Eley Karen A, Witherow Helen, Hayward Richard, Evans Robert, Young Karen, Clark Allan, Dunaway David
Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom.
J Craniofac Surg. 2009 Mar;20(2):275-8. doi: 10.1097/SCS.0b013e31819921b2.
Frontofacial advancement by distraction osteogenesis using the rigid external distraction device has become an accepted treatment for the deformity associated with craniofacial dysostoses (e.g., Crouzon, Apert, and Pfeiffer syndromes). The technical and physiological principles of osteogenesis distraction are well understood. This study documents the pattern of calcification at the osteotomy sites after distraction by analysis of serial three-dimensional computed tomography (CT) scans. The CT scans of 25 patients (11 with Crouzon, 6 with Apert, and 8 with Pfeiffer syndrome) were analyzed. Eleven individual areas along the osteotomy lines were assessed for evidence of bone formation. Scores were assigned within 4 categories ranging from no bone, calcification without bridge formation, a bony bridge, to complete bony infill (>95%). The scans were reviewed on 2 separate occasions by 2 independent assessors. There was high concordance both for intraobserver and interobserver scores. Rigid external distraction frame removal was undertaken after a 6-week consolidation period. All CT scan timings were calculated from this date. Of the 25 patients studied, 16 patients had CT scans available at 3 to 6 months, 12 at 9 to 12 months, and 7 at or more than 18 months. The scans were available in standard coronal slices with three-dimensional reconstructions. Bone formation is most consistently seen in the pterygoid region with calcification consistently occurring earlier and more completely in this area. Bone formation was often delayed in the orbital region and severely delayed or absent in the frontal region and zygomatic arches. There was no significant difference in the order or quality of bony union for the 3 underlying craniofacial dysostoses. This preliminary study confirms the clinical impression that bone formation after distraction is greatest in the pterygoid regions. The clinical implications of these findings are discussed.
使用坚固外固定牵张装置进行牵张成骨的额面部前移术已成为治疗与颅面骨发育不全相关畸形(如克鲁宗综合征、阿佩尔综合征和法伊弗综合征)的一种公认疗法。骨生成牵张的技术和生理原理已得到充分理解。本研究通过对系列三维计算机断层扫描(CT)进行分析,记录牵张后截骨部位的钙化模式。分析了25例患者(11例克鲁宗综合征、6例阿佩尔综合征和8例法伊弗综合征)的CT扫描结果。沿着截骨线的11个独立区域被评估是否有骨形成迹象。根据从无骨、无桥形成的钙化、骨桥到完全骨填充(>95%)的4个类别进行评分。由2名独立评估者在2个不同时间对扫描结果进行复查。观察者内和观察者间评分的一致性都很高。在6周的巩固期后拆除坚固外固定架。所有CT扫描时间均从此日期开始计算。在研究的25例患者中,16例患者在3至6个月时有CT扫描结果,12例在9至12个月时有结果,7例在18个月及以上时有结果。扫描结果以标准冠状位切片及三维重建形式呈现。骨形成在翼状区域最为常见,该区域钙化始终更早且更完全地出现。眼眶区域的骨形成常常延迟,额部区域和颧弓的骨形成严重延迟或缺失。对于3种潜在的颅面骨发育不全,骨愈合的顺序或质量没有显著差异。这项初步研究证实了临床印象,即牵张后翼状区域的骨形成最为显著。讨论了这些发现的临床意义。