Objective:To compare the efficacy of posterior atlas uniaxial and polyaxial screw instrumentation and fusion with bone graft for Gehweiler type IIIb atlas fracture.Methods:A retrospective cohort study was performed to analyze the clinical data of 36 patients with Gehweiler type IIIb atlas fracture admitted to Henan Provincial People′s Hospital from January 2015 to October 2020. There were 29 males and 7 females, with age range of 23-82 years [(48.8±15.5)years]. All patients were treated with posterior atlas screw-rod internal fixation and fusion with bone graft, of which 14 received atlas uniaxial screw internal fixation (uniaxial screw group) and 22 received atlas polyaxial screw internal fixation (polyaxial screw group). The operation time and intraoperative blood loss were compared between the two groups. The atlas fracture union rate and atlantoaxial posterior arch bone fusion rate were compared between the two groups at 3 months and 6 months after operation. The anterior atlantodental interval (ADI), basion-dens interval (BDI) and lateral mass displacement (LMD) were compared between the two groups to evaluate the reduction of fracture fragments before operation, at 1, 3, 6 months after operation and at the last follow-up. At the same time, the visual analogue scale (VAS) and neck dysfunction index (NDI) were compared between the two groups to evaluate neck pain and functional recovery. The postoperative complications were observed.Results:All patients were followed up for 12-44 months [(27.2±9.9)months]. There was no significant difference in operation time or intraoperative blood loss between the two groups (all P>0.05). The atlas fracture union rate and atlantoaxial posterior arch bone fusion rate were 85.7% (12/14) and 78.6% (11/14) in uniaxial screw group at 3 months after operation, insignificantly different from those in polyaxial screw group [72.7% (16/22) and 77.3% (17/22)] (all P>0.05). All patients in the two groups achieved bone union and fusion at 6 months after operation. There was no significant difference in ADI between the two groups before and after operation (all P>0.05). The BDI in the two groups did not differ significantly before operation ( P>0.05), but a significantly higher value was found in uniaxial screw group at 1, 3, and 6 months after operation and at the last follow-up [(5.9±1.3)mm, (5.8±1.3)mm, (5.9±1.2)mm and (5.8±1.2)mm] than in polyaxial screw group [(3.1±0.6)mm, (3.1±0.6)mm, (3.1±0.6)mm and (3.1±0.6)mm] (all P<0.01). The two groups did not differ significantly before operation ( P>0.05), but LMD at 1, 3, and 6 months after operation and at the last follow-up was (1.6±0.8)mm, (1.5±0.8)mm, (1.5±0.7)mm and (1.5±0.9)mm in uniaxial screw group, significantly lower than that in polyaxial screw group [(4.8±1.6)mm, (4.6±1.6)mm, (4.9±1.6)mm and (4.9±1.6)mm] (all P<0.01). There was no significant difference in VAS between the two groups before operation ( P>0.05). The VAS at 1, 3, and 6 months after operation and at the last follow-up was 3.0(3.0, 4.0)points, 2.0(1.0, 2.0)points, 1.0(0.8, 2.0)points and 1.0(0.0, 1.3)points in uniaxial screw group and was 3.5(3.0, 4.0)points, 2.0(2.0, 3.0)points, 2.0(1.0, 2.0)points and 2.0(1.0, 3.0)points in polyaxial screw group. In comparison, the VAS scored much lower in uniaxial screw group than in polyaxial screw group at 6 months after operation and at the last follow-up (all P<0.01). There was no significant difference in NDI between the two groups before operation ( P>0.05). The NDI at 1, 3, and 6 months after operation and at the last follow-up was 34.9±6.3, 23.4±6.2, 13.9±2.7 and 9.4±2.8 in uniaxial screw group and was 33.2±6.1, 24.4±6.3, 18.1±4.1 and 12.7±3.2 in polyaxial screw group, showing a significantly lower NDI in uniaxial screw group than in polyaxial screw group at 6 months after operation and at the last follow-up (all P<0.01). The complication rate was 21.4% (3/14) in uniaxial screw group when compared to 22.7% (5/22) in polyaxial screw group ( P>0.05). Conclusion:For Gehweiler type IIIb atlas fracture, both techniques can attain atlas fracture union and atlantoaxial posterior arch bone fusion, but the posterior atlas uniaxial screw instrumentation and fusion is superior in reduction of atlas fracture displacement and lateral mass separation, neck pain relief and functional improvement.

Objective:To investigate the effects of different surgical time points on the treatment efficacy of acute traumatic central cord syndrome (ATCCS).Methods:Retrospectively analyzed were the 84 ATCCS patients who had been treated at Department of Spinal Surgery, Henan Provincial People's Hospital from January 2013 to February 2021. According to the surgical timing, the patients were divided into 3 groups. In group A (surgery < 48 hours) of 16 cases, there were 11 males and 5 females, aged from 43 to 76 years; in group B (surgery within 3 to 7 days) of 41 cases, there were 31 males and 10 females, aged from 41 to 78 years; in group C (surgery within 8 to 14 days) of 27 cases, there were 15 males and 12 females, aged from 46 to 83 years. Anterior, posterior or combined anterior and posterior approaches were used according to their specific condition. The American Spinal Injury Association (ASIA) motor scores and Japanese Orthopaedic Association (JOA) scores at admission, 7 days and 12 months after operation, postoperative ICU duration, and complications were compared among the 3 groups.Results:There were no significant differences in the preoperative general information or surgical approaches among the 3 groups, showing they were comparable ( P> 0.05). In all patients, the ASIA motor scores and JOA scores at 7 days and 12 months after operation were significantly better than those at admission, and the ASIA motor scores and JOA scores at 12 months after operation were significantly better than those at 7 days after operation ( P<0.01). There was no significant difference in the ASIA motor score or JOA score between the 3 groups at 7 days or 12 months after operation ( P>0.05). The postoperative ICU duration in group A was 42 (26, 61) h, significantly longer than 23 (16, 35) h in group B and 24 (14, 38) h in group C ( P<0.05). There were no deaths in the 84 patients; there was no significant difference in the overall incidence of serious complications or in that of general complications among the 3 groups ( P>0.05). Conclusions:Surgery is safe and effective for ATCCS. However, decompression surgery within 2 weeks may achieve better outcomes.

Objective:To investigate the clinical efficacy of medial "in-out-in" axial pedicle screw in the treatment of atlantoaxial dislocation or instability during upper cervical spine surgery.Methods:Thirty-one patients with atlantoaxial dislocation or instability, admitted to our hospital from January 2017 to January 2020, were chosen in our study; 17 patients were with unilateral stenosis at the pedicle of vertebral arch, including 15 with dominant vertebral artery and 2 with unilateral vertebral artery, and medial "in-out-in" axis pedicle screw was placed on this side and conventionally axis pedicle screw was implanted on the other side; 14 patients were with bilateral stenosis at the pedicle of vertebral arch, including 13 with dominant vertebral artery and one with unilateral vertebral artery, and the medial "in-out-in" axial pedicle screw weas placed on the side of the dominant or unilateral vertebral artery and the medial or lateral "in-out-in" axial pedicle screw was inserted on the other side. X-ray, CT, and MRI were performed before, and 5 d and 3, 6, and 12 months after surgery to observe the fusion of bone grafts. Scores of visual analogue scale (VAS) and Japanese Orthopedic Association (JOA) were compared before surgery, and 7 d, and 3 and 6 months after surgery, and during the last follow-up to evaluate the clinical efficacy of these patients.Results:The surgical time was (164.2±28.3) min (136-224 min); the intraoperative blood loss was (283.6±74.5) mL (180-560 mL), and there was no spinal cord vascular injury or other serious complications. Two patients had cerebrospinal fluid leakage after surgery, the drainage tube was pulled out after a delay of 8 d, and the wounds healed at one stage. The distal end of the screw did not enter the vertebral body of one patient during the surgery, and the screw was inserted again after the distal end was tapped to enter the vertebral body. Follow up for 9-25 months was performed in all patients, with an average of 13 months. The imaging examination showed no loosening of internal fixation, fracture, or fusion of bone grafts at 6 months after surgery. Seven d, and 3 and 6 months after surgery and during the last follow-up, the VAS scores were 1.56±0.98, 1.13±1.01, 1.11±0.86 and 1.09±0.91, respectively, which were significantly lower than those before surgery (3.52±1.97, P<0.05); the JOA scores were 11.8±2.1, 12.3±1.9, 12.5±2.2, and 12.6±1.8, respectively, which were significantly improved as compared with those before surgery (8.2±1.7, P<0.05). Conclusion:The use of medial "in-out-in" axial pedicle screw posterior fusion and internal fixation has a positive clinical effect in treatment of atlantoaxial dislocation or instability during upper cervical spine surgery.

Objective:To evaluate the clinical efficacy of cervical anterior approach atlantodentoplasty for the treatment of irreducible atlantoaxial dislocation complicated with bony abnormality of atlanto-dental joint.Methods:Retrospective analysis was conducted to study the clinical data of 31 patients with irreducible atlantoaxial dislocation complicated with bony abnormality of atlanto-dental joint, including 7 males and 24 females; age ranged from 23 to 74 years, with an average of 49.0±12.0 years. All patients underwent cervical anterior approach soft tissue release, atlantodentoplasty and one-stage posterior occipito-cervical fixation and fusion. Twenty-one patients with atlantodental osteoarthritis underwent simplex atlantodental osteophyte resection, 5 patients with anterior tubercle hypertrophy of atlas and 5 patients with anterior tubercle hypertrophy of atlas and atlantodental osteoarthritis underwent atlantodental osteophyte resection and partial anterior tubercle resection. The operation time and blood loss of anterior procedure and total procedure were recorded. The anterior tubercle thickness (ATT), the atlantodental interval (ADI)were recorded before and 1 week after the operation. The available space of the cord (SAC), clivus-canal angle (CCA), cervicomedullaryangle (CMA), and the Japanese Orthopaedic Association (JOA) scores were recorded before the operation, 1 week, 3 months and 12 months after the operation, and at the last follow-up. The JOA improvement rate at the last follow-up was calculated, the time of postoperative bone graft fusion was recorded, and the complications were observed.Results:All patients were followed up for 12-60 months, with an average of 34.5±13.8 months. The operation time of anterior cervical atlantodentoplasty was 120.9±15.9 min, and the overall operation time was 315.1±31.4 min; The blood loss of anterior procedure was 101.2±31.2 ml, and that of overall procedure was 372.7±56.0 ml. The one week postoperative ATT (7.4±1.6 mm) of patients with anterior tubercle partial resection of atlas was lower than that before operation 10.8±1.5 mm ( t=4.94, P=0.001). The one week postoperative ADI 0.9±1.2 mm decreased compared with the preoperative ADI 8.3±2.2 mm ( t=17.91, P<0.001). The preoperative SAC was 10.4±2.8 mm, which increased to 19.2±3.6 mm one week after operation and 19.4±3.7 mm ( F=41.31, P<0.001) at last follow-up. The preoperative CCA was 119.4°±17.9°, which increased to 142.6°±13.0° one week after operation and 141.6°±12.2° ( F=35.86, P<0.001) at last follow-up. The preoperative CMA was 121.7°±14.1°, which increased to 148.9°±9.4° one week after operation and 149.4°±9.0° ( F=52.07, P<0.001) at last follow-up. The preoperative JOA score was 12.0±2.6, which was 14.3±1.3 one week after operation and 15.9±1.0 ( F=23.81, P<0.001) at last follow-up. JOA improvement rate was 78.9%±17.1%, while 23 cases were excellent (74.2%), 8 cases were good (25.8%), and the excellent and good rate was 100%; Thd fusion time of grafted bone was 5.7±1.5 months with the fusion rate of 100%; There were 12 patients with dysphagia after operation, all of which relieved spontaneously 5-10 days after operation; There were 3 cases of irritating choking after drinking or eating, and 2 cases were gradually alleviated 3-5 days after operation. One case was complicated with aspiration pneumonia due to stubborn choking, which gradually alleviated after 1 month of nasal feeding. No hardware failure or reduction loss, no serious complications such as esophageal injury, cerebrospinal fluid leakage, incision infection or vertebral artery injury occurred. Conclusion:Cervical anterior approach atlantodentoplasty for the treatment of irreducible atlantoaxial dislocation complicated with bony abnormality of atlanto-dental joint can anatomically reduce the atlantoaxial joint, and the clinical effect is satisfactory.

Objective:To evaluate the axial instrument strategy for atlantoaxial dislocation with complex vertebral artery variation.Methods:A total of 55 patients with atlantoaxial dislocation who underwent surgical treatment from January 2019 to December 2021 were retrospectively analyzed, including 14 males and 41 females, aged 54.0±12.8 years (range, 22-78 years). Among these patients, 10 patients with unilateral vertebral artery high ride with contralateral vertebral artery occlusion, 30 patients with bilateral vertebral artery high ride with single dominant vertebral artery, 15 patients with bilateral vertebral artery high ride. All patients underwent posterior reduction and internal fixation. Visual analogue scale (VAS) and Japanese Orthopaedic Association (JOA) score were used to evaluate the postoperative efficacy.Results:All patients completed the surgery successfully with a follow-up time of 14.6±5.5 months (range, 6-24 months). C 2 pedicle screw fixation was performed on the non-dominant side of unilateral vertebral artery high ride and the non-dominant side of bilateral vertebral artery high ride with one dominant vertebral artery (40 vertebraes). The dominant side of unilateral high vertebral artery and bilateral high vertebral artery with one dominant vertebral artery was fixed with C 2 medial "in-out-in" screw (10 vertebraes), C 2 isthmus screw (21 vertebraes), C 2 without screw (9 vertebraes) only extended the fixed segment. For bilateral vertebral artery high ride patients, one side was used C 2 "in-out-in" pedicle screws (right 10 vertebraes, left 5 vertebraes), and the other side was fixed with C 2 medial "in-out-in" screw (8 vertebraes), C 2 isthmus screw (5 vertebraes), C 2 without screw only extended the fixed segment (2 vertebraes). The JOA scores were 8.5±1.8, 13.9±1.3, and 14.4±1.1 preoperatively, 6 months postoperatively, and at the final follow-up, respectively, with statistically significant differences ( F=279.40, P<0.001). JOA at 6 months postoperatively and at the final follow-up was greater than preoperatively, and the differences were statistically significant ( P<0.05), whereas the differences in JOA scores at 6 months postoperatively and at the final follow-up was not statistically significant ( P>0.05). Preoperative, 6 months postoperatively and final follow-up cervical VAS scores were 3.7±1.9, 2.1±0.9 and 1.6±1.0, respectively, with statistically significant differences ( F=39.53, P<0.001). The cervical VAS at 6 months postoperatively and at the last follow-up was less than that before surgery, and the differences were statistically significant ( P<0.05). Cervical VAS scores at 6 months postoperatively were greater than at the last follow-up, with a statistically significant difference ( P<0.05). Conclusion:For patients with atlantoaxial dislocation with complex vertebral artery variation, C 2 lateral "in-out-in" screw, C 2 medial "in-out-in" screw, isthmus screw fixation or C 2 without screw only extended the fixed segment can obtain good clinical efficacy.

OBJECTIVE: To explore the coagulation deficit and genetic basis for a Chinese pedigree affected with Congenital dysfibrinogenemia (CD). METHODS: Peripheral venous blood samples of the proband and her family members (including 4 individuals from three generations) were subjected to routine blood test and assays of liver and kidney functions and viral hepatitis to exclude related diseases. Clauss method and DFg-PT method were used to determine the fibrinogen activity (Fg:C), and an immunoturbidimetric assay was used to determine the level of fibrinogen antigen (Fg:Ag). All of the exons (22 in total) and their flanking sequences of the FGA, FGB and FGG genes were amplified by PCR and directly sequenced. Variants in the coding regions of the three genes and transcriptional splicing sites were screened by using Mutation Surveyor^TM software. RESULTS: The Clauss method showed that Fg:C was significantly reduced in the proband and her father, whilst her mother and son were normal. With the DFg-PT method, the proband, her parents and son were all within the normal range. The Fg:C/Fg:Ag ratio of the proband and her father was lower than 0.7, whilst her mother and son were above 0.7. No significant change in the prothrombin time, activated partial thromboplastin clotting time and thrombin time was noted. Two genetic variants were detected, which included a homozygous missense variant in the FGA gene [c.991A>G (p.Thr331Ala)], which was predicted to be benign, and a heterozygous missense variant of the γ chain of the FGG gene [c.1211C>G (p.Ser404Phe)], which is located in a conserved region and unreported in the CLINVAR/HGMD/EXAC/1000G databases and literature. CONCLUSION This pedigree has conformed to the autosomal dominant inheritance of CD. The c.1211C>T (p.Ser404Phe) missense variant of the γ chain of the FGG gene probably underlay the pathogenesis of CD in this pedigree. The variant was unreported previously and named as "Fibrinogen Harbin II Ser404Phe".
Female ; Humans ; Afibrinogenemia/congenital* ; East Asian People ; Fibrinogen/genetics* ; Mothers ; Mutation ; Pedigree

Adult ; Aged ; Arsenicals ; pharmacology ; therapeutic use ; Bone Marrow Cells ; drug effects ; metabolism ; DNA Methylation ; drug effects ; Female ; Humans ; Inhibitor of Differentiation Proteins ; metabolism ; Male ; Middle Aged ; Myelodysplastic Syndromes ; metabolism ; therapy ; Oxides ; pharmacology ; therapeutic use ; Treatment Outcome ; Young Adult