Atlantoaxial instability and neurologic indicators in rheumatoid arthritis

Atlantoaxial subluxation in rheumatoid arthritis is characterized by pain, disability, and occasionally death. Surgical fusion of the offending vertebrae is the appropriate surgical remedy, but it is a procedure with a high failure and complication rate. Because cord compression and myelopathy are the main complications of this condition, it would be logical to use evidence of early neurologic deficit as an indicator for surgery. A group of 250 patients with rheumatoid arthritis were investigated to evaluate the neurologic status with respect to the degree of instability in the proximal cervical spine. Neurologic isolated signs such as hyperreflexia could not be correlated with the onset of cervical myelopathy or quadraparesis or to the radiographically measured degree of atlantoaxial instability. These signs should not be used as an indication for surgery. The positive indications are intractable pain, cervical myelopathy, and severe atlantoaxial instability.     

颈后路手术治疗类风湿性寰枢椎不稳

目的：对类风湿性寰枢椎不稳患者颈后路植骨融合、内固定手术进行探讨。方法：对21例类风湿性寰枢椎不稳患者采用颈后路植骨融合、内固定手术，其中7例可复性寰枢椎半脱位行寰枢椎间植骨钛缆或Apofix固定术；14例难以复位者行枕颈间植骨cervifix固定术。结果：随访6～28个月(平均18个月)。21例均无并发症发生，X线显示均获骨性融合，19例患者神经功能获不同程度改善，2例虽无改善但无神经损害发展。结论：颈后路植骨融合、内固定术可为类风湿性寰枢椎不稳的患者提供牢固的融合固定，且以早期手术为佳。     

Principles of surgical treatment of the cervical spine in rheumatoid arthritis

Summary About 20% of patients with rheumatoid arthritis complain about neck problems based on instability and deformity. As a consequence, pain, myelopathy, and severe neurological deficit may occur. Results reported in the literature were not encouraging as regards surgical decompression and stabilization. However, new surgical techniques allow a more aggressive strategy towards the complex problem of the instable cervical spine in rheumatoid arthritis. The most frequent instability of C1/2 can be stabilized by a posterior atlantoaxial screw fixation, a three-dimensional multidirectional construct with few complications. For the inclusion of the occiput into the fusion and the extension of the fusion down to the lower cervical spine, a titanium Y-plate is presented as a successful implant. While through a posterior approach, stability may be achieved, decompression is preferably done by anterior diskectomy or vertebrectomy. Encouraging results with a significant recovery of neurological deficits justify an early intervention in cases of instability of the cervical spine in rheumatoid arthritis.     

Transoral decompression, anterior plate fixation, and posterior wire fusion for irreducible atlantoaxial kyphosis in rheumatoid arthritis

STUDY DESIGN: Fifteen consecutive patients with irreducible atlantoaxial kyphosis caused by rheumatoid arthritis were treated by combined transoral odontoid resection, anterior plate fixation, and posterior wire fusion. OBJECTIVES: To investigate the clinical results of this new surgical procedure. SUMMARY AND BACKGROUND DATA: Irreducible atlantoaxial kyphosis in rheumatoid arthritis results from a destruction of the craniocervical joint ligaments and the anterior aspects of the lateral atlantoaxial joints. The development of a paradental synovial pannus and atlantoaxial joint impaction prevents reduction by conservative treatment, such as skull traction. Posterior surgical procedures for the treatment of the irreducible atlantoaxial kyphosis with spinal cord compression have been associated with high morbidity and mortality. METHODS: Fifteen consecutive patients were treated by transoral odontoid resection. The fixation was performed with anterior plating, according to the method of Harms in combination with posterior wire fusion according to Brooks. Before and after surgery, evaluation was performed using the parameters of pain (visual analog scale), range of motion, and subjective assessment of improvement and the Health Assessment Questionnaire. The neurologic deficit was defined according to the classifications proposed by Ranawat, Frankel, and Nurwick. Plain radiographs, including lateral flexion and extension views, and magnetic resonance scans were obtained. RESULTS: No perioperative fatality occurred. The average clinical and radiographic follow-up was 50.7 +/- 15.6 months (range, 26-77). Postoperative pain was relieved (mean pain score before surgery, 7.9 +/- 1.87; after surgery, 3.8 +/- 1.27), and the range of motion of all patients increased (mean 21.5 +/- 14.0 degrees for rotation; mean 17.2 +/- 5. 54 degrees for bending). The score on the Health Assessment Questionnaire increased in three patients, remained unchanged in three and decreased in six patients (three had died). All patients improved at least one Ranawat level after surgery, except a patient in Ranawat Class II, whose condition remained unchanged. All patients were satisfied with the procedure and reported subjective improvement. CONCLUSION: Transoral plate fixation combined with posterior wire fixation after transoral odontoid resectionis an effective, reliable, and safe procedure for the treatment of irreducible atlantoaxial kyphosis in rheumatoid arthritis.     

Rheumatoid arthritis in the cervical spine

The cervical spine often becomes involved early in the course of rheumatoid arthritis, leading to three different patterns of instability: atlantoaxial subluxation, atlantoaxial impaction, and subaxial subluxation. Although radiographic changes are common, the prevalence of neurologic injury is relatively low. The primary goal of treatment is to prevent permanent neurologic injury while avoiding potentially dangerous and unnecessary surgery. Strategies include patient education, lifestyle modification, regular radiographic follow-up, and early surgical intervention, when indicated. Magnetic resonance imaging is indicated when neurologic deficit (myelopathy) occurs or when plain radiographs show atlantoaxial subluxation with a posterior atlantodental interval < or =14 mm, any degree of atlantoaxial impaction, or subaxial stenosis with a canal diameter < or =14 mm. Surgery should be considered promptly for any of the following: progressive neurologic deficit, chronic neck pain in the setting of radiographic instability that does not respond to nonnarcotic pain medication, any degree of atlantoaxial impaction or cord stenosis, a posterior atlantodental interval < or =14 mm, atlantoaxial impaction represented by odontoid migration > or =5 mm rostral to McGregor's line, sagittal canal diameter <14 mm, or a cervicomedullary angle <135 degrees.     

Results of a longer than 10-year follow-Up of patients with rheumatoid arthritis treated by occipitocervical fusion

STUDY DESIGN: Evaluation of results a longer than 10-year follow-up of patients with upper cervical lesions due to rheumatoid arthritis who underwent occipitocervical fusion. OBJECTIVE: To determine the final outcome of patients with upper cervical lesions due to rheumatoid arthritis treated by occipitocervical fusion. SUMMARY OF BACKGROUND DATA: There are few studies reporting the final outcome of patients with rheumatoid arthritis treated by occipitocervical fusion and observed for longer than 10 years. METHODS: The subjects were 16 patients with rheumatoid arthritis with myelopathy who underwent occipitocervical fusion with a rectangular rod more than 10 years ago. All 16 patients had irreducible atlantoaxial dislocation, and 11 also had vertical dislocation of the axis. All patients had preoperative nuchal pain, and were classified into Class II (two patients), Class IIIA (nine patients), and class IIIB (five patients) according to Ranawat's preoperative neurologic classification. RESULTS: The atlas-dens interval remained the same as immediately after surgery. Vertical dislocation returned to the preoperative condition, despite successful surgical correction. Preoperative occipital pain disappeared or was reduced in all cases. Myelopathy improved in 12 of the 16 patients (75%) by more than one class in the Ranawat preoperative neurologic classification. Survival rate at 10 years after surgery was 38%; mean age at death was 70.7 years. The postoperative periods during which patients could walk by themselves ranged from 6 months to 13 years (mean, 7.5 years). CONCLUSIONS: Occipitocervical fusion for patients with rheumatoid arthritis is useful for decreasing nuchal pain, reducing myelopathy, and improving prognosis.     

Results after 24 years of prophylactic surgery for rheumatoid atlantoaxial subluxation

We evaluated the use of surgical stabilisation for atlantoaxial subluxation after a follow-up of 24 years in 50 rheumatoid patients who had some degree of pain but no major neurological deficit.The mortality of patients treated by atlantoaxial fusion was significantly lower than for those who received conservative treatment. The deaths resulted from infection or comorbid conditions. The significantly high relative risks of mortality from conservative treatment compared with surgical treatment were mutilating disease and susceptible factors on both of the HLA-DRB1 alleles. Relief from pain and neurological and functional recovery were better, and the radiological degree of atlantoaxial translocation was less in those who were surgically treated compared with those who were not. Two patients had superficial local infections after surgery. We conclude that prophylactic atlantoaxial fusion is better than conservative treatment in these patients.     

Spontaneous regression of periodontoid pannus mass in psoriatic atlantoaxial subluxation. Case report

STUDY DESIGN: A case report of a 41-year-old man with psoriasis who had cervical myelopathy caused by atlantoaxial subluxation and periodontoid pannus mass. OBJECTIVE: To describe the possible mechanism underlying the periodontoid pannus formation and the optimal treatment for such cases. SUMMARY OF BACKGROUND DATA: Atlantoaxial subluxation causing spinal cord compression at the craniocervical junction may develop in patients with rheumatoid or psoriatic arthritis. Periodontoid pannus formation plays an important role in compromising the anteroposterior diameter of the spinal canal and in causing neurologic deficits. Transoral transpharyngeal excision of the pannus is sometimes thought necessary for anterior decompression of the spinal cord. Spontaneous resolution of the periodontoid pannus after posterior atlantoaxial fusion and fixation has been documented in rheumatoid arthritis, but not in psoriatic arthritis. METHODS: The patient underwent posterior atlantoaxial fusion and Halifax fixation. RESULTS: The patient experienced clinical improvement. Regression of the periodontoid pannus mass was observed on magnetic resonance imaging. CONCLUSIONS: Posterior fusion and instrumentation resulted in spontaneous regression of the pannus mass and symptomatic relief. This report provides evidence that atlantoaxial instability may be the sine qua non for the formation of periodontoid pannus, and that amelioration of such instability leads to spontaneous resolution of the pannus mass.     

Atlantoaxial immobilization in rheumatoid arthritis: a prophylactic procedure?

Timing of surgical intervention in atlantoaxial instability due to rheumatoid arthritis is still controversial. An aim of this study was to investigate whether atlantoaxial fusion can prevent progression of instability and upward migration of the dens. Thirty-two patients with rheumatoid arthritis, who underwent posterior atlantoaxial fixation due to instability, were clinically and radiologically examined after a minimum follow-up of 5 years. The radiological measurements focussed on the extent of cranial vertical migration after atlantoaxial fusion. In none of the 20 patients available for follow-up examination was a vertical cranial migration observed, in spite of the ongoing course of the disease. These findings are in concordance with findings in the literature, and strongly suggest that, with atlantoaxial stabilization, the inflammatory process with destruction of the lateral masses of the atlas is able to prevent further deterioration with vertical cranial ¶migration.     

Upper cervical spine surgery in rheumatoid arthritis: retrospective study of 30 patients followed for two years or more after Cotrel-Dubousset instrumentation

PURPOSE: To evaluate the efficacy of upper cervical spine surgery in symptomatic atlantoaxial instability due to rheumatoid arthritis (RA). MATERIAL AND METHODS: Thirty RA patients (29 women and one man) with a mean age of 56 years were studied retrospectively. Symptomatic forward slippage of the atlas on the axis with a synovial pannus surrounding the odontoid and magnetic resonance imaging evidence of spinal cord compression was present in all 30 patients; 18 patients had vertical translocation of the odontoid and 14 had basilar invagination. Surgery, performed between 1991 and 1997, consisted of occipitocervical fusion in 18 patients and atlantoaxial fusion in 12. Cotrel-Dubousset instrumentation was performed in all 30 patients. RESULTS: Mean follow-up was four and a half years. All patients were satisfied with the procedure and exhibited marked functional gains and objective neurological improvement (by one class in the Ranawat scheme). Stable fusion was documented in all 30 patients. CONCLUSION: Cervical instrumentation and bone grafting seems to provide functional and neurological gains in carefully selected RA patients with atlantoaxial instability and spinal cord compression. Long term follow-up suggests that the benefits are sustained and that morbidity is low.     

Posterior upper cervical fusion in rheumatoid arthritis.

The purpose of this study was to determine fusion rates in patients who underwent posterior cervical fusion for instability of the upper cervical spine secondary to rheumatoid arthritis. A retrospective review of clinical and radiographic data was conducted. Nineteen patients underwent posterior cervical fusions limited to the upper cervical spine. There were 11 C1-C2 fusions and 8 occiput-C2 fusions. Instability with pain or neurologic deficits were the main indications. A uniform technique was used in all cases. Preoperative reduction in halo vest or cast was followed by a Gallie type fusion using autogenous iliac bone graft and wire, and postoperative halo vest or cast immobilization for 3 months. A fusion rate of 94% was achieved. The average follow-up was 5 years. Complete or partial relief of pain was obtained in all patients; 30% of those with preoperative deficits improved after surgery. A high fusion rate may be achieved with C1-C2 and occiput-C2 fusions in rheumatoid arthritis, with relief of pain and prevention of neurologic deterioration.     

Natural history of rheumatoid arthritis of the cervical spine

This article reviews the natural history of rheumatoid arthritis involving the cervical spine with special attention given to predictors of paralysis. Understanding the natural history of rheumatoid arthritis of the cervical spine is necessary to determine the benefit of various interventions. The primary treatment goal for cervical instability is prevention of irreversible neurologic injury. The natural history of rheumatoid arthritis for a period of 10 years or more is one of significant disease progression. The natural history of cervical instability in patients with rheumatoid arthritis is more variable, with only some patients having a neurologic deficit develop. Recent studies support prophylactic stabilization of the rheumatoid cervical spine to prevent paralysis in high risk patients. However, proponents for prophylactic arthrodesis must acknowledge that not all cervical instability in rheumatoid arthritis progresses to neurologic deficit, and surgical intervention in patients with rheumatoid arthritis incurs added morbidity and mortality. Identifying the risk factors for progression of cervical instability is the first step in eliminating morbidity and mortality from spinal cord and brain stem compression. Surgical stabilization is indicated not only for those patients with paralysis, but for the subgroups of patients with cervical rheumatoid disease who are at risk for spinal cord and brain stem compression. The posterior atlantodental interval is the most reliable screening tool and predictor of progressive neurologic deficit.     

带骨膜蒂枕骨外板翻转骨瓣在寰枢椎融合术中的应用

 目的评价带骨膜蒂枕骨外板翻转骨瓣在后路寰枢椎融合术中应用的可行性及其疗效。方法自2010年3月至2013年6月,对27例寰枢椎失稳患者在寰枢椎椎弓根钉棒系统内固定术中采用带骨膜蒂枕骨外板翻转骨瓣植骨融合,男16例,女11例;年龄23~56岁,平均45.6岁。12例新鲜齿状突骨折伴寰枢椎不稳（Anderson Ⅱ型9例、 Ⅲ型3例）,8例初次就诊时即有严重的骨折移位,4例轻度骨折移位行牵引复位、Halo-Vest支架固定后发生再移位;7例陈旧性齿突骨折伴寰椎前脱位（Anderson Ⅱ型5例、Ⅲ型2例）;先天性游离齿突7例;类风湿关节炎1例。采用疼痛视觉模拟评分（visual analogue scale,VAS）、Frankel分级评估术前与术后轴性颈痛和神经功能情况,采用正中矢状位CT观察植骨融合情况。结果所有患者均顺利完成手术,手术时间90~140 min,平均100 min。术中出血量100~600 ml,平均160 ml。术后随访18~39个月,平均32.6个月。骨性融合时间3个月19例、6个月7例、9个月1例。VAS 评分由术前（7.23±1.65）分降低到末次随访的（2.87±1.03）分,差异有统计学意义。术前9例存在脊髓神经功能损害者末次随访时7例恢复至完全正常,2例术前Frankel分级C级末次随访时恢复至D级。术中损伤椎旁静脉丛1例,使用明胶海绵及脑棉片行压迫止血。术后复查CT提示1例患者寰椎左侧椎弓根螺钉位置偏外,1例患者枢椎左侧椎弓根螺钉位置偏外,均紧邻横突孔,但此2例患者均无临床症状,未予以特殊处理。结论带骨膜枕骨外板翻转骨瓣植骨是寰枢椎固定植骨融合的有效方法之一,具有可行性及安全性。     

急性胸、腰段脊柱脊髓损伤外科手术治疗失误因素分析

目的 分析急性胸、腰段脊柱脊髓损伤手术治疗失误的主要因素。方法 通过对临床资料和Ｘ线片、ＣＴ等影像学资料的分析,回顾性研究８２例首次手术治疗失误的急性胸、腰段脊柱脊髓损伤患者的失误因素。结果 ８２例患者中,３０例脊柱骨折复位不良或脊柱畸形;３１例患者椎管残存压迫因素;１２枚椎弓根螺钉位于椎弓根以外,其中２枚穿入椎管,３枚顶压主动脉。同时,４１例患者主诉腰背疼痛,其中１６例伴有机械性不稳定,３例伴有     

胸、腰段脊柱脊髓损伤外科治疗失误的处理

目的:分析急性胸、腰段脊柱脊髓损伤外科治疗失误的主要原因,探讨处理原则。方法:对104例首次外科治疗失误的急性胸、腰段脊柱脊髓损伤患者的临床资料和影像学资料进行分析,并根据临床具体情况进行外科处理。结果:104例患者中,残存腰痛45例次,脊柱畸形30例次,脊柱不稳35例次,脊髓神经受压8例次,坐位失平衡8例次。经再次手术治疗后脊柱畸形患者明显改善;椎管残存压迫的不完全性脊髓损伤患者椎管减压术后运动指数和感觉指数均增加;30例脊柱不稳定患者重建了脊柱稳定性;45例腰痛患者中40例腰痛消失或基本消失,5例仍有轻度腰痛需应用止痛药物,但不影响轮椅活动。结论:胸、腰段脊柱脊髓损伤手术治疗失误的主要因素包括手术适应证选择不当、手术技术失误和保守治疗不当。再处理方案主要参考首次治疗主要失误因素来确定。     

Scoliosis Research Society. Multicenter spine fracture study.

This study consisted of 1,019 spinal fracture patients followed prospectively for 2 years. Sixty-four physicians from 12 countries participated. The purpose of the study was to determine: 1) the relationship between neurologic deficit and fracture type, level, and spinal canal compromise; 2) the neurologic outcome comparing surgical versus nonsurgical treatment and anterior versus posterior surgery; and 3) the relationship of pain to both kyphotic deformity and to surgical and nonsurgical treatment. The main findings of this study are as follows: 1) seat belts reduced the incidence of severe neurologic injury; 2) there was a higher incidence of neurologic deficit with fracture-dislocations and a higher incidence of neurologically intact patients with compression and flexion-distraction injuries; 3) there was a greater incidence of complete neurologic deficits caused by fractures at the spinal cord level, and a diminished incidence at the cauda equina level; 4) for burst fractures there was a weakly positive relationship between canal compromise and neurologic deficit, including bladder function; 5) surgical intervention led to a greater percentage of improved neurologic function than nonoperative treatment, but the rate of improvement was not statistically different; 6) anterior surgery was not more effective than posterior surgery in improving the neurologic function when function was assessed using the Frankel or Motor Index scales, but it was statistically significant when compared to the Manabe scale; 7) in patients who deteriorated before surgery and underwent surgery, there was a greater improvement neurologically, particularly for anterior surgery, compared to those patients treated nonoperatively or to the overall surgically treated group; 8) There was a statistically significant relationship between bladder function and fracture type, with an increased incidence of absent function seen with fracture-dislocations, of impaired function with burst fractures, and of intact bladder function with compression and flexion-distraction injuries; 9) anterior surgery was more beneficial in improving complete bladder impairment to partial impairment compared to posterior surgery; 10) a kyphotic deformity of greater than 30 degrees at 2-year follow-up was associated with an increased incidence of significant back pain; 11) patients who had surgery complained less of severe pain than those who were treated without surgery.     

Rheumatoid Arthritis of the Cervical Spine

Cervical involvement in patients with rheumatoid arthritis occurs primarily in the upper cervical spine. The characteristic deformities are atlantoaxial subluxation, vertical settling, and subaxial subluxation. The typical patient complaints are neck pain and occipital pain. Subtle signs of myelopathy may also be present. Useful radiologic studies include plain radiography, tomography, and functional magnetic resonance imaging. The most helpful radiographic measurements are the anterior atlantodens interval, the posterior atlantodens interval, and assessment of vertical settling. Atlantoaxial subluxation greater than 9 mm with vertical settling and a posterior atlantodens interval less than 14 mm correlate with neurologic deficit. Nonoperative management does not change the natural history of cervical disease. Traditional surgical indications include intractable pain and neurologic deficit. The author discusses more controversial indications and proposes a rationale and protocol for treatment. The primary surgical objectives are to achieve stabilization of the affected segments and to relieve neural compression by reduction of subluxations or direct decompression. Arthrodesis provides reliable pain relief. Neurologic recovery occurs more consistently in patients with lower grades of preoperative myelopathy.     

Atlantoaxial laxity in rheumatoid arthritis

We found that 14 of 162 rheumatoid arthritis patients with chronic occipitocervical pain had anterior atlantoaxial instability in the absence of any corresponding radiographic changes in the joint cartilage or subchondral bone. Our findings suggest that ligamentous instability is a prerequisite for this type of change. At the time of the detection of the instability, the median duration of disease was 12 (6-28) years. Rheumatoid occipitocervical pain may be initially caused by facet -joint arthritis or inflammation in the ligaments, and at a later stage also by irritation of the C2 nerve roots.     

Atlantoaxial laxity in rheumatoid arthritis

We found that 14 of 162 rheumatoid arthritis patients with chronic occipitocervical pain had anterior atlantoaxial instability in the absence of any corresponding radiographic changes in the joint cartilage or subchondral bone. Our findings suggest that ligamentous instability is a prerequisite for this type of change. At the time of the detection of the instability, the median duration of disease was 12 (6-28) years. Rheumatoid occipitocervical pain may be initially caused by facet-joint arthritis or inflammation in the ligaments, and at a later stage also by irritation of the C2 nerve roots.     

Rheumatoid arthritis of the cervical spine.

BACKGROUND CONTEXT: Rheumatoid arthritis affects over 2 million patients in the United States. It is the most common inflammatory disorder of the cervical spine. The natural history is variable. Women tend to be more commonly involved than men. Atlantoaxial instability is the most common form of cervical involvement and may occur either independently or concomitantly with cranial settling and subaxial instability. Cervical spine involvement can be seen in up to 86% of patients and neurologic involvement in up to 58%. Myelopathy is rare but when present portends a poor prognosis. What is frustrating for clinicians treating these patients is that pain cannot be equated with instability or instability with neurologic symptoms. The goal is to identify patients at risk before the development of neurologic symptoms. Both radiographic and nonradiographic risk factors play an important role in the surgical decision-making process. PURPOSE: We will describe the current concepts in rheumatoid arthritis of the cervical spine. Emphasis is placed on the natural history, anatomy, pathophysiology and decision-making process. STUDY DESIGN: A review of the current concepts of rheumatoid arthritis of the cervical spine. METHODS: MEDLINE search of all English literature published on rheumatoid arthritis of the cervical spine. RESULTS: Rheumatoid arthritis of the cervical spine was first described by Garrod in 1890. The prevalence has been estimated to be 1% to 2% of the world's adult population. Despite its prevalence, the etiology of rheumatoid arthritis remains unknown. Because of its potentially debilitating and life-threatening sequelae in advanced disease, rheumatoid arthritis in the cervical spine today remains a high priority to diagnose and treat. CONCLUSIONS: Many aspects of the natural history and pathophysiology of the rheumatoid spine remain unclear. The timing of operative intervention in patients with radiographic instability and no evidence of neurologic deficit is an area of considerable controversy. Continued surveillance into the natural history of the rheumatoid spine is required.