Outcomes for Periventricular Leukomalacia(PVL)

NicuGal, MSN, RN · Feb 6, 2009

PVL...a lot depends on where the cysts and "holes " are. Some kids have a lot of muscular issues, like CP, yet others are deaf and blind. The problem is that it is usually seen as echogenicity in areas of the brain, then they turn to cysts which fill up with macrophages and can be interpreted as "normal", so sometimes the docs say it is "gone". Sadly, PVL does not disappear at all. When the macrophages move out is when you see the "swiss cheese" holes in the brain, usually around ventricles. The extent of the damage is determined by what areas of the brain are eaten away, most commonly it is the neuromuscular tracts. These kids are either floppy or hypertonic, again depending on the area of damage. All of these kids should be followed by MRI to see the specific areas of damage, HUS's are NOT a reliable diagnositc tool to use, although most docs keep ordering them.

I've seen kids that have little problems to kids with profound CP. It is so sad when any kid is given this diagnosis. PVL can also happen in utero, so you may have a kid that shows the echogenicity on their first HUS and that kid warrants a close eye.

Jolie, BSN · Feb 6, 2009

I know this is anecdotal, but I once cared for a preemie with a long and complicated NICU course who was DC home with PVL. We expected him to be blind, have significant CP, etc. What a wonderful surprise it was to bump ino him a while later and see a baby who was developmentally appropriate, had normal muscle tone and while his vision was not perfect, he was not blind.

I think his miraculous condition stemmed from a dedicated mother who educated herself and did early intervention with him from the moment he was discharged. I believe she was able (with guidance from professionals) to "retrain" other areas of his bran to take over certain functions.

PVL can certainly be a grim diagnosis, but I do believe there is the possibility of improvement with intensive intervention.

Humbled_Nurse · Feb 7, 2009

Great info. in the previous posts.

I work NICU, but I also have 5 years experience working for a pediatric neurologist so we saw lots of "ex-preemies" affected by PVL. I also have a 10-year-old niece with PVL. She does have a moderate case of CP, but her intellect is normal. She had a pretty rough start at birth. She was a 31 weeker with undiagnosed pulmonary stenosis. They were treating her as if she had a PDA. I think most of her brain damage occured within that first week of life and possibly before she was born.

PVL is most definitely not a good thing to have. When there is a diagnosis of PVL the chances of having some form of CP is extremely high. Although it is hard to predict how severe the CP will be. It could be very mild and hardly noticeable or significant enough to result in the child being wheelchair bound. As the the PP mentioned PVL mostly affects the motor areas of the brain which is located around the ventricles resulting in some sort of motor disorder. It is most commonly affects the legs. Intellect is often spared. The majority of the kids I saw with PVL were very smart. Although, if the PVL is very extensive the chances of MR, blindness, deafness, seizures, etc... is obviously increased.

With early intervention kids with PVL have a much better chance. There is also treatments for the spasticity such as medications, botox, therapy, baclofen pumps, etc.....

I also agree that an MRI is the best for detecting PVL. HUS's are ok, but MRI's are much better. The neurologist I worked for always ordered an MRI. Most of the kids we saw never had an MRI done, only HUS's.

Also another thing to add is just because a baby has a "negative HUS" doesn't mean they are guaranteed normal neurological development in the future. There are so many processes (migration, myelinization, organization, etc..) that the brain in going through and when you are born premature those processes are disrupted. I think that is why there is a much higher rate of autism, senory disorders, ADD, etc... in "ex-preemies".

Hope that helps.

helicoptergal, BSN, RN · Feb 8, 2009

Thank you all for your very responses for the PVL thread. I really appreciate it. All of the responses were so interesting and thoughtful. I hope the little guy I am caring for defies the odds. :redpinkhe.

RNin2007 · Feb 10, 2009

Quick add....my 11 year old was a former 29 weeker and had/has PVL and hydrocephalus. He had his shunt removed at 2, and is completely normal. He scores above average on state testing in school and has NO medical issues from being a preemie.

littleneoRN · Feb 28, 2009

We talk a lot at work about MRIs vs. HUS for these kids. I'm wondering if I understand the issue correctly. Leaving the kids with progressing IVH, concerns for hydrocephalus, etc. aside for a moment, the major advantage of an MRI over a HUS is prognostic, meaning it gives us an idea of what the future may hold for this child. So many parents want some sort of picture, and the MRI can help some, although certainly we don't hold the magic 8 ball. Man if we did we would all be a lot richer! So anyway, my point being that some physicians will be inclined toward the MRI in order to help paint a picture for parents and further demonstrate and clarify the need for early intervention. Others might be less inclined, saying it won't change the plan of care in the NICU. It is a costly procedure, and if it requires sedation, there is risk. It gets safer if you let the kid get a little older before sedating. Any thoughts that can add to my understanding of this?

SingingFrog · Mar 26, 2009

Hello ladies! I stumbled across this thread searching information on PVL and find ALL of your posts to be extremely interesting. :yeah: I am a mom (actually a medical transcriptionist :typingat home-stopped going for RN when discovered new baby was on the way). I consider myself somewhat literate in the medical world and what I don't understand, I research. :banghead:

Tell me if this info raises any red flags with any of you...a mom with several kidney stones from her 7th to 9th month of her pregnancy, was sure she had a kidney infection in her 7th month when her first stone passed and doctors followed her very carefully. At full term, she delivered a V-back son who was code blue. He was able to leave NICU after a day when he "bounced back" and did not need a transfusion after all (according to well respected NICU doctor) and seemingly did fine in normal milestones and development. Had a 2 year old sister at the time of his birth who was "smart as a whip" and he learned to talk and do most things early. Preschool began and noticed issues with what seemed to be anger or tantrums. Speech was impaired and a speech and language pathologist worked with him in preschool. Mom had always kind of wondered about autistic tendencies with his behavior and talked with preschool teachers to see if they had noticed it as well. They did, so mom got a referral for a pediatric neurologist. At 4 years old, child had an MRI and thorough evaluation from pediatric neurologist who discovered on MRI that child had PVL. Mom was given horror story that her child would never be normal and she was in for a real fight. This particular doctor wanted to medicate child and mom disagreed with medication at 4 years old and both parents worked with a well respected behavior specialist to help child instead. Mom never revisited the pediatric neurologist after a very heated :argue:disagreement about medication and care. She went to school and immediately set up an IEP in kindergarten. Child continued with speech/language pathology and received necessary OT for fine motor skills, all the while developing intellectually normal. Child was "uncoordinated" and "very ADD" according to mom, but socially normal and progressed on honor roll in school, with very above average reading abilities.

Child is now age 11, in 6th grade, honor student, and has had state test scores in the "gifted" range according to teachers. He still has IEP that assists him only when he needs help. Both parents are and have been very active in his education and have communication with teachers regularly. He still has an "odd" way of running according to mom, shuffles his feet very fast instead of taking large strides (actually runs track at school and is competitive) and just learned how to ride a 2-wheel bike last year (age 10). Other diagnoses include Hashimoto's thyroiditis (with no diagnosis of hypothyroidism), positive ANA (very low titer), obsessive compulsive personality, very mild Tourette's, anxiety/panic, and ADD. Child is followed regularly by his pediatrician, pediatric rheumatologist, pediatric endocrinologist and a very well respected psychiatrist. He is on one medication that seems to be working well for the OCP/Tourette's tics/anxiety/and panic. He is a very cooperative and agreeable child, fixates on certain subjects, with terrible organizational skills/methods. One teacher stated that should he go on to become a "cardiologist" that she would certainly choose him to be her doctor since whatever he fixates on he will give 150%. Child is no longer in speech and language therapy and no longer needs/requires OT.

Mom has since learned that doctor who diagnosed PVL is questionable and is concerned that he has been followed for something that may not be accurate. However, in researching topic she states that several of the concerns that were discussed with her originally were accurate and indeed presented challenges, but feels that she has not been able to find other kids who have PVL and can function at this level. She read an article where it said that only 2% of kids with PVL could function at this level.

Any red flags here or concerns? Child definitely had and has struggles and challenges not listed that are present with PVL cases, however, what about when they are in puberty and a young adult? Can the brain heal itself in time? Can the effect of PVL lessen with age? Also, mom was told that it is very hard to diagnose by MRI after age of 5...anyone know anything about that? Mom has also asked for other professional opinions to find that several health professionals have not heard of it or are unfamiliar of deficits/lifestyle with the disease. There seem to be a lot of the same stories online about what it is, but what about outcomes for young adults or adults?

Your opinions are very valued on this topic, thank you for sharing

RNin2007 · Mar 26, 2009

Wow you surely know a lot about this child...but as you know we can't give out medical advice. I will just share a few "off topic" things about my own child who has PVL. He also scores above the standards on state testing. He is quite intelligent. He has ADD (not ADHD) which is treated by medication. He also only last year learned to ride a two wheel bike (he was very fearful up until this point of learning how to, despite my attempts to bribe him with $50 to ride LOL!). I think he has a sensory processing disorder due to his prematurity and MANY problems (8 surgeries before the age of two, need I say more). He isn't OCD, but he does chew his nails and would play video games 24/7 if I let him. I would say he also fixates on certain subjects, such as science. He is an overflowing river of knowledge when it comes to the most random of facts. His ADD is managed by a neurologist, and we see a pediatric psychiatrist every two years for testing, and he has an MRI every two years in addition d/t hydrocephalus (no shunt, he had an ETV at the age of 2). He also has a hard time making friends and he does talk nonstop. He just turned 12 last month and he was a 2 lb. 2 oz. 29 weeker. He is a handful but an amazing kid.

Again...no medical advice but thought I would just share with you - more on a mom level here I guess.

prmenrs, RN · Mar 26, 2009

Any opinions about the child described by singing frog would be speculative @ best, and it is against allnurses.com's Terms of Service to offer any legal or medical advice in any case. https://allnurses.com/terms-info.html

After many years of NICU experience, I can say one thing: HUSs (and other brain imagery) in the early NICU period are subject to interpretation, and not necessarily predictive of future performance. On the other hand, they can also be devastatingly correct.

There are outcome studies out there, but there are always exceptions and anecdotal stories of exceptional differences. Take it all w/a grain of salt.

SingingFrog · Mar 26, 2009

Okay, just to clarify...I am very close to this child and parents are not out to seek any legal advice anywhere or any medical advice from a blog/forum. The parents are actually questioning the accuracy of the diagnosis themselves since he has been able to achieve so much--they have never met anyone with a child or experience with PVL who can share their information with.

The last paragraph:

Any red flags here or concerns? Does this seem normal or out of the normal with anyone who has personal experience?

Child definitely had and has struggles and challenges not listed that are present with PVL cases, however, what about when they are in puberty and a young adult? Does anyone know of anyone in this timeframe and can share their struggles/achievements?

Can the brain heal itself in time? Can anyone direct to info where this can be read?

Can the effect of PVL lessen with age? Anyone know from personal experience, it seems to possibly be the case here in this situation if parents received accurate diagnosis of PVL 11 years ago.

Also, mom was told that it is very hard to diagnose by MRI after age of 5…anyone know anything about that? Does anyone know this to be the case or has anyone ever heard a neurologist state this in testing?

Mom has also asked for other professional opinions to find that several health professionals have not heard of it or are unfamiliar of deficits/lifestyle with the disease. There seem to be a lot of the same stories online about what it is, but what about outcomes for young adults or adults? Again, personal experience question.

Your opinions are very valued on this topic, thank you for sharing

I would like to state I am only seeking opinion, no profressional advice. If I want professional advice, I will seek out a professional in their office and pay the consultation fee for it. This is a network of health professionals that have actually seen this condition, worked with this condition, and have personal experience with this condition. I would like to get your opinions on it to have a general idea.

THANK YOU RNin2007 for your story-MOM LEVEL is wonderful-this is very interesting because this child also is a 24/7 video game strategist and has beaten many hard games within a few days of their purchase, leaving him very bored with it. It seems that the more it is a challenge, the greater his desire to conquer.

The bike riding is very interesting as well...glad I added that to the original description since I didn't know if others had experienced that. He was pretty upset about not being able to ride a bike until that age, but it finally happened. Is your child still a bit "clumsy" on the bike? Fall frequently? This one still does.

Also, were you instructed by his doctor that he needed to have regular MRIs or do you take it upon yourself as a mom to request it? And, were you at all suspicious with the things you listed that he had autism?

Thank you again, this info is very much appreciated :)

SingingFrog · Mar 26, 2009

THANK YOU RNin2007 for your story-MOM LEVEL is wonderful-this is very interesting because this child also is a 24/7 video game strategist and has beaten many hard games within a few days of their purchase, leaving him very bored with it. It seems that the more it is a challenge, the greater his desire to conquer it, sound familiar to you?

The bike riding is very interesting as well...glad I added that to the original description since I didn't know if others had experienced that. He was pretty upset about not being able to ride a bike until that age, but it finally happened. Is your child still a bit "clumsy" on the bike? Fall frequently? This one still does.

Also, were you instructed by his doctor that he needed to have regular MRIs or do you take it upon yourself as a mom to request it? And, were you at all suspicious with the things you listed that he had autism?

Thank you again, this info is very much appreciated and the similarities are wonderful to learn :)

I am a new member posting so I cannot respond to private messages yet, only read them If you would like to PM your email address I will certainly respond to your PM. Thank you :bow: