2 Pathophysiology of moyamoya disease

2 Pathophysiology of moyamoya disease

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Moyamoya disease (MMD) is a rare condition where the supraclinoid part of internal carotid artery is slowly and progressively becoming stenotic, and also it often involves both middle cerebral arteries and anterior cerebral arteries (Fig. 26.1) [1]. The term moyamoya means a puff of smoke in Japanese, and it was first reported by Takeuchi and Shimi...

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... too much. The ICA becomes tortuous, and frequently we will find the duplication of internal elastic lamina, and it does not show any inflammatory reaction or any atheromatous forming, resulting in artery stenosis/ occlusion [10]. The hypoxia induces supply from other parts to form collateral flow, and the arteries become tortuous and dilated ( Fig. 26.2). These "moyamoya" vessels have many fibrin deposits at its wall, but the media layer is thin, the elastic lamina becomes fragmented, and so there is a high chance to have microaneurysm formation. It has been reported that the aneurysm formation might be associated with over-expression of several growth factors such as matrix ...
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... course of the superficial temporal artery is plotted by using a Doppler probe. When parietal branch of STA is chosen as donor, the skin incision is planned over the artery along its course ( Fig. 26.3). If the frontal branch is the donor, the inferior portion of the incision follows the STA, but as the artery tracks anterior to the hairline, the incision stays posterior behind the hairline for cosmetic purposes. After shaving the hair over the planned incision, prepare and drape the surgical area. The operating microscope is ...
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... posterior behind the hairline for cosmetic purposes. After shaving the hair over the planned incision, prepare and drape the surgical area. The operating microscope is positioned and used for careful harvesting of the STA. A length of approximately 7 cm of STA together with a generous cuff of soft tissue to protect it is needed for the bypass (Fig. 26.4). Then the underlying temporalis fascia muscle is split and dissected from the frontotemporal bone. Burr holes are placed, the craniotomy bone flap is removed at frontotemporal area, and the dura is cut in a cruciate manner (Fig. ...
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... of approximately 7 cm of STA together with a generous cuff of soft tissue to protect it is needed for the bypass (Fig. 26.4). Then the underlying temporalis fascia muscle is split and dissected from the frontotemporal bone. Burr holes are placed, the craniotomy bone flap is removed at frontotemporal area, and the dura is cut in a cruciate manner (Fig. ...
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... of MCA (0.6 mm) emerging from Sylvian fissure is paramount for this procedure. The arachnoid overlying this cortical branch of the MCA is then microscopically opened. A 7 mm segment of M4 artery without branches is preferably chosen as the recipient, but any tiny branches arising from this segment can be coagulated and divided if necessary (Fig. 26.6). A jeweler-type bipolar is used. High-visibility background is placed under the M4 segment. Papaverine is intermittently instilled over the vessels to prevent spasm. Then the STA is temporarily occluded proximally and sectioned distally. The distal stump of the STA in the scalp is coagulated, and the STA is truncated to the proper ...
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... mean arterial pressure is raised, and then the M4 MCA branch is temporarily occluded with Sugita aneurysm clips. An arteriotomy is made in the M4 branch, removing an elliptical portion of the superior wall, and irrigated with heparinized saline. Anastomosis between an end of STA and the side of the MCA segment is done with 10-0 interrupted suture (Fig. 26.7). The Sugita clips are then removed, restoring flow. Flow in all vessels is confirmed with the intraoperative Doppler. An intraoperative indocyanine green (ICG) angiogram is performed by injecting 2 ml of ICG dye intravenously and visualizing the graft with the near-infrared camera on the ...
Context 7
... too much. The ICA becomes tortuous, and frequently we will find the duplication of internal elastic lamina, and it does not show any inflammatory reaction or any atheromatous forming, resulting in artery stenosis/ occlusion [10]. The hypoxia induces supply from other parts to form collateral flow, and the arteries become tortuous and dilated ( Fig. 26.2). These "moyamoya" vessels have many fibrin deposits at its wall, but the media layer is thin, the elastic lamina becomes fragmented, and so there is a high chance to have microaneurysm formation. It has been reported that the aneurysm formation might be associated with over-expression of several growth factors such as matrix ...
Context 8
... course of the superficial temporal artery is plotted by using a Doppler probe. When parietal branch of STA is chosen as donor, the skin incision is planned over the artery along its course ( Fig. 26.3). If the frontal branch is the donor, the inferior portion of the incision follows the STA, but as the artery tracks anterior to the hairline, the incision stays posterior behind the hairline for cosmetic purposes. After shaving the hair over the planned incision, prepare and drape the surgical area. The operating microscope is ...
Context 9
... posterior behind the hairline for cosmetic purposes. After shaving the hair over the planned incision, prepare and drape the surgical area. The operating microscope is positioned and used for careful harvesting of the STA. A length of approximately 7 cm of STA together with a generous cuff of soft tissue to protect it is needed for the bypass (Fig. 26.4). Then the underlying temporalis fascia muscle is split and dissected from the frontotemporal bone. Burr holes are placed, the craniotomy bone flap is removed at frontotemporal area, and the dura is cut in a cruciate manner (Fig. ...
Context 10
... of approximately 7 cm of STA together with a generous cuff of soft tissue to protect it is needed for the bypass (Fig. 26.4). Then the underlying temporalis fascia muscle is split and dissected from the frontotemporal bone. Burr holes are placed, the craniotomy bone flap is removed at frontotemporal area, and the dura is cut in a cruciate manner (Fig. ...
Context 11
... of MCA (0.6 mm) emerging from Sylvian fissure is paramount for this procedure. The arachnoid overlying this cortical branch of the MCA is then microscopically opened. A 7 mm segment of M4 artery without branches is preferably chosen as the recipient, but any tiny branches arising from this segment can be coagulated and divided if necessary (Fig. 26.6). A jeweler-type bipolar is used. High-visibility background is placed under the M4 segment. Papaverine is intermittently instilled over the vessels to prevent spasm. Then the STA is temporarily occluded proximally and sectioned distally. The distal stump of the STA in the scalp is coagulated, and the STA is truncated to the proper ...
Context 12
... mean arterial pressure is raised, and then the M4 MCA branch is temporarily occluded with Sugita aneurysm clips. An arteriotomy is made in the M4 branch, removing an elliptical portion of the superior wall, and irrigated with heparinized saline. Anastomosis between an end of STA and the side of the MCA segment is done with 10-0 interrupted suture (Fig. 26.7). The Sugita clips are then removed, restoring flow. Flow in all vessels is confirmed with the intraoperative Doppler. An intraoperative indocyanine green (ICG) angiogram is performed by injecting 2 ml of ICG dye intravenously and visualizing the graft with the near-infrared camera on the ...

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Article
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Moyamoya disease is a rare progressive cerebrovascular disorder caused by blocked Supraclinoid Internal Carotid Arteries at the base of the brain in the basal ganglia area. It is a chronic and progressive condition. The word “Moyamoya” is Japanese which refers to a hazy puff of smoke or cloud due to the appearance of blood vessels like a puff of smoke in angiograms of people suffering from this disease. The exact etiology of Moyamoya disease remains unclear, but there seems to be genetic and acquired forms. The first sign of Moyamoya disease is usually stroke or recurrent Transient Ischemic Attacks (TIAs) also called “mini-strokes”. Some other symptoms may include brain hemorrhage, headaches, developmental delays, aneurysm, involuntary movements, problems with cognitive abilities, problems with the senses, seizures, hemiparesis, ischaemic stroke or hemorrhagic stroke. Despite the etiology of Moyamoya disease being unclear, there are certain factors which may increase the risk of getting the disease, like Asian ancestry, family history of Moyamoya disease, other medical conditions, being female and being young. Moyamoya disease has a very unclear etiology and pathogenesis. Moyamoya disease can be diagnosed using tests like Cerebral Arteriography, Magnetic Resonance Angiography (MRA), Magnetic Resonance Imaging (MRI), etc. There is no cure for Moyamoya disease, but can be treated using drugs and surgical procedures. Hence, extensive studies need to be conducted in order to better determine the exact pathophysiology of the disease and also to find more effective treatment options that would further improve the prognosis in patients with Moyamoya Disease.
Article
Background: Moyamoya disease is a progressive, occlusive arteriopathy of the intracranial vessels causing an increased risk of stroke. It often results in functional impairment and decrease in quality of life, both in the presence and absence of stroke. Revascularisation is the accepted treatment for patients with symptomatic moyamoya disease, preventing further stroke. In Leeds Children's Hospital we use the encephalo-duro-arterio-myo-synangiosis (EDAMS) technique to facilitate revascularisation. We aim to assess the quality of life outcomes of patients who have undergone operative intervention at our unit for moyamoya disease. Methods: Paediatric patients with operated moyamoya disease from Leeds Children Hospital between February 2009 and January 2019 were included. Patient awaiting primary surgery were excluded. Patients were contacted via telephone and the Paediatric Quality of Life Inventory 4.0 (PedsQL) questionnaire administered via parent proxy. Quality of life outcomes were assessed using the PedsQL questionnaire score, which measures physical, emotional social and school functioning domains. This score was then converted in to a health-related quality of life (HRQOL) score. Scores in each domain and mean scores were compared to normative data. Results: This study included 11 children, 5 male, median age 6.8 years (range 22 months to 15.5 years) and 9 children underwent bilateral operations. Mean parent proxy PedsQL score was 66 (range 12.5-98.4), with a mean score of 61.9 in physical function, 88.9 in emotional function, 70.9 in social function and 58.7 in school function. This was lower than healthy controls overall and in each individual domain except emotional function which was similar to normative data. Conclusions: Children with moyamoya disease have a lower quality of life than healthy controls within this series. This suggests that children with moyamoya should be offered additional psychosocial support within the community.