Cervicomedullary decompression for foramen magnum stenosis in achondroplasia
Cervicomedullary decompression for foramen magnum stenosis in achondroplasia
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DOI：
10.3171/ped.2006.104.3.166
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发表时间：
2006-03-01
影响因子：
4.1
通讯作者：
Carson, BS
中科院分区：
文献类型：
--
作者：
Bagley, CA;Pindrik, JA;Carson, BS
物体。软骨发育不全是侏儒症最常见的遗传性形式，其特征是身材矮小、大头畸形和无数的骨骼异常。在儿科人群中，颈髓交界处的狭窄和压迫是常见的。本研究的目的是评估接受颈髓减压术的软骨发育不全儿童的预后。43名患有杂合性软骨发育不全和枕大孔狭窄的儿童患者在作者的研究所进行了11年的45次颈髓减压术。手术减压后，所有患者的术前症状均完全缓解或部分改善。接受治疗的患者中没有死亡病例。手术的发病率很低，通常包括硬脑膜被打开的患者的脑脊液(CSF)泄漏(无论是有意还是意外)。所有病例均通过局部处理(伤口缝合)或脑脊液改道成功处理。在这篇综述中，作者证明在软骨发育不全的情况下，颈髓交界处的减压术可以安全地完成，具有显着的临床益处和最低的发病率。
Object. Achondroplasia is the most common hereditary form of dwarfism, and is characterized by short stature, macrocephaly, and a myriad of skeletal abnormalities. In the pediatric population, stenosis and compression at the level of the cervicomedullary junction commonly occurs. The goal in this study was to assess the outcomes in children with achondroplasia who underwent cervicomedullary decompression.Methods. Forty-three pediatric patients with heterozygous achondroplasia and foramen magnum stenosis underwent 45 cervicomedullary decompressions at the authors' institution over an 11-year period. After surgical decompression, complete resolution or partial improvement in the preoperative symptoms was observed in all patients. There were no deaths in the treated patients. The surgical morbidity rate was low and usually consisted of a cerebrospinal fluid (CSF) leak in patients in whom the dura mater had been opened (either intentionally or accidentally). This problem was successfully managed in all cases with local measures (wound oversewing) or CSF diversion.Conclusions. In this review the authors demonstrate that decompression of the cervicomedullary junction in the setting of achondroplasia may be accomplished safely with significant clinical benefit and minimal morbidity.