Moyamoya disease: a review of the disease and anesthetic management

Moyamoya disease is a rare chronic cerebrovascular disease seen both in children and adults. It has a progressive course, but may have a variable clinical presentation. The disease causes ischemic stroke, intracranial hemorrhage, headache, seizures, and transient ischemia attack in children and in adults. Although the pathogenesis of the disease remains unknown, research suggests a genetic predisposition. There are also undefined systemic processes involved in this vasculopathy. Better noninvasive diagnostic techniques for diagnosis of the Moyamoya disease have been developed, but medical treatment can still be challenging. However, various surgical revascularization procedures have shown to provide symptomatic benefit in a majority of these patients. In addition, the anesthetic management of these patients has evolved over the years with an increased understanding of the disease. These have specifically resulted from the identification of risk factors for perioperative complications and outcomes related to the use of anesthetic agents. Finally, research in the last 3 decades has led to the recognition of the importance of pain control, the increased use of regional anesthesia, and better monitoring techniques in providing high quality and safe patient care to patients with Moyamoya disease. This article will provide a comprehensive review of the disease and its anesthetic management.     

Huntington''s disease: review and anesthetic case management.

Huntington's disease is a dominantly inherited progressive autosomal disease that affects the basal ganglia. Symptoms appear later in life and manifest as progressive mental deterioration and involuntary choreiform movements. Patients with Huntington's disease develop a progressive but variable dementia. Dysphagia, the most significant related motor symptom, hinders nutrition intake and places the patient at risk for aspiration. The combination of involuntary choreoathetoid movements, depression, and apathy leads to cachexia. Factors of considerable concern to the anesthesiologist who treats patients with Huntington's disease may include how to treat frail elderly people incapable of cooperation, how to treat patients suffering from malnourishment, and how to treat patients with an increased risk for aspiration or exaggerated responses to sodium thiopental and succinylcholine. The successful anesthetic management of a 65-yr-old woman with Huntington's disease who presented for full-mouth extractions is described.     

Moyamoya disease associated with Graves disease: special considerations regarding clinical significance and management

OBJECT: In moyamoya disease (MMD), ischemic events are usually precipitated by activities associated with hyperventilation or physical strain. The authors report on four patients with a rare combination of Graves disease-associated thyrotoxicosis and MMD, whose cerebrovascular ischemic events occurred while in a thyrotoxic state. The clinical correlation between MMD and Graves' thyrotoxicosis, and outcome after surgical intervention are described. METHODS: Four young women, ages 22 to 25 years, presented with profound cerebrovascular ischemic accidents. They had clinical and radiological features consistent with the diagnosis of MMD and were in the active thyrotoxic state of Graves disease. To prevent a future ischemic event, patients underwent superficial temporal artery-middle cerebral artery anastomosis combined with encephalomyosynangiosis or encephaloduroarteriosynangiosis after normalization of their hormonal conditions. All patients have been neurologically stable since revascularization procedures and lead a normal daily life. In patients with MMD, cerebrovascular ischemic events may be precipitated by thyrotoxicosis. One possible pathomechanism of cerebrovascular ischemic aggravation in the thyrotoxic state may be a hemodynamic compromise induced by an excessive increase in the cerebral metabolism and oxygen demand over the compensation of the cerebral blood flow deficit through collateral supply in MMD. CONCLUSIONS: Surgical revascularization after optimal control of thyrotoxicosis is thought to be an appropriate treatment in patients with MMD concurrent with Graves disease for the prevention of further ischemic events, especially in those with impaired cerebral perfusion and cerebral ischemic symptoms.     

Moyamoya disease

A 4 years old boy presented with acute left hemiplegia. Preliminary neuroimaging suggested an arterial ischemic process. Clinical and laboratory evaluation excluded haematologic, metabolic and vasculitic causes. Cerebral angiography confirmed the diagnosis of Moyamoya disease. Treatment included physiotherapy and close follow-up for recurrence.     

Hypertension in children with chronic kidney disease: pathophysiology and management

Arterial hypertension is very common in children with all stages of chronic kidney disease (CKD). While fluid overload and activation of the renin–angiotensin system have long been recognized as crucial pathophysiological pathways, sympathetic hyperactivation, endothelial dysfunction and chronic hyperparathyroidism have more recently been identified as important factors contributing to CKD-associated hypertension. Moreover, several drugs commonly administered in CKD, such as erythropoietin, glucocorticoids and cyclosporine A, independently raise blood pressure in a dose-dependent fashion. Because of the deleterious consequences of hypertension on the progression of renal disease and cardiovascular outcomes, an active screening approach should be adapted in patients with all stages of CKD. Before one starts antihypertensive treatment, non-pharmacological options should be explored. In hemodialysis patients a low salt diet, low dialysate sodium and stricter dialysis towards dry weight can often achieve adequate blood pressure control. Angiotensin-converting enzyme (ACE) inhibitors and angiotensin receptor blockers are first-line therapy for patients with proteinuria, due to their additional anti-proteinuric properties. Diuretics are a useful alternative for non-proteinuric patients or as an add-on to renin–angiotensin system blockade. Multiple drug therapy is often needed to maintain blood pressure below the 90th percentile target, but adequate blood pressure control is essential for better renal and cardiovascular long-term outcomes.     

非停跳冠状动脉搭桥术病生理改变及临床监测

非停跳冠状动脉搭桥术(OPCABG)因其避免了体外循环的不良反应,在临床上得到了广泛应用.术中心脏位置的变化和应用心脏固定器,会引起显著的血流动力学波动、心肌缺血等病生理改变,进而导致心室功能障碍.近年的研究表明,心室舒张功能障碍和右心功能不全是引起血流动力学变化的重要因素.临床上多采用肺动脉导管(PAC)和经食道超声心动图(TEE)来监测术中血流动力学和心功能的变化.新型的PAC能够将容量监测和压力监测结合起来,拓展了其监测范围和功能;而TEE在术中辅助诊断和监测的可靠性、有效性得到了广泛认可.心室压力一容量环作为一种非容量依赖性的方法,在监测心功能方面越来越受到重视.     

Gastroparesis: pathophysiology and management

The prevalence of gastroparesis is increasing. Diabetes mellitus and sequelae of ?sogastric surgery are the two main causes of gastroparesis. In some patients, gastroparesis seems a postinfectious disease after its sudden onset after a viral infection. In about one third of the patients, gastroparesis is considered as idiopathic. In diabetic patients, gastroparesis impairs glycaemic control. Due to the low positive predictive value of symptoms, a gastric emptying study is often necessary to confirm a suspected diagnosis of gastroparesis. The symptomatic efficacy of erythromycin is higher than that of other prokinetics. This efficacy is higher when erythromycin is given intravenously. Hyperglycaemia impairs this symptomatic effect. Due to a tachyphylaxis phenomenon, the clinical effect of erythromycin decreases with the duration of treatment. In refractory gastroperis, either duodenal or jejunal enteral feeding, or high-frequency gastric electrical stimulation are possible therapeutic options while endoscopic alternatives (intrapyloric botulinum injection or pyloric balloon dilation) give unsatisfactory results.     

Moyamoya disease and anesthesia

Moyamoya disease is a condition that results from bilateral stenosis or obstruction of the intracranial arteries at the base of the brain. Patients exhibit ischemic symptoms, and vascular reconstruction is the therapy of choice. Surgical treatment for Moyamoya disease is often complicated by cerebral ischemia, so the goal in perioperative management is to maintain the balance between oxygen supply and demand in the brain. This report presents three cases of Moyamoya disease in patients under 3 years of age, and discusses anesthesia management issues for pediatric patients with this condition.     

Moyamoya disease - a review of clinical experience and anaesthetic management

Moyamoya disease is a rare neurovascular condition that affects both children and adults. Increasingly these patients present for surgical management to improve the cerebral circulation since medical therapy is essentially ineffective. Because of the precarious cerebral circulation, these patients represent an anaesthetic challenge. In this report we review the pathophysiology of moyamoya disease, summarize our experience with seven patients and discuss the anaesthetic management.     

Moyamoya disease: recent progress and outlook

Moyamoya disease as a clinical entity has been known for more than 40 years. Constant efforts have been directed at clarifying the pathogenesis of this disorder and improving therapeutic methods for the ischemic and hemorrhagic stroke caused by the characteristic vasculopathy of this disease. Although much knowledge has been gained, unresolved problems remain, such as the true epidemiology of this disease, elucidation of the genetic mechanism, and prevention of repeated hemorrhagic events. In this paper, we review recent progress and discuss the outlook for this disorder.     

Moyamoya disease.

A case of Moyamoya disease is discussed, presenting in a young adult with subarachnoid hemorrhage. Moyamoya is a rare form of crebro-vascular occlusive disease, characterized by specific arteriographic findings. The etiology of the disorder is unknown.     

Pulmonary hypertension: pathophysiology, diagnosis, treatment and anesthetic considerations

Pulmonary artery hypertension is a rare entity but one that presents genuine challenges during anesthesia mainly because of the high risk of exacerbation and right heart failure during and after surgery. This review covers currently available treatments, the battery of diagnostic procedures at our disposal, and the basic precepts for the perioperative management of patients with this condition. Adequate hemodynamic control and the early use of selective pulmonary vasodilators are absolutely essential.     

Ejaculatory disorders: pathophysiology and management

Ejaculatory dysfunction (EjD) is one of the most common male sexual disorders, yet EjD is still frequently misdiagnosed or overlooked as a result of numerous patient and physician barriers. The wide spectrum of EjD ranges from premature or rapid ejaculation, through delayed ejaculation, to a complete inability to ejaculate--otherwise known as anejaculation--and includes retrograde ejaculation and painful ejaculation. Conventional algorithms for managing ejaculatory disorders are based either on an organic or psychogenic etiology, with the latter more traditionally considered the main cause. This paper reviews physiopathological, diagnostic and therapeutic aspects of ejaculation disorders, with a particular focus on the most prevalent disorder, premature ejaculation.     

Moyamoya disease and Moyamoya syndrome: experience in Europe; choice of revascularisation procedures

Objectives. To present our clinical experience in terms of disease presentation and choice of revascularisation procedure in our first group of 23 European Moyamoya angiopathy (disease and syndrome) patients. Method and patient selection. Twenty three patients were diagnosed and treated from 1997–2001 in our neurosurgical department. All patients underwent preoperative angiography, colour Doppler examination, cranial MRI and/or CT scans, HMPAO-SPECT or H ₂ ¹⁵O PET (baseline and Diamox challenge) scans.Nineteen patients presented with child-juvenile Moyamoya angiopathy with an average age at presentation of 8 years (range 1–17 years), in 4 patients adult Moyamoya was diagnosed with a mean age at presentation of 34 years (range 23–40 years). Results. In all but one patient bilateral affection was present at the time of diagnosis. All patients underwent direct and/or additional indirect revascularisation procedures. Twenty-one patients underwent bilateral revascularisation procedures. In two patients a unilateral procedure was performed.A direct STA-MCA bypass (superficial temporal artery branch – middle cerebral artery branch anastomosis) was performed in all patients. Additional direct STA-ACA bypass (STA – anterior cerebral artery branch anastomosis) was performed in 10 patients. Indirect arteriosynangiosis using the occipital artery was performed in the posterior cerebral artery (PCA) territory in 3 patients. The frontal branch of the STA was used for arteriosynangiosis in the frontal region in another 2 patients. Indirect frontal durasynangiosis was performed in 14 patients. The number and location of multiple revascularisation procedures was determined according to the angiographic findings as well as the site of decreased perfusion reserves seen on H ₂ ¹⁵O PET. A one stage revascularisation procedure was performed in 7 patients. Fourteen patients underwent two stage procedures. Following operation no complications were encountered in all but one adult patient who died postoperatively due to a massive middle cerebral artery infarct on the nonoperated side. Conclusions. As territorial hemodynamic disorder seems to be a characteristic in Moyamoya disease and/or syndrome, judging from our experience with European patients in our series, and several reports hitherto, treatment with multiple revascularisation procedures is considered to be justified.