Moyamoya disease (MMD) is a rare disease affecting the cerebral vasculature of the central nervous system (CNS) with a reported incidence of 0.35–0.94 per 100,000 populations. It was first reported from Japan and later from other parts of the world. The pathology is narrowing of blood vessels supplying anterior circulation and rarely posterior circulation. It was believed that the disease is genetic in origin, but environmental factors also play a role. Patients with this rare disease may present with ischemic or hemorrhagic symptoms. Ischemic symptoms account for the disease in most of the pediatric patients, whereas in adults, hemorrhage is more common. Diagnostic imaging like CT angiogram and magnetic resonance angiogram helps in demonstrating the narrowing or the collateral vessels like “a puff of smoke” (moyamoya) formed at the base of the brain. Moyamoya disease is treated medically and/or surgically. Aspirin is the main medication used. Surgical options are direct or indirect revascularization techniques to bypass the stenosis. The disease is progressive in majority of the patients, but if treated early, they can have good prognosis especially children.
Part of the book: Vascular Malformations of the Central Nervous System
Gastrointestinal endoscopy has become fundamental procedure for diagnosis and treatment of gastrointestinal tract diseases. Generally, the gastrointestinal endoscopy is minimally invasive procedure. However, it can cause considerable amount of discomfort and pain which make the procedure unsafe, complicated and refusal of follow up procedures if done without safe sedation. The sedation is required to alleviate anxiety, provide analgesia, amnesia and to improve endoscopic performance specifically in therapeutic procedures. The safe administration of sedative and analgesic medications, irrespective of the regimen used, requires knowledge of the individual needs of patients. The combination of benzodiazepines and opioids is now the most widely used sedation regimen for sedation in gastrointestinal endoscopic procedures. Generally, sedation for gastrointestinal endoscopy is considered safe, however, it has the potential for serious complications. Therefore, endoscopist should assess the patients properly before the endoscopy as well as should be aware of all possible complications and the risk factors. Furthermore, skilled staff and emergency equipment should be available in endoscopy suit. This chapter discuss in details all the aspects of safe procedural sedation during GI endoscopies.
Part of the book: Esophagitis and Gastritis