Severe Scoliosis Caused by Neurofibromatosis: Treatment Options and Risks

Multiple neurofibromas causing severe scoliosis of the spine?

Hello, my child is currently in the first year of junior high school and has had café-au-lait spots since birth.
Recently, we discovered a severe scoliosis of 45 degrees.
An MRI revealed that the cervical vertebra 7 and the area near the thoracic vertebrae have bone erosion due to the expansion of the spinal nerve sheath, resulting in a beak-shaped deformity, and surgery is required.
I would like to know if physical therapy is ineffective? What are the risks of surgery? If the scoliosis is caused by a neurofibroma, is it likely to recur? If surgery is unavoidable, where can we find an orthopedic surgeon with extensive experience in neurofibromatosis? Thank you...
Mr.
Kuo from Kaohsiung.

Guo Xiansheng, 40~49 year old female. Ask Date: 2015/02/09

Dr. Shi Guozheng reply Orthopedics

You can inquire at the pediatric orthopedic department of a medical center to see if National Taiwan University Hospital has experience in this area.

Reply Date: 2015/02/09

More Info

Hello Mr. Kuo,
Thank you for reaching out with your concerns regarding your child's severe scoliosis caused by neurofibromatosis. This is indeed a complex situation, and I appreciate your desire to understand the treatment options and associated risks.

Neurofibromatosis (NF) is a genetic disorder that can lead to the development of tumors on nerves, which can cause various complications, including scoliosis. In your child's case, the presence of café-au-lait spots and the MRI findings indicating nerve root involvement suggest that the scoliosis may be secondary to the neurofibromatosis, particularly if there is a neurofibroma affecting the spinal column.

Treatment Options
1. Observation: If the scoliosis is mild and not progressing, doctors may recommend regular monitoring. This is often the case in early stages or when the curve is less than 20 degrees.

2. Physical Therapy: While you mentioned that physical therapy has been ineffective, it is still a common first-line treatment for managing scoliosis. It can help improve posture, strengthen back muscles, and potentially slow the progression of the curve. However, it may not correct the underlying structural issues caused by neurofibromatosis.

3. Bracing: For moderate scoliosis (typically between 20-40 degrees), a brace may be recommended to prevent further curvature, especially in growing children. However, this is less effective in cases where the curvature is due to structural changes from neurofibromatosis.

4. Surgery: Given that your child's scoliosis is severe (45 degrees) and associated with structural changes in the vertebrae, surgical intervention may be necessary. The surgery typically involves spinal fusion, where rods and screws are used to stabilize the spine and correct the curvature.

Risks of Surgery
As with any surgical procedure, there are inherent risks involved, including:
- Infection: There is a risk of infection at the surgical site.

- Bleeding: Significant blood loss can occur during surgery.

- Nerve Damage: Given the proximity of the surgery to the spinal cord and nerves, there is a risk of nerve damage, which could lead to neurological deficits.

- Anesthesia Risks: General anesthesia carries its own set of risks, particularly in children.

- Scoliosis Recurrence: While surgery can correct the curvature, there is a possibility that scoliosis could recur, especially if the underlying neurofibromatosis continues to progress.

Recurrence and Follow-Up
If the scoliosis is indeed caused by neurofibromatosis, there is a chance that new tumors could develop, leading to further spinal deformities. Regular follow-ups with a specialist in neurofibromatosis and scoliosis are essential to monitor for any changes.

Finding a Specialist
For your child's condition, it is crucial to consult with an orthopedic surgeon who has experience in treating scoliosis related to neurofibromatosis. You may want to look for a pediatric orthopedic specialist at a major medical center or children's hospital, as they often have the most experience with complex cases. Institutions that have a dedicated neurofibromatosis clinic may also provide a multidisciplinary approach, involving neurologists, geneticists, and orthopedic surgeons.

In conclusion, while surgery may seem daunting, it can significantly improve your child's quality of life and prevent further complications from severe scoliosis. I recommend discussing all your concerns with your child's healthcare team to make an informed decision that aligns with your child's needs and circumstances.

Best wishes for your child's health and recovery. Please feel free to reach out if you have any further questions or need additional information.

Sincerely,
Doctor Q&A Teams

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