Surgical Management of Progressive Thoracolumbar Kyphosis in Mucopolysaccharidosis: Is a Posterior-only Approach Safe and Effective?

BACKGROUND: According to the current literature, the recommended surgical treatment is circumferential spinal fusion, including both anterior and posterior procedures, for progressive thoracolumbar kyphosis in mucopolysaccharidosis (MPS). The purpose of this study was to report our experience with the posterior-only approach and instrumented fusion for MPS kyphosis. METHODS: Six consecutive patients with MPS and thoracolumbar junctional kyphosis managed with the posterior-only approach were included. Demographic data, the type of MPS, medical comorbidities, and accompanying clinical manifestations were recorded. Measurements recorded on radiographs for the study included the presence of any coronal-plane deformity, fusion levels, changes in the local kyphosis angle (LKA), proximal and distal junctional kyphosis angles, and the apical vertebral wedge angle. RESULTS: The average age at the time of surgery was 6.6 (range, 4 to 12) years. The average follow-up duration was 52.6 (range, 44 to 64) months. The mean preoperative LKA of 63.1±15.8 (range, 48 to 92) degrees decreased to a mean of 16.6±8.4 (range, 5 to 30) degrees immediately after surgery. At the latest follow-up, the mean LKA was 19.6±8.8 (range, 8 to 34) degrees. Apical vertebral listhesis was reduced in all patients with surgical correction. The average apical vertebral wedge angle of 15 (range, 11 to 19) degrees at the early postoperative period decreased to 4.6 (range, 2 to 7) degrees at the latest follow-up. Adjacent-segment problems occurred in 2 patients. There were no neurological complications or implant failures in any of the patients. CONCLUSIONS: Posterior-only corrective techniques and instrumented fusion with pedicle screws for progressive thoracolumbar junctional kyphosis in MPS patients are safe and effective methods. Its results are comparable to those achieved with conventional circumferential fusion. However, patients should be monitored closely for adjacent-segment problems. LEVEL OF EVIDENCE: Level IV-retrospective case series.

Increased soluble selectins as a reflection of activated platelets and endothelium in Legg-Calve-Perthes disease.

BACKGROUND AND AIM: Legg-Calve-Perthes disease (LCPD) is still an enigma. Hemostatic abnormalities have been indicated in the pathogenesis. We had previously demonstrated enhanced tissue factor pathway inhibitor response, increased global fibrinolytic capacity, and an increase in thrombomodulin in patients with LCPD compared with healthy individuals. These studies emphasized the role of vascular endothelium in pathogenesis of the LCPD. P-selectin is expressed on activated platelets and endothelial cells, and E-selectin is expressed on activated endothelial cells. The aim of this study was to assess circulating E-selectin and P-selectin levels in LCPD patients, which might reflect an endothelium activation and/or injury. MATERIALS AND METHODS: The study included 85 pediatric patients. Group I consisted of 55 patients with LCPD and group II (control) consisted of 30 healthy children. Peripheral venous blood concentrations of E-selectin and P-selectin levels were measured with a commercially available assay. RESULTS: Mean age was 8.41±2.73 years in group I and 8.83±2.92 years in group II. Both E-selectin and P-selectin levels were higher in LCPD patients in comparison with the age-matched controls. E-selectin was 54.92±18.84 pg/mL in group I, 45.54±15.31 pg/mL in group II and P-selectin was 46.40±20.35 pg/mL in group I, 36.92±9.84 pg/mL in group II (P=0.022 and P=0.019, respectively). CONCLUSIONS: On the basis of our results, two important endothelium and platelet markers, E-selectin and P-selectin, are upregulated in LCPD. Our results suggested that activated platelets and possibly endothelial activation, as reflected by enhanced P-selectin/E-selectin kinetics, might contribute to the microvascular thrombosis and/or inflammation of LCPD.

The effect of dual growing rod instrumentation on the apical vertebral rotation in early-onset idiopathic scoliosis.

BACKGROUND AND AIM: Treating progressive early-onset idiopathic scoliosis is challenging. Surgical treatment is indicated in patients whose curves progress despite nonsurgical treatment. Dual growing rod (DGR) technique allows control of the curve while permitting continued spine growth and pulmonary development. Correction in coronal and sagittal planes with this technique has demonstrated both clinically and radiologically in previous studies. It is shown that apical vertebra rotation (AVR) increases with single-rod instrumentation technique. The effect of DGR technique on AVR has not been investigated, yet. The aim of our study was to assess the impact of DGR instrumentation technique on the apical AVR. METHODS: The study included 12 patients with early-onset idiopathic scoliosis treated with DGR technique. Mean follow-up was 74 months. Vertebral rotation angle in the apex of the curve in preindex surgery was measured with Perdriolle and Stokes' method. As pedicle shadows were masked by rods postoperatively, vertebral rotation angle of same levels in final computed tomography scans was measured using Aaro and Dahlborn's method. Standing anterior-posterior and lateral x-rays were measured for assessing Cobb angle, thoracic kyphosis, lumbar lordosis in coronal and sagittal planes preoperatively, postoperatively, and at the time of final follow-up. RESULTS: Mean age at the time of growing rod instrumentation was 69 (36 to 108) months. Mean follow-up was 77 (57 to 91) months. The mean preoperative AVR angle was 27 (18 to 38) degrees and decreased to 18 (4 to 35) degrees at the time of final follow-up. The difference between preoperative and final follow-up AVR was found to be significant (P=0.003). Preoperative mean Cobb angles were found to be 63.8 (40 to 98) degrees, 25 (10 to 46) degrees (60%) (P<0.001) after index surgery and at the time of final follow-up 20 (7 to 42) degrees (66%) (P<0.001). The mean thoracic kyphosis and lumbar lordosis angles were found to be 46 (20 to 90) and 34 (16 to 80) degrees at preoperative stage; 25 (12 to 50) and 22 (8 to 35) degrees at immediate postoperative stage; and 38 (16 to 83) and 37 (16 to 60) degrees at the time of final follow-up. CONCLUSIONS: This study proves that the DGR technique has no negative effect on transverse plane deformities. When compared with preoperative values, correction of the AVR during the treatment period suggests that DGR is effective in controlling the coronal and sagittal planes along with transverse plane deformities. Further studies are needed to prove that DGR treatment definitely prevents progression of AVR.