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Hydrocephalus


Hydrocephalus[1], also known as Water on the Brain, is a medical condition. People with hydrocephalus have an abnormal accumulation of cerebrospinal fluid (CSF) in the ventricles, or cavities, of the brain. This may cause increased intracranial pressure inside the skull and progressive enlargement of the head, convulsion, and mental disability. Hydrocephalus can also cause death.


Hydrocephalus was first described by the ancient Greek physician Hippocrates, but it remained an intractable condition until the 20th century, when shunts and other neurosurgical treatment modalities were developed. It is a lesser-known medical condition; relatively small amounts of research are conducted to improve treatments for hydrocephalus, and to this day there remains no cure for the condition.


Hydrocephalus affects both pediatric and adult patients. According to the NIH website, there are an estimated 700,000 children and adults living with hydrocephalus.


Pediatric hydrocephalus affects one in every 500 live births,[2] making it one of the most common developmental disabilities, more common than Down syndrome or deafness.[3] It is the leading cause of brain surgery for children in the United States. There are over 180 different causes of the condition, one of the most common acquired etiologies being brain hemorrhage associated with premature birth. Pediatric hydrocephalus may also be a heritable condition and runs in certain families mostly affecting boys.


One of the most performed treatments for hydrocephalus, the cerebral shunt, has not changed much since it was developed in 1960. The shunt must be implanted through neurosurgery into the patient's brain, a procedure which itself may cause brain damage. An estimated 50% of all shunts fail within two years, requiring further surgery to replace the shunts. In the past 25 years, death rates associated with hydrocephalus have decreased from 54% to 5% and the occurrence of intellectual disability has decreased from 62% to 30%.


Hydrocephalus is usually due to blockage of cerebrospinal fluid (CSF) outflow in the ventricles or in the subarachnoid space over the brain. In a person without hydrocephalus, CSF continuously circulates through the brain, its ventricles and the spinal cord and is continuously drained away into the circulatory system. Alternatively, the condition may result from an overproduction of the CSF fluid, from a congenital malformation blocking normal drainage of the fluid, or from complications of head injuries or infections.[4]


Compression of the brain by the accumulating fluid eventually may cause convulsions and mental retardation. These signs occur sooner in adults, whose skulls no longer are able to expand to accommodate the increasing fluid volume within. Fetuses, infants, and young children with hydrocephalus typically have an abnormally large head, excluding the face, because the pressure of the fluid causes the individual skull bones which have yet to fuse to bulge outward at their juncture points. Another medical sign, in infants, is a characteristic fixed downward gaze with whites of the eyes showing above the iris, as though the infant were trying to examine its own lower eyelids.[5] Hydrocephalus occurs in about one out of every 500 live births[2] and was routinely fatal until surgical techniques for shunting the excess fluid out of the central nervous system and into the blood or abdomen were developed. Hydrocephalus is detectable during prenatal ultrasound examinations.


Usually, hydrocephalus does not cause any intellectual disability if recognized and properly treated. A massive degree of hydrocephalus rarely exists in typically functioning people, though such a rarity may occur if onset is gradual rather than sudden.[6]Spontaneous intracerebral and intraventricular hemorrhage with hydrocephalus shown on CT scan[7]


The elevated intracranial pressure may cause compression of the brain, leading to brain damage and other complications. Conditions among affected individuals vary widely. Children who have had hydrocephalus may have very small ventricles, and presented as the "normal case".


If the foramina (pl.) of the fourth ventricle or the cerebral aqueduct are blocked, cereobrospinal fluid (CSF) can accumulate within the ventricles. This condition is called internal hydrocephalus and it results in increased CSF pressure. The production of CSF continues, even when the passages that normally allow it to exit the brain are blocked. Consequently, fluid builds inside the brain causing pressure that compresses the nervous tissue and dilates the ventricles. Compression of the nervous tissue usually results in irreversible brain damage. If the skull bones are not completely ossified when the hydrocephalus occurs, the pressure may also severely enlarge the head. The cerebral aqueduct may be blocked at the time of birth or may become blocked later in life because of a tumor growing in the brainstem.


Internal hydrocephalus can be successfully treated by placing a drainage tube (shunt) between the brain ventricles and abdominal cavity to eliminate the high internal pressures. There is some risk of infection being introduced into the brain through these shunts, however, and the shunts must be replaced as the person grows. A subarachnoid hemorrhage may block the return of CSF to the circulation. If CSF accumulates in the subarachnoid space, the condition is called external hydrocephalus. In this condition, pressure is applied to the brain externally, compressing neural tissues and causing brain damage. Thus resulting in further damage of the brain tissue and leading to necrotization.


Hydrocephalus treatment is surgical. It involves the placement of a ventricular catheter (a tube made of silastic), into the cerebral ventricles to bypass the flow obstruction/malfunctioning arachnoidal granulations and drain the excess fluid into other body cavities, from where it can be resorbed. Most shunts drain the fluid into the peritoneal cavity (ventriculo-peritoneal shunt), but alternative sites include the right atrium (ventriculo-atrial shunt), pleural cavity (ventriculo-pleural shunt), and gallbladder. A shunt system can also be placed in the lumbar space of the spine and have the CSF redirected to the peritoneal cavity (Lumbar-peritoneal shunt). An alternative treatment for obstructive hydrocephalus in selected patients is the endoscopic third ventriculostomy (ETV), whereby a surgically created opening in the floor of the third ventricle allows the CSF to flow directly to the basal cisterns, thereby shortcutting any obstruction, as in aqueductal stenosis. This may or may not be appropriate based on individual anatomy.

By definition, hypertension is present when there is a persistent elevation of either systolic blood pressure (>140 mm Hg) or diastolic blood pressure (>90 mm Hg), or when a patient is taking antihypertensive medication (regardless of the blood pressure level). The blood pressure level correlates directly with the magnitude of risk for clinical sequelae such as premature death, stroke, myocardial infarction, congestive heart failure, renal insufficiency, dementia, and peripheral vascular disease. Table 1 in Chapter 77 displays the blood pressure classification from the fifth report of the Joint National Committee on the Detection Treatment and Evaluation of High Blood Pressure expert panel. This scheme determines hypertension stage according to the highest pressure-either systolic or diastolic. The preponderance of epidemiological data suggests that systolic, not diastolic, blood pressure is the primary determinant of pressure-related risk.
 
Rational for Treatment


A compelling case can be made for pharmacological treatment of elevated blood pressure. Pharmacological blood pressure lowering reduces the risk of premature cardiovascular morbid and fatal events as well as all-cause mortality. Antihypertensive drug therapy has also been shown to prevent the gradual progression of mild hypertension to more severe elevations of blood pressure. The prevalence of pressure-related target-organ damage (i.e., elevated serum creatinine, left ventricular hypertrophy) is also greater at higher blood pressure levels. Hypertensives with pressure-related target-organ damage manifest a several-fold higher risk for pressure-related clinical complications at a given blood pressure level compared to hypertensives with similar levels of pressure without target-organ damage. Early treatment of hypertension favorably impacts long-term clinical risk, in part, by preventing the development of pressure-related target organ damage. It is not well appreciated that drug-treated hypertensive patients subjectively feel better (i.e., improved quality of life and fewer pressure-related symptoms) after successful blood pressure lowering. The evidence in support of this thesis is compelling.
 
General Therapeutic Principles


Several important therapeutic principles should be considered in treating hypertensive patients. In most hypertensives there is little to be gained from the pursuit of rapid blood pressure control. The most important therapeutic goal for the vast majority is to prescribe a combination of appropriate lifestyle modifications (weight loss, salt and alcohol restriction, and increased physical activity) plus the lowest doses of drug(s) that allow blood pressure normalization over the long term. The erroneous clinical perception that diastolic blood pressure is the predominant pressure mediator of clinical risk contributes to physician hesitancy to intensify treatment to normalize systolic blood pressure once diastolic blood pressure has been lowered to less than 90 mm Hg. Many antihypertensive medications have dose-related side effects. Drug acquisition costs also usually increase at higher dose levels. In certain instances, cost is a major barrier to patient compliance with prescribed drug therapies. The clinician and nursing staff should routinely ask patients whether they can afford the prescribed medications. Drug acquisition costs are but one consideration, albeit an important one, in embarking on a hypertension disease management strategy



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