Our Summary

This study looked at 42 patients suffering from a condition called Chiari malformation type II, which is usually found in patients with a birth defect known as myelomeningocele. This condition often doesn’t show symptoms. However, when symptoms do appear, they are generally more severe in newborns.

The first line of treatment is usually to ensure fluid drains properly from the brain. If this doesn’t work, a surgery called craniovertebral decompression might be needed. The exact timing and methods of these surgeries aren’t set in stone and the long-term outcomes aren’t well recorded. This study also looked at older patients, who aren’t often included in such research.

The patients in this study ranged from one week to 44 years old and the timing of their surgery depended on how severe their condition was. All patients first tried fluid drainage from the brain. This worked for 40% of patients. The rest needed the decompression surgery.

Out of all the patients, the only early deaths occurred in newborns. However, 92% of survivors saw an improvement in their condition. After following up with patients for 2 to 20 years, 10% had died, mainly from heart or respiratory failure. Over half needed additional surgery for issues with their original treatment and 15% needed another surgery due to their symptoms returning.

This study suggests that early treatment of Chiari II malformations can lead to good results, even in newborns. However, there is still a relatively high mortality rate over the patient’s lifetime. Therefore, programs and care processes for adults with spina bifida, a birth defect often associated with this condition, are very important.

FAQs

  1. What is Chiari malformation type II and how is it usually treated?
  2. What was the age range of the patients in this study and what were their treatments?
  3. What conclusions did the study reach about the effectiveness of early treatment of Chiari II malformations?

Doctor’s Tip

A helpful tip a doctor might tell a patient about detethering surgery is to discuss the potential risks and benefits with your healthcare team. It’s important to understand that while the surgery can improve symptoms in many patients, there is still a risk of complications and the need for additional surgeries in some cases. It’s also important to follow up with your healthcare team regularly after surgery to monitor your condition and address any new symptoms that may arise.

Suitable For

Overall, patients with Chiari malformation type II, particularly those with myelomeningocele, are typically recommended detethering surgery if they do not respond to other forms of treatment such as fluid drainage from the brain. The timing of the surgery depends on the severity of the patient’s condition, with newborns often requiring earlier intervention. This study showed that the majority of patients experienced improvement in their condition following surgery, but there is still a relatively high mortality rate over the patient’s lifetime, particularly in newborns. Therefore, it is important for healthcare providers to have programs and care processes in place for both pediatric and adult patients with this condition.

Timeline

Before detethering surgery:

  • Patients may experience symptoms such as headaches, neck pain, weakness, and difficulty walking
  • Initial treatment may involve fluid drainage from the brain or craniovertebral decompression surgery
  • Timing of surgery depends on the severity of the condition
  • Patients may need additional surgeries due to ongoing symptoms or complications

After detethering surgery:

  • Patients may see improvement in their symptoms, with 92% of survivors experiencing positive outcomes
  • Some patients may require additional surgeries for issues with their original treatment
  • Long-term follow-up shows a mortality rate of 10%, mainly from heart or respiratory failure
  • Programs and care processes for adults with Chiari malformation type II are important for long-term management and outcomes

What to Ask Your Doctor

Some questions a patient should ask their doctor about detethering surgery for Chiari malformation type II include:

  1. What are the risks and potential complications associated with detethering surgery?
  2. How long is the recovery process after detethering surgery and what can I expect during this time?
  3. What are the potential long-term outcomes of detethering surgery for Chiari malformation type II?
  4. Are there any alternative treatments or therapies that I should consider before undergoing detethering surgery?
  5. What is the success rate of detethering surgery for patients with Chiari malformation type II?
  6. How will the surgery impact my overall quality of life and daily functioning?
  7. Are there any specific guidelines or precautions I should follow post-surgery to ensure the best outcome?
  8. Will I need ongoing monitoring or follow-up care after detethering surgery?
  9. How experienced is the surgical team in performing detethering surgery for Chiari malformation type II?
  10. Can you provide me with any additional resources or information to help me better understand the procedure and its potential benefits and risks?

Reference

Authors: Talamonti G, Marcati E, Mastino L, Meccariello G, Picano M, D’Aliberti G. Journal: Childs Nerv Syst. 2020 Aug;36(8):1621-1634. doi: 10.1007/s00381-020-04675-7. Epub 2020 May 30. PMID: 32474814