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
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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。
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 ultra-sound 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 oth-er 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 malforma tions. 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 abnormalities6.67%(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.
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 ultra-sound 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 oth-er 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 malforma tions. 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 abnormalities6.67%(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.
引文
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4.Bollini G,Launay F,Docquier PL,et al.Congenital abnormalities associated with hemivertebrae in relation to hemivertebrae location[J].J Pediatr Orthop B,2010,19(1):90-94.
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6.Wu N,Ming X,Xiao J,et al.TBX6 null variants and a common hypomorphic allele in congenital scoliosis[J].N Engl J Med,2015,372(4):341-350.
7.季明亮,邱勇,钱邦平,等.先天性脊柱侧凸患者中胚层后方同源物2基因外显子突变的筛查[J].中国脊柱脊髓杂志,2012,22(3):258-264.
8.Shen J,Wang Z,Liu J,et al.Abnormalities associated with congenital scoliosis:a retrospective study of 226 Chinese surgical cases[J].Spine,2013,38(10):814-818.
9.Mohanty S,Kumar N.Patterns of presentation of congenital scoliosis[J].J Orthop Surg,2000,8(2):33-37.
10.郭建伟,仉建国,王升儒,等.后路半椎体切除治疗先天性脊柱侧凸的并发症及预防策略[J].中华外科杂志,2014,52(8):566-570.
11.Blake NS,Lynch AS,Dowling FE.Spinal cord abnormalities in congenital scoliosis[J].Ann Radiol,1986,29(3-4):377-379.
12.BealsRK,Robbins JR,Rolfe B.Anomalies associated with vertebral malformations[J].Spine,1993,18(10):1329-1332.
13.Nakamura H,Matsuda H,Konishi S,et al.Single-stage excision of hemivertebrae via the posterior approach alone for congenital spine deformity:follow-up period longer than ten years[J].Spine,2002,27(1):110-115.
14.Zhuang Q,Zhang J,Li S,et al.One-stage posterior-only lumbosacral hemivertebra resection with short segmental fusion:a more than 2-year follow-up[J].Eur Spine J,2016,25(5):1567-1574.
15.Li Y,Wang G,Jiang Z,et al.One-stage posterior excision of lumbosacral hemivertebrae:retrospective study of case series and literature review[J].Medicine,2017,96(43):e8393.
2.Mcmaster MJ,Singh H.Natural history of congenital kyphosis and kyphoscoliosis:a study of one hundred and twelve patients[J].J Bone Joint Surg Am,1999,81(10):1367-1383.
3.Bollini G,Docquier PL,Viehweger E,et al.Lumbosacral hemivertebrae resection by combined approach:medium-and long-term follow-up[J].Spine,2006,31(11):1232-1239.
4.Bollini G,Launay F,Docquier PL,et al.Congenital abnormalities associated with hemivertebrae in relation to hemivertebrae location[J].J Pediatr Orthop B,2010,19(1):90-94.
5.马兆龙,邱勇,王斌,等.先天性脊柱侧凸患者中的脊髓畸形和脊椎畸形[J].中国脊柱脊髓杂志,2007,17(8):588-592.
6.Wu N,Ming X,Xiao J,et al.TBX6 null variants and a common hypomorphic allele in congenital scoliosis[J].N Engl J Med,2015,372(4):341-350.
7.季明亮,邱勇,钱邦平,等.先天性脊柱侧凸患者中胚层后方同源物2基因外显子突变的筛查[J].中国脊柱脊髓杂志,2012,22(3):258-264.
8.Shen J,Wang Z,Liu J,et al.Abnormalities associated with congenital scoliosis:a retrospective study of 226 Chinese surgical cases[J].Spine,2013,38(10):814-818.
9.Mohanty S,Kumar N.Patterns of presentation of congenital scoliosis[J].J Orthop Surg,2000,8(2):33-37.
10.郭建伟,仉建国,王升儒,等.后路半椎体切除治疗先天性脊柱侧凸的并发症及预防策略[J].中华外科杂志,2014,52(8):566-570.
11.Blake NS,Lynch AS,Dowling FE.Spinal cord abnormalities in congenital scoliosis[J].Ann Radiol,1986,29(3-4):377-379.
12.BealsRK,Robbins JR,Rolfe B.Anomalies associated with vertebral malformations[J].Spine,1993,18(10):1329-1332.
13.Nakamura H,Matsuda H,Konishi S,et al.Single-stage excision of hemivertebrae via the posterior approach alone for congenital spine deformity:follow-up period longer than ten years[J].Spine,2002,27(1):110-115.
14.Zhuang Q,Zhang J,Li S,et al.One-stage posterior-only lumbosacral hemivertebra resection with short segmental fusion:a more than 2-year follow-up[J].Eur Spine J,2016,25(5):1567-1574.
15.Li Y,Wang G,Jiang Z,et al.One-stage posterior excision of lumbosacral hemivertebrae:retrospective study of case series and literature review[J].Medicine,2017,96(43):e8393.