Osteogenesis imperfecta (OI) is characterized by brittle bones, premature hearing loss, blue sclera, dental abnormalities, and short stature. Maxillofacial pathology is marked in many OI patients and includes a high incidence of class III malocclusion secondary to a retrusive maxilla relative to both the mandible and cranial base.
Review of literature shows that most of the orthognathic surgeries performed in the setting of OI are double jaw surgeries, in the form of maxillary advancement and mandibular setback. However, severe maxillary hypoplasia is usually not correctable with single-stage maxillary advancement. Distraction osteogenesis (DO) is a technique that relies on the normal healing process that occurs between controlled, surgically osteotomized bone segments and it is a relatively widely used technique in modern management of craniofacial conditions.
Distraction osteogenesis has been reported in only several patients with OI. There is only 1 previously documented case of maxillary distraction in the craniofacial literature. The authors present here the successful management of a patient with OI and severe class III malocclusion using LeFort I osteotomy and DO with an external rigid distractor.
At 12 months follow-up, the patient had no complications and maintained stable maxillary position with normal occlusion, improvement of facial appearance, obstructive airway symptoms, speech, and chewing.
This case serves to reinforce the safety and efficacy of DO in patients with OI. The authors did not significantly change our distraction protocol and did not have any complications, therefore the authors believe that DO should be the preferable treatment technique for severe malocclusion in OI patient population.