Beyond the Curve: Intra-spinal Rib Head Herniation as a Critical Complication of Dystrophic Scoliosis in Pediatric Patients With Neurofibromatosis Type 1.

Neurofibromatosis type 1 (NF1) is frequently associated with a multitude of skeletal abnormalities including dystrophic scoliosis. A rare but severe complication of dystrophic scoliosis in NF1 is the herniation of rib heads into the spinal canal, potentially leading to devastating spinal cord compression. We present two pediatric cases of NF1-associated dystrophic scoliosis with intra-spinal herniation of rib heads. Case 1 involves a teenage male with progressive thoracolumbar scoliosis and protrusion of T10 and T11 rib heads into the spinal canal, who underwent successful posterior spinal fusion (T3-L3) with instrumentation, osteotomies, and rib head resection. Case 2 involves a teenage female with progressive thoracolumbar scoliosis and intra-spinal protrusion of T4 and T5 rib heads, who remains neurologically intact despite worsening curvature. Intra-spinal herniation of rib heads is a clinically important complication of NF1-associated dystrophic scoliosis requiring close surveillance. Familiarity with this complication is important as imaging findings may be subtle early on. Surgical management typically involves both spinal fusion and resection of rib heads to prevent neurological compromise, though timing may vary based on symptoms and progression. Multidisciplinary care is essential.