Purpose or Case Report: A 10-year-old female with neurofibromatosis type 1 and severe dysplastic scoliosis, presented with a two-month history of difficulty ambulating due to lower back pain. The patient did not have neurological symptoms. An MRI of the thoracolumbar spine demonstrated subluxation of the right tenth and eleventh ribs through the neural foramina, causing mass effect on the thecal sac, but at T9-T10 without abnormal cord signal or cord compression. Several clusters of neurofibromas were present along the right ribs and paravertebral soft tissues at these levels. CT evaluation was performed for greater osseous definition, which confirmed the findings. The patient underwent complex posterior spine fusion and segmental spinal instrumentation, which included T9-T10 thoracoplasty, rib resections, and multiple Ponte osteotomies.
Cases of rib head protrusion into the central canal in the setting of dystrophic scoliosis have been documented in only a limited sample of case reports. The angulated short-segment curvature in dystrophic scoliosis causes vertebral body rotation, foraminal enlargement, spindling of transverse process, and penciling of the apical ribs. These changes can alter the articulation of the rib along the spinous process. The enlarged foramina also create a space into which a rib may displace. As a result, in most reported cases, the subluxed ribs were on the convex apex of the curve in the mid-to-lower thoracic region. The risk of cord injury makes complete depiction of the anatomy crucial for surgical management.
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