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- Title
Craniosynostosis in primary metabolic bone disorders: a single-institution experience.
- Authors
Punchak, Maria A.; Sarris, Christina; Xu, Emily; Flanders, Tracy M.; Lang, Shih-Shan; Swanson, Jordan W.; Taylor, Jesse A.; Heuer, Gregory G.
- Abstract
Purpose: The incidence of metabolic bone diseases in pediatric neurosurgical patients is rare. We examined our institutional experience of metabolic bone diseases along with a review of the literature in an effort to understand management for this rare entity. Methods: Retrospective review of the electronic medical record database was performed to identify patients with primary metabolic bone disorders who underwent craniosynostosis surgery between 2011 and 2022 at a quaternary referral pediatric hospital. Literature review was conducted for primary metabolic bone disorders associated with craniosynostosis. Results: Ten patients were identified, 6 of whom were male. The most common bone disorders were hypophosphatemic rickets (n = 2) and pseudohypoparathyroidism (n = 2). The median age at diagnosis of metabolic bone disorder was 2.02 years (IQR: 0.11–4.26), 2.52 years (IQR: 1.24–3.14) at craniosynostosis diagnosis, and 2.65 years (IQR: 0.91–3.58) at the time of surgery. Sagittal suture was most commonly fused (n = 4), followed by multi-suture craniosynostosis (n = 3). Other imaging findings included Chiari (n = 1), hydrocephalus (n = 1), and concurrent Chiari and hydrocephalus (n = 1). All patients underwent surgery for craniosynostosis, with the most common operation being bifronto-orbital advancement (n = 4). A total of 5 patients underwent reoperation, 3 of which were planned second-stage surgeries and 2 of whom had craniosynostosis recurrence. Conclusions: We advocate screening for suture abnormalities in children with primary metabolic bone disorders. While cranial vault remodeling is not associated with a high rate of postoperative complications in this patient cohort, craniosynostosis recurrences may occur, and parental counseling is recommended.
- Subjects
CRANIOSYNOSTOSES; METABOLIC bone disorders; MEDICAL record databases; LITERATURE reviews; ELECTRONIC health records; CHILDREN'S hospitals
- Publication
Child's Nervous System, 2023, Vol 39, Issue 12, p3583
- ISSN
0256-7040
- Publication type
Article
- DOI
10.1007/s00381-023-06059-z