authors.affiliation: Department of Plastic Surgery, School of Medicine, Reconstructive and Aesthetic Surgery, Ege University, Izmir, Turkey.
authors.forename: Ufuk
authors.initials: U
authors.lastname: Bilkay
authors.forename: Yigit Ozer
authors.initials: YO
authors.lastname: Tiftikcioglu
authors.forename: Candan
authors.initials: C
authors.lastname: Mezili
mesh_terms: D005260:Female; D006801:Humans; D015508:Nasal Obstruction / Q000209:etiology / Q000601:surgery; D009666:Nose / Q000002:abnormalities / Q000601:surgery*; D010013:Osteogenesis Imperfecta / Q000150:complications*; D010027:Osteotomy / Q000379:methods; D012225:Rhinoplasty / Q000379:methods*; D055815:Young Adult