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非综合征性单一颅缝早闭中的 Chiari 畸形——小题大做?

Chiari malformation in nonsyndromal single craniosynostosis--much ado about nothing?

机构信息

Department of Oral and Maxillofacial Surgery, University Hospital Heidelberg, Im Neuenheimer Feld 400, 69120, Heidelberg, Germany.

出版信息

Acta Neurochir (Wien). 2012 Oct;154(10):1803-7. doi: 10.1007/s00701-012-1439-5. Epub 2012 Jul 13.

DOI:10.1007/s00701-012-1439-5
PMID:22790904
Abstract

BACKGROUND

The significance of Chiari malformation in nonsyndromal-isolated craniosynostosis is still not well documented. Hence, in the present study we investigated the incidence of Chiari malformation in a larger series of patients with nonsyndromic-isolated single-suture craniosynostosis over a 9-year period using preoperative magnetic resonance imaging (MRI).

METHODS

Of 215 children who had undergone surgery for nonsyndromic-isolated craniosynostosis, 89 cases (41.4 %) had MRI prior to surgery. All MRIs were screened for Chiari malformation.

RESULTS

Only one patient (1.1 %) with isolated lambdoid synostosis showed Chiari malformation preoperatively, which was defined as a cerebellar tonsillar descent greater than 5 mm below the foramen magnum. However, no clinical symptoms were associated with Chiari malformation in this patient.

CONCLUSIONS

As Chiari malformation is more likely to be associated with syndromic craniosynostosis, nonsyndromic bilateral coronal synostosis, or synostosis of the lambdoid suture, a general use of MRI as a screening tool for Chiari malformation should not be recommended for patients with nonsyndromic-isolated craniosynostosis who lack clinical symptoms.

摘要

背景

Chiari 畸形在非综合征型孤立性颅缝早闭中的意义尚未得到充分证实。因此,在本研究中,我们使用术前磁共振成像(MRI)检查了 9 年间更大系列的非综合征型孤立性单一颅缝早闭患者 Chiari 畸形的发生率。

方法

在 215 例接受非综合征型孤立性颅缝早闭手术的儿童中,89 例(41.4%)在术前进行了 MRI 检查。所有 MRI 均进行 Chiari 畸形筛查。

结果

仅 1 例(1.1%)孤立性人字缝早闭患者术前发现 Chiari 畸形,定义为小脑扁桃体下疝超过 5mm 至枕骨大孔以下。然而,该患者无 Chiari 畸形相关临床症状。

结论

由于 Chiari 畸形更可能与综合征型颅缝早闭、双侧冠状缝早闭或人字缝早闭相关,因此对于无临床症状的非综合征型孤立性颅缝早闭患者,不建议将 MRI 作为 Chiari 畸形的常规筛查手段。

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