脊髓纵裂的诊断与治疗

１９８５年１月～１９９２年１２月两院共收治先天性脊柱侧凸２１９例，其中并存的脊髓纵裂１３例（５．９％）。作者认为在矫正脊柱畸形之前应认真排除此病，以免在畸形矫治中发生脊髓损伤，并对该病的诊断要点及治疗原则作了讨论。     

先天性脊柱侧凸合并脊髓纵裂的手术治疗

目的 :探 讨先 天 性脊 柱侧 凸 合并 脊髓 纵 裂患 者的 矫 形手 术治 疗 方法 及效 果 。方 法:回顾 性 分析 31 例 先天性 脊柱 侧 凸合 并脊 髓 纵裂 患者 的 临床 资料 、手术 方 法 及 治疗 结 果 。 结果 :31 例 患 者中 合 并 骨 性纵 裂 4 例 ,纤维性 纵裂 23 , 例 混合 性 纵裂 例 纵 裂涉 及 胸段 4 , 10 ,例 腰段 例 胸 段 及腰 段 6 , 14 , 例 颈 段到 腰 段 例 1 。 矫形 手术方 法 包 括 前路 矫 形 内 固定 2 例 ,后 路 矫 形 内固 定 14 例 ,前 路 松 解 联合 二 期 后 路矫 形 内 固 定 10 例 ,一 期 前 后路半 椎体 切 除联 合后 路 矫形 内固 定 例 仅 例 患者 在 后路 矫形 手 术时 先行 切 除了 骨嵴 ,纤 维 性纵 裂均 未 予处 5 。 1理。 手术 前 、后 脊柱 侧 凸主 弯冠 状 面 Cobb 角、 顶椎 偏 距、 顶 椎 旋 转度 、 躯 干 偏移 平 均 分 别为65.4° 35.1° 和 ;51.2m m 和32.2m m ;1.3° 1.2°13.5m m 和 ; 和 8.9m m ,主弯冠 状 面矫 形率 平 均为 49.1%。术 后均 无 神经 功能 缺 陷发生或 神经 功 能缺 陷加 重 。结论 :根 据 合并 脊髓 纵 裂的 类型 、患 者的 神 经功 能状 态 选择 相应 的 治疗 方法 ,先 天 性脊柱侧 凸可 以 获得     

The faun tail and the plastic surgeon

Congenital spinal abnormalities seen with spinal dysraphism, when not recognized and treated in a timely fashion, can cause devastating neurological deficits. These anomalies are often accompanied by cutaneous markers, e.g., faun tail. Early recognition of the association of these lesions with the underlying spinal anomalies will reduce the morbidity associated with this condition. At times, the management of the cutaneous marker will be of primary importance. A review of Roman mythology surrounding the name ascribed to this clinical condition is included.     

Comparison of prenatal and postnatal MRI findings in the evaluation of intrauterine CNS anomalies requiring postnatal neurosurgical treatment

Aim To assess the diagnostic capability of fetal magnetic resonance imaging (MRI) in children suspected antenatally to harbor central nervous system (CNS) defects that require immediate postnatal neurosurgical treatment. Materials and methods Between 2003 and 2005, 13 fetal MRI scans were performed in mothers suspected to have fetuses with congenital CNS defects that would require surgery soon after birth. Comparisons between antenatal and postnatal scans were made with emphasis on diagnostic accuracy of antenatal examinations. Results All mothers were scanned using heavily T2-weighted fat-saturated sequences, allowing rapid acquisitions to avoid movement artefacts. Imaging quality was satisfactory in all patients. Diagnoses made antenatally were: myelomeningocele in seven, meningocele in one, diastematomyelia in one, occipital meningocele in one, and isolated hydrocephalus in three children. Of the seven children with antenatal diagnosis of myelomeningocele, one proved to have spinal lipoma postnatally. The patient who antenatally was diagnosed with meningocele proved to have spinal lipoma postnatally. These two were early antenatal MR scans. Antenatal diagnosis of hydrocephalus was made in five of the six confirmed myelomeningocele patients, which was verified postnatally. Antenatal diagnosis of Chiari II malformation was made in all six confirmed myelomeningocele patients. The antenatal diagnoses of occipital meningo-encephalocele and isolated hydrocephalus were verified postnatally. Antenatal diagnosis of diastematomyelia was not verified postnatally. Conclusion Fetal MRI scanning is an effective, noninvasive method of assessing in-utero CNS abnormalities. The diagnostic accuracy has improved to allow prediction of clinical outcome and counseling for possible treatment, but is not perfect yet to allow counseling for termination of pregnancy. The material of this paper was presented at the 34th Annual Meeting of the International Society for Paediatric Neurosurgery, Taipei, Taiwan, 10–14 September 2006.     

脊髓纵裂畸形CT诊断和临床分析

目的 探讨CT在脊髓纵裂畸形诊断和治疗中的价值。方法 回顾分析12例经手术病理或临床证实的脊髓纵裂畸形CT表现和临床特点。结果 根据CT表现脊髓纵裂分为有间隔的Ⅰ型和无间隔的Ⅱ型，Ⅰ型中完全性骨性中隔7例，不完全性骨性中隔1例，软骨性1例，纤维性2例，Ⅱ型中无间隔1例，均合并有不同程度的脊柱先天性畸形，诊断正确率92％。10例手术切除纵裂间隔，总有效率90％。结论 CT具有特征性表现，并可显示存在的合并畸形，可为临床制订手术方案提供依据。     

Diagnosis of diastematomyelia with high-resolution spinal ultrasound

Two children with diastematomyelia were diagnosed before operation with high-resolution spinal ultrasonography. Neonates with evident or suspected diastematomyelia can be effectively examined by high-resolution spinal ultrasonography and spinal radiography, reserving more invasive techniques for those for whom the method proves inadequate.     

Spina bifida occulta: Radiographic and operative correlation

The clinical and myelographie evaluation in 24 children with spina bifida occulta has been correlated with the operative findings. Myelograms were done using Myodil in 11 patients and Metrizamide in 13 patients. The diagnostic quality of myelograms done with Metrizamide was comparatively better. Myodil myelography failed to demonstrate diastematomyelia in one case and a durai sac in 3 cases of lipomeningomyelocele, whereas, Metrizamide failed to demonstrate a sac in one case of lipomeningomyelocele only. Worsening of the neurological status was observed in only 3 cases in whom myelography was done with Myodil. The radiographie and operative correlation has been discussed.     

脊髓纵裂手术前后胫后神经体感诱发电位分析

２０例脊髓纵裂患者手术前后进行了两下肢胫后神经皮层体感诱发电位（ＣＳＥＰ）检查，并选择了２０例正常人作为对照组，结果发现手术组与对照组ＣＳＥＰ有显著性差异，手术治疗后患者ＣＳＥＰ的Ｐ４０峰潜伏期及波幅明显改善，患者两下肢间的ＣＳＥＰ亦有明显差异。表明ＣＳＥＰ是一敏感、客观、可靠的诊断指标，可用来判断神经损害的程度，评价手术疗效。文中并讨论了神经缺陷的机理。     

Magnetic Resonance Imaging in Occult Spinal Dysraphism

A prospective study was carried out in 100 cases of suspected occult spinal dysraphic anomalies with Magnetic Resonance Imaging in order to determine its diagnostic efficacy as the initial imaging modality. MR imaging provided accurate preoperative information in 91 out of 92 cases (98.9%). Some of the unusual and interesting findings in the series were: presence of intrinsic cord abnormality in 19 out of 21 cases (90.4%) with a normal plain radiograph, 4 cases of diastematomyelia with a dermoid in the dorsal and lumbar region associated with syringohydromyelia, intradural fibrous/glial bands, sgringo-hydromyelia/myelomalacia of the conus with tethered cord syndrome having a normally placed conus, and myelocystocele. It is concluded that MRI is an excellent primary diagnostic tool, together with a plain radiograph, for complete preoperative evaluation of mid-tine spinal anomalies.     

Diastematomyelia combined with disc herniation at T 6/7 in an adult. Case report

A case of thoracic diastematomyelia associated with acute disc herniation is reported. The female patient noted sensory and motor symptoms at 49 years of age, four months before hospitalization. Myelography, myelo-computerized tomography and nuclear resonance tomography of the thoracic spine and cord showed the region of diastematomyelia with an additional disc herniation at T 6/7. The bone spur and the disc was successfully excised. Post-operatively, the patient's deficits improved.