Our Summary

This research paper discusses neuromuscular diseases (NMDs), a group of conditions affecting the nerves and muscles in the body. Some of these conditions are particularly important to children’s orthopedic surgeons because they can cause physical signs, like walking on tiptoes, or deformities, like a high arch in the foot or a curved spine. The diseases can also lead to other issues that require orthopedic treatment.

The main NMDs that orthopedic surgeons deal with are infantile spinal muscular atrophy (a disease that affects motor neurons), peripheral neuropathies (mainly Charcot-Marie-Tooth disease), congenital muscular dystrophies, progressive muscular dystrophies, and Steinert myotonic dystrophy (or myotonic dystrophy type 1).

All of these conditions share a common symptom: muscle weakness. Therefore, a pediatric orthopedic surgeon needs to be familiar not just with the musculoskeletal system, but also with other areas like respiratory function, heart function, and nutrition. These areas can affect treatment and require careful management before surgery.

The surgeon must also understand the natural progression of each NMD to determine the best timing for treatment. This is particularly important when treating spinal deformities caused by muscle weakness during growth. Depending on the disease, treatment might involve non-surgical methods or the use of growing rods, followed by spinal fusion.

Due to the complexity of treating NMDs, a team-based approach is necessary. As survival rates for these diseases have improved, more attention also needs to be given to preparing patients for adult life. This includes treating orthopedic issues before patients leave the pediatric environment and helping them transition to adult healthcare.

FAQs

  1. What are the main neuromuscular diseases (NMDs) that pediatric orthopedic surgeons deal with?
  2. How does a pediatric orthopedic surgeon determine the best timing for treatment of NMDs?
  3. Why is a team-based approach necessary for treating neuromuscular diseases in children?

Doctor’s Tip

A helpful tip a doctor might tell a patient about pediatric scoliosis surgery is to ensure they follow all pre-operative instructions provided by their healthcare team. This may include maintaining a healthy diet, staying physically active, and avoiding certain medications. Additionally, it is important for the patient to communicate openly with their healthcare team about any concerns or questions they may have before and after the surgery. Following a proper rehabilitation plan post-surgery is also crucial for a successful recovery.

Suitable For

In general, pediatric scoliosis surgery is typically recommended for patients with neuromuscular diseases that are causing severe spinal deformities or compromising the patient’s overall health and function. Patients with conditions such as infantile spinal muscular atrophy, Charcot-Marie-Tooth disease, congenital muscular dystrophies, progressive muscular dystrophies, and myotonic dystrophy type 1 may benefit from scoliosis surgery if their spinal curvature is progressing rapidly, causing pain, affecting lung function, or impacting their ability to perform daily activities.

It is important for pediatric orthopedic surgeons to carefully evaluate each patient’s individual case and consider factors such as the severity of the spinal deformity, the patient’s age and overall health, the progression of the neuromuscular disease, and the potential risks and benefits of surgery. A multidisciplinary team approach, involving specialists in orthopedic surgery, neurology, physical therapy, and other related fields, can help ensure that each patient receives comprehensive and personalized care.

Ultimately, the decision to recommend pediatric scoliosis surgery for patients with neuromuscular diseases should be made on a case-by-case basis, taking into account the unique circumstances and needs of each individual patient.

Timeline

Before pediatric scoliosis surgery:

  • Patient may experience symptoms such as muscle weakness, walking on tiptoes, or deformities like a curved spine
  • Orthopedic surgeon assesses the patient’s condition and determines the best course of treatment
  • Non-surgical methods or the use of growing rods may be considered before surgery
  • Patient undergoes pre-operative evaluations, including assessments of respiratory and cardiovascular function, as well as nutritional status

After pediatric scoliosis surgery:

  • Patient undergoes surgery to correct the spinal deformity caused by the neuromuscular disease
  • Post-operative care includes pain management, physical therapy, and monitoring for complications
  • Patient may require a period of rehabilitation and recovery before returning to normal activities
  • Long-term follow-up is necessary to monitor the patient’s progress and address any potential complications or issues related to the NMD

Overall, the timeline for a patient with pediatric scoliosis surgery involves a thorough assessment and preparation before surgery, followed by post-operative care and long-term management to ensure the best possible outcomes for the patient.

What to Ask Your Doctor

Questions a patient should ask their doctor about pediatric scoliosis surgery related to neuromuscular diseases may include:

  1. Can you explain how my child’s specific neuromuscular disease is affecting their spine and the potential risks of not treating the scoliosis surgically?
  2. What are the different surgical options available for treating pediatric scoliosis in children with neuromuscular diseases, and which would be most suitable for my child?
  3. How will the surgery impact my child’s overall health and their ability to manage their neuromuscular condition?
  4. What are the potential complications or risks associated with scoliosis surgery in children with neuromuscular diseases, and how will they be managed?
  5. How long will the recovery process be for my child, and what kind of physical therapy or rehabilitation will they need post-surgery?
  6. Will my child need ongoing orthopedic care or additional surgeries in the future due to their neuromuscular condition?
  7. How will the surgery and subsequent treatment plan be coordinated with other healthcare providers managing my child’s neuromuscular disease?
  8. How will my child’s transition from pediatric to adult healthcare be managed, and what support will be provided during this transition period?
  9. Can you provide information about the long-term outcomes and potential quality of life improvements for children with neuromuscular diseases who undergo scoliosis surgery?
  10. Are there any specific lifestyle changes or precautions my child will need to take following the surgery to ensure the best possible outcome for their neuromuscular condition?

Reference

Authors: Mary P, Servais L, Vialle R. Journal: Orthop Traumatol Surg Res. 2018 Feb;104(1S):S89-S95. doi: 10.1016/j.otsr.2017.04.019. Epub 2017 Nov 28. PMID: 29196274