Incidence of delayed intracranial hypertension in children with isolated sagittal synostosis following open calvarial vault reconstruction

Purpose Delayed intracranial hypertension (DIH) occurs most frequently in children with syndromic or multi-suture synostosis after surgical correction. The rarity of DIH in children with isolated non-syndromic sagittal synostosis (ISS) warrants follow-up evaluation by large craniofacial centers until skeletal maturity. This study reports the incidence of DIH in children following open repair for ISS by our center's craniofacial team. Methods A single-center retrospective study of patients who underwent open calvarial vault remodeling (CVR) for ISS at our institution between November 2000 and November 2012 was performed. Syndromic and multi-suture synostosis patients were excluded. Demographic and follow-up data were extracted from the medical record for analysis until July 2017. Results One hundred five patients with ISS were identified who had undergone CVR in the aforementioned timeframe. Average age at initial surgery was 11.7 +/- 15.32 months. Mean follow-up in our craniofacial clinic was 4.94 +/- 3.53 years, with 69 patients (65.7%) having follow-up in craniofacial clinic >= 3 years and 74 (70.5%) having follow-up >= 3 years in any clinic at our institution. Four patients (3.8%) had intracranial pressure (ICP) monitors placed for symptoms concerning for DIH, one of which (0.95%) had confirmed DIH and underwent a second surgical procedure at 7.4 years of age. The patient presented late initially, having his first operation at 1.56 years of age. Conclusion One patient out of 105 (0.95%) developed DIH, confirmed by ICP monitoring, and required reoperation. The occurrence of DIH, albeit rare, remains an important topic to include in parental discussions and mandates long-term follow-up in this population.