Morphometric analysis and outcomes following posterior cranial vault distraction in syndromic and multisuture craniosynostosis.

BACKGROUND: Turribrachycephaly is a common feature in many syndromic and multisuture craniosynostoses and is traditionally treated with total cranial vault reshaping and fronto-orbital advancement. A staged approach with posterior cranial vault distraction as a primary procedure followed by anterior cranial vault reshaping has the advantage of reducing the vertical dimension of the skull in a controlled and gradual manner while expanding the cranial volume. The purpose of this study was to evaluate outcomes following posterior cranial vault expansion using distraction osteogenesis at a single tertiary pediatric center.

METHODS: This retrospective review included all cases of posterior cranial vault distraction at a single institution from 2008 to 2022 performed by one surgeon. Morphometric outcomes such as Turricephaly Index (TI) and posterior cranial volume (PCV) were assessed from pre- and postoperative computerized tomography scans for patients who underwent posterior cranial vault distraction as a primary first-stage operation. Clinical outcomes and complications were collated.

RESULTS: A total of 41 patients (25 females, 16 males; mean age 11 months) with syndromic craniosynostosis (n = 32) and nonsyndromic craniosynostosis (n = 9) were included. The main indication for posterior cranial vault distraction in this cohort was turricephaly (63%). The mean distraction distance was 25.9 mm and the mean decrease in TI was 18%. The estimated increase in PCV from distraction in this cohort was 19.7%. In this cohort of patients, 13 patients (32%) experienced complications but there were no mortalities.

CONCLUSION: Posterior cranial vault distraction osteogenesis is an effective surgical procedure to increase intracranial volume and correct turricephaly in children with syndromic and nonsyndromic craniosynostosis. This procedure is considered a safe and effective first-stage cranial operation in children with syndromic craniosynostosis with increased intracranial pressure and or turricephaly.