PMC10077782_01
Patient unable to sit without support. Notice the locked posture while sitting in (a), and dislocation in the bilateral foot (b), knees (c), elbow(d), and wrist (e)
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F1
A 17-year-old male with Larsen syndrome suffered from myelopathy due to cervical kyphosis and neuromuscular scoliosis (NMS) that made him unable to sit erect without support [Figure 1a]. On examination, he was conscious with average intelligence, carrying typical syndromic facies with congenital dislocation of multiple limb joints [Figure 1b-e], including the bilateral knee and elbow that made him dependent on others (his mother) for all his daily activities.
X-ray confirms a long C-type scoliosis (a), with multiple joint dislocations (b-d); the severe cervical kyphosis and cord compression can be appreciated in this T-2 weighted sagittal MRI (e)
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F2
The radiograph of the patient's spine and deformed joints is shown in Figure 2a-d. The spine's magnetic resonance imaging (MRI) revealed cervical and thoracic kyphoscoliosis [Figure 2e] with severe spinal canal stenosis (AP diameter was 2 mm at C4-C5).
Sagittal CT following first surgery showing corpectomy with cage and plate (a); the final postoperative radiograph is shown in AP view (b), Lateral view (c)
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F3
Eventually, the patient underwent second and third corrective spine surgery [Figure 3a-c] after 3 and 4 months, respectively, and recovered well without any anesthetic and surgical complications, but unfortunately, he succumbed to COVID because of the severe respiratory compromise.