Hello all,
I thought I would update you with Aneeshas recovery after her duroplasty that took place on the 9th of Juliy 2014. After the surgery she was walking and feeling fine ofcourse except for the headaches and not being able to focus and balance issues , and sleep apnea…which occurred two weeks after surgery…When taken to get her staples removed an MRI showed that she had developed substantial hydrocephalus…her doc said to wait n watch…as he said the brain has strange ways of adjusting to changes and that he hoped things would get better on their own…well her focussing problem resolved and so did her headaches…she is no longer complaining so the nerve twitches she felt on the right side of her face…but is very upset about the balancing part…she has to be held when she goes to the washroom with one hand…she has osteogenesis Imperfecta but was able to walk on her own before…but she has also got scoliosis…she feels very tired most of the time…otherwise other issues have been resolved…My question is that have you ever heard of some one becoming better on their own after they have developed hydrocephalus due to duroplasty…?We are giving her some homeopathic meds for the hydrocephalus and a tablet to keep the CSF flow from increasing…but they say this tab can lesson the potassium in the body…Please do share what ever you know about this…she wants to start college but we feel she is too frail right now…how soon did you guys start your normal life after this surgery?..I am so confused…please guide me for the do’s and don’ts also…love n blessings…Ambareen
Hydrocephalus after intracranial surgery(in Aneesha's case, Chiari decompression with duroplasty) occurs due to a blockage of CSF flow at some level(typically at the surgical level). It is certainly reasonable to wait as long as she can tolerate to see if any post-operative swelling improves and she is possibly able to resume her normal CSF flow. It sounds like your neurosurgeon started her on Diamox(a medicine that reduces CSF production), the generic name is acetazolamide. Again, all that makes sense.
However, what set off alarm bells for me in your story is Aneesha's osteogenesis imperfecta. Decompression is those patients is notoriously difficult due to the soft nature of their bones. the most common CVJ finding in the OI patient population is basilar invagination, not Chiari. A Chiari decompression can weaken the CVJ and cause basilar invagination(which, if bad enough, can lead to hydrocephalus). You are describing brain stem dysfunction(ataxia, sleep apnea, head-aches, and facial nerve irritability).
I would suggest reconfirming recommended follow up with the neurosurgeon, let them know she is not improving to the point that she can attend school, and did they recommend sooner follow-up or repeat imaging. Make sure they know the same symptoms you describe in your above description. It is probably worthwhile even specifically asking about basilar invagination. There is a high risk of needed a posterior occipito-cervical fusion in the OI patient population(and a much higher failure rate of fusion due to bone quality).
Yes, someone needs to address the described symptoms in a fragile patient. Few neurosurgeons are comfortable with patients with OI so I would strongly recommend follow up with the operating neurosurgeon but don't stop without images(at least plain X-rays of the cervical spine to assess stability).
Beeba said:
Obviously would never have more to add medically than what dr Trumble is advising you. Dr T - if her ns pushes this aside would you suggest seeking a second opinion, or even third until things make sense?
As for returning to school - personally I would wait. I would want to make sure everything is okay and will not interrupt her school year, that could hurt her academically and possibly financially. My first decompression was incomplete and I couldn't even check my mailbox at the end of the driveway. I can't imagine hiking around a campus with heavy books. I did try and listen to everyone and get back to living. I found myself stuck in the center of a very large mall just in tears thinking I couldn't make it back to my car. It was very scary. It was not anxiety although that certainly follows this is a physical thing. I was 42 when that happened. A young person will push themself much harder for normalcy and get in situations that are over their head. Perhaps she could take just 2 classes or so online so she can work at her own pace and still feel she is moving forward with life but until this gets better I couldn't have done it. The surgery even at its best really takes a great deal out of you physically and not to mention the emotional strain.
I would do whatever dr Trumble suggested.
Dear Doctor Tumble,
Thankyou so much for your valuable opinion and helpful advice.Aneesha had the duroplasty and decompression with out the occipital fusion as the surgeon said that her C1 and C2 we went strong enough to hold the screws as he had shaved them during the previous decompression surgery where he had not done the duroplasty but just removed a part of her skull…but this didn’t help…so to get rid of the syrinx her had to do the duroplasty…Aneesha already had basilar Invagination prior to this surgery but no Chiari…so this surgery didn’t have anything to do with Chiari…he did the duro Plasty and hoped that the syrinx which was from the C2 to C 7 would dissipate…but after this surgery Aneesha developed hydrocephalus…as the odontoid peg impinged upwards…the doc says it might get better with time…she is able to see properly now and has no more facial nerve twitches but bearable head aches sometimes…the main issue is with her balance…as she can’t walk on her own…we will be taking an MRI…in a month or so…so let’s see what the results bring in…I will update her doc as well…is it common for such a complication to take place after her surgery…Aneeshas surgeon asked us to show her to a neuro physician for her sleep apnea issues…which we aren’t really sure were there before the surgery…he was the one who suggested the medicine to lesson the CSF flow to avoid more hydro cephallus…her surgeon didn’t seem to keen onit …said her potassium needs to be monitored and he doesn’t really think it would make a difference…what do you say about this?is it ok to continue giving her the meds?..we really don’t want to go in for a shunt and are hoping and praying that the syrinx goes away and Her walk stables…Another thing I would like to take your advice on is her sleep apnea…is it something serious?.. Hyde neuro physician said that it very common to have it…but I am not really convinced…i would really appreciate it if you could again advice me regarding these issues that I am facing,
kind regards,
Ambareen
Dear Doctor Tumble,
Thankyou so much for your valuable opinion and helpful advice.Aneesha had the duroplasty and decompression with out the occipital fusion as the surgeon said that her C1 and C2 we went strong enough to hold the screws as he had shaved them during the previous decompression surgery where he had not done the duroplasty but just removed a part of her skull…but this didn’t help…so to get rid of the syrinx her had to do the duroplasty…Aneesha already had basilar Invagination prior to this surgery but no Chiari…so this surgery didn’t have anything to do with Chiari…he did the duro Plasty and hoped that the syrinx which was from the C2 to C 7 would dissipate…but after this surgery Aneesha developed hydrocephalus…as the odontoid peg impinged upwards…the doc says it might get better with time…she is able to see properly now and has no more facial nerve twitches but bearable head aches sometimes…the main issue is with her balance…as she can’t walk on her own…we will be taking an MRI…in a month or so…so let’s see what the results bring in…I will update her doc as well…is it common for such a complication to take place after her surgery…Aneeshas surgeon asked us to show her to a neuro physician for her sleep apnea issues…which we aren’t really sure were there before the surgery…he was the one who suggested the medicine to lesson the CSF flow to avoid more hydro cephallus…her surgeon didn’t seem to keen onit …said her potassium needs to be monitored and he doesn’t really think it would make a difference…what do you say about this?is it ok to continue giving her the meds?..we really don’t want to go in for a shunt and are hoping and praying that the syrinx goes away and Her walk stables…Another thing I would like to take your advice on is her sleep apnea…is it something serious?.. Hyde neuro physician said that it very common to have it…but I am not really convinced…i would really appreciate it if you could again advice me regarding these issues that I am facing,
kind regards,
Ambareen
Here's the largest article I'm aware of with OI and basilar invagination.
http://www.ncbi.nlm.nih.gov/pubmed/9171173
It was published in 1997 but hasn't been updated. Arnold Menezes(the senior author) is the leading expert in craniovertebral junction issues(although I don't think he operates anymore). the first author(his resident) is Paul Sawin, the adult spine neurosurgeon who works in Orlando with me now.
The bottom line is, all those patients required fusion. Even with fusion, basilar invagination can be progressive in the OI patient population. Posterior decompression destabilizes OI patients due to their bone disease.
Aneesha's head is literally sinking into her neck. You describe "the odontoid peg impinged upwards", causing hydrocephalus and a syrinx(by definition, enough pressure to prevent CSF flow and, if fluid can't get around, imagine what the pressure is doing to the spinal cord), worsening brain stem function(inability to walk), respiratory dysfunction(sleep apnea). You are essentially checking off every red flag in a neurosurgeon's book.
I would strongly suggest returning to your neurosurgeon, asking them to see the pictures, and looking how much her odontoid is now pressing on the brain(you can see that on plain films so, if the expense of MRIs is an issue, plain X-rays are adequate for the bony structures).
Ask about fusion. Ask about life expectancy. Ask if going to sleep and not waking up is a risk of waiting. and ask if there is anyone in your neurosurgeon's practice with similar issues. If not, perhaps a referral to someone with more experience would be prudent(both for you and the neurosurgeon). If yes, ask to meet them(because I would be shocked if they were telling the truth).
In the US, I don't think anyone would posteriorly decompressed an OI patient with basilar invagination. Most are anteriorly decompressed(a MUCH bigger operation than the posterior decompression), following by a fusion. Prior decompression doesn't exclude fusion, it means the operation is a bigger operation and must go down lower in the cervical spine.
The fact that Aneesha has done so well for so long with all her difficulties is a tribute to your love and support. You may need to get a little pushier than you are comfortable with for her sake now.