New method for quantification of severity of isolated scaphocephaly linked to intracranial volume

Childs Nerv Syst. 2021 Apr;37(4):1175-1183. doi: 10.1007/s00381-020-04932-9. Epub 2020 Oct 18.


Purpose: The aim is to implement Utrecht Cranial Shape Quantificator (UCSQ) for quantification of severity of scaphocephaly and compare UCSQ with the most used quantification method, cranial index (CI). Additionally, severity is linked to intracranial volume (ICV).

Methods: Sinusoid curves of 21 pre-operative children (age < 2 years) with isolated scaphocephaly were created. Variables of UCSQ (width of skull and maximum occiput and forehead) were combined to determine severity. CI was calculated. Three raters performed visual scoring for clinical severity (rating of 6 items; total score of 12 represents most severe form). Pearson's correlation test was used for correlation between UCSQ and visual score and between both CIs. ICV was calculated using OsiriX. ICV was compared to normative values and correlated to severity.

Results: Mean UCSQ was 22.00 (2.00-42.00). Mean traditional CI was 66.01 (57.36-78.58), and mean visual score was 9.1 (7-12). Correlations between both traditional CI and CI of UCSQ and overall visual scores were moderate and high (r = - 0.59; p = 0.005 vs. r = - 0.81; p < 0.000). Mean ICV was 910 mL (671-1303), and ICV varied from decreased to increased compared to normative values. Negligible correlation was found between ICV and UCSQ (r = 0.26; p > 0.05) and between ICV and CI and visual score (r = - 0.30; p > 0.05 and r = 0.17; p > 0.05, respectively).

Conclusion: Our current advice is to use traditional CI in clinical practice; it is easy to use and minimally invasive. However, UCSQ is more precise and objective and captures whole skull shape. Therefore, UCSQ is preferable for research. Additionally, more severe scaphocephaly does not result in more deviant skull volumes.

Keywords: Cranial suture; Quantification; Scaphocephaly; Severity; Synostosis.

MeSH terms

  • Child
  • Child, Preschool
  • Craniosynostoses* / diagnostic imaging
  • Humans
  • Infant
  • Skull / diagnostic imaging
  • Tomography, X-Ray Computed*