Treatment of the Syndromic Midface: A Long-Term Assessment at Skeletal Maturity

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Abstract

Background:

Midfacial advancements improve function and normalize appearance in growing children with syndromic craniosynostosis yet carry substantial risks while often offering only transient benefits. The authors sought to evaluate outcomes and the number of advancements required, at skeletal maturity.

Methods:

The authors performed a retrospective review of all children undergoing rigid external distraction Le Fort III advancement who had reached skeletal maturity, including examinations, photographic scoring, and cephalometric, anthropometric and polysomnography data.

Results:

Over 15 years, 104 children underwent distraction and 32 met skeletal maturity criterion (mean age, 18.6 years). Thirty-four percent had prior conventional Le Fort III advancement (mean age, 5.6 years), primarily by other surgeons. Primary distractions were performed on average at 9.1 years. Ten children presented with obstructive sleep apnea; eight normalized, two improved, but 30 percent subsequently developed recurrent apnea. Per anthropometry, no sagittal growth was noted after a Le Fort III. With a mean follow-up of 9.4 years, almost half (15 of 32) underwent two midfacial advancements; however, following primary distractions, only 25 percent required repeated advancements. Associated with repeated procedures were a greater degree of midfacial hypoplasia (−2.4 versus −1.6, p = 0.01), advancement at a younger age (7.1 versus 9.5 years, p = 0.04), and failure to overcorrect midfacial position (p = 0.03).

Conclusions:

These data suggest that sagittal midfacial growth ceases after advancement. Operation before 8 years old and failure to overadvance the midface were both associated with need for subsequent midfacial procedures. Although distraction effectively eliminated apnea, subsequent ventilatory degradation may occur, suggesting the need for continued surveillance.

CLINICAL QUESTION/LEVEL OF EVIDENCE:

Therapeutic, IV.

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