title: Monobloc minus Le Fort II for single-stage treatment of the Apert phenotype.
abstract: Treatment of the Apert syndrome phenotype aims to correct airway obstruction, exorbitism, elevated intracranial pressure, midface hypoplasia, and malocclusion. Cranial vault expansion prevents elevated intracranial pressure, normalizes head shape, and protects the globes, but variation exists in surgical timing and osteotomy to treat the midface. We present the case of an 11-year-old female patient with Apert syndrome and no prior surgical interventions who presented with severe turribrachycephaly, exorbitism, severe midface retrusion, and apertognathia. A monobloc distraction with simultaneous Le Fort II distraction was planned using computer-aided design and modeling (CAD/CAM) techniques to provide for concurrent distraction of the segments in independent vectors without bony interferences. Monobloc minus Le Fort II distraction was performed without intraoperative complications. Surgical time was 340 minutes with an estimated blood loss of 1100 mL. Distraction began on postoperative day 5 at a rate of 1.5 mm/day for the Le Fort II via an external Halo distractor and 1 mm/day for the monobloc segment via internal distractors anchored bitemporally. The monobloc was distracted a total of 17 mm in a horizontal vector, while the Le Fort II segment was distracted 18 mm horizontally and 5 mm inferiorly. The Halo distractor was removed 3 months following the procedure and the internal distractors 1 month later. Monobloc minus Le Fort II distraction enables correction of the Apert phenotype with a single-stage approach, potentially decreasing the burden of care with improved results. Utilization of CAD/CAM modeling allows for accurate planning of multisegment distraction in independent vectors without concerns for bony interferences.
authors.affiliation: Division of Plastic Surgery, The Perelman School of Medicine, University of Pennsylvania, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA.
authors.forename: James Thomas
authors.initials: JT
authors.lastname: Paliga
authors.forename: Jesse A
authors.initials: JA
authors.lastname: Goldstein
authors.forename: Phillip B
authors.initials: PB
authors.lastname: Storm
authors.forename: Jesse Adam
authors.initials: JA
authors.lastname: Taylor
mesh_terms: D000168:Acrocephalosyndactylia / Q000601:surgery*; D002648:Child; D003198:Computer Simulation; D017076:Computer-Aided Design; D005094:Exophthalmos / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008313:Malocclusion, Angle Class III / Q000601:surgery; D008437:Maxilla / Q000601:surgery; D024343:Open Bite / Q000601:surgery; D009915:Orbit / Q000601:surgery; D019857:Osteogenesis, Distraction / Q000379:methods*; D019340:Osteotomy, Le Fort / Q000379:methods*; D010347:Patient Care Planning; D017060:Patient Satisfaction; D025321:Surgery, Computer-Assisted