Monobloc截骨牵引成骨和颅骨重塑治疗Crouzon综合征

Monobloc distraction osteogenesis and cranial vault remodeling in a pediatric patient with severe Crouzon＇ s syndrome

目的总结Monobloc截骨外置牵引和颅骨重塑治疗1例Crouzon综合征伴颅缝早闭额部后缩病例的经验体会。方法经颅内外联合径路，额部颅骨截骨前移重塑加植骨，Monobloc截骨并外置牵引器，将额、双侧眼眶和面中部颧骨上颌骨整体逐步前移，牵引结束后固定4个月。结果截骨牵引手术顺利，额眶、面中部牵引前移达20mm，患儿突眼、反咬合得到完全纠正，术前重度阻塞性睡眠呼吸暂停综合征转为轻度。结论Monobloc截骨外置牵引和颅骨重塑为治疗Crouzon综合征提供了一种安全有效的方法。

Objective To report the treatment of a case of severe Crouzon＇s syndrome using monobloc distraction osteogenesis and cranial vault remodeling. Methods Through intra-and extra-cranial approach, monobloc osteotomy was performed and external distractor was placed. Distraction began on the 7th postoperative day at a rate of I mm a day, two times a day. The distractor removed after consolidation for 4 months. Results The distraction distance attained 20mm. The exophthalmos and cross bite were corrected completely. The severe obstructive apnea improved markedly. Conclusions Monbloc distraction osteogenesis and cranial vault remodeling are effective and safe procedure for Crouzon＇s syndrome.