Presentation
A known patient with cutaneous cafe au lait patches underwent thoracic spine surgery for removal of the huge left paraspinal mass.
Patient Data
Severe spinal deformity as kyphoscoliosis of the thoracolumbar spine
Opacity within the heart shadow due to posterior thoracic mass.
Multiple bilateral neurofibromas in the thoracic spinal canal, in the neural foramina and paravertebral regions dominantly on the left side
Numerous similar small intramuscular and subcutaneous plexiform neurofibroma on the left side
Most of the lesions show hypointense signal on T1, high signals on T2 with central hypointense central foci giving target appearance.
Surgical resection of most parts of the left thoracic paraspinal and intramuscular mass
Fluid equivalent signal rim enhancing area along with left posterior thoracic region at the site of surgery
Left-sided pleural effusion.
Pathology proved the case of plexiform neurofibroma associated with neurofibromatosis type I.
Case Discussion
A case of neurofibromatosis type I with plexiform neurofibromas of the spine, kyphoscoliosis, and cafe au lait spots.