Microsurgical efficacy in 326 children with tethered cord syndrome: a retrospective analysis

Tethered cord syndrome is a progressive disease with a typically insidious onset in infants and children, and which can lead to persistent progress of neurological deficits and a high rate of disability without timely intervention. The purpose of this study was to investigate the curative effect of microsurgery in children with different types of tethered cord syndrome. In this study, we analyzed 326 patients with tethered cord syndrome, aged from 2 months to 14 years old, who were followed for 3-36 months after microscopic surgery. Based on clinical manifestations and imaging findings, these patients were classified into five types： tight ilium terminale （53 cases）, lipomyelome- ningocele （55 cases）, lipomatous malformation （124 cases）, postoperative adhesions （56 cases）, and split cord malformation （38 cases）. All patients underwent microsurgery. Curative effects were measured before and 3 months after surgery by Spina Bifida Neurological Scale based on sensory and motor functions, reflexes, and bladder and bowel function. The results showed that Spina Bifida Neurological Scale scores improved in all five types after surgery. Overall effective rates in these patients were 75%. Effective rates were 91% in tight ilium terminale, 84% in lipomyelomeningocele, 65% in lipomatous malformation, 75% in postoperative adhesion, and 79% in split cord mal- formation. Binary logistic regression analysis revealed that types of tethered cord syndrome （lipoma-type or not） and symptom duration before surgery were independent influencing factors of surgical outcome. These results show that therapeutic effect is markedly different in patients with different types of tethered cord syndrome. Suitable clinical classification for tethered cord syndrome will be helpful in predicting prognosis and guiding treatment. This trial has been registered in the Chinese Clinical Trial Registry （registration number： ChiCTR1800016464）.

Thoracolumbar surgery for degenerative spine diseases complicated with tethered cord syndrome:A case report

BACKGROUND Tethered cord syndrome(TCS) secondary to split cord malformation(SCM) is rare in adulthood.There is as yet no consensus about the optimal treatment method for adult patients with SCMs and degenerative spine diseases such as lumbar stenosis,spondylolisthesis and ossification of the ligamentum flavum(OLF).The tethered cord poses a great challenge to the decompression and fusion procedures for the intraoperative stretching of the spinal cord,which might lead to deteriorated neural deficits.Here,we report on a case to add our treatment experience to the medical literature.CASE SUMMARY We treated a 67-year-old female patient with type II SCM suffering from lumbar disc herniation,degenerative lumbar spondylolisthesis and thoracic OLF.The patient underwent thoracolumbar spinal fusion and decompression surgery for severe lower back pain,extensive left lower limb muscle weakness and intermittent claudication.After the thoracolumbar surgery,without stretching the tethered cord,the patient achieved complete relief of pain and lower extremity weakness at final follow-up.CONCLUSION For adult patients with underlying TCS secondary to SCM coupled with thoracic OLF and lumbar spondylolisthesis,a thoracolumbar fusion surgery could be safe and effective with the tethered cord untreated.It is critical to design individualized surgical protocols to reduce the stretch of the low-lying spinal cord.

Association between chromosomal aberration of COX8C and tethered spinal cord syndrome:array-based comparative genomic hybridization analysis

Copy number variations have been found in patients with neural tube abnormalities.In this study,we performed genome-wide screening using high-resolution array-based comparative genomic hybridization in three children with tethered spinal cord syndrome and two healthy parents.Of eight copy number variations,four were non-polymorphic.These non-polymorphic copy number variations were associated with Angelman and Prader-Willi syndromes,and microcephaly.Gene function enrichment analysis revealed that COX8 C,a gene associated with metabolic disorders of the nervous system,was located in the copy number variation region of Patient 1.Our results indicate that array-based comparative genomic hybridization can be used to diagnose tethered spinal cord syndrome.Our results may help determine the pathogenesis of tethered spinal cord syndrome and prevent occurrence of this disease.

Tethered spinal cord syndrome with symptomatic onset in adulthood

Tethered spinal cord syndrome （TCS） is a condition of overstretching or compression of the caudal part of the spinal cord caused by various spinal lesions, such as a tight filum terminale or an intraspinal lipoma.l-9 Though it is a well-recognized cause of neurological deterioration in childhood, its symptomatic onset in adulthood is uncommon. Eleven cases of TCS are presented here. In addition, their related clinical features, surgical procedures and outcomes are investigated.