METHODS: We analyzed the clinical and radiological data of 260 children with different types of spinal cord malformations in combination with vertebral abnormalities.
RESULTS: Among 260 individuals, approximately 109 presented with open neural tube defects (ONTDs), 83 with split cord malformations (SCMs), and 83 with different types of spinal lipomas. Pathological spina bifida emerged as the most frequent vertebral anomaly, affecting 232 patients, with a higher prevalence in ONTD. Vertebral segmentation disorders, including unsegmented bars, butterfly vertebrae, and hemivertebrae, were present in 124 cases, with a higher prevalence in SCM. The third most common spinal anomaly group consisted of various forms of sacral agenesis (58 cases), notably associated with blunt conus medullaris, spinal lipomas, and sacral myelomeningocele. Segmental aplasia of the spinal cord had a typical association with segmental spinal absence (N = 17).
CONCLUSIONS: The association between SCM and neuroenteric cyst/canal and vertebral segmentation disorders is strong. High ONTDs often coincide with pathological spina bifida posterior. Type 1 spinal lipomas and focal spinal nondisjunction also correlate with pathologic spina bifida. Segmental spinal absence or dysgenesis involves localized spinal and spinal cord aplasia, sometimes with secondary filar lipoma.
方法:我们分析了260例不同类型的脊髓畸形合并椎体畸形的儿童的临床和放射学数据。
结果:在260个人中,大约109例出现开放性神经管缺陷(ONTDs),83例脊髓分裂畸形(SCM),83例不同类型的脊髓脂肪瘤.病理性脊柱裂是最常见的椎体异常,影响232名患者,ONTD患病率较高。椎体分割障碍,包括未分段的钢筋,蝴蝶椎骨,和半椎骨,出现在124例病例中,SCM患病率较高。第三常见的脊柱异常包括各种形式的骶骨发育不全(58例),特别是与延髓钝锥有关,脊髓脂肪瘤,和骶骨脊髓膜膨出。脊髓节段性发育不全与节段性脊髓缺失有典型关联(N=17)。
结论:SCM与神经肠囊肿/肠管和椎骨分割障碍之间有很强的关联。高ONTDs通常与病理性脊柱裂并发。1型脊髓脂肪瘤和局灶性脊髓非分离也与病理性脊柱裂有关。节段性脊髓缺失或发育不全涉及局部脊髓和脊髓发育不全,有时伴有继发性丝状脂肪瘤。