Characteristics of Syndromic Craniosynostosis and Its Contemporary Treatment

顱縫過早融合症候群之顱顏特性與治療

戴楸霏(Ivy Tai-Chiew Hui) ； 柯雯青(Ellen Wen-Ching Ko) ； 黃炯興(Chiung-Shing Huang)

《中華民國齒顎矯正學雜誌》 22卷2期 (2010/06) Pp. 4-10

https://doi.org/10.30036/TJO.201006.0001

摘要

顱縫過早融合因發生的顱骨縫部位不同而產生特殊型態的頭部形狀異常。顱縫過早融合可分為原發性單純性的顱縫融合和顱縫過早融合症候群(syndromic craniosynostosis, SCS)兩大類。顱縫過早融合症候群佔了顱縫過早融合比例的15%，大部份為自體顯性遺傳的顱顏疾病。除了頭部和臉部，有些甚至產生四肢異常。常見的SCS包括克魯仲氏症(Crouzon syndrome)亞伯氏症(Apert syndrome)、菲佛氏症(Pfeiffer syndrome)以及傑克森－衛斯氏症(Jackson-Weiss syndrome)等等。顱骨內體積的減少、顱內壓力增加、眼球突出、上呼吸道狹窄、視神經被壓迫、中顏面發育不良等都是常見的手術適應症。本文獻回顧將常見的SCS與手術治療方式做整理，並比較傳統截骨手術與顏面牽引成骨治療(distraction Osteogenesis)之優劣點。

關鍵字

顱縫過早融合症候群 ； 顱內壓力增加 ； 截骨手術 ； 牽引成骨治療

並列摘要

The main characteristics of craniosynostosis were premature fusion of cranial sutures with specific skull shapes. There are divided into nonsyndromic and syndromic craniosynostosis. Syndromic craniosynostosis (SCS) was an inherited congenital craniofacial disorder and approximately 15% of craniosynostosis had associated anomalies in the face and limbs. Crouzon syndrome, Apert syndrome, Pfeiffer syndrome and Jackson-Weiss syndrome were the examples of SCS. Increased intracranial pressure (IICP), decrease intracranial volume, corneal exposure, decrease upper airway space, compression of optic nerve and midface hypoplaisa were the indications of surgical intervention. Current treatment modalities such as traditional surgical methods, including monobloc and LeFort III osteotomies as well as craniofacial distraction osteogenesis were reviewed and discussed.

並列關鍵字

Syndromic craniosynostosis ； increased intracranial pressure IICP ； osteotomy ； distraction osteogenesis