Monobloc分段截骨双向牵引治疗婴儿Crouzon综合征

目的 总结Monobloc截骨、外置牵引和上颌骨延长在婴儿Crouzon综合征伴颅缝早闭后缩病例的治疗经验。方法 对1例9个月的女婴经颅内外联合径路进行手术。设计Monobloc 截骨线,将整个面骨分成额部、眶部、上颌3段截骨,固定后前移重塑颅面骨,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,同时内置延长器向下延长上颌骨,牵引结束后固定3个月。结果 患儿顺利完成Monobloc截骨牵引,牵引器调节螺丝杆前移达18 mm,三维影像测量颅面骨实际前移12 mm。牵引结束时、拆除牵引器后1个月及6个月均显示患者面部轮廓有6 mm的回缩,头部畸形和面中部严重凹陷得到良好纠正,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改善,但上颌向下延长长度有限。结论 Monobloc截骨外置牵引和上颌骨延长可以应用于婴儿Crouzon综合征,并且为呼吸困难型的Crouzon综合征患儿提供了一种早期的安全有效的治疗方法。

Segmental monobloc osteotomy and bi-directional distraction for the treatment of Crouzon syndrome in an infant

Objective To discuss the segmental monobloc osteotomy and bi-directional distraction for the treatment of Crouzon syndrome in an infant. Methods A 9-month-old female infant underwent monobloc osteotomy through combined intra- and extra-cranial way. The facial skull was divided into frontal, orbital and maxillary segments. The external distractor was used to move the frontal segment, orbital segment and midface forward. The internal distractor was used to move the maxilla downward. The consolidation lasted for 3 months after distraction. Results The osteotomy and distraction were successfully completed. The distraction distance reached 18 mm, showing by distractor. The real distraction distance of facial bone was 12 mm, documented by 3-D image. The skull deformity and severe depression of midface improved a lot. The exophthalmos and underbite were corrected. The obstructive sleep apnea also improved markedly. However, the downward movement of maxilla was limited. Conclusions Monobloc osteotomy with external distractor, as well as maxillary distraction, could be used for Crouzon syndrome in infant. It is safe and effective method which can be performed in an early age for Crouzon syndrome with obstructive sleep apnea.