Mucopolysaccharidosis VI: Evaluation After 2 Years of Treatment

Abstract Introduction: Mucopolysaccharidosis VI (MPS VI) is the result of the absence of arylsulfatase B leading to the abnormal lysosomal accumulation of glycosaminoglycans. Two different phenotypes have been described to date, namely, rapidly progressive and slowly progressive. Aim: To present the evolution of a slowly progressive phenotype of MPS VI in a patient after 2 years of enzyme replacement therapy. Case report: A 26-year-old man diagnosed with MPS VI at 9 years of age started enzyme replacement therapy with galsulfase due to cardiac, pulmonary, neurologic, and joint involvement. After 10 months of treatment, improvement in quality-of-life scales and walk test was evident. Because of persistent symptomatology associated with narrow cervical spinal canal, decompressive surgery was performed. After 2 years of treatment, there was a clear improvement in the respiratory, motor, and cardiac functions as well as in the spinal symptoms. Discussion: The evolution of our patient leads to the conclusion that the combined treatment of galasulfase and decompressive surgery should be indicated at an early stage in order to achieve best outcome for the patient.

Curative effect evaluation of senile osteoporotic vertebral compression fractures with balloon kyphoplasty / 局解手术学杂志

Objective To study the curative effect and safety of senile osteoporotic vertebral compression fractures with balloon ky-phoplasty. Methods The clinical data of 80 patients in our hospital during July 2008 to July 2012 with senile osteoporotic vertebral compres-sion fractures were analysed retrospectively. And the clinical effect was evaluated by observing the charges of visual analog scale (VAS), height of vertebral bodies and Cobb’ s angle, the quality of life score in patients respectively before and after operation. Results The pain of the patients were controlled effectively after operation, and the patients had significant and sustained improvement in anterior and midline ver-tebral body height after operation, with (56. 02 ± 12. 08) % and (58. 19 ± 13. 11) % before preoperation respectively, and (72. 10 ± 16. 19) % and (78. 33 ± 19. 02) % after surgery respectively. VAS score reduced from (8. 31 ± 1. 22) to (1. 65 ± 0. 33) after surgery;Cobb’s angle reduced from (24. 12 ± 3. 28)° to (13. 56 ± 2. 05)° after operation. The differences of the frontal height and flange height, VAS scores, and Cobb’s angle before and after operation were statistically significant (P<0. 05). The incidence of complications reduced from 23. 75% to 1. 25% after operation, which indicates significant difference (P<0. 01). According to QLQC-30 quality of life score, the postoperative life of patients were much better than that of the preoperative life, and the difference of all statistical index were of statistical significance (P<0. 05). Conclusion Balloon kyphoplasty in the treatment of senile osteoporotic vertebral compression fractures has signifi-cant effect and high safety, it should be popularized and applied in clinical.

Analysis of Osteoporotic Spinal Compression Fractures in Whole Spine Sagittal MR Images

OBJECTIVES: To analyze the fracture type and adjacency of accompanied fractures in acute osteoporotic spinal compression fracture using whole spine sagittal MRI (WS-MRI), and to know the relation of sagittal vertical axis (SVA), body mass index (BMI), and lumbar bone mineral density (BMD). METHODS: From June 2007 to December 2010, 167 patients who had acute spinal compression fractures confirmed by WS-MRI divide in three groups. 82 patients (M/F : 25/57) who had acute fractures only were in group 1, 79 patients who had acute and old fractures were in group 2, and 20 patients who had acute fractures after vertebroplasy were in group 3. To analyze the fracture type and adjacency of accompanied fractures in acute osteoporotic spinal compression fractures which combined chronic fracture or vertebroplasty using the WS-MRI and compared with the control group who had no spinal fractures measured the SVA. In all groups, we measured BMI, and BMD. RESULTS: In WS-MRI, 31 patients in group 1 had L1 which was the most common fracture site. Twenty two patients had chronic fractures and more than 3 remote levels at the acute fracture site in group 2 and especially among there 14 patients have each level in cervicothoracic and lumbar vertebrae. In group 3 had old fracture and adjacent fracture was 14 and 11 patients. Compared with the control group, all groups had increased SVA, especially in group 2 which has acute and chronic compression fractures. They have no significantly difference of BMI in each group, but group 2 and 3 had a significant lower BMD than group 1. CONCLUSION: Fourteen patients of coexisting fractures in acute osteoporotic spinal compression fractures with WS-MRI which could be missed in the conventional MRI. Additionally this study suggests that longer SVA causes sagittal imbalance, and BMD is more relative than BMI in refractures of chronic compression fracture patients.

Advance in Spinal Cord Compression Injury (review) / 中国康复理论与实践

@#Spinal cord compression injury (SCCI) is common in orthopedics manifested as system dysfunction, and even Results in disability and death. This article would review the recent researches for SCCI.

Efficacy and Safety of Balloon Kyphoplasty in the Treatment of Osteoporotic Vertebral Body Compression Fractures: Compared with Vertebroplasty

OBJECTIVE: Kyphoplasty and vertebroplasty are two minimally invasive procedures for osteoporotic vertebral compression fractures. The purpose of this retrospective study was to compare the radiological findings and clinical outcomes between two procedures. METHODS: Osteoporotic vertebral fractures were treated in 76 vertebrae, using kyphoplasty (n=35 vertebrae) and using vertebroplasty (n=41 vertebrae). Fractured vertebral bodies were diagnosed by correlating the clinical symptoms with radiologic study. The responses of pain symptoms were measured by a self-reported Visual Analog Scale (VAS) score. Plain X-rays were checked preoperatively and postoperatively at admission and 6 months. The vertebral body height and kyphotic angle were measured to assess the reduction of the sagittal alignment. RESULTS: The mean pain scores were decreased significantly for both procedures postoperatively, but there were no significant differences between two groups. Kyphoplasty led to a significant reduction of the vertebral body height and improvement of kyphotic angle. There were no neurological deficits after kyphoplasty, but one patient experienced paraparesis after vertebroplasty. During the 6 months follow-up both procedures provided stabilization of the sagittal alignment. CONCLUSION: Kyphoplasty and vertebroplasty are considered effective minimally invasive techniques for the stabilization of osteoporotic vertebral body fractures, leading to a statistically significant reduction in pain. Kyphoplasty significantly restore sagittal alignment. Also, complications and the incidence of bone cement leakage are significantly lesser than vertebroplasty. Therefore, kyphoplasty seems to be reasonable procedure for osteoporotic vertebral body compression fractures when medical treatment fail.

The Effects of Facet Joint Injection in Osteoporotic Spinal Compression Fractures

OBJECTIVE: To evaluate the effects of facet joint injection in the conservative management of osteoporotic spinal compression fractures METHOD: Among 27 patients with osteoporotic spinal compression fractures which were confirmed by plain radiography and bone densitometry (dual energy x-ray absorptiometry), 9 patients were control group and 18 patients received facet joint injection treatment. Facet joint injection of thoracolumbar spine was done under fluoroscopic guide with 1% lidocaine 1 ml and triamcinolone 10 mg at each joint above and below the level of compression fracture at both side. Main outcome measures were visual analog scale (VAS), spinal movement (modified Schober's and lateral bending test), and physical activity from bed-ridden state (grade I) to outdoor activity without pain (grade V). The treatment outcomes were assessed before injection, 2 weeks and 4 weeks after injection. RESULTS: There were significant decrease in VAS at 2 weeks and 4 weeks after injection in the study group (p<0.05). Physical activity was significantly improved at post injection 2 weeks and 4 weeks (p<0.05). There were no significant differences between the two groups in spinal movement. CONCLUSION: These results suggest that facet joint injection of thoracolumbar spine is useful method in the conservative management of painful osteoporotic compression fractures.