Modified Lefort Distraction Osteogenesis for the Treatment of Nager Syndrome-Associated Midface Hypoplasia: Technique and Review

The surgical management of midface hypoplasia in the setting of Nager syndrome remains a significant challenge for craniofacial surgeons. This study describes a novel technique using distraction osteogenesis and modified osteotomies for the treatment of midface bony defects in an 11-year-old child with Nager syndrome. Presurgical 3-dimensional planning was performed to design the osteotomies and placement of distractors. The surgical approach required upper buccal sulcus and extended transconjunctival incisions only. Osteotomies were performed from the pyriform aperture through the orbit to include the lateral orbital wall, with bilateral osteotomy of the zygomas through the anterior arch via the transconjunctival incision. Distraction of the en bloc midface segment was successfully performed using external distractors. Bone grafting was not required. There were no complications. External distraction was well tolerated and there were no intraoperative or postoperative complications. The distractors were removed uneventfully after consolidation. The midface was successfully advanced without the need for bone grafting or bicoronal incision. The occlusal plane was leveled and the aesthetic appearance of the child was improved. Symmetrical midface hypoplasia in the context of Nager syndrome can be successfully corrected with en bloc distraction osteogenesis of the maxilla and bilateral zygomas through modified osteotomies that exclude the upper nasal pyramid. The approach is simplified and the need for bicoronal incision and bone grafting is mitigated in this technique, which the authors have named Lefort 2.5. This is an open access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal. https://ift.tt/OBJ4xP Address correspondence and reprint requests to Barbu Gociman, MD, PhD, Division of Plastic and Reconstructive Surgery, School of Medicine, University of Utah, 30 North 1900 East, 3B400 Salt Lake City, UT 84132; E-mail: andrew.simpson@utah.edu Received 1 March, 2018 Accepted 10 May, 2018 This case was presented at the 17th Biennial Congress of the International Society of Craniofacial Surgery in Cancun, Mexico on October 26, 2017. The authors report no conflicts of interest. © 2018 by Mutaz B. Habal, MD.

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