MEDICAL CASE
ABOUT MOYAMOYA SYNDROME
Núria Climent Huguet
INTRODUCTION
Hello, I’m Núria Climent Huguet.
First of all, I would like to thank everyone for this
project we are building, and encourage you to continue it because, as Clàudia
said last week, is a great opportunity to Increase our knowledge about medicine
which we all love, and our English level.
I chose an article from the serial Grey’s Anatomy,
which I think most of us have seen and so it’s interesting to know more about a
chapter we have seen. However, if you have not seen the series, it’s a
perfectly understandable and entertaining clinical case.
I would be grateful if you answer some questions after
reading it:
·
Do you think that it is more important to research on
rare diseases or should invest more money in the most frequent ones?
·
What treatment would you prefer to apply to your child
if he would suffer the Moyamoya disease?
·
Would you like to attend a procedure omental transposition
surgery?
I wish you like the article, thank you everyone!
Iris suffers from Moyamoya disease.
Also known as Moyamoya syndrome and mostly seen in children, the disease is a rare condition in which the walls of the internal carotid arteries (the vessels that provide the blood supply to the brain) thicken and narrow. As the arteries narrow, less oxygen-rich blood reaches the brain, increasing the risk for the patient to have a complete blockage and suffer a stroke.
Due to the narrowing arteries, the brain will attempt to create new blood vessels in order to continue to provide oxygen-rich blood to the areas of the brain in need. When viewed on an angiogram, these tiny additional blood vessels resemble wisps – hence, the Japanese doctors named the disease Moyamoya, which means "puff of smoke" in Japanese. However, even though the brain forms these new vessels, they tend to be much more fragile and can even break off and bleed into the brain, causing hemorrhages.
As part of the progression of her disease, Iris lost muscle strength from having transient ischemic attacks (T.I.A.s or "mini-strokes").
During a T.I.A., a blockage occurs, disrupting the flow of blood to the brain. Symptoms appear similar to those of a stroke, such as:
• Weakness on one side of the body.
• Headache.
• Seizures.
• Slurred speech.
• Blurry vision.
Symptoms may come and go, depending on the individual patient and any treatment pursued. A rare and serious complication can be a brain hemorrhage. Warning signs include:
• Vomiting.
• Nausea.
• Severe headache.
• Numbness in part of the body.
• Fatigue.
• Vision changes.
Iris came to Grey-Sloan Memorial to have surgery to establish a new blood supply to her brain.
Unfortunately, Moyamoya disease is a progressive condition, and currently no medication can reverse the artery damage in the brain. However, some medications and various surgical treatments can successfully manage symptoms. Many doctors recommend children with Moyamoya disease regularly take aspirin to prevent blood clots from forming. Additionally, calcium channel blockers may be prescribed; these medications help lower blood pressure by keeping calcium from entering the cells in the heart and blood vessel walls. A lowered blood pressure can be instrumental in decreasing the headaches and possible T.I.A.s associated with the disease.
Surgery aims to either bypass narrowed arteries or create entirely new blood supplies for the affected areas of the brain. Surgical options can be divided into two groups – direct or indirect procedures. Cerebral bypass procedures are known as the direct method – a blood vessel from outside the brain will be connected to a vessel inside the brain to reroute the flow of blood around one of the damaged arteries. Surgeons commonly use a blood vessel from the patient's scalp (the superficial temporal artery) and join it to the middle cerebral artery in the brain. Abbreviated the STA-MCA, this bypass procedure may show improvement in blood flow in just a few months.
Derek and Alex performed an indirect procedure for little Iris with the goal of introducing a new blood supply to the brain over time. In many cases of a young pediatric patient, an indirect procedure is chosen because their arteries are not yet large enough for a bypass procedure. Several types of indirect surgeries exist, such as:
• The omental transposition procedure (what Iris had) uses the omentum – the lining of the abdominal organs, which serves as an excellent source of blood supply. The omentum is separated from the abdomen and placed on the surface of the brain; these vessels will then hopefully grow into the brain.
• The EDAS (encephalo-duro-arterio-synangiosis) procedure takes the superficial temporal artery and sutures it to the dura – the tissue that covers the brain. Over time, small arterial vessels will form.
• The EMS (encephalo-myo-synangiosis) procedure takes the temporalis muscle from the side of the head and places that onto the surface of the brain.
• Dural inversion involves flipping the flaps of the patient's dural tissue on the meningeal vessel, a large artery in the skull. Once reversed, the blood vessels on the outer surface provide supply to the starved areas of the brain.
Also known as Moyamoya syndrome and mostly seen in children, the disease is a rare condition in which the walls of the internal carotid arteries (the vessels that provide the blood supply to the brain) thicken and narrow. As the arteries narrow, less oxygen-rich blood reaches the brain, increasing the risk for the patient to have a complete blockage and suffer a stroke.
Due to the narrowing arteries, the brain will attempt to create new blood vessels in order to continue to provide oxygen-rich blood to the areas of the brain in need. When viewed on an angiogram, these tiny additional blood vessels resemble wisps – hence, the Japanese doctors named the disease Moyamoya, which means "puff of smoke" in Japanese. However, even though the brain forms these new vessels, they tend to be much more fragile and can even break off and bleed into the brain, causing hemorrhages.
As part of the progression of her disease, Iris lost muscle strength from having transient ischemic attacks (T.I.A.s or "mini-strokes").
During a T.I.A., a blockage occurs, disrupting the flow of blood to the brain. Symptoms appear similar to those of a stroke, such as:
• Weakness on one side of the body.
• Headache.
• Seizures.
• Slurred speech.
• Blurry vision.
Symptoms may come and go, depending on the individual patient and any treatment pursued. A rare and serious complication can be a brain hemorrhage. Warning signs include:
• Vomiting.
• Nausea.
• Severe headache.
• Numbness in part of the body.
• Fatigue.
• Vision changes.
Iris came to Grey-Sloan Memorial to have surgery to establish a new blood supply to her brain.
Unfortunately, Moyamoya disease is a progressive condition, and currently no medication can reverse the artery damage in the brain. However, some medications and various surgical treatments can successfully manage symptoms. Many doctors recommend children with Moyamoya disease regularly take aspirin to prevent blood clots from forming. Additionally, calcium channel blockers may be prescribed; these medications help lower blood pressure by keeping calcium from entering the cells in the heart and blood vessel walls. A lowered blood pressure can be instrumental in decreasing the headaches and possible T.I.A.s associated with the disease.
Surgery aims to either bypass narrowed arteries or create entirely new blood supplies for the affected areas of the brain. Surgical options can be divided into two groups – direct or indirect procedures. Cerebral bypass procedures are known as the direct method – a blood vessel from outside the brain will be connected to a vessel inside the brain to reroute the flow of blood around one of the damaged arteries. Surgeons commonly use a blood vessel from the patient's scalp (the superficial temporal artery) and join it to the middle cerebral artery in the brain. Abbreviated the STA-MCA, this bypass procedure may show improvement in blood flow in just a few months.
Derek and Alex performed an indirect procedure for little Iris with the goal of introducing a new blood supply to the brain over time. In many cases of a young pediatric patient, an indirect procedure is chosen because their arteries are not yet large enough for a bypass procedure. Several types of indirect surgeries exist, such as:
• The omental transposition procedure (what Iris had) uses the omentum – the lining of the abdominal organs, which serves as an excellent source of blood supply. The omentum is separated from the abdomen and placed on the surface of the brain; these vessels will then hopefully grow into the brain.
• The EDAS (encephalo-duro-arterio-synangiosis) procedure takes the superficial temporal artery and sutures it to the dura – the tissue that covers the brain. Over time, small arterial vessels will form.
• The EMS (encephalo-myo-synangiosis) procedure takes the temporalis muscle from the side of the head and places that onto the surface of the brain.
• Dural inversion involves flipping the flaps of the patient's dural tissue on the meningeal vessel, a large artery in the skull. Once reversed, the blood vessels on the outer surface provide supply to the starved areas of the brain.
Hello Nuria ! Very interesting, I did not know this disease.
ResponderEliminarI think both types of research are important, but nowadays I consider that there is a lack of research on rare diseases and perhaps too much investment by the pharmaceutical industry on very prevalent diseases are as hypertension and diabetes, on which we already have several resources to treat them. But really this is a difficult problem to solve, because as all we know a lot of money and labor are spent for the creation of new drugs, so it is difficult to invest money in such rare diseases where profits can never compensate the expenses.
Regarding the second question, I think that the best treatment will depend on each particular case but I personally might have preferred the surgery because it seem me to be the best long term solution.
Finally, yes! I would like to attend a procedure omental transposition surgery I think it is a very interesting surgery.
Joana Gonçalves
Thanks Nuria for this article, because now I know something about this pathology and I think I should follow Grey's Anatomy.
ResponderEliminarI really like your first question, because I think that it's an interesting theme to discuss. I'm agree with Joana, both kinds of diseases are important to research, but I think we must investigate more than we do nowadays in non-frequent diseases. However, people only want to research in that when they know a near case, an example is Ebola. We needed to investigate about Ebola when it arrived to the first world, while Ebola was in Africa it wasn't so important. And this is only a one example.
The second questions is really difficult to me to answer because I don't know these surgeries but I think I'd choose the first one, the omental transposition, because it seems less aggressive than others. I find interesting to attend this procedure because I'm ignorant of it.
ANDREA GARCÍA
Hello Núria, I found your article very interesting, because it is about a disease that is not very well-known.
ResponderEliminarRegarding the first question, my opinion is that all research is useful and necessary. However, it is also true that there are many factors that make researching increasingly more difficult such as economic restrictions and lack of resources, for example.
The second question is difficult for me to answer, because I know very little about this disease. I feel that the omental transposition surgery seems to be less aggressive than the alternative, but I'm not sure.
After knowing this disease, I would like to learn more about it, and I would like to attend a surgery.
Dunia
Good morning Núria, thank's for your report!
ResponderEliminarWhen I've read the title I've felt confused, I didn't know anything about the topic! Most of us aren't aware of Moyamoya disease, so you can guess how many non-medical people are informed about this syndrome as much as other rare disease! Writing my little introduction, I've answered part of your first question.
In my opinion, rare disesase should be researched as well as prevalent ones; they'd never be forgotten by society. Although, the reasonable thing is investing more money in the prevalent disease because much more people will be beneficiated.
We would like to have a hundred per cent health coverage all around the world, but if the resources are limitated we will have to choose. In this case, we will focus on prevalent and popular diseases.
As my partners have said before about the second interrogation, these treatments were totally unknown for me so I'm in the dark. Of course, I'd choose a surgery treatment, accepting all it morbidity, looking for a long-term solution.
I would like to know more about this rare syndrome and of course I'd like to attend a omental transposition surgery too, this procedure must be a delicate, detailed and interesting work.
María Lamana.
Hi Nuria, thank you for this article. It was useful for me since I had never heard about this disease, and I really find it quite interesting.
ResponderEliminarRegarding to the first question I think it´s a very controversial topic. Maybe in an ideal world where economic interesting and gains are not the most important goals, rare diseases can be researched. But, a big invest is needed for any research, and if the regarding is not positive, this can´t be done, since there will be loose of money and time. On the other hand, is logical that researches are focused on the most prevalent diseases, because besides money, more people will be beneficiated. However, rare disease can´t be forgotten.
Talking about the second question, I have to say that I will need more information of each treatment, because what I will prefer will be a best quality of life for my kid. So the one that gives me this, will be the treatment I choose. Of course, this has to be decided with the doctors because they are the ones who know about this; and maybe more treatments will appear in the future, since science is developing so fast, so there might be more options.
Finally, answering to your last question, I love surgery. Actually, vascular is one of my options of specialties, so this must be an amazing opportunity. Besides, attending to this surgery has to be incredible, talking about the techniques and the difficult of it. However, since it´s not a very common disease, and in not all hospitals this surgery can be done, it can be difficult to attend to one. But, if I have the chance, definitely, I won´t say no.
Elisa Salazar
Hi Núria! I had never heard about this syndrome before and has left me speechless, wanting to know more about the Moyamoya's Syndrome.
ResponderEliminarWith regard to the first question, I think that even though the economic situation in which we live today is not the best, the research should not be affected, and although there are many research groups looking for the solution and the treatments of the most prevalent diseases, rare diseases should not be set aside. So I'd like to think that despite the current circumstances, the investigation will continue moving forward both rare diseases and the most prevalent ones.
If my child had this disease, I don't know what I will choose, I think that I will need more information of each treatment and the ones with the best results could be an option.
However, after reading the information that you give us, I would say I could accept a surgery op. like EDAS (encephalo-duro-arterio-synangiosis) procedure which will give my son the irrigation that he lost.
To end up, as you all know I love surgery and of course I would like to attend an Omental Transposition Surgery. It must be a complicated surgery with a high risk, anyway it will be a good experience. Although, as my classmate Elisa said, is not a very common disease so this surgical technique will only be performed in specific cases but whether I had the opportunity I will be glad to be part of the surgeon team without any doubt.
Mercè
Hi!
ResponderEliminarIt's the first time I've read about Moyamoya syndrome.
I think blood vessel diseases are very interesting, as they're kind of paradoxical. The point I find the most curious is that its treatment has to pursue a correct fluidity of the blood (preventing from clot formation) but not exceed the limit, because it would increase the risk of hemorrhage.
As said in the article, this syndrome –mostly seen in children- can course with symptoms similar to those of a stroke. It has to be really shocking to visit a young patient with that symptomatology, which is more common in elderly people due to different causes to Moyamoya.
I guess rare diseases don’t catch our attention until we don’t know someone who’s suffering from one of them. Then, we may realize how necessary the investigation in this field is. Answering to Nuria’s question, I would say that all diseases are worth of being studied as there are ill people behind them. However, I understand economic issues make research invest more in frequent health problems than in the rare ones.
First of all, I need to thank you because of the acceptance of the article, and I’m glad you liked it.
ResponderEliminarI’m a passionate about surgery and I’m happy about knowing most of you too.
As most of you said, infrequent illness may not be forgotten, but I also agree with you regarding the fact that we can’t research everything because of limited resources. So, lack of money forces us to choose the best cost-benefit research.
On the other hand, I understand you when you say that you’d need much more information about the techniques before choosing one to be used with your hypothetical children. We’ll become doctors and we would like to take care about our children as well as possible, and mainly about their health. However, with the information we have with the article, I’d probably choose the omental transposition too.
To finish, it’s clear that I’ll be grateful to assist this kind of surgery and I’m surprised most of you too, so I’m proud of this.
Thank you all for your attention and implication. I love sharing knowledge with you.
See you tomorrow in class!
MARTA CORDOBA
ResponderEliminarHi Nuria! I found your article really interesting and I think you had a very original idea to get the case of a TV programe.
In answer to your questions, I will say this:
1. In my opinion both are important. I think our society is enough advanced and we have enough scientists and economic recourses to research most of diseases that have no cure.
2. I'm not sure because I haven’t all the knowledge or sufficient information about the different treatments. However it seems to me that the EDAS is the procedure most simply and I think if the blood vessels are not larger enough medical treatment can be used to keep the temporal artery growing until it gets large enough to make the synangiosis.
3. As many of you know, I’m not into surgery because I prefer having a closer relationship with patients, even though I think it’s good for my medical training.