Years of training, painful moments of learning culminate in the making of any professional; more so in the field of neurosurgery. As with many other specialities of medicine, a neurosurgeon is often vexed with problems concerning life and death, treading a fine path between hope and utter despair. The decision taken on a particular moment can act as a verdict for his whole life. Decades are spent on mastering the art of performing a surgery, but often the best surgeon is one who knows when not to operate. It takes wisdom, strength and courage not to intervene. Any decision to operate should always weigh in the favour of benefit to the patient and his family against a possible harm. I would like to share a story where such a decision not to operate was taken, while working in a tertiary care institute.
Tanuja was a young mother from a remote village in West Bengal. She and her husband were peasants working in a paddy farm and were earning their livelihood with hard work. It was the happiest moment in their lives when she gave birth to her first baby. The delivery was completed with great difficulty with the help of a ‘village dai’. The birth it seems was complicated due to the large head of the baby. At the end of all pain and suffering she was extremely satisfied to see the face of her little one. The happiness was short lived as the elderly people found that the child was not as active as babies of that age, and the child would not cry loudly. She thought that everything will be okay and used to get comforted by the smile of her precious one. The baby was not holding the head even after 5 months and she was finding that the head was heavier, even difficult to hold. She felt that something was wrong but they had no medical facilities nearby and hence continued the care with some native medicines. By the time, the child was about one year old, his head seemed to increase in size more than his body and he had not even become prone. He used to cry a lot especially if somebody touched his head or turned him in the bed. After much persuasion from her, they decided to take the kid to a health centre about 100 kilometres from their home. The doctor diagnosed the child to have ‘water trapped inside the head’ and quickly referred to a higher centre for further investigation and treatment. They returned home as travelling to Kolkata and getting treated in a hospital was out of question for a poor peasant family who were already finding it difficult to meet the ends.
She returned home in despair but the couple had a resolve to save their earnings, so that they could give some treatment to their child. The child was so dependent on the mother and could hardly move without her help, because of his large, heavy head. His eyes were rolled down like sunset though the child recognised and smiled at her. She was very much bonded with the child and wound spend hours nursing him. The child was now about two years but could not sit or stand, could only cry to express his needs.
A boy in the neighbourhood who had come for vacation from his work place in Bangalore saw the plight of the child and started mobilising the villagers in order to help the child as possible. With great difficulty they managed to collect enough funds and they travelled up to Kolkata. They could meet a neurologist in a government medical college, who advised them to take an MRI scan. After much waiting in the hospital corridors, they could get an MRI done for the child, but they could not get admitted in the hospital due to shortage of beds. It was at this juncture that the neighbourhood boy who was working in Bangalore suggested them to travel to Bangalore for better treatment.
They reached Bangalore after travelling in a train for two days. It was extremely difficult to take care of the child during the journey as he would cry almost incessantly. After waiting in the queue for several hours, they reached the Neurosurgery OPD. I was destined to see the child brought by this family with lots of hope and a few medical records. The first question that came to my mind was why this family came to us so late. Is it a neglected child due to uncordial family relationships, a common scenario in the modern world? The boy translated the naïve Bengali dialect, the mother had expressed. I could read her anxiety, concern and finally her hope. The child was almost listless and skinny, he was hardly able to turn in the bed. It seemed that he was burdened by the weight of the head and winced when I palpated the soft head. The circumference of the head was almost that of an adult and he could not even look straight. The eyeball was deviated down, the ‘sunset sign’ and veins were prominent over the scalp. After seeing the MRI scan, I could quickly spot the water trapped in the brain known as ‘Hydrocephalus’ in the medical jargon and the thinned out brain. The brain which should have almost fully developed by this age was looking like a paper bag holding water. She asked me whether I could do anything to set the things right. She had been advised by somebody that there is some kind of ‘shunt surgery’ for this disease. As with most cases of neglected hydrocephalus, his prognosis was heart breaking.
I was perplexed for a while on how to convey the message that there is not much to be done at this stage. That the Brain of that child will not recover whatever surgery is performed. That draining the water in brain, by putting in a tube in the brain will lead to lot more complications like bleeding inside a decompressed brain with vacant space, giving more torture to the already distressed child. That there are limits to what medical science can offer. After much rumination, I reached the conclusion that I am not going to operate on this child. The hardest part was to convey the message to the mother who had come all her hope. But life is at times testing and you have to act on the best interests of the patient. “Primum non nocere” – (Firstly, do not harm) Hippocrates.
Cerebrospinal fluid (CSF) is the water that completely surrounds and bathes the exposed surfaces of the brain. It supports, cushions and nourishes the brain. It is produced in specialised structures called ‘choroid plexus’, clusters of capillary blood vessels which are present in the cavities of the brain called ‘ventricles’. The ventricles are an interconnected system of CSF filled spaces. The uppermost is the lateral ventricle (One on each side), then comes the third ventricle and lastly the fourth ventricle behind. CSF is filtered out of the blood by the choroid plexus at a rate of about 0.5 ml per minute. In young children, the total CSF volume ranges between 65 and 150 ml. However, it recycles once every 4–6 hours. This is because, the rate of removal normally keeps pace with the rate of production. The CSF circulates inside the ventricles through various channels and aqueducts to reach the subarachnoid space surrounding the brain and spinal cord. It then travels up near the middle of the head called superior sagittal sinus and is absorbed through the arachnoid villi into the blood. So it is produced from the blood and returns to the blood.
NORMAL HEAD GROWTH:
The head growth of a child can be indirectly assessed by using a measuring tape around the broadest part of the baby's forehead, just above the ears and at the midpoint of the back of the head. (Occipito-frontal circumference). Normally this head circumference is 35 cms at birth and it becomes almost 47 cms by the age of one year. This 12 cms increase is distributed over the first year in the following manner. In the first three months it increases 2 cms per month, next three months it is 1 cm per month and the next six months, it is 0.5 cms per month. Later it increases @ 1 cm per year and reaches the adult size of 55 cms at about five years of age.
HYDROCEPHALUS: Hydrocephalus is a condition where there is an excessive accumulation of cerebrospinal fluid (CSF) under pressure, resulting from:
• Impaired circulation or absorption of CSF into the blood
• Obstruction to the flow of CSF in the ventricle
• Rarely from increased production of CSF, by tumors in choroid plexus.
Over time, the portions of the ventricular system above the site of obstruction gradually dilate. This causes compression and thinning the surrounding brain, thereby damaging it. Gradually the pressure inside the system decreases and it becomes an arrested hydrocephalus. Brain development is much affected in these cases. There is not much treatment to be offered at this stage. The brain development will not recover with whatever surgical exercise we do. This is a scenario where the doctor has to take a decision NOT to operate.
TREATMENT: Usually, the treatment depends on the cause of hydrocephalus and location of the obstruction. In many cases the block is permanent or is unable to be removed (e.g. most tectal gliomas, aqueduct stenosis). A permanent CSF diversion may be required in these cases. Treatment options include:
• Shunting or bypass of obstruction, most commonly with a Ventriculo Peritoneal shunt
• Third ventriculostomy is useful in patients with non-communicating obstructive hydrocephalus at or below the level of the aqueduct connecting the third and fourth ventricle.
VENTRICULO-PERITONEAL SHUNT:
In this, a tube is passed permanently from the high pressure, dilated ventricular cavity inside the brain to the low pressure peritoneal cavity in the abdomen, thereby bypassing the block and ensuring continuous flow of CSF. The CSF is absorbed into the blood circulation at the abdomen, completing the cycle. Rarely, the tube can be placed into the chest cavity (Pleural) or the heart (atrial) in some cases. This is a simple method of treating hydrocephalus but can often get complicated due to infection or block of the tube. This may require revision or replacement of the tube. In some cases the pressure inside the brain may not be high enough to drive enough fluid through the tube. Sometimes, the tube may take out more fluid or over drain. These problems can be taken care of by using a variable pressure tube called ‘programmable shunt tube’.
ENDOSCOPIC THIRD VENTRICULOSTOMY:
The Endoscopic Third Ventriculostomy procedure is used as an alternative to a ventriculoperitoneal shunt. This involves operating inside the ventricle using an endoscope, a camera system. A hole in made in the thinnest portion of the ventricle- the transparent floor. The CSF comes out of the ventricle thereby bypassing the block. Chance of infection is less as there is no shunt tube. The success of the procedure is dependent on the pressure gradient
The story is narrated to emphasise that sometimes in medical practice, not performing a procedure is equally important in relieving the patient of the distress.
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