1 month

Exactly one month from today we will be in Boston, done with Malachi's pre-op appointment and probably eating dinner somewhere. It's so close I can almost taste the yummy sea food. HA! But it's also so close that I can taste the fear, the worry and the tears running down my face. My sweet pea that has already been through so much is going to be getting ready for the biggest event in his life thus far. And I am worried sick about it.



This is the man that I am entrusting Malachi's life with. He is the guru of Moyamoya disease and the most sought after neurosurgeon in the country. He is the expert and the brains behind Pial Synangiosis surgery and in 1 month we will me him, shake his hand and trust him with our life.

So what is this Pial Synangiosis you talk of? I will let Boston's Neurosurgical Foundation explain it to you.  (all information was taken from http://www.boston-neurosurg.org/publications/faq/moyamoyaRMS.shtml

What is a Pial Synangiosis?
     
     This operation is designed to take advantage of the tendency of the brains of children with      moyamoya syndrome to attract new blood vessels from any source that is made available by the surgeon. We make an incision on the scalp over an artery supplying blood only to the skin over the head. We separate this artery from the tissues around it, keeping blood flowing through it. We open up a window of bone beneath the artery, and then use a microscope to carefully open all of the coverings of the brain right down to the brain surface. The artery is then placed directly onto the brain, and the tissues surrounding its walls are sewn to the brain surface with tiny sutures to keep it in contact with the brain. Then the bone window is replaced securely, and the skin incision closed. In some patients, we may also place an extra small hole (a "burr hole") in the skull remote from the first incision, and at this hole we also make tiny openings in all of the coverings of the brain before closing the incision. In about 75% of patients, new blood vessels will also grow into the brain through this small opening, and the short additional operating time required to do this extra procedure seems worthwhile. To operate on one side of the brain takes about 3 to 4 hours; in many patients, we will try to do both sides of the brain on the same day, under the same anesthesia.


Is it dangerous to operate on both sides of the brain at once?


     Operating on both sides of the brain during the same anesthesia probably reduces the patient's risk of having a stroke with surgery. Most of the patients with moyamoya syndrome have a very tenuous blood supply to their brain that can be reduced very easily. Anesthesia can alter brain blood flow, and in particular, the starting and ending of the anesthesia are critical times when blood flow to the brain can be dramatically changed. It is our belief that reducing the number of anesthetic inductions is essential for the child with moyamoya. We also monitor the patient's brain waves ("EEG") throughout the procedure using small button electrodes placed over the scalp except in the areas where we are operating. As we are carrying out the surgery, we can tell whether our anesthetic or surgical techniques are affecting the patient's brain function, and alter either to correct a potential problem. If all is going well after the first side is completed, we will immediately operate on the other side to avoid having to administer another anesthetic on a separate occasion.


What is the chance of another stroke during or following the operation? 


      The surgery has been safe, and has a low complication rate, but approximately 7% of patients will suffer new strokes of varying degrees of severity either at the time of surgery or during the first post-operative month. Most of these patients had been neurologically unstable before surgery, having frequent strokes or numerous transient ischemic attacks ("TIA's") -- brief periods of neurological dysfunction that are often warning signs of an impending stroke - in the months or weeks before the operation. We believe that such patients are at a higher risk for stroke during a surgical procedure than patients who have had no recent events of this type. For this reason, we usually wait for four to six weeks after any stroke before we proceed with surgery. In four patients, I thought that unstable blood pressure or medication problems were the likely causes of the new post-operative strokes. Some patients have had new strokes while waiting for surgery that had to be delayed or postponed for various reasons; because of the way operative complications are reported, these patients are also included in this group. Most of these patients, by the way, have made excellent recoveries.


Why does the surgery work?


     The surgery works by inducing the development of new blood vessels from the donor scalp artery in the area of the area of the synangiosis which provides an additional source of blood to the underlying brain. These blood vessels develop not only from the scalp artery, which is the major source of new blood, but also from blood vessels which sprout from the coverings of the brain around the skull opening. That is why even making a small skull opening (the "burr hole" mentioned above) can also help these patients. We are not sure what makes these new blood vessels sprout and grow, and this response is usually not seen in patients having brain surgery for other reasons. Our research work has demonstrated that in the fluid surrounding the brain (CSF) of patients with moyamoya syndrome are growth factors which seem to induce the development of the new blood vessels. Our research work in this area is ongoing, and we have recently reported on the results of some of this work involving growth factors and other substances in the cerebrospinal fluid that seem to be related to a chronic inflammation of some type in these patients. We hope that eventually it might be possible to enhance the effect of surgery by treating patients before or during surgery with some of these substances.