(482.21) Left - Right Asymmetry of the Jugular Foramen in Multisuture Syndromic Craniosynostosis

Poster Board Number: C136
Introduction: AAA has separate poster presentation times for odd and even posters.
Odd poster #s – 10:15 am – 11:15 am
Even poster #s – 11:15 am – 12:15 pm

Katherine Woolridge (LSUHSC-NO), Jason Mussell (LSUHSC-NO)

Introduction

Apert and Crouzon syndromes are among the most common craniosynostosis syndromes found in children. Mutations have been found in the fibroblast growth factor receptor 2 (FGFR2) gene in both syndromes. Although different mutations, the common mutated receptor raises suspicion for its involvement in the cranial morphogenesis found in these syndromic craniosynostosis patients.

The shapes of these children’s skulls have long been thought to be the result of early fusion of the synchondroses. The syndromic morphology of the skulls in these patients, in particular Apert, results from a combination of abnormal development of the skull base, neural tissue, and calvaria. Investigations of these effects has led multiple studies to find decreased volumes of the posterior fossa in craniosynostosis patients and effects on the basicranial foramina of the skull, in particular the jugular foramen.

Craniosynostotic children develop elevated intracranial pressure (ICP), as well as, other complications such as hydrocephalus and tonsillar herniation related to this increased pressure and skull dysmorphology. Narrowing of the jugular foramen is a potential cause of venous hypertension and may contribute to the ICP.

This study analyzes the size of the jugular foramina in an effort to relate the severity of the foraminal stenosis to timing of synchondroses fusion, seen earlier in Crouzon patients. Additionally, as right jugular foramina have been found to be larger than left in the general population, our study analyzes the right and left jugular foramen sizes in relationship to each other in each Apert and Crouzon patient.

Methods

Twelve preoperative CT scans of individuals diagnosed with either Apert or Crouzon syndrome were individually segmented and volume of the right and left jugular foramina estimated in Avizo. Volumes were analyzed using Wilcoxon Rank Sum and Kruskal-Wallis one-way ANOVA tests in SPSS. Scatter plots of the ratio of right to left foraminal volumes were created using Excel. This study was conducted under LSUHSC IRB #920.

Results and Discussion

Initial results found no significant differences between right and left jugular foramina, however, absolute measurement of volumes most often recorded larger volumes on the left, contrary to that seen in the normal population. Scatter plots revealed two distinct clusters based on ratio of left-right asymmetry which may reflect a difference in diagnosis.