Open Access Open Access
Restricted Access Access granted
Restricted Access Subscription or Fee Access


This article is devoted to the abnormalities of the visual function associated with the rare and severe congenital pathology - craniosynostosis in the hildren. We observed three clinical cases of partial craniosynostosis: scaphocephaly, plagiocephaly, and trigonocephaly. In addition to the standard ophthalmologic examination, we carried out the electrophysiological study to measure visual evoked potentials r (VEP). In the case of trigonocephaly, we documented significant changes in the amplitude-time characteristics of VEP and revealed partial atrophy of the optic nerves. Strabismus was detected in the cases of plagiocephaly and trigonocephaly. We obtained evidence of the association between different types of craniosynostosis and the anatomical features, such as location of the cranial sutures, and the manifestations of the pathological process underlying the changes in VEP especially its amplitude and temporal characteristics. They included the prolonged implicit time of the PI00 component most pronounced in the cells with the angulsar size of 8 minutes and the decrease of the PI00 peak amplitude. The damage to the optic nerves, especially the secondary one, due to the papilledema can be prevented by the early diagnostics of craniosynostosis. When craniosynostosis is suspected in the children, they need to undergo the ophthalmological examination. Early diagnostics with the use of not only the standard methods of ophthalmological studies but also of electrophysiological investigations allows to significantly improve the prognosis in different types of craniosynostosis in the children.

Full Text

Restricted Access

About the authors

N. V Kurenkova

Children medical center, General Management Department of the Administration of the President of the Russian Federation

Moscow, 109012, Russian Federation

I. V Zolnikova

The Helmholtz Moscow Research Institute of Eye Diseases, Russian Ministry of Health

Moscow, 105062, Russian Federation

G. A Klitvina

Children medical center, General Management Department of the Administration of the President of the Russian Federation

Moscow, 109012, Russian Federation

O. N Demenkova

Children medical center, General Management Department of the Administration of the President of the Russian Federation

Moscow, 109012, Russian Federation


  1. Лопатин А.В., Ясонов С.А. Общие вопросы ранней диагностики краниосиностозов. Методические рекомендации для врачей. М: ЗАО «ПроМедиа»; 2005.
  2. Lajeunie E., Le Merrer M., Bonaiti-Pellie C. et al. Genetic study of syndromic coronal craniosynostosis. Am. J. Med. Genet. 1995; 13; 55 (4): 500-4.
  3. Steinberger D., Reinhartz T., Unsold R. et al. FGFR2 mutation in clinically non-classifiable autosomal dominant craniosynostosis with pronounced phenotypic variation. Am. J. Med. Genet. 1996; 2, 66 (1): 81-6.
  4. Суфианов А. А., Гаибов С. С.-Х., Суфианов Р. А. Несиндромальные краниосиностозы: современное состояние проблемы. Российский вестник перинатологии и педиатрии. 2013; 58 (6): 33-7.
  5. Буркова Р.С., Гусева О.И. Несиндромальная скафоцефалия: особенности диагностики. Пренатальная диагностика. 2016; 15 (1): 44-7.
  6. Крючкова Т. А., Агаркова Г. Л. Клинический случай синдрома Пфайффера у ребенка раннего возраста. Научные ведомости Белгородского государственного университета. Серия Медицина. Фармация. 2015; 31 (16): 277-81.
  7. Колтунов Д.Е. Синдром Пфайффера: клинические проявления и этиология Вопросы диагностики в педиатрии. 2010; 2 (3): 42-6.
  8. Patton M.A., Goodship J., Hayward R., Lansdown R. Intellectual development in Aper’s syndrome: a long term follow up of 29 patients. J. Med. Genet. 1988; 25: 164-7.
  9. Cohen M.M. Jr. Craniosynostosis: Diagnosis, Evaluation and Management. New York: Raven Press; 1986: 157-89.
  10. Winter RM, Baraitser M. The London Dysmorphology Database. Oxford: Oxford University Press; 1996.
  11. Renier D., Sainte-Rose C. et al. Intracranial pressure in craniostenosis. J. Neurosurg. 1982; 57: 370-7.
  12. Marchac D., Renier D. “Lefront flottant”: Traitement precoce des facio-craniostenoses. Ann. Chir. Plast. 1979; 24: 121.
  13. Tessier P. The definitive plastic surgical treatment of the severe facial deformities of craniofacial dysostosis Crouzon’s and Apert’s diseases. Plast. Reconstr. Surg. 1971; 48 (5): 419-42.
  14. Denis D., Genitori L., Bolufer A. et al. Refractive error and ocular motility in plagiocephaly. Childs Nerv. Syst. 1994; 10 (4): 210-6.
  15. Diamond G.R., Katowitz A., Whitaker L.A. et al. Ocular and adnexal complications of unilateral orbital advancement for plagiocephaly. Arch. Ophthalmol. 1987; 105 (3): 381-5.
  16. Hromadkova L., Rehurek J., Uherkova E. Vertical strabismus in plagiocephaly. Cesk. Oftalmol. 1992; 48 (3): 181-5.
  17. Pensler J.M., Balich S.M., Greenwald M.J. Ocular abnormalities associated with unilateral coronal synostosis. Ann. Plast. Surg. 1994; 33 (2): 162-5.
  18. Robb R.M., Boger W.P. Vertical strabismus associated with plagiocephaly. Pediatr. Ophthalmol. Strabismus. 1983; 20 (2): 58-62.
  19. Gosain A.K., Steele M.A., McCarthy J.G., Thorne C.H. A pro-spective study of the relationship between strabismus and head posture in patients with frontal plagiocephaly. Plast. Reconstr. Surg. 1996; 97 (5): 881-91.
  20. Hinojosa J. Endoscopic-assisted treatment of trigonocephaly. Childs Nerv. Syst. 2012; 28: 1381-7.
  21. McCulloch D.L., Marmor M.F., Brigell M.G. et al. ISCEV Standard for full-field clinical electroretinography (2015 update). Docum. Ophthalmol. 2015; 130 (1): 1-12.
  22. Khan S.H., Nischal K.K., Dean F. et al. Visual outcomes and amblyogenic risk factors in craniosynostotic syndromes: a review of 141 cases. Br. J. Ophthalmol. 2003; 87 (8): 999-1003.
  23. Newman S.A. Ophthalmic features of craniosynostosis. Neurosurg. Clin. N. Am. 1991; 2: 587-610.
  24. Nguyen T., Shock A. L., Missoi T., Muzaffar A. Incidence of аmblyopia and its risk factors in children with isolated metopic craniosynostosis. Cleft Palate-Craniofac. 2016; 53: 14-7.
  25. Mursch K., Brockmann K., Lang J. et al. Visually evoked potentials in 52 children requiring operative repair of craniosynostosis. Pediatr. Neurosurg. 1998; 29: 320-3.
  26. Thompson D., Liasis A., Hardy S. et al. Prevalence of abnormal pattern reversal visual evoked potentials in craniosynostosis. Plast. Reconstr. Surg. 2006; 118 (1): 184-92.
  27. Liasis A., Walters B., Thompson D. et al. Visual field loss in children with craniosynostosis. Childs Nerv. Syst. 2011; 27: 1289-96.
  28. Liasis A., Nischal K., Walters B. et al. Monitoring visual function in children with syndromic craniosynostosis. A comparison of 3 methods. Arch. Ophthalmol. 2006; 124: 1119-26.
  29. Gupta S., Ghose S., Rohatgi M., Das A. The optic nerve in children with craniosynostosis. Docum. Ophthalmol. 1993; 83 (4): 271-8.
  30. Li S., Hertzler R., Lawhon W. et al. Visual evoked potential (VEP) testing and craniofacial synostosis (CS): results in 67 patients. DOI:



Abstract - 88

PDF (Russian) - 0


Article Metrics

Metrics Loading ...


  • There are currently no refbacks.

Copyright (c) 2017 Eco-Vector

This website uses cookies

You consent to our cookies if you continue to use our website.

About Cookies