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Progressive Scoliosis and Multiple Epiphyseal Dysplasia

History

The patient is a 16-year-old female with multiple epiphyseal dysplasia who was diagnosed with scoliosis at age 12. No treatment was recommended at that time. She presents with progressive back pain, left hip pain, and worsening posture.

Examination

  • Motor and sensory exams were normal.
  • She had no pathologic reflexes and no leg-leg discrepancy.
  • She has short stature and a slightly waddling gait.
  • There was an obvious left lumbar prominence on bending test.
  • No shoulder asymmetry was appreciated.

Pre-Treatment Imaging

Standing scoliosis series demonstrated a right main thoracic curve of 28-degrees that corrected to 22 on bending films, and left lumbar curve measuring 62-degrees with minimal correction (Figure 1).

  • Thoracic kyphosis: 15-degrees (Figure 2)
  • Lumbar lordosis: 44-degrees
  • Pelvic incidence: 45-degrees
  • Risser score: 5

MRI lumbar spine shows multilevel lumbar stenosis, worst at L4-L5 (Figures 3, 4).

standing anterior x-ray shows thoracic lumbar scoliosisFigure 1. Standing anterior x-ray shows a right main scoliotic curve of 28-degrees and left lumbar curve measuring 62-degrees.

standing lateral x-ray shows 15-degrees thoracic kyphosisFigure 2. Standing lateral thoracic kyphosis measures 15-degrees.

lumbar sagittal MRI demonstrates multilevel spinal stenosis, worst at L4-L5Figure 3. Lumbar sagittal MRI demonstrates multilevel spinal stenosis; worst at L4-L5.

lumbar axial MRI demonstrates spinal stenosis L4-L5Figure 4. Lumbar axial MRI demonstrates spinal stenosis at L4-L5.

Diagnosis

Lumbar scoliosis in the setting of multiple epiphyseal dysplasia.

Suggest Treatment

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Selected Treatment

Correction of the lumbar curve with L4-L5 decompression and T10-Ilium fixation.

Surgeon's Comments
The patient has progressive scoliosis, back and left hip pain. It was unclear whether the pain pattern was radicular but, given spinal stenosis on MRI, a decompression was planned.

X-rays showed a rather rigid fractional curve and this, along with the need for decompression, influenced the decision to anchor distally to the ilium.

Intraoperatively, there was arthrosis of the facet joints and the curve was not flexible. Multilevel facetectomies and Ponte osteotomies were performed to facilitate correction.

Post-Surgical Imaging

post-operative scoliosis surgery standing anterior and lateral x-raysFigures 5A, 5B. Post-operative standing anterior (5A) and lateral (5B) x-rays. Lumbar scoliotic curve reduced from 62-degrees to 23-degrees.

Outcome

The patient did well immediately post-operatively with improvement in mechanical back and hip pain. The lumbar curve was corrected from 62- to 23-degrees with improvement in truncal shift as well.

Peer Discussion

Dr. Baaj presents a very unique patient with the combination of multiple epiphyseal dysplasia, scoliosis and lumbar stenosis. The patient clearly had an operative lumbar curve measuring 62-degrees, with a compensatory thoracic curve.

The decision of whether or not to include the thoracic curve involves factors such as coronal magnitude, sagittal kyphosis and axial rotation. Since all appeared favorable, Dr. Baaj's decision not to include it in the fusion appears sound.

Although concomitant lumbar decompression with deformity correction is not common in the pediatric population, the marked stenosis mandated a combined procedure. The post-operative x-rays demonstrate excellent overall balance and curve correction.

Community Case Discussion (5 comments)

SpineUniverse invites spine professionals to share their thoughts on this case.


Interesting case .. Thanks for sharing. The decision to include the pelvis in those types of cases is often challenging.
I've done similar cases during fellowship at HSS and there were often different opinions in conference.

As the patient had no pelvic obliquity preop and although syndromic it's not a frank neuromuscular patient , I would have tried to explore the option of stopping at L4. She's young but skeletally mature. Leaving two mobile segments would likely improve her overall function through her early adult life.

Ofcourse it's debatable what to do for those patients .. Thanks again for sharing.

Excellent comment, thank you. If there was no need to perform a laminectomy/decompression at L4/5 I would've not included the sacrum in the fixation. I did not feel comfortable with leaving a laminectomized level just below a long construct.

Indeed , the decompression does add more complexity to the decision.
Thanks again for sharing.

Thanks for the nice case.
However it seems there is now a slight sagital imbalance. Scoliosis can be looked at as a way to gain additional lordosis and derotating the curve can induce a loss of this mechanism.
Any thought on that regarding this case, and ways to prevent it?

Thank you Dr. Neves for the excellent comment. You're correct in the sense that sometimes correcting in one plane can affect another as scoliosis is a three-dimensional entity. As these are early post-operative films, I typically see additional changes in alignment several months later after patients "compensate" after surgery.

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