Osteochondritis dissecans and Osgood Schlatter disease in a family with Stickler syndrome

<p>Abstract</p> <p>Purpose</p> <p>Stickler syndrome is among the most common autosomal dominant connective tissue disorders but is often unrecognised and therefore not diagnosed by clinicians. Despite much speculation, the cause of osteochondrosis in general and osteochondritis dissecans (OCD) and Osgood Schlatter syndrome (OSS) in particular remain unclear. Etiological understanding is essential. We describe a pair of family subjects presented with OCD and OSS as a symptom complex rather than a diagnosis.</p> <p>Methods</p> <p>Detailed clinical and radiographic examinations were undertaken with emphasis on the role of MRI imaging. Magnetic resonance imaging may allow early prediction of articular lesion healing potential in patients with Stickler syndrome.</p> <p>Results</p> <p>The phenotype of Stickler syndrome can be diverse and therefore misleading. The expectation that the full clinical criteria of any given genetic disorder such as Stickler syndrome will always be present can easily lead to an underestimation of these serious inheritable disorders. We report here two family subjects, a male proband and his aunt (paternal sister), both presented with the major features of Stickler syndrome. Tall stature with marfanoid habitus, astigmatism/congenital vitreous abnormality and submucus cleft palate/cleft uvula, and enlarged painful joints with early onset osteoarthritis. Osteochondritis dissecans (OCD) and Osgood Schlatter syndrome (OSS) were the predominating joint abnormalities.</p> <p>Conclusion</p> <p>We observed that the nature of the articular and physeal abnormalities was consistent with a localised manifestation of a more generalised epiphyseal dysplasia affecting the weight-bearing joints. In these two patients, OCD and OSS appeared to be the predominant pathologic musculoskeletal consequences of an underlying Stickler's syndrome. It is empirical to consider generalised epiphyseal dysplasia as a major underlying causation that might drastically affect the weight-bearing joints.</p

Degenerative spondylolisthesis does not influence surgical results of laminoplasty in elderly cervical spondylotic myelopathy patients

The objective of the study was to investigate the comorbidity of degenerative spondylolisthesis (DS), in elderly cervical spondylotic myelopathy (CSM) patients in our hospital, and the correlation between surgical results and preoperative DS. There are few studies on the outcome of laminoplasty for CSM with DS. A total of 49 elderly patients (>65 years old) who eventually had surgical treatment for CSM were evaluated. A slippage displacement of more than 2.5 mm at least at one level was classified to have a positive DS on flexion/extension radiographs (DS group). A slippage displacement less than 1.0 mm was considered a negative DS (non-DS group). Seventeen patients who had slippage of 1.0–2.5 mm were excluded from the study. The DS group (n = 15) included cases with DS at preoperation, while the remaining cases (n = 17) belonged to the non-DS group. The flexion/extension radiographs of the two groups were compared for range of motion and clinical results at 3 years after the operation. Of all elderly patients, 30.6% had DS. There was no significant difference between the two groups based on the clinical results. The range of motion of all cervical spines (DS group and non-DS group) was significantly limited. However, there was no significant difference between the two groups. New postoperative DS appeared in four patients, of which two were from the DS group and two from the non-DS group. These data suggest that degenerative spondylolisthesis does not influence surgical results in elderly cervical spondylotic myelopathy patients