Blog Topic: Spinal Deformity in Neurofibromatosis Type 1

Surgical Case

7-12-2022

The case presented is a 13 year old male with NF-1 who has a severe, progressive, painful kyphoscoliosis.

There is some inherent spinal flexibility as the thoracic kyphosis of 91 degrees improves when he lays on his back and hyperextends. 

The below selected MRI cuts demonstrates he does not have significant dural ectasias which could complicated surgery.  The axial MRI cut shows the spinal cord very eccentric in the canal, resting against the pedicle.  The spinal cord is slightly out-of-round, which elevates the risk of neurologic issues during surgery.

The below coronal CT scan cuts nicely shows the apex of the scoliosis having very abnormal vertebra.  Instead of being rectangular they are trapezoidal or triangular, which makes the scoliosis have a very tight turn.

The below axial CT scan cuts demonstrates the very abnormal pedicles.  Several of these pedicles (R T7, R T8 and R T9) are very difficult to place straight pedicle screws.  The reason these can be cannulated safely is due to the bone being malleable or bendable, and the pedicles can be bent straight (within reason).

The patients underwent 4 weeks of in-patient halo-gravity traction, with a maximum traction weight of 28 lbs. Despite the spine improving above and below the apex of the scoliosis, and the kyphosis improving, there still was a stiff apex.

Surgical treatment was a T3-L3 posterior spinal fusion and a T10 vertebral column resection, which means the entire T10 vertebra was removed.  This technique disconnects the spine and dramatically increases the flexibility of the spinal deformity.  After the spine was straightened a metal cage was placed in the front to help attain and maintain correction.

The patient is one year out from surgery and his doing well without pain.