Session: Free papers Session VII - Exotropia et misc.

Prevalence of ocular torticollis in patients with non-syndromal unicoronal craniosynostosis and its ophthalmic features

Sjoukje LOUDON¹, Parinaz ROSTAMZAD^1,2, Yasmine ESSER^1,2, Mieke PLEUMEEKERS², Emily TAN¹

¹Erasmus MC Rotterdam, dept. Ophthalmology, Rotterdam, Netherlands
²Erasmus MC Rotterdam, dept. Plastic and Reconstructive Surgery, Rotterdam, Netherlands

Introduction

Unicoronal craniosynostosis (UCS) patients often show a torticollis. Several reasons both ocular and non-ocular have been postulated. This study aims to establish the prevalence of an ocular torticollis (OT) in non-syndromic UCS patients and possible associated ocular features. In addition, MRI was employed to investigate changes in the extraocular muscles.

Methods

Medical records of all non-syndromic UCS patients treated at the Erasmus MC University Medical Center Rotterdam between 1994-2022 were retrospectively reviewed. Data was collected from electronic medical records. Patients with other craniofacial disorders or incomplete orthoptic data were excluded. Patients were categorized as having an OT based on their orthoptic diagnosis. MRI brain data were acquired with a 1.5T Unit.

Results

Overall, data of 146 patients was included (mean age at initial examination was 3.5±4.4yrs) of whom 57 exhibited a torticollis, with an ocular cause identified in 54 cases. Torticollis was first identified at age 2.9±2.7yrs. The prevalence of OT was 37% (n=146; 95% CI [0.292-0.454]). The primary cause of the torticollis was incomitant strabismus (n=47; 87%) followed by concomitant strabismus (n=6; 11%) and congenital nystagmus (n=1; 2%). Pseudo-superior oblique palsy was the most common subtype (n=34; 59.6%). Significant associations were observed between OT and ocular motility abnormalities (p<0.001), alphabetical patterns (p<0.001), strabismus (p<0.001) and amblyopia (p=0.002). A subset of 24 patients had MRI scans pre-craniofacial surgery, revealing the presence of all extraocular and oblique muscles. A smaller and asymmetric superior oblique muscle was seen on the ipsilateral side of the closed suture.

Conclusion

A third of the patients with UCS had torticollis. Torticollis in patients with UCS is predominantly ocular related and revealed at different ages. MRI analysis revealed a reduced volume of the superior oblique muscle on the side of the closed suture.