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.
Highlights
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
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
Summary
Ключевые слова: клинический случай, синдром Крузона, вывих глазного яблока, блефарорафия, остеотомия, дистракционный остеогенез. (Для цитирования: Зрячкин Н.И., Бучкова Т.Н., Чеботарёва Г.И., Кулешова Т.И. ОБОСНОВАНИЕ Генетические заболевания в последнее время становятся серьезной проблемой современного мира. Crouzon syndrome is incurable illness that demands timely functional and cosmetic correction (For citation: Zryachkin N.I., Buchkova T.N., Chebotareva G.I., Luleshova T.I. Crouzon Syndrome: Clinical case. ПЕДИАТРИЧЕСКАЯ ФАРМАКОЛОГИЯ /2019/ ТОМ 16/ No 1 стей, редкие кривые зубы, неполное смыкание зубов, Физическое развитие низкое, резко дисгармоничвысокое и узкое твердое нёбо, выступающий вперед ное: масса тела — 12 250 г, рост — 93 см, окружность подбородок, также имеет место расщелина твердого головы — 52 см, окружность груди — 52 см. Синдром Крузона — неизлечимое заболевание, ный порок сердца, вторичный дефект межпредсердной требующее функциональной и косметической коррек- перегородки (диаметр, d, 0,7 см).
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
