Late-Presenting Sagittal Craniosynostosis: An Update to a Standardized Treatment Protocol

Abstract

Background: The authors previously published their protocol to treat patients who present with sagittal craniosynostosis after the age of 1 year. The purpose of this article is to present a follow-up and update of this cohort to evaluate outcomes of their treatment protocol. Methods: Patients with isolated sagittal craniosynostosis who presented after the age of 1 year between July of 2013 and April of 2021 were included. Results: A total of 108 patients met inclusion criteria. The average age at presentation was 5.2 ± 3.4 years, and 79 patients (73.1%) were male. The indications for imaging were head shape (54.6%), headache (14.8%), trauma (9.3%), seizure (4.6%), papilledema (2.8%), and other (13.9%). Of the 108 patients, 12 (11.1%) underwent surgery following their initial consultation: five for papilledema, four for elevated intracranial pressure, two for severely scaphocephalic head shapes, and one for abnormal funduscopic findings. Two of these patients underwent additional reconstructive surgery, one for the recurrence of papilledema and headache and the other for progressive scaphocephaly. The average length of time between operations was 4.9 years. Of the 96 patients who were managed conservatively, four (4.2%) underwent surgery at an average of 1.2 ± 0.5 years later (average age, 4.4 ± 1.5 years) for brain growth restriction (n = 2), aesthetic concerns (n = 1), and refractory headaches (n = 1). The average follow-up of all patients with craniofacial surgery was 2.7 ± 2.3 years (median, 2.1 years; interquartile range, 3.7 years). Conclusions: Patients with late-presenting sagittal craniosynostosis require surgical correction less often than younger patients, likely because of milder phenotype. Few patients in the conservative treatment arm ultimately required surgery (4%). CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.