Cauda equina syndrome in ankylosing spondylitis (the CES-AS syndrome): meta-analysis of outcomes after medical and surgical treatments.

The cauda equina syndrome in ankylosing spondylitis (the CES-AS syndrome) is marked by slow, insidious progression and a high incidence of dural ectasia in the lumbosacral spine. A high index of suspicion for this problem must be maintained when evaluating the patient with ankylosing spondylitis with a history of incontinence and neurologic deficit on examination. There has been disagreement in the literature as to whether surgical treatment is warranted for this condition. A meta-analysis was thus performed comparing outcomes with treatment regimens. Our results suggest that leaving these patients untreated or treating with steroids alone is inappropriate. Nonsteroidal antiinflammatory drugs may improve back pain but do not improve neurologic deficit. Surgical treatment of the dural ectasia, either by lumboperitoneal shunting or laminectomy, may improve neurologic dysfunction or halt the progression of neurologic deficit.

Dural ectasia and conventional radiography in the Marfan lumbosacral spine.

OBJECTIVE: To determine how well conventional radiographic findings can predict the presence of dural ectasia in Marfan patients. DESIGN AND PATIENTS: Twelve Marfan patients without dural ectasia and 21 Marfan patients with dural ectasia were included in the study. Five radiographic measurements were made of the lumbosacral spine: interpediculate distance, scalloping value, sagittal canal diameter, vertebral body width, and transverse process width. RESULTS: The following measurements were significantly larger in patients with dural ectasia: interpediculate distances at L3-L4 levels (P<0.03); scalloping values at the L1 and L5 levels (P<0.05); sagittal diameters of the vertebral canal at L5-S1 (P<0.03); transverse process to width ratios at L2 (P<0.03). Criteria were developed for diagnosis of dural ectasia in Marfan patients. These included presence of one of the following: interpediculate distance at L4 > or = 38.0 mm, sagittal diameter at S1 > or = 18.0 mm, or scalloping value at L5 > or = 5.5 mm. CONCLUSION: Dural ectasia in Marfan syndrome is commonly associated with several osseous changes that are observable on conventional radiographs of the lumbosacral spine. Conventional radiography can detect dural ectasia in patients with Marfan syndrome with a very high specificity (91.7%) but a low sensitivity (57.1%).

The lumbar interpediculate distance is widened in adults with the Marfan syndrome: data from 32 cases.

32 patients with Marfan syndrome, diagnosed with DePaepe's criteria, volunteered for this study. All patients underwent standard anteroposterior radiographs of the lumbar spine. Interpediculate distances (IPD) at each level were compared to those of previously established norms. Criteria were developed to determine the presence of Marfan using "cut-off values" for the IPDs at each lumbar level. The IPDs were significantly larger in the Marfan patients at all lumbar levels. Cut-off values were calculated setting the specificity at 95% at each lumbar level. The cut-off value at L4 yielded the greatest sensitivity. We conclude that the IPDs are widened in patients with Marfan syndrome. The IPD at L4 is a good criterion for Marfan with specificity of 95% and sensitivity of 75%. One must consider using this as a skeletal criterion or a screening tool for Marfan.

Thoracolumbar spinal abnormalities in Stickler syndrome.

STUDY DESIGN: Retrospective review of clinical and radiographic records of patients with Stickler syndrome. OBJECTIVES: To describe thoracolumbar spinal abnormalities and their correlation with age and back pain among patients with Stickler syndrome. SUMMARY OF BACKGROUND DATA: Stickler syndrome (hereditary arthro-ophthalmopathy) is an autosomal dominant connective tissue disorder characterized by skeletal, ocular, oral-facial, cardiac, and auditory manifestations. Prevalence is approximately 1 in 10,000 (similar to that of Marfan syndrome). No one has investigated spinal abnormalities in a large series of patients. METHODS: A single-center evaluation of 53 patients from 24 families with Stickler syndrome (age range, 1-70 years) in a multidisciplinary genetics clinic. Thoracolumbar radiographs were analyzed for spinal abnormalities and correlation with age and back pain. RESULTS: Thirty-four percent of patients had scoliosis, 74% endplate abnormalities, 64% Schmorl's nodes, 43% platyspondylia, and 43% Scheuermann-like kyphosis. Sixty-seven percent of patients and 85% of adults reported chronic back pain. Endplate abnormalities and Schmorl's nodes were associated with adult age; endplate abnormalities, Schmorl's nodes, and adult age were associated with back pain. Only one adult patient was free of spinal abnormalities. CONCLUSIONS: Spinal abnormalities are nearly uniformly observed in Stickler syndrome, progress with age, and are associated with back pain. Although common, scoliosis is generally self-limited (only one patient needed surgical treatment). Correct diagnosis of this syndrome facilitates early identification and management of other potentially severe systemic manifestations and genetic counseling for affected families. Moreover, recognition of Stickler syndrome allows accurate prognosis for skeletal abnormalities and anticipation of potential surgical complications.

Early failure associated with the use of Hylamer-M spacers in three primary AMK total knee arthroplasties.

We report 3 cases of early failure associated with AMK total knee arthroplasties with the use of Hylamer-M spacers. In 2 of these cases, massive osteolysis of the posterior condyles was noted; revision with the use of allograft bone to fill in the cavitary defects yielded excellent results. In all cases, large areas of pitting and delamination of the Hylamer spacers were noted. Given these cases and the previous literature on early failure of hip arthroplasties with Hylamer inserts, we recommend caution in using Hylamer for knee arthroplasty surgery.

Osseous anatomy of the lumbosacral spine in Marfan syndrome.

STUDY DESIGN: This study examines pedicle widths, laminar thicknesses, and scalloping values for lumbosacral spine elements in Marfan volunteers. Comparisons were made between these measurements and norms as well as measurements between Marfan patients with and without dural ectasia. OBJECTIVES: To determine if the lumbosacral vertebral elements are altered in the patient with Marfan syndrome. SUMMARY OF BACKGROUND DATA: Several abnormalities have been noted in Marfan lumbar spine, including pedicular attenuation and widened interpediculate distances. This may be due to abnormalities of growth or presence of dural ectasia. Given the large numbers of Marfan patients requiring spinal surgery and the high postoperative failure rate, better understanding of the bony anatomy of Marfan lumbar spine is necessary, especially if use of instrumentation is anticipated. METHODS: Thirty-two volunteers with Marfan syndrome based on the Ghent criteria underwent spiral computed tomography of the lumbosacral spine. Images were evaluated for dural ectasia, and measurements of pedicle width, laminar thickness, and vertebral scalloping were made. RESULTS: Pedicle widths and laminar thicknesses were significantly smaller in Marfan patients at all levels (P<0.001). Mean pedicle widths at L1-L3 were smaller than the smallest available pedicle screw (5 mm). In Marfan patients with dural ectasia, laminar thickness from L5-S2 and pedicle widths at all lumbar levels were significantly reduced (P<0.01). Vertebral scalloping at S1 was significantly greater in Marfan patients with dural ectasia (P = 0.02). CONCLUSION: Lumbar pedicle width and laminar thickness are significantly reduced in Marfan individuals. Those with dural ectasia demonstrate increased bony erosion of anterior and posterior elements of lumbosacral spine. Preoperative planning and routine computed tomography scans are recommended when operating on Marfan lumbosacral spine.

Dural ectasia is associated with back pain in Marfan syndrome.

STUDY DESIGN: A cross-sectional age- and sex-matched study comparing the prevalence and size of dural ectasia in two groups of patients with Marfan syndrome. Group I comprised patients with moderate to severe back pain and Group II comprised patients without back pain. OBJECTIVES: To determine whether the presence and size of dural ectasia is associated with back pain in patients with Marfan syndrome. SUMMARY OF BACKGROUND DATA: Dural ectasia is present in more than 60% of patients with Marfan syndrome. Moderate to severe back pain is present in more than 50% of patients with Marfan syndrome. Most cases of significant low back pain in patients with Marfan syndrome do not have a clear cause. It would be useful for the clinician to know whether dural ectasia may be a cause of back pain in patients with Marfan syndrome with no other source. METHODS: Thirty two volunteers aged 30-50 with Marfan syndrome were enrolled as age- and sex-matched pairs with significant back pain (Group I) and without back pain (Group II). A completed questionnaire, physical examination, and magnetic resonance image of the lumbosacral spine were obtained. Dural volume caudal to L5 was calculated from the magnetic resonance data by specially designed software. RESULTS: Dural ectasia was present in 76% of the patients in Group I, and 41% of the patients in Group II. The proportion of patients with dural ectasia was significantly higher in Group I. Furthermore, the mean dural volume was significantly higher in Group I, and a significant correlation between dural volume and Oswestry pain score was noted. CONCLUSIONS: The presence and size of dural ectasia are associated with back pain in the Marfan syndrome. However, a high prevalence of dural ectasia (41%) exists even in patients with Marfan syndrome without back pain. The mere presence of dural ectasia therefore does not necessarily mean the patient will be symptomatic even though the two are associated.

Dural ectasia in the Marfan syndrome: MR and CT findings and criteria.

PURPOSE: To create criteria for detecting dural ectasia on MR or CT images in adult Marfan patients. METHODS: Images were analyzed using a workstation. Parameters that predicted dural ectasia were included in our criteria. RESULTS: Major criteria include: (1) width of dural sac below L5 > width above L4; (2) anterior sacral meningocele. Minor criteria include: (1) L5 nerve root sleeve diameter > 6.5 mm and (2) S1 scalloping > 3.5. Dural ectasia exists if 1 major or 2 minor criteria are present. CONCLUSION: MR and CT diagnose dural ectasia with high specificity and sensitivity. Our criteria accurately diagnose dural ectasia in adult Marfan patients.