• Journal of Chest Surgery
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• v.36 no.10
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• pp.772-775
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• 2003

Moyamoya disease is an unusual cerebrovascular disorder characterized by occlusive intimal dysplasia of the distal internal carotid and proximal cerebral arteries, but the etiology remains unclear. Angiographic characteristics include bilateral stenosis or occlusion of the terminal portions of the intracranial internal carotid arteries and bilateral development of fine collateral vessels at the base of the brain known as ‘Moyamoya vessels’. Cardiac surgery using cardiopulmonary bypass due to coronary artery disease and others among patients with moyamoya disease is very rare, and cardiac surgery for such patients has a potential risk of intraoperative and perioperative brain ischemia. We successfully treated a patient who underwent artrial septal defect closure and coronary artery bypass graft using the cardiopulmonary bypass, so we report this case with a brief literature review.

• Journal of Chest Surgery
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• v.35 no.3
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• pp.231-234
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• 2002

Moyamoya disease is an unusual cerebrovascular disorder characterized by occlusive intimal dysplasia of the distal internal carotid and proximal cerebral arteries as well as other collateral arteries. However, moyamoya diseases are recently being reported as a systemic process. We experienced one case of coronary artery occlusive disease affected by moyamoya disease. The patient was a 35-year-old female, experiencing intermittent NYHA class ll dyspnea and exertional chest pain for 6 months and right paresthesia for 1 month before admission. Cerebral artery angiogram showed abnormal cerebrovascular systems and confirmed moyamoya disease with cerebral infarction of the left frontal lobe. In coronary artery angiogram, left coronary artery was not visualized due to total occlusion of the left main ostium and left coronary blood flow was supplied from normal right coronary artery. CABG was performed with OPCAB. Both internal mammary arteries were used for LAD and LCx. Intraoperative coronary artery findings showed intimal hyperplasia and no definite thrombi, and nondiseased coronary arteries were good and patent. We concluded that this patient's coronary artery disease was affected by moyamoya disease, and moyamoya disease should be evaluated in the extracerebral cardiovascular system.

• Childhood Kidney Diseases
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• v.11 no.2
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• pp.294-298
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• 2007

Moyamoya disease is a progressive cerebrovascular disorder with stenosis or occlusion of the bilateral internal carotid arteries with abnormal vascular networks at the base of the brain. Previous reports have shown that there are extracranial vascular involvements in Moyamoya disease, especially in the renal artery. We report a 7-year-old patient with Moyamoya disease associated with renovascular hyper tension, who presented in infancy with seizures and hemiparesis. Renal angiography showed multiple stenoses of the right renal artery. Although renal artery stenosis in Moyamoya disease has been effectively treated with balloon angioplasty, stent implantation, or surgery, bat-loon angioplasty could not be done in this patient due to multiple stenoses. His blood pressure was successfully controlled with medical treatment, and remained normotensive during the follow up period of 6 months.

• Child Health Nursing Research
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• v.21 no.2
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• pp.123-130
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• 2015

Purpose: The purpose of this study was to understand the intellectual and psychological features of children with Moyamoya disease who were patients in the Department of Pediatric Neurosurgery of Severance Hospital. Methods: From December 2010 through December 2012, 63 patients with diagnoses of Moyamoya disease and 59 children in a normal group were enrolled. This study was conducted using the Korean-Wechsler Intelligence Scale for Children, Rey-Kim Memory Test for children, Korean Child Behavior Checklist and Pediatric Quality of Life Inventory$^{TM}$4.0. Results: The results showed that the intellectual and psychological profiles of children with Moyamoya disease were lower than the average of the normal control group. The tested patients showed significantly lower scores for Performance Intelligence Quotient cognition level. Also, in terms of quality of life, children with Moyamoya disease had lower levels of physical and school functionality. The results were in line with those of previous studies involving psychological tests of children with chronic diseases. Conclusion: Considering the intellectual and psychological characteristics of children with Moyamoya disease, integrated psychological intervention plans including elements such as supportive therapy for patients and programs for parental education are required.

• Childhood Kidney Diseases
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• v.17 no.2
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• pp.143-148
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• 2013

Neurofibromatosis type 1 (NF-1) is an autosomal dominant neurocutaneous disorder, which can affect different organs or systems of the body, including the cardiovascular system. One of the more serious aspects of the disease relates to arterial involvement. In particular, renal artery stenosis is one of the most common vascular abnormalities in patients with NF-1, and the manifestations vary, ranging from no symptoms to end-stage renal failure. Treatment usually consists of antihypertensive drugs, percutaneous transluminal angioplasty, or surgery. Other causes of hypertension should be ruled out and the patient followed up for close monitoring and proper management. We report a case of bilateral renal artery stenosis and hypertension in a patient with moyamoya disease associated with neurofibromatosis type 1. This report discusses the literature available on the current subject, its clinical features, diagnosis, and treatment.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.15 no.1
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• pp.40-44
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• 2019

Moyamoya disease (MMD) is a chronic, occlusive cerebrovascular disease of unknown etiology characterized by progressive stenosis at the terminal portion of the internal carotid artery and an abnormal vascular network at the base of the brain. The clinical presentations of MMD include transient ischemic attacks (TIA), ischemic stroke, hemorrhagic stroke, seizures, headache, and cognitive impairment. MMD is the most important cause of stroke or TIA in children in East Asian countries. A 5-year-3-month old boy with MMD experienced cerebral infarctions five times. Cerebrovascular anastomosis surgery was performed on him four years ago. He had dysphagia, developmental delay, hemiplegia, and strabismus. Besides, a number of dental caries in primary dentition were identified during clinical oral examination. Dental treatment under general anesthesia using sevoflurane was performed due to his lack of cooperation and underlying systemic disease. MMD is associated with various medical diseases requiring thoughtful consideration during dental treatment. Crying and hyperventilation in MMD patients may cause hypocapnia and have a cerebral vasoconstrictive effect. If dental treatment is required, control of pain and anxiety is very important. General anesthesia may be considered for dental treatment in uncooperative or very young patients with MMD.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.14 no.2
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• pp.142-149
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• 2014

Purpose: There are 15 types of Glycogen storage disease (GSD) that have been identified, and GSD type Ia is the most common type. There are several studies of Korean GSD type Ia patients' long-term complications. The aim of this study to find out clinical symptoms and prognosis of GSD type Ia patients. Methods: We performed clinical, biochemical and genetic analysis retrospectively on five patients diagnosed with GSD type Ia in a Soonchunhyang University Hospital from July 2002 to July 2014. Results: All patients had hepatomegaly at diagnosis, and they were all confirmed to have fatty liver at abdomen USG. They had no developmental delay, but two of them had growth retardation. Elevated blood lactate, triglyceride, and uric acid levels can find out all patients, but only one patient had hypoglycemia. They are diagnosed with GSD through gene analysis, and by gene analysis, they have c.648G>T (homozygote, splicing mutation), c.122G>A/c.648G>T, c.248G>A/c.648G>T mutations. Treatment with three times meals, three times snacks and four to six times use of uncooked constarch for all patients. Following the progress, one of them resulted in hypothyroidism, other one had renal stones. A patient diagnosed at 16 years old had liver cirrhosis and started having hemodialysis for ESRD. Conclusion: GSD type Ia patients had hepatomegaly, hyperlipidemia, hyperuricemia, and lactacidemia. Therefore patients who have such these symptoms are recommended gene analysis. A patient diagnosed at 16-years-old had liver cirrhosis and ESRD in progress, early diagnosis and treatment are important for GSD type Ia patients.