Crouzon Syndrome: Clinical case

Abstract

Relevance. Crouzon syndrome is a craniostenosis due to combination of cranial bones hypoplasia and premature ectocranial sutures fusion resulting in deformation of cerebral and facial cranium. Deformations of facial cranium are responsible for exophthalmos and corneoconjunctival xerosis and can lead to spontaneous eyeball dislocation. Clinical case description. The clinical case of bilateral eyeball dislocation in a patient (right eye at the age of 1 year 2 months, left — at 1 year 4 months) due to untimely original reconstructive surgery is presented. The blepharorrhaphy and osteotomy of cranio-orbital-zygomaxillary complex with installation of two distraction correction instruments on maxillary bone and two on frontal bone, distance osteogenesis, fronto-temporal region remodelling were performed at the age of 3 years 2 months. Conclusion. Crouzon syndrome is incurable illness that demands timely functional and cosmetic correction. Prognosis for this disease is unfavourable. These days the child is 8 years old: physical development delay and mental retardation progression as well as psychoneurological disorders are recorded.