Intra-abdominal hypertension (IAH) and abdominal compartment syndrome (ACS) are continuation of the same pathological and physiological processes that are largely unrecognized in critical patients. From an era of indistinct definitions and recommendations, this condition has been studied extensively and experts have come forward with clear definitions and recommendations for management. IAH is graded in four grades and ACS is IAH above 20 cm H2O with new organ dysfunction. IAH/ACS can present as acute, hyperacute, or chronic and aetiologically can be classified into primary, secondary and tertiary. It affects various body systems including respiratory, cardiovascular, central nervous, gastrointestinal, renal and hepatic systems adversely and results in deleterious consequences. Management of IAH/ACS is based on the evacuation of intra-luminal and extra-luminal contents, improving the abdominal wall compliance. There are various surgical techniques recommended for preventing the development of IAH/ACS and mitigating the negative consequences. New medical therapies such as octreotide, tissue plasminogen activator, melatonin and vitamin C are being investigated and non-pharmacological methods such as continuous negative abdominal pressure (CNAP) have been introduced recently but are still experimental and not recommended for routine use.
Part of the book: Intensive Care
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