先天性腰骶部半椎体患者中脊髓畸形和脊髓外畸形的发生率

The incidence of intraspinal abnormalities and other systemic anomalies in patients with lumbosacral hemivertebra

中文关键词:  腰骶部半椎体  脊髓畸形  脊柱外异常

英文关键词:Lumbosacral hemivertebra  Intraspinal abnormality  Extraspinal anomaly

中文摘要:

  【摘要】 目的：探讨继发于腰骶部半椎体的先天性脊柱侧凸患者中脊髓畸形及脊髓外畸形的发生率。方法：回顾性分析2002年1月～2016年5月在我院收治的75例腰骶部半椎体患者，其中男35例，女40例，平均年龄13.8±9.1岁（3~41岁）。术前均行全脊髓MRI、心脏及泌尿生殖系统多普勒超声检查，统计腰骶部半椎体患者脊髓畸形及脊髓外畸形的发生情况。脊髓畸形包括脊髓纵裂、脊髓空洞、Chiari畸形、脊髓拴系、低位圆锥等。脊髓外畸形包括心脏异常、泌尿生殖系统异常、骶骨发育不良、双下肢不等长。分别按患者性别（男性与女性）、半椎体所处侧别（左侧与右侧）、半椎体分节情况（完全分节、半分节与未分节）及是否伴发其他部位半椎体（单发半椎体与多发半椎体）分组比较脊髓畸形及脊髓外畸形的发生率。结果：脊髓畸形的发生率为14.67%（11/75），其中脊髓纵裂6.67%（5/75），脊髓拴系5.33%（4/75），脊髓空洞4.0%（3/75），Chiari畸形1.33%（1/75），低位圆锥1.33%（1/75）；3例（4.0%）患者同时合并两种脊髓畸形。按性别、半椎体所处侧别、半椎体分节情况及是否伴发其他部位半椎体比较脊髓畸形的发生率均无显著性差异（P>0.05）。脊髓外畸形的发生率为20%（15/75），其中心脏异常6.67%（5/75），泌尿生殖系统异常4.0%（3/75），双下肢不等长4%（3/75），骶骨发育不良6.67%（5/75）。按性别、半椎体所处侧别、半椎体分节情况及是否伴发其他部位半椎体分组比较脊髓外畸形的发生率无显著性差异（P>0.05）。结论：在腰骶部半椎体患者中，脊髓畸形的发生率为14.67%，脊髓外畸形的发生率为20%。骶骨发育不良、双下肢不等长、心脏及泌尿生殖系统异常是发生率较高的脊髓外畸形。

英文摘要:

  【Abstract】 Objectives: To investigate the incidence of intraspinal abnormalities and other systemic anomalies in congenital scoliosis(CS) patients secondary to lumbosacral hemivertebra. Methods: From January 2002 to May 2016, the clinical data of 75 CS patients with lumbosacral hemivertebra who received correction surgery were reviewed. There were 35 males and 40 females with a mean age of 13.8±9.1(3-41) years. All of the patients underwent magnetic resonance image(MRI) of the whole spine, echocardiography and urogenital ultrasound before surgery. The incidences of intraspinal abnormalities and other systemic anomalies were analyzed. The patients were grouped according to gender, side, segmentation and number of hemivertebrae. The incidences of intraspinal and other systemic anomalies in different groups were compared. Intraspinal abnormalities were classified as diastematomyelia, tethered cord, syringomyelia, Chiari malformation and low conus. And other systemic anomalies were recorded including cardiac abnormalities, genitourinary abnormalities, discrepancy of lower extremities and sacrum dysplasia. Results: The incidence of intraspinal abnormalities was 14.67%(11/75), including diastematomyelia 6.67%(5/75), tethered cord 5.33%(4/75) syringomyelia 4.0%(3/75), Chiari malformation 1.33%(1/75) and low conus 1.33%(1/75). Three patients presented with 2 intraspinal malformations. There was no significant statistical difference in the incidence of intraspinal abnormalities between males and females, nor between left side and right side hemivertebra, nor between fully-segmented, semi-segmented and non-segmented hemivertebra, nor between single and multiple hemivertebrae(P>0.05). The incidence of other organ anomalies in patients with lumbosacral hemivertebra was 20%(15/75), with cardiac abnormalities 6.67%(5/75), genitourinary abnormalities 4.0%(3/75), discrepancy of lower extremities 4.0%(3/75) and sacrum dysplasia 6.67%(5/75), respectively. No significant statistical difference of the incidence of other systemic anomalies was found between males and females, nor between left side and right side hemivertebra, nor between fully-segmented, semi-segmented and non-segmented hemivertebra, nor between single and multiple hemivertebrae(P>0.05). Conclusions: The incidence of intraspinal abnormalities is 14.67% in CS patients with lumbosacral hemivertebra, while the incidence of other organ anomalies is 20%. Sacrum dysplasia, discrepancy of lower extremities, cardiac abnormalities and genitourinary abnormalities are the most common other systemic anomalies.