The Kaleidoscope of Midface Management in Apert Syndrome: A 23-Year Single-Institution Experience.

BACKGROUND

This study assesses operative trends over time and outcomes of 5 osteotomy techniques used to treat the midface in Apert syndrome. Using clinical and photogrammetric data, the authors present their institution's selection rationale for correcting specific dysmorphologies of the midface in Apert syndrome based on the individual phenotype.

METHODS

The authors retrospectively reviewed patients with Apert syndrome who underwent midface distraction from 2000 through 2023. Patients were temporally divided by 2012 to assess differences in surgical approach. Postoperative facial dimension changes, surgical and perioperative characteristics, and complications data were compared across techniques.

RESULTS

A total of 39 patients with Apert syndrome underwent 41 midface distraction procedures (23 [56%] in the early cohort and 18 [44%] in the late cohort). The use of segmental osteotomies for frontofacial advancement increased from 0% before 2012 to 61% from 2012 onwards ( P < 0.001). Monobloc with bipartition was the only technique associated with decreased intercanthal distance ( P = 0.016), and Le Fort II with zygomatic repositioning achieved the greatest median change in bilateral canthal tilt of 8.7 degrees (interquartile range, 1.3, 8.7 degrees; P = 0.068). Monobloc with Le Fort II achieved the greatest median change in facial convexity of -34.9 degrees (interquartile range, -43.3, -29.2 degrees; P = 0.031). Severity of complications, stratified by Clavien-Dindo grade, was greater in transcranial than subcranial procedures, but similar between segmental and nonsegmental osteotomies ( P = 0.365).

CONCLUSIONS

In studying the midface in Apert syndrome and attempting to resolve its varying functional and aesthetic issues, the authors document an evolution toward multipiece osteotomies over time. With an appreciation for differential midface hypoplasia, segmentation is found to be associated with more effective normalization of the face in Apert syndrome.

CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.