Aryanpur J, Hurko O, Francomano C, Wang H, Carson B
Department of Neurosurgery, Johns Hopkins Medical Institutions, Baltimore, Maryland.
J Neurosurg. 1990 Sep;73(3):375-82. doi: 10.3171/jns.1990.73.3.0375.
The congenital osseous abnormalities associated with achondroplasia include stenosis of the foramen magnum and the upper cervical spinal canal. In the pediatric achondroplastic patient, such stenosis may lead to cervicomedullary compression with serious sequelae, including paresis, hypertonia, delayed motor mile-stones, and respiratory compromise. Using a standardized protocol the authors have treated 15 young achondroplastic patients with documented cervicomedullary compression by craniocervical decompression and duroplasty. Following this procedure, significant improvement in presenting neurological or respiratory complaints was noted in all patients. The mortality rate in this series was zero. The major cause of morbidity associated with this procedure was perioperative cerebrospinal fluid (CSF) leakage from the surgical wound, presumably related to coexisting abnormalities of CSF dynamics. This problem was successfully managed by temporary or, when necessary, permanent CSF diversion procedures. It is concluded that craniocervical decompression is an effective and safe treatment for young achondroplastic patients with cervicomedullary compression.
与软骨发育不全相关的先天性骨异常包括枕骨大孔和上颈椎管狭窄。在小儿软骨发育不全患者中，这种狭窄可能导致颈髓受压并产生严重后果，包括轻瘫、张力亢进、运动发育迟缓以及呼吸功能不全。作者采用标准化方案，对15例有记录的颈髓受压的小儿软骨发育不全患者进行了颅颈减压和硬脑膜成形术治疗。术后，所有患者的主要神经或呼吸症状均有显著改善。该系列的死亡率为零。与该手术相关的主要发病原因是手术伤口的围手术期脑脊液漏，可能与脑脊液动力学的并存异常有关。这个问题通过临时或必要时的永久性脑脊液分流手术得到了成功解决。结论是，颅颈减压术是治疗颈髓受压的小儿软骨发育不全患者的一种有效且安全的方法。
