Tamura Goichiro, Morota Nobuhito, Ihara Satoshi
Division of Neurosurgery, Tokyo Metropolitan Children's Medical Center, Tokyo, Japan.
J Neurosurg Pediatr. 2017 Sep;20(3):289-297. doi: 10.3171/2017.5.PEDS16719. Epub 2017 Jul 7.
OBJECTIVE Sacrococcygeal dimples in neonates and infants are of uncertain pathological import. Previously they were believed to be rarely associated with intraspinal anomalies. Recent studies using MRI, however, revealed that 6%-7% of pediatric cases of sacrococcygeal dimples were associated with anatomical tethered spinal cord (TSC). Because the prevalence of tethered cord syndrome is still unclear, there is no consensus among pediatric neurosurgeons on the management of children with sacrococcygeal dimples. The authors performed an analysis of MRI and urodynamic studies to validate their management strategy for pediatric cases of sacrococcygeal dimples. METHODS A total of 103 Japanese children (49 male and 54 female, median age 4 months, range 8 days-83 months) with sacrococcygeal dimples who were referred to the Division of Pediatric Neurosurgery between 2013 and 2015 were included in this study. The lumbosacral region of all the patients was investigated using MRI. Anatomical TSC was defined as a condition in which the caudal end of the conus medullaris is lower than the inferior border of the L2-3 intervertebral disc. Patients with minor spinal anomalies (e.g., anatomical TSC, filum lipoma, thickened filum, or filar cyst) underwent further urodynamic studies to ascertain the presence of neurogenic bladder (NGB). In this study, the presence of NGB without anatomical TSC but with other minor spinal anomalies was defined as "functional TSC." The prevalence of anatomical and functional TSC was investigated. The association of the following cutaneous findings with spinal anomalies was also assessed: 1) depth of the dimple, 2) deviation of the gluteal fold, and 3) other skin abnormalities (e.g., discoloration, angioma, or abnormal hair). RESULTS The children were classified into 4 groups: Group 1, patients with anatomical TSC; Group 2, patients with functional TSC; Group 3, patients without anatomical or functional TSC but with other minor spinal anomalies; and Group 4, patients with no spinal anomaly. There were 6 patients (5.8%) in Group 1, 8 patients (7.8%) in Group 2, 10 patients (9.7%) in Group 3, and 79 patients (76.7%) in Group 4. Twenty-four patients (23.3%; Groups 1, 2, and 3) showed MRI abnormalities, including filum lipoma (14 cases), filar cysts (5 cases), thickened filum (2 cases), and anatomical TSC without other spinal anomalies (3 cases). Untethering of the spinal cord was indicated for 14 patients (13.6%; Groups 1 and 2) with anatomical and functional TSCs. Preoperative NGB was found in 12 patients and improved postoperatively in 7 (58.3%). None of the associated lumbosacral skin findings predicted the presence of underlying spinal anomalies. CONCLUSIONS The prevalence of tethered cord syndrome among children with sacrococcygeal dimples was, for the first time, revealed to be higher than previously thought. MRI and supplemental urodynamic studies may be indicated for children with sacrococcygeal dimples to identify patients with symptomatic TSC.
目的 新生儿和婴儿的骶尾部酒窝的病理意义尚不明确。以前认为它们很少与脊髓内异常相关。然而,最近使用MRI的研究表明,6% - 7%的小儿骶尾部酒窝病例与解剖学上的脊髓栓系(TSC)有关。由于脊髓栓系综合征的患病率仍不清楚,小儿神经外科医生对于骶尾部酒窝患儿的治疗尚无共识。作者进行了MRI和尿动力学研究分析,以验证他们对小儿骶尾部酒窝病例的治疗策略。
方法 本研究纳入了2013年至2015年间转诊至小儿神经外科的103例日本骶尾部有酒窝的儿童(49例男性,54例女性,中位年龄4个月,范围8天至83个月)。对所有患者的腰骶部进行了MRI检查。解剖学上的TSC定义为脊髓圆锥尾端低于L2 - 3椎间盘下缘的情况。有轻微脊柱异常(如解剖学上的TSC、终丝脂肪瘤、终丝增厚或终丝囊肿)的患者接受了进一步的尿动力学研究,以确定是否存在神经源性膀胱(NGB)。在本研究中,无解剖学上的TSC但有其他轻微脊柱异常的NGB存在被定义为“功能性TSC”。研究了解剖学和功能性TSC的患病率。还评估了以下皮肤表现与脊柱异常的相关性:1)酒窝深度,2)臀褶偏移,3)其他皮肤异常(如色素沉着、血管瘤或异常毛发)。
结果 患儿被分为4组:第1组,有解剖学上的TSC的患者;第2组,有功能性TSC的患者;第3组,无解剖学或功能性TSC但有其他轻微脊柱异常的患者;第4组,无脊柱异常的患者。第1组有6例患者(5.8%),第2组有8例患者(7.8%),第3组有10例患者(9.7%),第4组有79例患者(76.7%)。24例患者(23.3%;第1、2和3组)显示MRI异常,包括终丝脂肪瘤(14例)、终丝囊肿(5例)、终丝增厚(2例)以及无其他脊柱异常的解剖学上的TSC(3例)。14例(13.6%;第1和2组)有解剖学和功能性TSC的患者需要进行脊髓松解术。术前发现12例患者有NGB,术后7例(58.3%)有所改善。没有任何相关的腰骶部皮肤表现能够预测潜在脊柱异常的存在。
结论 首次发现骶尾部有酒窝的儿童中脊髓栓系综合征的患病率高于先前的认识。对于有骶尾部酒窝的儿童,可能需要进行MRI和补充尿动力学研究,以识别有症状的TSC患者。