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By Dr. Damien Jonas Wilson, MDReviewed by Susha Cheriyedath, M.Sc.
A disruption in the flow, formation, or absorption of cerebrospinal fluid (CSF) may lead to hydrocephalus. It is a very dangerous condition with life-threatening consequences if left untreated. The disturbance in the hydrodynamics of CSF may be acute, subacute, or chronic, occurring over days, weeks, and months to years, respectively. The disorder has a bimodal distribution in its peak incidence, peaking first in infancy and usually associated with a congenital malformation. Its second peak incidence occurs after the sixth decade of life.
The signs and symptoms that accompany hydrocephalus vary with the patient’s age. In addition to this, the causative factor of the hydrocephalus and the rapidity of the buildup of fluid inside the brain as well as the duration are key determinants to the clinical features seen. Infants present with symptoms such as poor feeding, vomiting, irritability, and reduced activity. Toddlers, older children, and adults show decreased mental capacity and may experience headaches, neck pain, vision disturbances, drowsiness, and unstable balance.
Diagnosis may be made with radiographic imaging techniques such as ultrasound in children less than 12 months old and MRI or CT scans in everyone else. The preferred therapeutic option is surgical placement of shunts to re-channel CSF or removal of blockages that can be removed. Alternatively, choroid plexus cauterization combined with third ventriculostomy may also be performed to reduce the amount of CSF that is produced by the choroid plexus.
Outcomes of Surgery
The mortality rate for hydrocephalus and associated therapy ranges from 0 to 3%. This rate is highly dependent on the duration of follow-up care. The shunt event-free survival is approximately 70% at 12 months and is nearly half that at 10 years, post-operatively. Shunts that are placed to channel CSF to other parts of the body may fail due to malfunction or infection. Infections occur in less than 10% of all surgeries.
Malfunctions that may be seen include over- and under-drainage as well as obstruction and hidden shunt failure. The occurrence of malfunction is noted as being duration-dependent and may generally be regarded as actuarial survival. Malfunctions can be managed with or without further surgery. For instance, over-drainage can be resolved with simple valve adjustment. Infections leading to shunt failure may be classified as incisional, deep-incisional and organ-space infections. Infection is ascertained when there is a diagnosis based on clinical findings and/ or culture that lead to further operation or death.
Long-Term Outlook
The triad of early detection, prompt treatment, and taking the necessary steps to avoid surgical site infections is the cornerstone to ensuring a great long-term outlook. It particularly helps in reducing the chances of developing clinically significant neurological damage. Failure to catch hydrocephalus on time and treat it accordingly may lead to long-term neurological deficits that require multidisciplinary medical teams to assist patients with developmental and lasting cognitive impairment. Neurological damage that may have occurred prior to treatment is unfortunately irreversible and can have a significant impact on functional social outcomes such as social integration, schooling, and marriage.
Sources
Further Reading
- All Hydrocephalus Content
- What is Hydrocephalus?
- Hydrocephalus Research
- Hydrocephalus in Children
Last Updated: Dec 30, 2022
Written by
Dr. Damien Jonas Wilson
Dr. Damien Jonas Wilson is a medical doctor from St. Martin in the Caribbean. He was awarded his Medical Degree (MD) from the University of Zagreb Teaching Hospital. His training in general medicine and surgery compliments his degree in biomolecular engineering (BASc.Eng.) from Utrecht, the Netherlands. During this degree, he completed a dissertation in the field of oncology at the Harvard Medical School/ Massachusetts General Hospital. Dr. Wilson currently works in the UK as a medical practitioner.
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Comments
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Question about living alone with NPH...
Willow
says:
September 30, 2020 at 6:22 AM
Hi,
I have an appointment with a neurologist coming up for a loved one (age 37) whose symptoms resemble the beginning stages of NPH. They have fatigue, vision issues, a shuffling gait, balance issues, muscle weakness, light sensitivity, headaches and some very very mild forgetfulness. They were recently taken off work because of the vision problems and balance issues. They live alone and other than fatigue, balance and headaches are ok. I have a couple of questions, if they require a shunt will they still be able to maintain their independence living alone? And will there still be mental decline if the shunt works properly? Any information you cna provide would eb immensely appreciated. Thank you.0 0
Reply
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I was 37 (in 2004) when my first shunt was placed in an emergency situation
Yvonne DeBartola
says:
October 3, 2020 at 1:03 PM
I was 37 (in 2004) when my first shunt was placed in an emergency situation - although I had symptoms I was not diagnosed properly until it became a crisis situation. At the time I was married and also had assistance from my mother. I divorced in 2009 and have lived alone just fine since then. In my experience I had to work at getting my mental faculties restored, and I did this mostly by doing crossword puzzles and Sodoku - things I thought I couldn't do but eventually became quite good at. Jigsaw puzzles and learning new skills seem to help as well and these are things I continue to do. Anything that challenges your loved one is good to try. I'm not sure what will happen as I get older, but I'm hopeful that I can continue to do most of the things I do now. After my divorce I bought a fixer-upper and learned basic plumbing and electrical skills - I replaced toilets and vanities in two bathrooms and numerous switches, outlets, and light fixtures throughout my house. I also bought a miter saw and replaced some of the trim. I do my own yard work. Your loved one will be just fine. It sounds like he/she has been diagnosed early enough that they won't have to do as much to get back to normal as I did. I think you should help as needed if you can, but I don't think you need to worry about his/her independence unless they express a concern to you. I hope this is helpful to you.
0 0
Reply
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Questions about BPH...
Willow
says:
September 30, 2020 at 6:33 AM
I have a loved one age 37 who is showing symptoms of BPH such as balance issues, shuffling gait, vision issues, head aches, extreme fatigue, light sensitivity and very very mild short term memory issues. They were recently taken off work because of the balance and visual issues. They have an appointment coming up soon at UK with a neurologist to see what the issue maybe. My questions are if someone requires a shunt for BPH can they still live alone independently as they do now? Is there still cognitive decline after a shunt is installed or is it just slowed down? Any information your expertise could provide will be immensely appreciated. Thank you.
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FAQs
What is the prognosis for hydrocephalus? ›
Hydrocephalus can be very serious, and even fatal, if left untreated. Fifty percent of those who fail to have their hydrocephalus treated will die. The other half survive with what is called arrested hydrocephalus. Those who are not treated and survive may have serious brain damage and physical disabilities.
Can you be normal with hydrocephalus? ›With appropriate early treatment, however, many people with hydrocephalus lead normal lives with few limitations. Hydrocephalus can occur at any age, but is most common in infants and adults age 60 and older.
Does hydrocephalus get worse over time? ›Symptoms of Hydrocephalus in Older Adults
In older adults, symptoms usually get worse over time. They can include: Difficulty walking or slow shuffling. Loss of bladder control.
Normal pressure hydrocephalus is one of the few causes of dementia that can be controlled or reversed with treatment.
Who has the longest lifespan with hydrocephalus? ›The longest living hydrocephalic is Theresa Alvina Schaan (Canada) who was born on the 17 March 1941 and diagnosed with having congenital hydrocephalus. Also known as "water on the brain," it is a condition in which there is extra cerebrospinal fluid (CSF) around the brain and spinal cord.
How bad can hydrocephalus get? ›Excess buildup of CSF can keep the brain from functioning properly and cause brain damage or even death. Symptoms of hydrocephalus can vary significantly from person to person and mostly depend on age.
What should you not do with hydrocephalus? ›Any activity that involves being grabbed around the neck is not advised, as the shunt tubing in the neck can crack. Judo may not be advisable, but other martial arts may be safe, depending on what is involved.
How often is hydrocephalus misdiagnosed? ›NPH Misdiagnosed as Dementia
Yet despite the availability of surgical treatment options, according to the Hydrocephalus Association in Bethesda, Maryland, the largest private funder of hydrocephalus research in the U.S., fewer than 20 percent of people with NPH receive an appropriate diagnosis.
- Getting on with others (social difficulties).
- Managing how they feel about things (emotional difficulties).
- Knowing how to act (behavioural difficulties).
Early diagnosis and treatment of hydrocephalus can improve the survival rate of patients. Most hydrocephalus patients lead long, problem-free lives. However, if left untreated, hydrocephalus may prove to be fatal. The longer the symptoms persist, the more difficult it becomes to treat.
Is hydrocephalus considered a disability? ›
Your child should qualify for disability benefits if he or she has been diagnosed with non-compensated hydrocephalus. If you can't find a listing in the Blue Book that matches your hydrocephalus symptoms, you will have to undergo a residual functional capacity test (RFC).
Is hydrocephalus high risk for COVID? ›Hydrocephalus by itself has not been identified as a risk factor for severe COVID-19. Persons with normal pressure hydrocephalus (NPH) should consider themselves at high risk for severe COVID-19, but not because they have NPH.
Does hydrocephalus lower IQ? ›Hydrocephalus appears to decrease the IQ further in patients with MMC, although some studies have shown that if it is corrected in early age, the IQ can return to MMC baseline though not to normal levels.
Does hydrocephalus lead to dementia? ›In some cases, NPH is caused by other brain disorders such as a tumor, head injury, hemorrhage, infection or inflammation. But in most cases, the cause of the fluid buildup remains unknown. NPH is one of the few causes of dementia that can be controlled or reversed with treatment.
What is the survival rate of hydrocephalus in adults? ›For hydrocephalus cases that are not associated with tumors, it has been discovered that the survival rate is up to 95% with treatment.
What is the success rate of shunt for hydrocephalus? ›After 1 year, 91.2% of patients had an improvement of clinical outcomes after shunting and our results are consistent with the previous studies reporting the clinical benefit of shunt, ranging from 31% to 96% [18,19].
Can you live a normal life with a brain shunt? ›Many people with normal pressure hydrocephalus enjoy a normal life with the help of a shunt.