No evidence of association with HLA-B27Associated with HLA-B8 (common in patients with DISH and diabetes)
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Average 4.4 of 51 Ratings
All of the following are characteristics of juvenile ankylosing spondylitis EXCEPT?
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Juvenile Ankylosing spondylitis (AS) one of the spondyloarthropathies that is characterized by sacroiliitis, spondylitis, enthesitis, HLA B-27, kyphosis, asymmetric lower extremity inflammatory arthritis, decreased chest expansion, and uveitis, but NOT Urethritis (which is typical of the triad of Reiter syndrome).
The Azouz article reviews juvenile spondyloarthropathies, including AS, which is characterized by increased rates of enthesitis, sacroilitis, and peripheral arthropathies compared to the adult form of AS.
The Gensler article stresses the relationship of enthesitis to juvenile AS, poorer functional outcomes compared to adult AS, and reports effective treatment with tumor necrosis factor-alpha blockers.
The Kredich article stresses early diagnosis for proper treatment with a good chance to return to normal function.
Azouz EM, Duffy CM.
Skeletal Radiol. 1995 Aug;24(6):399-408. PMID: 7481895 (Link to Abstract)
Azouz, SRAD 1995
Gensler L, Davis JC Jr.
Curr Opin Rheumatol. 2006 Sep;18(5):507-11. PMID: 16896291 (Link to Abstract)
Kredich D, Patrone NA.
Clin Orthop Relat Res. 1990 Oct;(259):18-22. PMID: 2208854 (Link to Abstract)
Kredich, CORR 1990
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Average 3.0 of 34 Ratings
A patient with ankylosing spondylitis and a hip flexion contracture undergoes uneventful right total hip replacement using a Kocher (posterior) approach. This patient is at increased risk for which of the following complications post-operatively?
Posterior hip dislocation
Anterior hip dislocation
Degenerative joint disease due ankylosing spondylitis (AS) with a concomitant hip flexion contracture increases post-operative rates of anterior hip dislocations. Correction of hip flexion contracture with THA can restore sagittal balance. However, when positioning the acetabular component in a patient with AS, one must account for the relationship of the pelvis to the lumbar spine in the sagittal plane in order to avoid an excessively hyperextended hip once the patient resumes an upright position.
Tang el al followed 95 primary THA's in patients with AS for over 10 years. Their series had 3 dislocations, 2 of which were anterior. They noted that, because of the presence of relative hyperextension of the hips after THA, AS patients are more prone to anterior dislocation when acetabular components are placed in their normal position relative to the pelvis.
In a more recent study, Bhan et al retrospectively reviewed 54 patients (92 hips) who underwent cementless total hip arthroplasty for bony ankylosis in AS via a posterior surgical approach. At an average of 8.5 years follow-up, they noted that anterior dislocation occurred in 4 (4.3%) of the hips and that there were no posterior dislocations.
Tang WM, Chiu KY.
J Arthroplasty. 2000 Jan;15(1):52-8. PMID: 10654462 (Link to Abstract)
Tang, JARTHO 2000
Bhan S, Eachempati KK, Malhotra R
J Arthroplasty. 2008 Sep;23(6):859-66. PMID: 18722294 (Link to Abstract)
Bhan, JARTHO 2008
Average 2.0 of 80 Ratings
A 75-year-old man presents to your office complaining of inability to lift his head and look ahead. He states that initially he was unable to turn his head sideways, and that this progressed to his current state. A clinical photo is provided in Fig A. Radiographs of his cervical spine and lumbar spine are provided in Figure B and C. What is the most appropriate management?
Anterior osteotomy, anterior decompression and fusion
Halo traction for 6 weeks only
Posterior fusion in situ
Anterior osteotomy, posterior instrumentation
Posterior extension osteotomy, then posterior fusion and instrumentation
Figure A shows a man with "chin-on-chest" deformity caused by increasing kyphosis at the cervicothoracic junction. Figure B shows a severely kyphotic cervicothoracic junction while Figure C shows the bamboo spine deformity commonly seen in ankylosing spondylitis (AS). Although AS is generally more evenly distributed in the thoracic spine, focal deformity at the cervicothoracic junction can cause chin-on-chest deformity.
Belanger et al. retrospectively review 26 patients who underwent posterior extension osteotomy, with the average patient achieving 38 degrees of sagittal correction and 9 of 10 patients with preoperative neurological deficits achieving some degree of neurologic improvement. The authors strongly recommend rigid internal fixation to prevent catastrophic subluxation at the osteotomy site.
Simmons et al. describe the specifics of the surgical technique, recommending the extension osteotomy be performed at the C7-T1 junction due to various anatomic advantages. The vertebral vessels are anterior to the spine, the spinal canal is wider at this level, and the C8 nerve root tolerates migration better than nerve roots higher in the cervical spine.
Answer 1: Anterior osteotomy is technically difficult and does not allow the necessary access to the C7 pedicle needed to achieve adequate sagittal correction.
Answer 2: Halo traction will not correct the severe kyphotic deformity.
Answer 3: Posterior fusion in situ may prevent further deformity but will not improve the patient's functional or neurologic status.
Answer 4: Anterior osteotomy does not allow resection of the C7 pedicle which is needed to achieve the needed sagittal correction.
Belanger TA, Milam RA, Roh JS, Bohlman HH
J Bone Joint Surg Am. 2005 Aug;87(8):1732-8. PMID: 16085612 (Link to Abstract)
Belanger, JBJS 2005
Clin Orthop Relat Res. 1977 Oct;(128):65-77. PMID: 598177 (Link to Abstract)
Simmons, CORR 1977
A 61-year-old man with ankylosing spondylitis falls and hits his forehead while getting out of the shower. On arrival to the emergency room he complained of neck pain, but his neurologic exam is normal. A CT scan shows a nondisplaced extension-type fracture of the lower cervical spine and no evidence of epidural hematoma. He is treated with a cervical orthosis and admitted for pain management. Seven hours later he reports increasing paresthesias in his upper and lower extremities. Examination now shows weakness in his upper and lower extremities, including 3+/5 ankle dorsal and ankle plantar flexion. An MRI scan is performed emergently and is shown in Figure A . What is the most appropriate next step in management.
Methylprednisolone at 30 mg/kg over 1st hour followed by 5.4 mg/kg/hr drip for 23 hours
Repeat MRI in twelve hours with serial neurologic exam
Anterior cervical fusion
Posterior cervical laminectomy
Posterior cervical laminectomy and fusion with instrumentation
Patients with ankylosing spondylitis are prone to spinal fracture due to their rigid spine.
The most common fracture pattern seen are extension-type fractures of the cervicothoracic junction. These fractures can appear nondisplaced or minimally displaced initially, making them difficult to diagnose. The vertebral bony anatomy of patients with AS make them vulnerable to epidural bleeding. In this case, the MRI scan reveals an dorsal epidural hematoma that is leading to cord compression. Because the patient has a a progressive neurological deficit and radiographic evidence of compression treatment should include surgical decompression. Because the source of compression is posterior, a posterior laminectomy is treatment of choice. These fracture patterns are usually unstable so decompression should be combined with an instrumented fusion.
The Weinstein reference is a retrospective review of 105 patients with ankylosing spondylitis (AS) diagnosed over a 6-year period. They argue that in patients with cervical trauma and a progressive neurologic deficit, early diagnosis and appropriate therapy to decompress, reduce, and immobilize unstable spinal fractures may result in reduction of the mortality rate and permanent neurological deficits.
J Bone Joint Surg Am. 1979 Dec;61(8):1119-42. PMID: 511875 (Link to Abstract)
Bohlman, JBJS 1979
Weinstein PR, Karpman RR, Gall EP, Pitt M.
J Neurosurg. 1982 Nov;57(5):609-16. PMID: 7131059 (Link to Abstract)
Weinstein, JNEURS 1982
Average 3.0 of 30 Ratings
A 32-year-old man presents with low back and hip pain that has been gradually worsening over the past year. He reports the symptoms are worse in the morning. Radiographs are shown in Figure A. Laboratory studies show a positive HLA-B27. What additional finding will help confirm the diagnosis?
Positive Rheumatoid Factor
Elevated urine phosphoethanolamine
The radiograph in Figure A shows bilateral sacroiliitis. Bilateral sacroiliitis (with or without uveitis) and a postive HLA-B27 is diagnostic of ankylosing spondylitis.
Ankylosing spondylitis is characterized by a positive HLA-B27 with a negative RF titer. It typically presents in the 4th decade of life and is more common in men than women. Low back pain usually precedes the radiogaphic findings of bilateral sacroiliitis. Of note, HLA-B27 is positive in ~6% of the white population.
Rudwaleit et al, looked at variables that could help make an early diagnosis of ankylosing spondylitis. They found the highest likelihood ratio was found in patients with a positive HLA test and positive MRI findings.
Burgos-Vargas et al, studied the clinical and radiographic features of sacroiliac and spinal involvement in patients with seronegative enthesopathy and arthropathy. Based on their findings, they recommend periodical measurements of the spinal flexion and radiographs of the pelvis from age 3 in high risk children.
Answer 1: Erythema marginatum is a major criteria for Acute Rheumatic Fever.
Answer 2: HLA-DR3 is associated with SLE.
Answer 4: RF is found in rheumatoid arthritis, Sjogren's, sarcoid, and SLE.
Answer 5: Elevated urine phosphoethanolamine is found in hypophosphatasia.
Rudwaleit M, van der Heijde D, Khan MA, Braun J, Sieper J
Ann. Rheum. Dis.. 2004 May;63(5):535-43. PMID: 15082484 (Link to Abstract)
Rudwaleit, ANRD 2004
Burgos-Vargas R, Clark P.
J Rheumatol. 1989 Feb;16(2):192-7. PMID: 2526221 (Link to Abstract)
A 69-year-old man falls on the ice. On arrival to the emergency room he is found to have a 2 cm laceration on the back of his head. He complains of neck pain, but is oriented to place and time and his neurologic exam is normal. Cervical and lumbar radiographs are shown in Figures A-C. What is the next most appropriate step in treatment?
Obtain flexion-extension radiographs
Obtain a CT scan of the lumbar spine
Obtain a CT scan of the cervical spine
Obtain a technetium bone scan
Treat with soft collar and discharge patient to home
The radiographs are consistent with ankylosing spondylitis. In these patients, due to the stiffness of the spine, there is an increased risk for cervical fractures.
A cervical fracture in a patient with ankylosing spondylitis is often very difficult to see on plain radiographs. In addition, there is a high mortality rate secondary to epidural hemorrhage. Therefore, in a patient with AS and a high suspicion for a neck injury, plain radiographs should be supplemented with additional imaging studies to look for acute fracture and epidural hemorrhage.
In the references, Colterjohn and Bednar identified flexural mechanisms of injury and chronic multilevel arthritis with ankylosis as being risk factors for sustaining motor neurological deterioration after cervical trauma. In their study, routine three-view cervical radiographs were insensitive in detecting cervical injury in this patient group. The authors’ recommendations are that cervical spine should be immobilized until there is unequivocal evidence that there is no cervical spine injury based on radiographic and clinical exam. If these criteria cannot be met, the cervical spine should be immobilized until secondary imaging with a CT scan is performed to exclude cervical spine injury. The authors did not investigate the sensitivity of MRI in detecting these injuries because MRI was unavailable at the spine injury referral center.
Colterjohn NR, Bednar DA.
Spine (Phila Pa 1976). 1995 Nov 1;20(21):2293-7. PMID: 8553116 (Link to Abstract)
Colterjohn, SPINE 1995
Average 4.0 of 18 Ratings
A 45-year-old man with ankylosing spondylitis presents with fixed sagittal imbalance and difficulty with horizontal gaze. His kyphotic deformity is localized to the thoracolumbar spine. Which of the following procedures allows the most correction in the sagittal plane at a single level without having to resect the intevertebral disc?
Pedicle subtraction osteotomy (PSO)
Vertebral column resection (VCR)
Single-level opening wedge osteotomy
Multi-level opening wedge osteotomies
Pedicle subtraction osteotomy (PSO) provides greater sagittal correction than single-level opening wedge osteotomy and Smith-Petersen osteotomies, with the advantage of working at a single level and not having to resect the intevertebral disc.
Kyphotic spinal deformity is a common orthopaedic manifestation of ankylosing spondylitis. It results from multiple microfractures, and can be localized to the to the cervicothoracic region or thoracolumbar region. Goals of treatment are to restore horizontal gaze and sagittal balance. Deformities in the thoracolumbar region are best treated with a lumbar osteotomy as it allows correction without disrupting the thoracic cage (these patients often have poor pulmonary function) and without risking an iatrogenic injury to the spinal cord. Greater correction is also an advantage due to a longer lever arm from the lumbar spine to eye level.
Thomasen first described the transpedicular wedge resection osteotomy without opening the disc or discs in front. A wedge consisting of the spinous processes and laminae of L2 and the upper part of L3 and the synostosed articular processes of L2-3 with the pedicles of L2 is resected. Through the base of the resected pedicles, bone is removed from the back part of the body of L2.
Bridwell et al evaluated twenty-seven consecutive patients in whom sagittal imbalance was treated with lumbar pedicle subtraction osteotomy. They found the average increase in lordosis was 34.1 degrees.
Arun et al compared pedicle subtraction closing wedge osteotomy, polysegmental posterior lumbar wedge osteotomies, and Smith Peterson's open wedge osteotomy for correction of deformities in AS. They found better radiographic correction was noted with pedicle subtraction closing wedge osteotomy.
Enercan et al provide a review of the different types of surgical treatment that can be used in patients with rigid severe spinal deformity. They report the VCR provides the greatest amount of correction among other osteotomy types with complete resection of one or more vertebral segments with posterior elements and entire vertebral body including adjacent discs.
Illustration A depicts a Pedicle subtraction osteotomy (PSO). Illustration B depicts a Vertebral column resection (VCR).
Answer 1: PSO provides greater correction than Smith-Petersen osteotomies.
Answer #3: VCR provide the greatest amount of resection, but requires resection of the intervertebral disc.
Answer 4 & 5: PSO provide greater correction at a single level than Single-level opening wedge osteotomy and Multi-level opening wedge osteotomies.
Arun R, Dabke HV, Mehdian H
Eur Spine J. 2011 Dec;20(12):2252-60. PMID: 21800034 (Link to Abstract)
Arun, ESPNJ 2011
Clin Orthop Relat Res. 1985 Apr;(194):142-52. PMID: 3978906 (Link to Abstract)
Thomasen, CORR 1985
Enercan M, Ozturk C, Kahraman S, Sarıer M, Hamzaoglu A, Alanay A.
Eur Spine J. 2013 Mar;22 Suppl 2:S254-64. Epub 2012 May 11. PMID: 22576156 (Link to Abstract)
Enercan, ESPNJ 2013
Bridwell KH, Lewis SJ, Rinella A, Lenke LG, Baldus C, Blanke K.
J Bone Joint Surg Am. 2004 Mar;86-A Suppl 1:44-50. PMID: 14996921 (Link to Abstract)
Bridwell, JBJS 2004
Average 3.0 of 21 Ratings