Surgical management of congenital thoracic kyphosis and multilevel bilateral thoracic pedicle aplasia: case report

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Pedicle aplasia is an uncommon congenital anomaly most frequently involving the absence of a single pedicle at a single vertebral level. Bilateral pedicle aplasia at multiple levels is exceedingly rare and has only been described once previously in the literature. While single-level pedicle aplasia is often asymptomatic and discovered incidentally, pedicle aplasia of multiple levels may produce severe spinal deformities and neurological deficits. Due to the rarity of this condition, optimal management remains uncertain. In this case report, the authors describe the surgical management of a healthy 9-year-old boy who presented with frequent falls, difficulty running, and severe thoracic kyphotic deformity and was found to have bilateral pedicle aplasia from T3 to T9. A review of the literature regarding pedicle aplasia is also presented.

ABBREVIATIONS VCR = vertebral column resection.

Article Information

Correspondence Darryl Lau: University of California, San Francisco, CA. darryl.lau@ucsf.edu.

INCLUDE WHEN CITING Published online October 5, 2018; DOI: 10.3171/2018.7.PEDS18222.

D.L. and C.L.D.O. contributed equally to this work.

Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

© AANS, except where prohibited by US copyright law.

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Figures

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    Preoperative radiographs demonstrating severe fixed focal kyphosis and absent pedicles at multiple levels. Left: Anteroposterior view demonstrating normal coronal plane alignment. Right: On the lateral view, pedicles cannot be visualized from T3 to T9. The patient had regional T1–12 kyphosis of 39°, segmental T3–9 kyphosis of 58°, and focal T6–7 kyphosis of 81°.

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    Preoperative CT scans demonstrating multilevel bilateral pedicle aplasia. Sagittal images (left) demonstrating severe focal kyphosis at T6 and T7 with narrowing of the canal from T5 to T8. Axial images (right) at the levels of T3 (A), T7 (B), and T8 (C), demonstrating absence of pedicles.

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    Preoperative images demonstrating bilateral, multilevel pedicle aplasia with severe kyphosis and draping of cord across kyphotic apex. A: Sagittal T2-weighted MR image demonstrating severe kyphotic deformity with severe canal stenosis resulting in spinal cord compression and spinal cord flattening over the kyphotic apex of T6–7. B–D: Sagittal CT images demonstrating absence of pedicles from T3 to T9.

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    Radiographs obtained immediately postoperatively, demonstrating distal junctional failure and residual kyphosis. Left: Anteroposterior view demonstrating normal coronal balance. Right: Lateral view demonstrating ongoing kyphosis from a lever arm effect.

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    Follow-up imaging after revision and extension of the distal construct, demonstrating extension of the instrumentation distally to T11. Left: Lateral view demonstrating significant improvement in thoracic kyphosis. Regional T1–12 kyphosis was 31°, T3–9 segmental kyphosis was 23°, and focal kyphosis (inferior T5 and superior T8 [in between cage]) was 7°. Right: Anteroposterior view demonstrating good coronal plane balance with intact hardware.

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