20-YEAR EXPERIENCE WITH EARLY SURGERY FOR CRANIOSYNOSTOSIS .2. THE CRANIOFACIAL SYNOSTOSIS SYNDROMES AND PANSYNOSTOSIS - RESULTS AND UNSOLVED PROBLEMS
20-YEAR EXPERIENCE WITH EARLY SURGERY FOR CRANIOSYNOSTOSIS .2. THE CRANIOFACIAL SYNOSTOSIS SYNDROMES AND PANSYNOSTOSIS - RESULTS AND UNSOLVED PROBLEMS
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DOI：
10.1097/00006534-199508000-00005
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发表时间：
1995-08-01
影响因子：
3.6
通讯作者：
ZIDE, BM
中科院分区：
文献类型：
--
作者：
MCCARTHY, JG;GLASBERG, SB;ZIDE, BM
作为两部分系列的第二部分，对76例全面融合和颅面融合综合征的患者进行了回顾分析。诊断包括全融合(7例)、颅额鼻发育不良(8例)、Apert综合征(24例)、Crouzon综合征(15例)和Pfeiffer综合征(15例)。所有患者在小于18个月(平均6.1个月)时接受了初级额眶推进颅顶重建术。28例患者(36.8%)需要二次颅顶手术(平均年龄28.4个月)。此外，5例患者需要进行大型三级手术以处理持续的不可接受的颅面形态。为了解决与面中部发育不全相关的发现，64.8%(n=35)的患者接受了Le Fort III面中部推进术或为他们推荐了该手术。其余的人正在等待合适的年龄进行重建。图中显示了全智症和颅面综合征组更广泛的病理改变。与先前报道的孤立性颅面融合组相比，主要二次手术(36.8比13.5%)、围手术期并发症(11.3比5.0%)、随诊并发症(44.7比7.7%)、脑积水(42.1比3.9%)、分流(22.4比1.0%)和癫痫发作(11.8比2.9%)的发生率显著增加。反复遇到复杂的问题，包括颅内压升高，呼吸道阻塞，反复出现的头颅或颅顶发育不良。此外，早期额眶前移-颅顶重建未能促进面中部发育和该区域发育不良，这在颅面综合征组中几乎是一致的发现。术后平均随访时间为6年。根据Whitaker等人评估手术结果的分类，在最近的评估中，73.7%的患者被认为至少具有满意的头面部形状(I-II类)。提出了一种以早期手术干预为关键因素的算法方法来治疗所有的颅缝融合患者。
As the second of a two-part series, 76 patients with pansynostosis and craniofacial synostosis syndromes were retrospectively analyzed. Diagnoses included pansynostosis (7), craniofrontonasal dysplasia (8), and Apert (24), Crouzon (15), and Pfeiffer (15) syndromes.All patients underwent primary fronto-orbital advancement-calvarial vault remodeling procedures at less than 18 months of age (mean 6.1 months). Twenty-eight patients (36.8 percent) required a secondary cranial vault operation (mean age 28.4 months). Additionally, a major tertiary procedure was necessary in 5 patients to deal with persistent unacceptable craniofacial form. To address the associated finding of midface hypoplasia, 64.8 percent (n = 35) of patients underwent Le Fort III midface advancement or had that procedure recommended for them. The remainder were awaiting appropriate age for this reconstruction.The more extensive pathologic involvement of the pansynostosis and craniofacial syndrome group is illustrated. As compared with the isolated craniofacial synostosis group previously reported, the incidence of major secondary procedures (36.8 versus 13.5 percent), perioperative complications (11.3 versus 5.0 percent), follow-up complications (44.7 versus 7.7 percent), hydrocephalus (42.1 versus 3.9 percent), shunt placement (22.4 versus 1.0 percent), and seizures (11.8 versus 2.9 percent) was significantly increased. Complex problems including those of increased intracranial pressure, airway obstruction, and recurrent turricephaly or cranial vault maldevelopment are repeatedly encountered. In addition, that early frontoorbital advancement-cranial vault remodeling failed to promote midface development and hypoplasia of this region is almost a consistent finding in the craniofacial syndromic group.The average length of postoperative follow-up was 6 years. According to the classification of Whitaker et al., which assesses surgical results, 73.7 percent of patients were considered to have at least satisfactory craniofacial form (category I-II) at latest evaluation.An algorithmic approach to the treatment of all patients with craniosynostosis is presented utilizing early surgical intervention as the key element.