HYDROCEPHALUS

STORY ABOUT HYDROCEPHALUS

This is also my story from August 1959, I was still posted in the septic labour ward, and the ward was where the women who were neglected in labour outside the hospital came in for further care and management.  Sarla a beautiful 19 year old was one such woman. She never had any care for her pregnancy. She had been in labour for two days; her waters had also broken two days ago. The baby was not coming, her Doula looking after her asked her to go to the big hospital as she was unable to do anything for her. It was early morning when she arrived, I was on duty, when I saw her, she appeared to be a fit young woman. I was unable to hear the foetal heart sounds therefore I assumed that the baby was gone. At that time we had no sonic aids, CTG, machines or any x-ray facilities.

On abdominal examination it felt like a big head when I did a pelvic examination, I felt a tense bag of fluid. I was not sure if this could be a hydrocephalic head or a bag of membranes, which may not have ruptured. (Please do not forget that I had graduated only few days ago)

I requested the nurse, for A lumber puncture needle (a long fine needle used to do a spinal puncture). With this needle I punctured the tense bag of fluid. With a great rush, lots of fluid and a baby came out hitting me on my chest. Obviously it was a hydrocephalic head.

In the human brain there are spaces which are fluid filled called the Ventricles. The spaces in the Heart are also called the ventricles.

There is a set of four ventricles in the brain, they are all connected with each other and a central canal of the spinal cord. The fluid that flows thorough these canals is called cerebrospinal fluid (for short CSF). Of these, there are two lateral ventricle right and left on each side of the front part of the brain, there is a third ventricle in the middle part of the brain which is connected to the lateral ventricles by a small opening called ,Foramen  of  Monro . The fourth ventricle lies in the posterior part of the brain. It is connected to the third ventricle by a very narrow channel called aqueduct of sylvius.

Besides these, there are other spaces called cisterns and foramens. All these together allow the free flow of CSF.

In each ventricle there is a network of specialised blood vessels with special cells called ependymal cells. It is the choroid plexus which produce two thirds of CSF the rest is produced by the lining of the ventricles and a special space around the brain called subarachnoid space.

CSF contains the same amount of sodium like blood . It has much less protein. Its osmolarity is the same as the blood. 500 ml of CSF is produced each day. It constantly moves all the time, 3.7 times each day, the resultant fluid present at any one time is 100 to 160 mls

CSF serves very important functions for the brain. It keeps the buoyancy of the brain, weight of the brain is 1400 gms, however when it floats in the CSF it records only 25 gms. It protects the brain from injury during sport and accidents. It also maintains the chemical stability of the brain and protects it from infections.

It protects the brain from ischemia, if by chance the CSF volume  drops CNS pressure drops, it then sets up a blood flow. Besides all this CSF supplies the nutrition to the brain and removes waste products and toxins. It also acts as basic immunological protector

It seems that normal head and CSF is very important for the normal human life and function. After all the brain is the band master of the body orchestra. So why we get hydrocephalus? And how do we deal with it. Again this means too much water(hydro)around the head(cephalus)

Congenital hydrocephalus means hydrocephalus present at birth. It is not an inherited disorder; it only means it is present at birth. It usually happens if the CSF cannot move normally and one of the passages is blocked. This often happens if the baby is born with other brain abnormalities. These together are called neural tube defects. One of the commonest one is spinabifida others are less common such as hydrancephaly .

This can happen if the

1) Foetus has a haemorrhage

2) Aqueductal stenosis

3) Blocking of the cisterns or foramina

4) Neural tube defects encephaocoele

5) Infections in the mother, Toxoplasmosis or syphilis

6) Tumours in the foetus or baby

7) Genetic abnormalities

So obviously the flow of the CSF is obstructed or even too much is being produced. At the time when I had a demised hydrocephalic foetus we had no tests not even a post-mortem to see if any abnormalities where present.

Now of course you can suspect a hydrocephalic head very early in pregnancy, and also look for any other abnormalities.

Now we do a chromosome analysis. These are found in 20% of cases of hydrocephalus. Further assessment can be done by MRI. If the problem is complicated a termination of pregnancy is offered. If it is isolated hydrocephalus, progress of ventricles is monitored. Delivery is done at a place where there are facilities for neonatal intensive care. Surgical drainage is generally done for the blocked ventricles. Foetal shunting has been attempted, but needs further  improvement . Mothers Needs to be advised to take 400 micrograms of folic acid when they want to get pregnant again.

The above photo is by ultrasound. It is floating choroid plexus and dilated ventricles.