True lambdoid craniosynostosis: long-term results of surgical and conservative therapy.
True lambdoid craniosynostosis: long-term results of surgical and conservative therapy.
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真正的人字形颅缝早闭：手术和保守治疗的长期结果。
DOI：
10.1097/01.prs.0000278043.28952.e8
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发表时间：
2007
影响因子：
3.6
通讯作者：
Bartlett,ScottP
中科院分区：
文献类型：
--
作者：
SmarttJr,JamesM;Reid,RussellR;Singh,DavinderJ;Bartlett,ScottP
背景：真正的板样融合是一种罕见的畸形。很少有临床报告检验保守或手术治疗在这些患者护理中的有效性。方法：所有在1990年至2005年期间在费城儿童医院由资深作者(SPB)治疗的被诊断为真性样肌腱融合的患者都纳入了这项研究。无论是保守治疗还是手术治疗，都进行了颅面生长的定性和定量评估。定性评估是基于术前和术后的照片、计算机断层扫描和对患者病历的回顾。使用颅骨测量分析和适当的统计学检验对耳朵位置进行定量评估。结果：研究包括9名患者-6名接受手术干预，3名迄今未接受经颅手术。作者对患者数据的分析显示，可预见的颅面畸形表现为枕骨扁平、同侧枕乳突起和明显的半面部缺陷。总的来说，这些畸形在手术治疗后得到了改善。对手术前后耳位置的定量分析显示，垂直或前后移位在统计学上没有显著差异。在1例未经治疗的患者中，真性板样融合导致颅顶和面部骨骼持续严重的不对称。结论：真性板样融合如果不治疗，会导致明显的颅面不对称。虽然临床表现多种多样，但诊断特征包括枕骨变平、同侧枕乳突隆起和对侧面部畸形。后穹隆转换颅骨成形术是一种有效的治疗真正的板状突融合，如果在生命的第一年进行。
Background:True lambdoid synostosis is a rare malformation. Few clinical reports have examined the efficacy of conservative or surgical management in the care of these patients.Methods:All patients with a diagnosis of true lambdoid synostosis treated by the senior author (SPB) at The Children’s Hospital of Philadelphia between 1990 and 2005 were included in the study. Both qualitative and quantitative assessments of craniofacial growth were performed following either conservative or surgical management. Qualitative assessments were made based on preoperative and postoperative photographs, computed tomographic scans, and a review of patient charts. A quantitative assessment of ear position was performed using craniometric analysis and the appropriate statistical tests.Results:The study included nine patients—six who underwent surgical intervention and three who did not undergo transcranial surgery to date. The authors’ analysis of patient data revealed a predictable craniofacial dysmorphism manifest as occipital flattening, an ipsilateral occipitomastoid bulge, and a pronounced hemifacial deficiency. Generally, these malformations improved following operative management. A quantitative analysis of preoperative and postoperative ear position displayed no statistically significant vertical or anteroposterior displacement. In one untreated patient, true lambdoid synostosis resulted in persistent severe asymmetry of the cranial vault and facial skeleton.Conclusions:True lambdoid synostosis, if left untreated, results in pronounced craniofacial asymmetry. Although a diversity of clinical presentations exists, diagnostic features include occipital flattening, an ipsilateral occipitomastoid bulge, and a contralateral hemifacial deficiency. Posterior vault switch cranioplasty is an effective treatment for true lambdoid synostosis when performed in the first year of life.