The recent case of Roona Begum, an 18 months old Tripura girl suffering from a rare illness that has caused her head to swell to more than double its normal size is talk of the town in the Medical fraternity. Doctors across India have expressed amazement at this rare medical condition. This particular illness is termed as `Hydrocephalus` or in a layman’s language commonly termed as ` water on the brain`. This water is basically the cerebrospinal fluid (CSF) which serves important functions:
-It protects the brain from damage
-It removes waste products from the brain
-It provides the brain with the nutrients it needs to function properly
Hydrocephalus affects about 1 out of every 500 children. Here we bring before you all that you need to know about this disease.
What is Hydrocephalus?
Hydrocephalus is a brain condition which occurs when cerebral spinal fluid is unable to drain from the brain. It then pools, causing a backup of fluid in the skull. This may cause increased intracranial pressure inside the skull and progressive enlargement of the head, convulsion, tunnel vision, and mental disability and ultimately death in many cases.
The causes of hydrocephalus are still not well understood. Hydrocephalus may result from inherited genetic abnormalities or developmental disorders. In most cases, the exact cause of this condition is difficult to get detected. Some kids can develop hydrocephalus without a known cause.
In other cases, this disease can occur if a blockage exists in any of the brain ventricles, the cerebral spinal fluid backs up and causes an excess of fluid in the brain. This accumulation of fluid can also happen when the choroid plexus (the area of the brain that produces CSF) is in overdrive or if the fluid fails to be properly absorbed by the bloodstream.
Acquired hydrocephalus is caused by cranial hemorrhage or bleeding in the brain. This can happen in premature babies or kids who`ve undergone traumatic head injuries.
Types of hydrocephalus
There are three main types of hydrocephalus:
Congenital hydrocephalus :Hydrocephalus that`s present at birth caused by birth defects, such as spina bifida, or as a result of an infection that the mother develops during pregnancy, such as mumps or rubella.
Acquired hydrocephalus :Hydrocephalus that develops after birth, usually after an injury or illness.
Normal pressure hydrocephalus: Hydrocephalus that usually only develops in older people and affects people over 50 years old. It can sometimes develop after an injury or a stroke, but in most cases the cause is unknown.
Hydrocephalus is easy to detect. The most obvious indication of hydrocephalus in children and infants is often a rapid increase in head circumference or an unusually large head size. The head swells to accommodate the excess fluid. The cerebral spinal fluid builds up in the central nervous system, causing the fontanelle (soft spot) to bulge and the head to be larger than expected.
Other than the obvious physical signs other symptoms may also include:
Changes in facial appearance and eye spacing, eyes that appear to gaze downward
Irritability and Seizures
Sleepiness, Headache and Vomiting
Changes in personality, memory, or the ability to reason or think which leads to irritability, poor temper control and loss of coordination and trouble walking
Slow or restricted movement
Hydrocephalus is diagnosed through clinical neurological evaluation and by using cranial imaging techniques such as ultrasonography, computed tomography (CT), magnetic resonance imaging (MRI), or pressure-monitoring techniques. The appropriate diagnostic tool is decided by the doctor based on an individual`s age, clinical presentation, and the presence of known or suspected abnormalities of the brain or spinal cord.
Hydrocephalus is most often treated by surgically inserting a shunt system. Shunt procedures involve surgically implanting one end of a catheter (flexible tube) into a ventricle of the brain and placing the other end in the abdominal cavity, chambers of the heart, or space around the lungs where fluid is drained and absorbed by the bloodstream. A valve in the shunt system regulates flow to prevent over-draining and under-draining.
Though touted as the most effective treatment for this disease, Shunt system has chances of failure and complications. Symptoms can recur again and the child will have to undergo surgery to correct the problem.
An alternative treatment for obstructive hydrocephalus in selected cases is the Endoscopic third ventriculostomy in which 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.
Thus timely, effective and proper treatment can enable kids with hydrocephalus go on to lead normal lives. Also intensive research is being led into this direction. Much of this research focuses on finding better ways to prevent, treat, and ultimately cure such rare disorder and hopefully Medical Science will come up with a reliable and guaranteed cure for it soon.
Compiled by: Ritu Singh