Long-term outcomes after surgical correction of anterior plagiocephaly secondary to isolated frontosphenoidal synostosis: a systematic review and two illustrative cases

Purpose: Anterior plagiocephaly ( AP), secondary to isolated frontosphenoidal synostosis (IFS), represents one of the rarest forms of craniosynostosis documented in medical literature. Therefore, there is a lack of comprehensive understanding regarding the long-term (> 2 years) postoperative outcomes of this minor suture synostosis.

Methods: This study presents the long-term outcomes of two patients previously treated for IFS, detailing their clinical, imaging, and postoperative characteristics. Additionally, a systematic review was conducted following PRISMA criteria to summarize existing literature on the topic. The review specifically focuses on long-term aesthetic outcomes, reoperation rates, and complications following surgical intervention for IFS.

Results: Four articles encompassing 12 patients were included in the systematic review. The review highlights clinical features, postoperative complications, reoperation rates, and long-term aesthetic outcomes. The mean age at surgery was 17.5 months (range 9.5-24 months), with an average age at diagnosis of 9.3 months (range 6-10 months). Fronto-orbital advancement was the predominant surgical approach described across all cases. The reoperation rate among patients was 16.6%, with only two cases reporting postoperative complications. In one of our cases, an intraoperative CT scan was utilized to illustrate real-time improvement in frontal bone alignment according to the surgical plan, which significantly contributed to positive long-term clinical outcomes observed during a follow-up period exceeding 24 months.

Conclusion: IFS, is a rare minor suture synostosis, significantly affects the aesthetic appearance of the forehead and necessitates consideration in the management of AP cases. Surgical intervention has shown minimal reoperation rates and excellent long-term prognosis. These findings are reinforced by recent institutional data and a comprehensive systematic review of the current literature.