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Question: We have an 11 month old daughter with Crouzon syndrome. She has had 4 surgeries already (craniosynostosis repair, VP shunt for hydrocephalus, tracheostomy, and gastrostomy) and we are at an impasse as to what the next surgery should be.
We have seen 2 teams of doctors and Team A says to do a frontal orbital advancement as the next surgery. The Team B wants to do a Chiari decompression surgery and then the frontal orbital advancement. The Team B did a limited MRI and the neurosurgeon noted crowding in the area. The Team A routinely uses the frontal orbital to try and provide more room for the brain.
Currently our daughter shows no signs of a Chiari malformation, central sleep apnea or spinal cord issues. We are reluctant to put her through any unnecessary surgeries. I guess our questions would be whether or not to do the frontal orbital first or the chiari decompression? If it's the frontal orbital how would you approach the Team B's neurosurgeon who wants to do the decompression surgery first?
Answer: I am so sorry that your child has required so many surgeries at such a young age. As a father of 8, I know how hard that can be on both the child and your family as a whole. I think you are right to want to minimize the number of trips to the operating room and the number of general anesthetics.
Without seeing your child or reviewing her medical records and imaging, I cannot give specific medical advice for your daughter. However, I do know that in my own practice and in my fellowship training, I have observed some improvements in Chiari malformations when the cranial vault is expanded. This is probably most true for posterior expansions and to a lesser degree is also observed with anterior expansion. I think that the reason you may see improvement is that by expanding the vault, you improve the volume mismatch between the brain and the closed space it lives in. If the space gets bigger, sometimes that allows the brain to “ride-up” a bit and the Chiari to improve.
I am not sure there is an absolute right or wrong answer in your case regarding which approach to take. Your two teams sound as though they have both put forth thoughtful plans, and both approaches make sense. My best advice to you is to not be afraid to ask a lot of questions to your team (or teams) as you seek to make a good decision regarding your daughter’s care.
Since I tend to favor limiting the number of surgeries or performing surgeries that may have multiple benefits, I understand the appeal of fronto-orbital advancement. That is the only option that has been put forward that offers a possibility of doing one surgery to address both the forehead position and the Chiari. However, it must be said that with fronto-orbital advancement, the improvement in the Chiari may be modest at best. Furthermore, anterior vault surgery (such as fronto-orbital advancement) probably shouldn’t be seen as adequate treatment for a major Chiari malformation or a symptomatic Chiari malformation, in my opinion. However, if the Chiari is judged by your team to be small and if it is asymptomatic, it may be reasonable to do the fronto-orbital advancement first with the expectation that you may see some improvement in the Chiari. If this strategy is selected, it is mandatory that post-operative imaging such as an MRI be done to document any change and that she continue to be monitored over time to be sure that the Chiari doesn’t worsen or become symptomatic. You should also not be disappointed if she later requires a more formal Chiari decompression.
As for how to approach the neurosurgeon who has recommended decompression first, I would say that good communication is key. Don’t be afraid to be your child’s advocate and ask lots of questions. I would try to understand why the neurosurgeon feels so strongly that decompression be done first. (It may be that there is a factor in your daughter’s case that clearly mandates treatment of the Chiari right away.) I would also advise you to explain your rationale for considering the other approach and your strong desire to minimize the number of surgeries that your daughter has. If it is safe to wait on formal decompression, your pediatric neurosurgeon may be more supportive of your position than you think, especially since fronto-orbital advancement may conceivably improve the Chiari, too.
Although it is always hard to make these sorts of health care decisions for your child, you are fortunate to have access to more than one team to give you options and help you decide what’s best for your daughter. Remember also that your teams know the details of your daughter’s situation better than anyone and are the ones best suited to help you make a good decision. Best of luck!
We have seen 2 teams of doctors and Team A says to do a frontal orbital advancement as the next surgery. The Team B wants to do a Chiari decompression surgery and then the frontal orbital advancement. The Team B did a limited MRI and the neurosurgeon noted crowding in the area. The Team A routinely uses the frontal orbital to try and provide more room for the brain.
Currently our daughter shows no signs of a Chiari malformation, central sleep apnea or spinal cord issues. We are reluctant to put her through any unnecessary surgeries. I guess our questions would be whether or not to do the frontal orbital first or the chiari decompression? If it's the frontal orbital how would you approach the Team B's neurosurgeon who wants to do the decompression surgery first?
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Without seeing your child or reviewing her medical records and imaging, I cannot give specific medical advice for your daughter. However, I do know that in my own practice and in my fellowship training, I have observed some improvements in Chiari malformations when the cranial vault is expanded. This is probably most true for posterior expansions and to a lesser degree is also observed with anterior expansion. I think that the reason you may see improvement is that by expanding the vault, you improve the volume mismatch between the brain and the closed space it lives in. If the space gets bigger, sometimes that allows the brain to “ride-up” a bit and the Chiari to improve.
I am not sure there is an absolute right or wrong answer in your case regarding which approach to take. Your two teams sound as though they have both put forth thoughtful plans, and both approaches make sense. My best advice to you is to not be afraid to ask a lot of questions to your team (or teams) as you seek to make a good decision regarding your daughter’s care.
Since I tend to favor limiting the number of surgeries or performing surgeries that may have multiple benefits, I understand the appeal of fronto-orbital advancement. That is the only option that has been put forward that offers a possibility of doing one surgery to address both the forehead position and the Chiari. However, it must be said that with fronto-orbital advancement, the improvement in the Chiari may be modest at best. Furthermore, anterior vault surgery (such as fronto-orbital advancement) probably shouldn’t be seen as adequate treatment for a major Chiari malformation or a symptomatic Chiari malformation, in my opinion. However, if the Chiari is judged by your team to be small and if it is asymptomatic, it may be reasonable to do the fronto-orbital advancement first with the expectation that you may see some improvement in the Chiari. If this strategy is selected, it is mandatory that post-operative imaging such as an MRI be done to document any change and that she continue to be monitored over time to be sure that the Chiari doesn’t worsen or become symptomatic. You should also not be disappointed if she later requires a more formal Chiari decompression.
As for how to approach the neurosurgeon who has recommended decompression first, I would say that good communication is key. Don’t be afraid to be your child’s advocate and ask lots of questions. I would try to understand why the neurosurgeon feels so strongly that decompression be done first. (It may be that there is a factor in your daughter’s case that clearly mandates treatment of the Chiari right away.) I would also advise you to explain your rationale for considering the other approach and your strong desire to minimize the number of surgeries that your daughter has. If it is safe to wait on formal decompression, your pediatric neurosurgeon may be more supportive of your position than you think, especially since fronto-orbital advancement may conceivably improve the Chiari, too.
Although it is always hard to make these sorts of health care decisions for your child, you are fortunate to have access to more than one team to give you options and help you decide what’s best for your daughter. Remember also that your teams know the details of your daughter’s situation better than anyone and are the ones best suited to help you make a good decision. Best of luck!
As someone with Crouzon, cleft palate, craniosynostosis, and Chiari Malformation 1, thank you so much for posting this. I am so thankful for all information posted about such rare disorders. I will pray for this family.
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