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Delayed Presentation of Sagittal Synostosis: A Management Algorithm and Long-Term Follow-Up
Justin W. Beiriger, BSE1; Madeleine K. Bruce, BA1; Allison Mak, BA1; Casey Zhang, BA; Janina Kueper, MD1; John Smetona, MD1; Miles Pfaff, MD1, MHS; Zhazira Irgebay, BA1; Joseph E. Losee, MD1; Jesse A. Goldstein, MD1
1UPMC, Children's Hospital of Pittsburgh, Department of Plastic Surgery

Background: We previously published our protocol to treat patients who present with sagittal craniosynostosis after the age of one year. The purpose of this study is to present a follow-up and update of this cohort to evaluate outcomes of our treatment protocol.
Methods: Patients with isolated sagittal craniosynostosis who presented after the age of one year between July 2013 and April 2021 were included.
Results: 108 patients met inclusion criteria. The average age at presentation was 5.2 3.4 years and 79 (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: 5 for papilledema, 4 for elevated ICP, 2 for severely scaphocephalic head shapes, and 1 for abnormal fundoscopic 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 surgeries was 4.9 years. Of the 96 patients who were conservatively managed, 4 (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, IQR 3.7 years).
Conclusion: Patients with late-presenting sagittal craniosynostosis require surgical correction less often than younger patients, likely due to milder phenotype. Few patients in the conservative treatment arm ultimately required surgery (4%).


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