儿童Chiari畸形Ⅰ型的治疗规范及争议

Management of Chiari malformation type I in children: Standards and controversies

摘要:

儿童Chiari畸形Ⅰ型由于缺乏国内、外专家共识和指南，以及术前评估不规范和治疗手段选择不当，致使临床疗效不佳或需二次翻修手术。儿童Chiari畸形Ⅰ型患者的手术治疗方案应基于术前临床症状结合多模态影像学精准评估后实施个体化治疗，包括：单纯骨性减压，硬膜扩大成形术，硬膜扩大成形术+扁桃体切除或电凝（伴闩部探查），骨性减压+经后路寰枕融合植骨内固定术等多种术式。手术治疗儿童症状型Chiari畸形Ⅰ型的核心：一是确保枕下减压的同时维持颅颈交界区的稳定性，二是对于合并脊髓空洞的患儿术中闩部探查确保术毕脑脊液循环通畅至关重要。临床医师应根据Chiari畸形患儿的具体情况进行个体化评估并采取不同的手术策略，以期达到临床治疗微创化和疗效最佳化。

Abstract:

There are currently no guidelines or expert consensus on the management of children with Chiari malformation type Ⅰ， and a lack of standard preoperative evaluation and improper selection of treatment method often lead to poor treatment outcome or revision surgery. The surgical treatment regimen for children with Chiari malformation type Ⅰ should be selected based on preoperative clinical symptoms and multi-modality neuroimaging， and individualized treatment should be performed， including bony decompression alone， expansive duraplasty， expansive duraplasty+tonsillar resection/cautery （including obex exploration）， and bony decompression+posterior atlanto-occipital fusion and bone grafting internal fixation. The core of surgical treatment of Chiari malformation type Ⅰ in children is to maintain the stability of craniovertebral junction while ensuring decompression and perform intraoperative obex exploration for children with syringomyelia to ensure normal cerebrospinal fluid circulation after surgery. Clinicians should perform individualized assessment and select different surgical strategies based on the actual conditions of the child， so as to achieve the optimal outcome of minimally invasive treatment.