Visual Prognosis in Craniosynostosis Patients: A 20-year Retrospective Cohort Study at a Tertiary Referral Center in Thailand

Authors

  • Thammanoon Surachatkumtonekul Department of Ophthalmology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand
  • Subongkoch Subhadhirasakul Department of Ophthalmology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand
  • Wasawat Sermsripong Department of Ophthalmology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand

DOI:

https://doi.org/10.33192/smj.v76i10.269614

Keywords:

Craniosynostosis, visual outcome, ophthalmologic finding, strabismic surgery

Abstract

Objective: This study aims to evaluate the visual prognosis in craniosynostosis patients in order to identify ophthalmologic manifestations in craniosynostosis patients and strabismus surgery outcomes in these patients.

Materials and Methods: The medical record charts of craniosynostosis patients were reviewed retrospectively from January 2000 to June 2023. All the relevant patient data, such as age, sex, visual acuity, ophthalmologic examinations, and strabismus surgery outcomes were recorded.

Results: In total, 123 patients were included in the study, among whom 65 (52.84%) were male. Overall, there were 70 cases (56.91%) of non-syndromic craniosynostosis, and 53 cases of syndromic craniosynostosis, comprising Crouzon syndrome (15 cases), Apert syndrome (8 cases), Pfeiffer syndrome (2 cases), and other syndromes (28 cases). The mean age at first examination was 3.11 years old. Out of 107 cases with visual impairment at the first visit, 92 (85.98%) showed no visual impairment at the last visit. Among the 87 patients with strabismus, 28 (32.18%) had strabismus at the primary position, with exotropia being the most common type (18 cases). Eight cases underwent strabismus surgery, and 4 cases achieved success (deviation < 10 prism diopters). Other ophthalmologic manifestations were lagophthalmos, exposure keratopathy, ptosis, proptosis, and nasolacrimal duct obstruction.

Conclusion: Among the craniosynostosis cases, 85.98% had no visual impairment, with the common ophthalmologic manifestations being refractive errors, strabismus, lagophthalmos, and exposure keratopathy. The syndromic craniosynostosis cases showed worse visual outcomes and a higher incidence of ocular diseases than non-syndromic cases. The success rate for strabismus surgery in craniosynostosis patients was 50%.

References

Steinberger D, Reinhartz T, Unsold R, Muller U. FGFR2 mutation in clinically non-classifiable autosomal dominant craniosynostosis with pronounced phenotypic variation. Am J Med Genet. 1996;66(1):81-6.

Garza RM, Khosla RK. Nonsyndromic craniosynostosis. Semin Plast Surg. 2012;26(2): 53-63.

Shlobin NA, Baticulon RE, Ortega CA, Du L, Bonfield CM, Wray A, et al. Global Epidemiology of Craniosynostosis: A Systematic Review and Meta-Analysis. World Neurosurg. 2022;164:413-23.e3.

Hinds AM, Thompson DA, Rufai SR, Weston K, Schwiebert K, Panteli V, et al. Visual outcomes in children with syndromic craniosynostosis: a review of 165 cases. Eye (Lond). 2022;36(5):1005-11.

Khan SH, Nischal KK, Dean F, Hayward RD, Walker J. Visual outcomes and amblyogenic risk factors in craniosynostotic syndrome: a review of 141 cases. Br J Ophthalmol. 2003; 87(8):99-1003.

Rafique Ali AA, Ismail F, May CM, Abdullah AAM, Khaliddin N, Hariri F, et al. Ophthalmic features of craniosynostosis: A Malaysian experience. Eur J Ophthalmol. 2022;32(3):1417-23.

Tay T, Martin F, Rowe N, Johnson K, Poole M, Tan K, et al. Prevalence and causes of visual impairment in craniosynostotic syndromes. Clin Exp Ophthalmol. 2006; 34(5): 434-40.

World Health Organization. ICD-11: International classification of diseases (11th revision). 2022.

Diamond GR, Katowitz JA, Whitaker LA, Quinn GE, Schaffer DB. Variations in extraocular muscle number and structure in craniofacial dysostosis. Am J Ophthalmol. 1980;90(3):416-8.

Kampanartsanyakorn S, Surachatkumtonekul T, Dulayajinda D, Jumroendararasmee M, Tongsae S. The outcomes of horizontal strabismus surgery and influencing factors of the surgical success. J Med Assoc Thai. 2005;88 Suppl 9:S94-9.

Liu Q, Li Y, Wang S, Zheng W, Ye H, Li W, et al. Surgical treatment and muscle protein analysis of V-pattern exotropia in craniosynostosis. Sci Rep. 2022;12(1):11524.

Rostamzad P, Arslan ZF, Mathijssen IMJ, Koudstaal MJ, Pleumeekers MM, Versnel SL, et al. Prevalence of Ocular Anomalies in Craniosynostosis: A Systematic Review and Meta-Analysis. J Clin Med. 2022;11(4):1060.

Ntoula E, Nowinski D, Holmström G, Larsson E. Strabismus and refraction in non-syndromic craniosynostosis – A longitudinal study up to 5 years of age. Acta Ophthalmol. 2024;102(5):564-72.

Published

01-10-2024

How to Cite

Surachatkumtonekul, T. ., Subhadhirasakul, S. ., & Sermsripong, W. . (2024). Visual Prognosis in Craniosynostosis Patients: A 20-year Retrospective Cohort Study at a Tertiary Referral Center in Thailand. Siriraj Medical Journal, 76(10), 679–686. https://doi.org/10.33192/smj.v76i10.269614

Issue

Section

Original Article

Categories