用牵引成骨矫正Crouzon综合征性严重突眼畸形

 目的介绍经颅外或经颅内外手术途径用牵引成骨技术矫正Crouzon综合征性严重突眼畸形病例的术式.方法病例1:采用改良Le FortⅢ型骨切开术,安装外固定头架,通过结扎于双侧颧骨体和梨状孔旁支抗钉的两对钢丝,分别经皮和经口穿出,连接到头架的加力螺杆,术后第1天开始按0.5 mm/次、2/d的速率向前牵引14 d、向前下牵引17 d.病例2:经冠状切口掀起额骨骨瓣,经颅内外和口内行眶上、外、下缘骨切开术,在保留眶内骨膜附着下使双眶整体游离.将额骨瓣前徙以扩大颅腔,微型接骨板固定.安装头架后,将结扎于眶上缘和颧骨体的两组钢丝经皮穿出,连接于加力螺杆上.术后第5天开始加力,速率同上,共17 d.结果病例1的眶外、下缘和面中部前徙14 mm,下旋7 mm,纠正了突眼、面中部凹陷和开反(牙合)畸形.病例2的前额由向后斜变圆,双眶前徙14 mm,眶缘移至眼球赤道之前,闭眼无障碍.除术后脑脊液漏持续了6 d外,无任何并发症.结论根据患者特征,用牵引成骨技术矫正Crouzon综合征性颅颌面畸形安全可行,效果显著.

Treatment of severe exophthalmos in patients with crouzon syndrome by traction osteogenesis technique by extra- and/or intra- cranial approach

Objective To introduce two traction osteogenesis techniques in the treatment of severe exophthalmos in patients with Crouzon syndrome by extra-and/or intra-cranial approaches.Methods For the first patient,a young man,a modified Le Fort III osteotomy was performed and a rigid external tractor was fabricated.Traction was begun immediately after operation at 1 mm twice daily.The directions of traction were forward in the former 14 days and downward in the later 17 days.For the second patient,a boy,the osteotomy was performed by both intra-and extra-cranial approach to free upper-lateral-lower orbital rim en bloc bilaterally with periosteum intact.The frontal bone segment was fixed in a way to expand the skull cavity. The same device was fixed cranially and connected to 2 pairs vectors of tractor with wire to keep balance of both orbital framework.The traction started in the 5th day postoperatively at 1 mm twice daily.Results Both patients were cured uneventfully except that CSF leakage occurred in the boy for 6 days. The young man's midface advanced for 14 mm and turned downward for 7 mm.His midface contour became protruded from retrusion. His occlusion was fully corrected. The snoring during sleep disappeared. The boy's oblique frontal bone was expanded into round in shape. His orbital framework advanced for 14 mm. He could easily close eyes during sleep. There was no relapse found during more than 6 month's follow-up.Conclusion Both traction osteogenesis techniques are clinically practicable and effective.Long-term follow-up is needed to observe its effect on the development of children.