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Getting the Diagnosis, Part 2


The following excerpt is taken from Chapter Two of Hydrocephalus: A Guide for Patients, Families, and Friends by Chuck Toporek & Kellie Robinson, copyright 1999 by O'Reilly & Associates, Inc. For book orders/information, call 1-800-998-9938. Permission is granted to print and distribute this excerpt for noncommercial use as long as the above source is included. The information in this article is meant to educate and should not be used as an alternative for professional medical care.

This second of a three-part series focuses on emotional responses to the diagnosis.


Although many cases of hydrocephalus are diagnosed either in utero or at birth, it can also affect people at any age. There are three possible times when a person could be diagnosed with hydrocephalus: at or before birth (known as congenital hydrocephalus), as a child (known as childhood onset hydrocephalus), or later in life as an adult (known as adult onset hydrocephalus). Although the basis of the condition is the same-an abnormal accumulation of cerebrospinal fluid-the situations in which a person is diagnosed can differ sharply.

Story: A diagnosis of congenital hydrocephalus

Each year, 1 in 1,000 children are born and diagnosed with hydrocephalus. That seems like a fairly high number, especially when the latest statistics reveal that a child is born in the U.S. every 8.1 seconds. This would mean that a child is born with hydrocephalus every 135 minutes. With statistics like that, it's amazing just how few people know what hydrocephalus is, and how little information there is out there for people to find out more about it.

Cameron was a total surprise to us. You see, we already had four children, the youngest being 13, so we were sure our baby-raising days were over. In my seventh month, I began to feel that something was wrong. The baby was still moving, but the quality of the kicks had changed. My obstetrician kind of blew me off. He did a few non-stress tests, but they were inconclusive.

On January 9, 1996, our son Cameron was born at 4:30 a.m. Our tears of joy quickly turned to tears of fear when Cameron went into respiratory distress. He was rushed into the nursery where they could work on him, and later taken to another hospital 20 miles away which had a neonatal intensive care unit (NICU). I was left to recover from the birth and pray for our little boy. The prognosis was not good.

My husband went to the other hospital with our son, and when he came back, we were both sad and afraid. He brought me a Polaroid picture of Cameron they took in the nursery. I held that picture all night long and cried. The nurses in the hospital didn't know how to handle me, so they just closed the door and left me alone.

Two and a half weeks later, they started to wean him from the ventilator, and he started to breathe on his own. They did an MRI to see why he had a seizure on the day he was born. That is when they found out that he had a stroke before he was born. Because of the damage that was done, they said Cameron would never walk or move his left side. They were pretty sure he had cerebral palsy. Our world stopped at that moment. I felt like someone had punched me in the stomach.

On February 5, the pediatrician called in a neurosurgeon because the ultrasound of Cameron's head was abnormal. They did a spinal tap two days later and informed us that Cameron had hydrocephalus. However, they said the fluid looked fine, so the neurosurgeon discharged him. We finally got to take our baby home! Four days later they did another ultrasound. The neurosurgeon's office phoned and told me to bring him back for emergency surgery. They did the surgery that night at 8 p.m., and by 10 p.m. we were in the recovery room holding our baby. He was supposed to be in the hospital for five days after they placed the shunt, but he was doing so well they sent him home the following day.

Cameron has continued to surprise everyone. Every time he sees any of his doctors, they shake their heads in amazement. There is no sign of cerebral palsy. Our life with him has been a wonder. He really is a miracle and a blessing. There isn't a person who meets him who doesn't tell me he is the most beautiful boy. I am still afraid every minute that our luck will change. Every day seems like a time bomb. I know that the shunt could cause problems at any time. I look at Cameron and believe in miracles. I pray every day that God will continue to watch over our baby.

Cameron experienced an intracranial hemorrhage either prior to or at the time of birth, causing acquired communicating hydrocephalus.

Story: A diagnosis of childhood onset hydrocephalus

Many factors can cause hydrocephalus in children. The most common cause of hydrocephalus is the result of a congenital form of aqueductal stenosis that restricts the flow of cerebrospinal fluid within the ventricles of the brain. With some, hydrocephalus can be caused as a result of other congenital defects, such as myelomeningoceles.

Other fairly common causes of hydrocephalus in children are cysts and brain tumors. Porencephalic cysts are commonly found within the brain, next to the ventricular system. Arachnoid cysts can be found either within the ventricular system or in the subarachnoid space. Brain tumors in children are most frequently found in the posterior fossa. Since cysts and brain tumors can grow over a long period of time, they are difficult to detect until they begin to block the passage of cerebrospinal fluid.

As a parent, it can be difficult to understand why one day your happy, active child has suddenly changed.

At the end of July 1996, late on a Wednesday afternoon, B.J. complained of a headache and did not feel well or hungry. He had a little soup, I told him to drink some water, and he went to lie down. I thought he just got too much sun--we live in Jerusalem, Israel, and the summer sun here is fierce. He was up all night with a steadily worsening headache and vomited a few times. That night I thought he wasn't feeling well because of his exposure to the heat and sun, but by Thursday morning I was beginning to fear that it could be meningitis.

I called my pediatrician. She said to bring him in right away. She examined him. About five minutes later she was on the phone with the local hospital asking if they had a CT scan--she used to be a pediatric neurologist. After a few simple neurological tests, she asked him to touch his chin to his chest-he couldn't. When she looked into B.J.'s eyes, she saw massive papilledema (swelling of the optic nerve) from high intracranial pressure (ICP). My pediatrician knew right away there was a blockage, probably based in the third ventricle. This is where his tumor actually was.

We went to the main medical center in the city where they handle all of the neurosurgery cases. After a few hours the doctors came and told us there was definitely something wrong, but first they needed to deal with the immediate crisis of the extremely high ICP.

The neurosurgeon said that inserting the shunt would be a simple procedure that would give B.J. immediate relief and solve the pressing issue of his severe headaches. After that, the doctor said, they could take their time and do careful diagnostics to figure out what the underlying cause of his headaches were. His shunt, a PS Medical high-pressure system, was inserted around midnight of the same day. We were extremely fortunate. He started to feel ill and was diagnosed within 24 hours, which is extremely rare.

It wasn't until later that B.J.'s neurosurgeon discovered that he had a benign juvenile pilocytic astrocytoma (JPA). The tumor filled the third ventricle and extended out of all three openings into both lateral ventricles and down into the fourth ventricle.

B.J. is now nine years old. In the weeks and months since B.J. received his first shunt, he has had others placed and removed--being shunt independent for a month or so over the summer. However, when he began showing signs of intracranial pressure again, his neurosurgeon placed another shunt system. Aside from having a slight headache when he wakes up in the morning--which is normally gone by the time he gets to school--he feels absolutely wonderful.

Story: A Diagnosis of adult onset hydrocephalus

The two most common forms of hydrocephalus in adults are hydrocephalus ex-vacuo and normal-pressure hydrocephalus (NPH). Hydrocephalus ex-vacuo occurs when there is damage to the brain caused by stroke or injury. In some cases, there may also be shrinkage of brain substance, particularly with the elderly and individuals with Alzheimer's disease. When reduction in brain matter occurs, the amount of intracranial CSF increases to fill up the space. NPH is caused when there is a gradual blockage of the CSF drainage pathways in the brain, such as in the subarachnoid space or within the ventricular system. Although the ventricles may become enlarged, the pressure of the CSF remains within normal range.

The symptoms first started to appear when I was about 40 years old. My feet would tingle and feel totally numb when I was working out in aerobics class. I was certain the numbness was caused by the running shoes I was wearing, so I started buying new ones, trying to find the perfect fit. But that didn't work either. A few months and four pairs of shoes later, the tingling and numbness were still there.

That spring, we went on a holiday to Disney World with our 16-year-old daughter, and we stayed in the same hotel room. At the time, I was experiencing "urine hesitancy," or incontinence, during the night. I didn't think much of it, but it really bothered my daughter and my husband. They suggested I get this problem checked out. I went to a bladder specialist when we got back home and all of the tests came back negative, so I decided it was just the way I urinated. I never dreamt the numbness in my feet and bladder problems were related to one another.

A few months later I was going down some steps in our home, and my legs completely disappeared from beneath me. I dropped everything and collapsed. I just figured I was really clumsy. The same thing happened during the middle of the night when I got up to go to the bathroom a few times. I collapsed, but thought it was caused by my legs being asleep.

This continued on for a few more months, and during this time I also suffered from nagging headaches (not excruciating by any means), but my husband noticed how much Tylenol we were going through--and I was the main person in the family taking it. Still, I didn't put all these symptoms together.

Then one day, I was driving to work, and lost sensation in my hands; they felt like pins and needles. I went to rub my right hand on the steering wheel, and to my surprise, my hand was not on the steering wheel, it was on the seat beside me. This really gave me a scare, so I headed straight to my family doctor's office. She was not too pleased with me, because in one of my previous visits, she arranged for me to see a neurologist--an appointment which I had canceled because I didn't think there was anything wrong with me. She made another appointment with a neurologist, but it took another month for it to be scheduled.

The neurologist did a bunch of tests. I believe she was testing me for multiple sclerosis (MS) as I had all the symptoms of that disease. The final test was a CT scan, which I had to wait three months for. We lived in Edmonton, Alberta, at the time and it was impossible to get tests like that unless you were practically dying.

During those three months, my condition kept getting worse. I would double-book appointments at work (I was a sales representative for a large international corporation). I would phone my friends twice in a row and repeat the same conversation. They were very worried about me, but figured I was just being absent-minded.

My husband and I went on another holiday to San Francisco. This holiday was about two weeks before my scheduled CT scan. My condition during the trip was really bad. I passed out at the airport in Vancouver while waiting for our connecting flight. I walked like a total invalid (I guess they call that "gait"). We visited an aunt while in San Francisco and she really noticed that something was wrong. My condition was gradually deteriorating0--something that my family didn't notice because they saw me every day. The symptoms would come and go. One day my eyesight could be practically gone, and the next it could be perfectly fine.

I had the CT scan when we returned from San Francisco, and was immediately admitted to the hospital for surgery. According to my neurosurgeon, the ventricles of my brain were so large they were ready to explode. I had quite a few complications after the surgery as my body tried to reject the foreign object, but after a month everything seemed to settle down.

I was diagnosed with adult onset, normal-pressure, communicating hydrocephalus and received a ventriculoperitoneal (VP) shunt. I was 42 years old at the time and am now 44. I had one revision a year later when I began experiencing the same symptoms again. I had another CT scan just recently and the ventricles are back to normal size, but I still suffer from frequent headaches.

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