OBJECTIVETo investigate the pulmonary dysfunction patterns in patients of scoliosis associated with neurofibromatosis type I (NF1) and to identify factors affecting the pulmonary function in patients with scoliosis associated with NF1.

METHODSPreoperative pulmonary function tests (PFTs) were evaluated in 100 patients with scoliosis [NF1 group, 36 cases; idiopathic scoliosis (IS) group, 64 cases] from January 2003 to June 2009. According to location of apical vertebra and dystrophic change in patients with NF1, the parameters of pulmonary function [vital capacity (VC), forced vital capacity (FVC), forced expiratory volume in one second (FEV1), maximal mid-expiratory flow (MMEF), maximal voluntary ventilation (MVV)] were compared between NF1 group and IS group, and between the subgroups of NF1. The correlation between pulmonary function parameters and radiographic parameters of scoliosis was analyzed.

RESULTSThe VC, FVC, FEV1, MMEF, MVV in NF1 group and IS group were of no significant difference (P > 0.05). In NF1 patients, the pulmonary dysfunction was more severe in thoracic subgroup than non-thoracic subgroup (P < 0.05), while there was no difference between dystrophic scoliosis and non-dystrophic scoliosis (P > 0.05). The location of apical vertebra and the severity of scoliosis correlated significantly with the pulmonary dysfunction in NF1 group.

CONCLUSIONSThe pattern of pulmonary dysfunction in scoliosis associated with NF1 is similar with IS. Pulmonary dysfunction is more severe in thoracic scoliosis. The location of apical vertebra and the severity of scoliosis are the risk factors influencing the pulmonary dysfunction.

Adolescent ; Child ; Female ; Forced Expiratory Volume ; Humans ; Lung ; physiopathology ; Male ; Neurofibromatosis 1 ; complications ; physiopathology ; Respiratory Function Tests ; Scoliosis ; complications ; physiopathology ; Vital Capacity ; Young Adult

OBJECTIVETo investigate the clinical outcome and fusion rate in patients with idiopathic thoracolumbar/lumbar scoliosis treated with anterior correction and interbody fusion with calcium phosphate cement.

METHODSFrom October 2006 to March 2008, 24 cases undergoing anterior correction and interbody fusion with calcium phosphate cement were enrolled. All of them were female, with an age ranged from 12 to 25 years. The mean Cobb angle of main curve was 46° ± 5° (range, 40° - 56°) before surgery. During operation, the most proximal and distal disc spaces were filled with rib autograft, while the remaining levels were filled with calcium phosphate cement. The interbody fusion rate, coronal correction and sagittal profile reconstruction were evaluated respectively by using χ² test and t test.

RESULTSInterbody fusion was performed in 103 levels, including 48 levels with rib autograft and 55 levels with calcium phosphate cement. The mean follow-up period was 23.8 months (range, 12 - 33 months) in this series. At the follow-up of 6 months, fusion rate was found as 54.2% in the levels filled with rib autograft, while 50.9% in those filled with calcium phosphate cement. Solid fusion of the whole instrumented area was achieved in all cases with a minimum one-year follow-up. No instrumentation-related complications occurred. The correction rate of main curve was on an average of 76% ± 11% after surgery. A significant difference was found between preoperative and immediate postoperative value in terms of the main curve magnitude (46° ± 5° vs. 14° ± 5°, t = -26.95, P < 0.05). The correction loss of the main curve was -5.1° - 10.4° at the final follow-up. The coronal balance and lower instrumented vertebra tilting were significantly improved after operation. The thoracolumbar kyphosis was significantly reduced postoperatively (t = 3.11, P < 0.05).

CONCLUSIONSatisfactory bone fusion and correction maintenance can be achieved in idiopathic thoracolumbar/lumbar scoliosis treated by anterior instrumentation combined with interbody fusion using calcium phosphate cement.

Adolescent ; Adult ; Bone Cements ; therapeutic use ; Calcium Phosphates ; therapeutic use ; Child ; Female ; Follow-Up Studies ; Humans ; Lumbar Vertebrae ; surgery ; Scoliosis ; surgery ; Spinal Fusion ; methods ; Thoracic Vertebrae ; surgery ; Treatment Outcome ; Young Adult

OBJECTIVETo investigate the association between the tissue inhibitor of metalloproteinase-2 (TIMP-2) gene polymorphisms with the predisposition and disease severity of thoracic adolescent idiopathic scoliosis (AIS).

METHODSThree hundred and fifty-four female thoracic AIS patients treated from January 2007 to March 2009 and 210 healthy female who underwent health examination during March 2005 to June 2006 as normal controls were recruited in this study. One SNP-418G/C (rs8179090) in the promoter region were selected for TIMP-2 gene. PCR- RFLP was used for genotyping.

RESULTSNo significant differences of genotype and allele frequency distribution were found between AIS patients and normal controls (P > 0.05). In AIS patients, the frequency of C allele of the patients with the body mass index (BMI) < 17 kg/m(2) was significantly higher than those with the BMI > or = 17 kg/m(2) (P < 0.05), and the frequency of C allele of cases with the major Cobb angle > or = 40 degrees was significantly higher than that with Cobb angle < 40 degrees (P < 0.05). Among the patients who reached skeletal maturity without any interference of natural history, significantly higher average maximum Cobb angle was found in patients with GC and CC genotype compare with those with GG genotype.

CONCLUSIONSThe SNP-418G/C (rs8179090) in the promoter region of TIMP-2 gene may be associated with abnormal growth pattern and curve progression of thoracic AIS. TIMP-2 gene is a disease-modifier gene of thoracic AIS.

Adolescent ; Case-Control Studies ; Female ; Genetic Predisposition to Disease ; Genotype ; Humans ; Polymorphism, Genetic ; Polymorphism, Single Nucleotide ; Scoliosis ; genetics ; Tissue Inhibitor of Metalloproteinase-2 ; genetics

OBJECTIVESTo compare the sagittal profiles between thoracic idiopathic scoliosis (IS) patients with different curve progression and to determine the risk factors associated with curve progression.

METHODSA total of 83 thoracic IS patients from September 2009 to May 2010 were included in this study and were divided into 3 groups according to different curve progression. All the patients did not receive any previous treatments. There were 26 skeletally mature patients whose Risser sign were 5 degree with Cobb angle < 40° in non-curve progression group (NCP group), 29 mature patients whose Risser sign were 5 degree with Cobb angle ≥ 40° in moderate curve progression group (MCP group) and 28 immature patients whose Risser sign ≤ 3 degree with Cobb angle ≥ 40° in severe curve progression group (SCP group). Five sagittal parameters, including thoracic kyphosis (TK), lumbar lordosis (LL), sacral slope (SS), pelvic incidence (PI) and pelvic tilt (PT) were measured on the lateral X-ray films. Analysis of variance was used to compare these parameters among the 3 groups.

RESULTSThe average thoracic Cobb angle was significantly smaller in NCP group when compared with MCP group (P < 0.01) or SCP group (P < 0.01), but not significantly different between the 2 latter groups (P = 0.619). The average TK was 19° ± 7° in NCP group, 13° ± 6° in MCP group and 8° ± 5° in SCP group. The average TK was significantly smaller in SCP group when compared with MCP group (P = 0.011) or NCP group (P < 0.01), while the average TK was significantly smaller in MCP group when compared with NCP group (P < 0.01). None of the other 4 parameters showed any significant difference between the 3 groups (P > 0.05).

CONCLUSIONSThoracic hypokyphosis is strongly associated with curve progression in thoracic IS patients. Pelvic sagittal profile may not be involved in the underlying mechanism of curve progression in thoracic IS patients.

Adolescent ; Adult ; Female ; Humans ; Male ; Radiography ; Scoliosis ; diagnostic imaging ; pathology ; Thoracic Vertebrae ; diagnostic imaging ; pathology ; Young Adult

OBJECTIVETo investigate whether the titrate-resistant acid phosphatase 5 (ACP5) gene polymorphisms were associated with the occurrence or curve severity of adolescent idiopathic scoliosis (AIS).

METHODSThere were 372 AIS patients from January 2006 to December 2008 and 239 normal controls from March 2005 to August 2006 were recruited. The Cobb angles were ≥ 10° in all AIS patients. Using the haplotype data of Han population from the Hapmap Project, two tag SNPs (rs2229531, rs2071484) were defined for ACP5 gene. PCR-restriction fragment length polymorphism was used for the genotyping.

RESULTSNo polymorphism in rs2229531 was found in this study. The genotype and allele frequency distribution in rs2071484 were similar between AIS patients and normal controls (χ(2) = 3.336 and 1.438, P > 0.05). The mean maximum Cobb angles of different genotypes of rs2071484 in ACP5 gene were 38° ± 19° in AA, 34° ± 14° in AG and 38° ± 21° in GG, which were similar with each other among AIS patients who reached skeletal maturity or received surgery treatment (P = 0.157).

CONCLUSIONThe ACP5 gene is neither associated with the occurrence nor the curve severity of AIS.

Acid Phosphatase ; genetics ; Adolescent ; Child ; Female ; Humans ; Isoenzymes ; genetics ; Male ; Polymorphism, Genetic ; Scoliosis ; genetics ; Tartrate-Resistant Acid Phosphatase

BACKGROUND: Scoliosis secondary to neurofibromatosis type 1 (NF1) in children aged <10 years is an important etiology of early-onset scoliosis (EOS). This study was performed to investigate the curve evolution of patients with EOS secondary to NF1 undergoing bracing treatment and to analyze high-risk indicators of rapid curve progression. METHODS: Children with EOS due to NF1 who underwent bracing treatment from 2010 to 2017 were retrospectively reviewed. The angle velocity (AV) at each visit was calculated, and patients with rapid curve progression (AV of >10°/year) were identified. The age at modulation and the AV before and after modulation were obtained. Patients with (n = 18) and without rapid curve progression (n = 10) were statistically compared. RESULTS: Twenty-eight patients with a mean age of 6.5 ± 1.9 years at the initial visit were reviewed. The mean Cobb angle of the main curve was 41.7° ± 2.4° at the initial visit and increased to 67.1° ± 8.6° during a mean follow-up of 44.1 ± 8.5 months. The overall AV was 6.6° ± 2.4°/year for all patients. At the last follow-up, all patients presented curve progression of >5°, and 20 (71%) patients had progressed by >20°. Rapid curve progression was observed in 18 (64%) patients and was associated with younger age at the initial visit and a higher incidence of modulation change during follow-up (t = 2.868, P = 0.008 and <0.001, respectively). The mean AV was 4.4° ± 1.2°/year before modulation and 11.8° ± 2.7°/year after modulation (t = 11.477, P < 0.010). CONCLUSIONS Curve progression of >10°/year is associated with younger age at the initial visit, and modulation change indicated the occurrence of the rapid curve progression phase.
Braces ; Child ; Child, Preschool ; Disease Progression ; Humans ; Neurofibromatosis 1/complications* ; Retrospective Studies ; Scoliosis/diagnostic imaging* ; Treatment Outcome

OBJECTIVETo measure the correlative parameters of vertebral pedicles from L₁ to S₁ by CT scan in the patients with thoracolumbar kyphosis secondary to ankylosing spondylitis(AS) and disc degenerative disease(DDD), and analyze their anatomical difference in order to provide the selection and placement of pedicle screw during operation.

METHODSThe clinical data of 30 male AS patients (AS group) with the mean age of(35.7±9.5) years (ranged, 23 to 51) and 30 male DDD patients (DDD group) with the mean age of(52.4±8.9) years(ranged, 39 to 64) underwent surgery in our institution from March 2012 to November 2014 were analyzed. The CT scans of lumbar and sacrum were performed before surgery. The parameters of vertebral pedicle from L₁ to S₁ were measured and compared, including pedicle width (PW), pedicle screw path length (PL), pedicle height (PH), pedicle transverse angle (EA), and pedicle inclined angle (FA). Paired sample t-test was used to detect the divergence in the above-mentioned data between left and right sides. In addition, results between two groups were compared using independent sample t-test.

RESULTSThe study showed that a gradual increase in the average pedicle width both AS group and DDD group from L₁ to S₁. The average PW of AS group was bigger than DDD group in L₅ and S₁(<0.05), it was(16.47±2.66) mm and (21.76±2.97)mm vs. (14.51±2.11)mm and (18.87±2.14) mm respectively;the average PL of DDD group was smaller than AS group from L₁ to S₁(<0.05); the both maximum of PL were in L₃ segment; the average EA of AS group was smaller than DDD group from L₁ to S₁; the average FA of AS group was significantly smaller than DDD group from L₃ to S₁, (<0.05), was(-2.88±10.24)°, (-7.88±10.22)°, (-7.70±10.40)°, (-5.15±10.25)° vs. (4.05±2.21)°, (7.79±4.38)°, (7.07±3.21)°, (12.62±3.21)°, respectively.

CONCLUSIONSIncreasing the strength of internal fixation is feasible to insert the larger and bigger pedicle screws in low lumbar and S₁ among AS patients, while the EA should be decreased properly and the direction on the sagittal plane should be adjusted.