A Suture-specific Photo Score for Metopic Synostosis.

Head shape assessments in children with metopic synostosis are a relevant outcome measure in addition to functional measures, such as neurocognitive outcomes, behavioral outcomes, and visual functioning outcomes. However, consensus on head shape assessments in children with metopic synostosis is lacking. The aim of this study is to develop a reproducible and reliable suture-specific photo score that can be used for cross-center comparison of phenotypical severity of metopic synostosis and evaluation of esthetic outcome of treatment later in childhood. We conducted a retrospective study among nonsyndromic metopic synostosis patients aged <18 years. Preoperative and postoperative photosets of patients with metopic synostosis from 6 expert centers were included. The photo score was discussed in the group of expert craniofacial plastic surgeons and pediatric neurosurgeons. Interrater reliability was determined with modified weighted Fleiss' kappa and intraclass correlation coefficients. Correlation between individual photo score items with overall phenotype was assessed using Spearman correlation analyses. The metopic synostosis photo score contained the following items: "wedging of the forehead", "hypotelorism", "temporal hollowing", "biparietal widening,"and an assessment of "overall phenotype". Items were scored on a 4-point ordinal scale ranging from normal to severe. We found moderate interrater reliability for all items, but substantial agreement for the summed scores. Correlation with overall phenotype was lowest for biparietal widening. To conclude, although agreement on individual photo score items was suboptimal, the agreement on the summed score was substantial, which indicates there is consensus on the overall severity of the metopic synostosis phenotype.