Alelyani Fayez, Aronyk Keith, Alghamdi Hashim, Alnaami Ibrahim
Department of Neurosurgery, King Khalid University Medical City, King Khalid University, Abha 61413, Saudi Arabia.
Department of Neuroscience, University of Alberta, Edmonton, AB T6G 2B7, Canada.
Children (Basel). 2022 Jul 29;9(8):1138. doi: 10.3390/children9081138.
Split notochord syndrome (SNS) is an exceedingly rare type of spinal dysraphism. SNS is sometimes associated with other congenital dysraphic defects but, as in our case, the association with spinal cord lipoma, tethered cord, and spinal deformity in the form of spinal column duplication would be exceedingly rare. Herein, the authors report a three-year-old child presented with SNS associated with complex spinal deformity and other associated congenital anomalies. The patient underwent microsurgical release of the tethering element with excellent short- and long-term outcomes.
A male newborn with healthy nonconsanguineous parents was born with multiple gastrointestinal and genitourinary anomalies, and duplicated vertebral columns at the lumbosacral area consistent with split notochord syndrome. The patient was initially managed for the gastrointestinal and genitourinary anomalies. As there was no obvious neurological deficit initially, the neurosurgical intervention was postponed till the child reached 30 months of age, when he underwent uneventful release of both spinal cords at their spit point.
SNS is an exceedingly rare developmental anomaly that is usually associated with varying degrees of complex congenital dysraphic defects. Early clinical diagnosis, understanding of the pathophysiology of spinal cord tethering, and microsurgical cord untethering are the important steps in optimal management.
分裂脊索综合征（SNS）是一种极为罕见的脊柱裂类型。SNS有时与其他先天性脊柱裂缺陷相关，但如我们的病例所示，与脊髓脂肪瘤、脊髓栓系以及脊柱重复形式的脊柱畸形相关则极为罕见。在此，作者报告一名3岁儿童，患有SNS并伴有复杂脊柱畸形及其他相关先天性异常。该患者接受了栓系因素的显微手术松解，短期和长期效果均良好。
一名男婴，父母非近亲结婚且身体健康，出生时伴有多种胃肠道和泌尿生殖系统异常，腰骶部脊柱重复，符合分裂脊索综合征。患者最初针对胃肠道和泌尿生殖系统异常进行治疗。由于最初无明显神经功能缺损，神经外科干预推迟至患儿30个月大时，此时他在脊髓分叉点顺利接受了双侧脊髓松解术。
SNS是一种极为罕见的发育异常，通常与不同程度的复杂先天性脊柱裂缺陷相关。早期临床诊断、对脊髓栓系病理生理学的理解以及显微手术松解脊髓是最佳治疗的重要步骤。
