Affiliations 

  • 1 Department of Oral and Maxillofacial Clinical Sciences, Faculty of Dentistry, Universiti Malaya
  • 2 Department of Oral and Maxillofacial Clinical Sciences, Faculty of Dentistry, Universiti Malaya; firdaushariri@um.edu.my
  • 3 Mathematics Division, Centre for Foundation Studies in Science, Universiti Malaya
J Vis Exp, 2026 Nov 03.
PMID: 42980251 DOI: 10.3791/73292

Abstract

Syndromic craniosynostosis (SC), such as Apert and Crouzon syndromes, causes midface hypoplasia that severely limits nasal cavity development. A computed tomography (CT)-based volumetric protocol for nasal cavity volumetry and choanal airway classification in pediatric SC patients, using landmark-based image segmentation software, is described and tested for intra-rater reproducibility. The protocol was applied to 10 SC patients (5 Apert, 5 Crouzon; 2-48 months; paired pre-/postoperative CT) and 10 reference children (60-168 months), not age-matched because CT is rarely indicated in healthy children. Intra-rater reliability was excellent (ICC = 0.987 and 0.909; 95% CI 0.934-0.997 and 0.689-0.976). Preoperative nasal cavity volume was significantly lower in SC patients (median 2,063.77 mm3; Q1-Q3 1,275.53-3,457.18) than in the reference group (median 35,502.13 mm3; Q1-Q3 28,811.21-39,167.15) (Mann-Whitney U = 0.000, Z = -3.780, p < 0.001). Nasal cavity volume increased between the paired CT scans in nine of ten patients (Wilcoxon Z = -2.701, p = 0.007; difference in medians +1,584.25 mm3; +76.8%), remaining at only 10.3% of the reference group median postoperatively. A weighted regression sensitivity analysis (log[Volume] vs log[Age], w = 1/Age; n = 10), accounting for the age disparity between cohorts, yielded an exploratory, model-based, non-normative estimate of 7,580 mm3 at the SC median age (8.5 months), extrapolated below the reference group age range; the SC median represented 27.2% of this estimate. Preoperative volume varied descriptively by choanal category (normal, stenosis, atresia) without tracking narrowing severity, possibly reflecting global hypoplasia; small subgroups precluded inferential testing. Nasal cavity volume is an anatomical measure and does not by itself indicate functional airway obstruction. The protocol offers a standardized workflow for larger, age-matched multicenter studies.

* Title and MeSH Headings from MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.