Prasad G L, Borkar Sachin A, Satyarthee G D, Mahapatra A K
Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi, India.
J Pediatr Neurosci. 2012 Sep;7(3):167-70. doi: 10.4103/1817-1745.106469.
Split cord malformations (SCM) with a dorsally located bony spur are a very rare entity. The authors report a series of four such cases. The literature is reviewed regarding the pathogenesis and management of this uncommon variant of SCM. The presenting features include - scoliosis with motor and autonomic dysfunction (n = 1), scoliosis with cutaneous patch (n = 1), hypertrichotic area (n = 1), and motor deficits alone (n = 1). The location of spur was thoracic and lumbar in two patients (50%) each. Low-lying conus was present in three patients. Long segment syrinx was present in one patient. With respect to the bony anomalies, two patients had a hypertrophied posterior arch (HPA) and one patient had a dysraphic spine. All patients underwent surgical excision of the spur with detethering, if a low-lying conus was present. Two patients developed transient worsening of the neurological status after surgery which recovered at the time of the last follow-up; in both these patients, there was a HPA and a low-lying conus. Surgical excision of the spur with detethering of filum, in cases of low-lying conus, is the treatment of choice. Risk of post-operative worsening of the neurological status is increased in cases in which there is concomitant presence of HPA.
伴有背侧骨赘的脊髓纵裂畸形（SCM）是一种非常罕见的疾病。作者报告了一系列4例此类病例。本文对这种不常见的SCM变异型的发病机制和治疗方法进行了文献综述。其临床表现包括：伴有运动和自主神经功能障碍的脊柱侧弯（n = 1）、伴有皮肤斑片的脊柱侧弯（n = 1）、多毛区域（n = 1）以及仅存在运动功能缺损（n = 1）。骨赘的位置在两名患者（各占50%）中位于胸段和腰段。三名患者存在低位圆锥。一名患者存在长节段脊髓空洞。关于骨骼异常，两名患者有肥大的后弓（HPA），一名患者有脊柱裂。如果存在低位圆锥，所有患者均接受了骨赘切除并松解栓系手术。两名患者术后出现神经功能状态短暂恶化，在最后一次随访时恢复；在这两名患者中，均存在HPA和低位圆锥。对于存在低位圆锥的病例，骨赘切除并松解终丝是首选的治疗方法。伴有HPA的病例术后神经功能状态恶化的风险会增加。
