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The Kaleidoscope of Midface Management in Apert Syndrome: A 23-Year Single-Institution Experience
Meagan Wu, MA, Benjamin B. Massenburg, MD, Jinggang J. Ng, MA, Dominic J. Romeo, MA, Jordan W. Swanson, MD MSc, Scott P. Bartlett, MD, Jesse A. Taylor, MD;
Children's Hospital of Philadelphia, Philadelphia, PA, USA
Purpose: This study assesses operative trends over time and outcomes of five osteotomy techniques used to treat the Apert midface. Using clinical and photogrammetric data, we present our selection rationale for correcting specific dysmorphologies based on the individual phenotype.
Methods: We identified patients with Apert syndrome who underwent midface distraction from 2000 to 2023, temporally dividing them by the year 2012 to assess differences in surgical approach. Postoperative facial dimension changes, perioperative characteristics, and complication profiles were compared across techniques.
Results: Thirty-nine patients with Apert syndrome underwent 41 procedures: 23 (56%) in the early cohort and 18 (44%) in the late cohort. The use of segmental osteotomies increased from 0% before 2012 to 61% from 2012 onwards (p<0.001). Only monobloc with bipartition decreased intercanthal distance (p=0.016). Le Fort II with zygomatic repositioning achieved the greatest median change in bilateral canthal tilt of 8.7° (IQR 1.3°, 8.7°; p=0.068). Monobloc with Le Fort II achieved the greatest median change in facial convexity of -34.9° (IQR -43.3°, -29.2°; p=0.031). Severity of complications, stratified by Clavien-Dindo grade, was greater in transcranial than subcranial procedures but similar between segmental and non-segmental osteotomies (p=0.365).
Conclusions: In studying the Apert midface and attempting to resolve its varying functional and aesthetic issues, we document an evolution towards multi-piece osteotomies. With an appreciation for differential midface hypoplasia, segmentation is associated with more effective normalization.
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