Archer Jacob, Jea Andrew
Section of Pediatric Neurosurgery, Riley Hospital for Children, Department of Neurological Surgery, Indiana University School of Medicine, Goodman Campbell Brain and Spine, Indianapolis, Indiana.
Oper Neurosurg (Hagerstown). 2019 Jun 1;16(6):E168. doi: 10.1093/ons/opy247.
Tethered cord syndrome is a constellation of signs and symptoms involving sensory and motor dysfunction of the lower extremities, bladder, and bowel. The pathophysiology may be from a mechanical stretch of the distal spinal cord causing deleterious changes in blood supply and metabolism. The standard of care for tethered cord syndrome has been tethered cord release. However, drawbacks of traditional tethered cord release include neurological injury, cerebrospinal fluid leak, and intradural scarring and retethering of the spinal cord (secondary tethering). As such, spinal column shortening was described to avoid these risks.1 We applied spinal column shortening to children and transitional adults with secondary tethered cord syndrome.1 As shown in cadaveric biomechanical studies, the objective of surgery is to shorten by the spinal column by 1.5 to 2.5 cm, to obtain the equivalent relief of traction on the spinal cord and nerve roots as direct circumferential untethering.2 In this video, we present the case of an 11-yr-old boy with a history of VATERL (vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal anomalies, and limb abnormalities) and tethered cord syndromes manifest as bladder dysfunction. Our patient had segmentation and formation anomalies of the lumbosacral junction, imperforate anus, and leg length discrepancy. He had already undergone multiple tethered cord releases with no durable improvement in bladder function. After discussion of the advantages and disadvantages of spinal column shortening with the patient and his father, we decided to proceed with this treatment alternative. At 9 mo after surgery, the patient has satisfactory clinical and radiographic outcomes. Informed consent was obtained from the parents of the patient.
脊髓拴系综合征是一系列涉及下肢、膀胱和肠道感觉及运动功能障碍的体征和症状。其病理生理学可能源于脊髓远端的机械性牵拉，导致血液供应和代谢发生有害变化。脊髓拴系综合征的标准治疗方法是脊髓拴系松解术。然而，传统脊髓拴系松解术的缺点包括神经损伤、脑脊液漏、硬膜内瘢痕形成以及脊髓再拴系（继发性拴系）。因此，有人描述了脊柱缩短术以避免这些风险。我们将脊柱缩短术应用于患有继发性脊髓拴系综合征的儿童和青少年成人。如尸体生物力学研究所示，手术的目的是使脊柱缩短1.5至2.5厘米，以获得与直接环形松解术对脊髓和神经根相同的牵引缓解效果。在本视频中，我们展示了一名11岁男孩的病例，他有VATERL（脊柱缺陷、肛门闭锁、心脏缺陷、气管食管瘘、肾脏异常和肢体异常）病史，脊髓拴系综合征表现为膀胱功能障碍。我们的患者存在腰骶部连接的节段性和形成性异常、肛门闭锁以及腿长差异。他已经接受了多次脊髓拴系松解术，但膀胱功能没有得到持久改善。在与患者及其父亲讨论了脊柱缩短术的利弊后，我们决定采用这种治疗方案。术后9个月，患者的临床和影像学结果令人满意。已获得患者父母的知情同意。
