PVNS: experience and causes
Posted , 91 users are following.
I recently got diagnosed with the fairly rare condition called Pigmented villonodular synovitis (PVNS) a disorder which affects joints. In my case, it was my knee joint, as is often the case.
I'm posting this to help others better identify and understand this condition, and also to explore any possible causes.
PVNS is very easily misdiagnosed by GP's. It was misdiagnosed twice in my case, by two different doctors.
Because it's more common in the 30's and 40's age group, and the symptoms are similar to arthritis, it can be misdiagnosed as the early onset of arthritis of the knee/joint in people who are in the upper end of this age bracket.
In the case of the PVNS affecting the knee joint which results in a tumour growth at the back of the knee (as in my own situation), because the condition often arises after a trauma to the knee joint, the condition can be misdiagnosed as a simple lingering knee injury, leaking joint fluid, giving rise to a Bakers Cyst.
The symptoms of PVNS are both sporadic and very similar to both an ordinary knee injury and arthritis as the condition progresses. The joint becomes stiff and movement restricted. It's usually painless in the early stages but as it progresses it causes mild to moderate discomfort with a persistent burning pain around the joint area.
If left untreated, PVNS in its advanced stages, can render the joint completely unusable, requiring a complete joint replacement.
Here's my own experience of PVNS:
I was taking Tramadol (a painkiller) for back pain and was heavily into exercise and was also carrying a heavy bicycle every day up and down stairs. I injured my knee. This was due to the Tramadol I believe. My pain tolerance threshold was at a much higher level, so the injury didn't fully register and I continued exercising and carrying the bicycle, further straining the already injured knee.
Two years later, the knee joint gradually became stiffer and a lump appeared at the back of the knee. It became so stiff I could no longer crouch or bend down to tie my shoe laces. I went to the doctor and was misdiagnosed as mentioned. The second GP ruled out arthritis from an x-ray and to confirm his diagnosis of a leaking joint and Bakers Cyst, he requested an ultrasound scan of the joint. The scan showed an overgrowth on the lining of the knee joint and a tumour growth, resulting in a PVNS diagnosis by an orthopedic consultant.
A proof-positive diagnosis is carried out from sample by examining cells under the microscope from the joint which have the typical PVNS-like abnormal characteristic.
There's two forms of PVNS, localised and diffused. The localised version is what I have. It is a lot easier to treat using key-hole surgery with a better prognosis for recovery. Diffused PVNS affects the entire joint lining, requiring open surgery to remove all of the knee lining and it has a much higher risk (almost 50%) of returning, and requiring extensive physiotherapy.
PVNS is quite a rare condition as I mentioned. Therefore it's not encountered very much by GP's and there hasn't been much research done into the causes of the condition or why it returns in many sufferers. It's not thought to be hereditary, and as I mentioned, trauma to the affected joint is a common feature.
This leads me to wonder if certain medicines, or activities, may cause the condition. As I mentioned, in my own case I was taking Tramadol (at a normal dose of 50mg once daily) for several years, both prior to, and after my knee injury.
I'd be interested to hear from anyone else out there who suffers with PVNS and their backgrounds, in particular:
Anyone who has developed PVNS while being on Tramadol or similar drugs?
Anyone who has developed PVNS after a sports or work related injury, which they ignored?
How much time elapsed from any initial injury to PVNS symptoms and diagnosis?
5 likes, 296 replies
morphix
Posted
There's several similarities interestingly between our stories... I also just turned 40 when I got diagnosed and after symptoms manifested...
I had the arthroscopy and a biopsy taken from the knee 2 months ago. The tumour biopsy result showed to be benign as expected with PVNS. The procedure actually improved considerably the mobility and use of my leg again.. after the op within a couple of weeks I felt like 3 years had been rolled back and I was even able to go jogging daily.. the stiffness was almost gone, and no burning pain, so I've been able to come off painkillers.
At the post-op arthroscopy consultation the doctor told me that it's common with that procedure for the symptoms to improve but emphasised it would be temporary and eventually surgery would be needed. How temporary he didn't say and I forgot to ask. As you say though, it's probably different for everyone and hard for doctors to give a general answer.. it could be weeks, months or a year or two for some lucky people maybe, depending on how progressive their PVNS and how active they are.
I subsequently contacted the Royal Orthopedic Hospital in Birmingham (a great hospital by the way, one of the best in world and they know this condition well) and basically asked them how long I can expect before I need surgery and the symptoms return. Well I got my answer before the hospital replied. The daily joogging and other activities which involved bending the knee a lot and putting pressure on it, caused swelling and the symptoms have begun to return gradually in the last 2 weeks. The joint is getting stiffer, some burning pain is back...but still nowhere near as bad as before the arthroscopy procedure during which I I believed they drained some fluid.
I was fortunate? to have the localised version of PVNS and not the more difficult to treat and progressive version you have, the diffused type of PVNS. I was told initially from my scans that the treatment would be very straightforward, by arthroscopy, one small hole. They told me I'd be back to normal in 3 weeks and not need any physiotherapy. However, after I had the explorative arthroscopy procedure, they said the PVNS was more progressed then they first thought.. I've been told that open surgery will be required and half my knee joint will be taken away.. I'm looking at 3-6 months of physiotherapy.
Now I'm facing a dilemma. Do I go ahead with the surgery and just get it over with and have a terrible summer.. or I do ease off the physical activities I enjoy so much (exercising etc) and just hope the symptoms don't get worse and ride it out until after summer.. I planned to travel overseas this summer, but obviously that will be no fun with a crutch.
apeksha85641 morphix
Posted
hi
is there any specialist consultant for PVNS in UK? you mention the birmingham hospital - anyone is specific?
necessitea
Posted
If I were you I would eliminate any kind of exercising that has significant impact on the knee. No more running! Especially because it can cause an unnecessary muscle tear. I'm not sure that it caused by localized or diffused but it's not a fun side effect. I had one last summer and it was awful. Also a girl that lives near me with PVNS was told to not try to have kids because she has a job where she stands all day.
I find that riding bicycles, swimming and dancing are the best ways to stay active without the stress on the joint that can instigate growth of the PVNS in the joint. Also yoga seems good for getting back to normal. I heard physical therapy before surgery helps you get back to normal quicker after surgery. So I would say go for surgery after the weather turns. I think planning your life around the disorder is the name of the game.
alice56
Posted
As I was informed by the surgeons there it is not caused by anything in particular however most forums I have read the patients have been very active and I was a keen dancer, dancing EVERY day before this.
I actually regret the surgery to some degree as I was not told before there was a 50% chance of it returning, and I am now having trouble with my other knee. Before the operation I was not in too much pain and was managing to do most of my day to day tasks (except for dancing) but I now realise that is probably not a career option for me now.
morphix
Posted
I'm astonished they didn't tell you about the 50% risk of return... that was brought up early by my consultant even before we had the biospy results..
Then after my biospy they gave me the choice, and basically said, you can have the surgery now, or wait a while until the disease progresses worse, because of the risk of it coming back and needing further surgery some people decide to postpone it if they feel improved after the biospy.. rather than rush into surgery and then have it come back a year or so later then have to go through it all again.
After the biospy I felt much mobility improvement due to the fluid draining from swelling etc and was able to do jogging again, something I love doing daily.. when I couldn't jog for a year I got very unfit and depressed.. so I can understand how you must feel not being able to do your dancing, something you enjoy doing.
Maybe when you're fully recovered from surgery you will be lucky not to have it return and can do your dancing again but be careful not to overdo exercise like I did which could cause injury and trigger a new PVNS episode.
Keep optimistic!
Paul
morphix
Posted
A quick update for anyone following this thread..
After my biospy in March I decided to postpone surgery.
Reason being, I was told it would be full open surgery instead of the key-hole surgery I had been told initially. Open surgery is a whole different scenario to key-hole. The risk of months of physiotherapy would interfere in my plans for this summer for a holiday/home move. I'm also in the middle of a business degree course and work full time...
Since I had greatly improved mobility afer the biospy, I was able to go jogging again (or so I thought) and lead a fairly normal life.. no pain in the knee hardly, just stiffness.. I wondered how long this would last and hoped it would last the summer at least. Unfortunately the PVNS came back with a vengence this month. I'm pretty sure this was due to the jogging and increased physical activity. If I had avoided that, I would have lasted the summer at least I'm sure before I needed surgery.
I called my consultant and got a quick surgery date within a week at the Birmingham Royal Orthopedic and had my surgery last Thursday. I didn't get out from hospital until last Sunday, even though I was told I would be out the same or next day. That was due to the drain being kept in longer.
OK so here's what to expect...(for anyone undergoing open surgery on the knee for PVNS)
Prepare yourself. Don't expect to be walking out of the hospital with a stick as I rather naively did. Don't expect to be leading a normal life again quickly within days. You need to be realistic. You will have to adapt your life around your recovery and physiotherapy for at least a month, maybe longer.
SURGERY & HOSPITAL STAY
I don't want to scare anyone who is awaiting open surgery, but it's no walk in the park. The pain is terrible. So terrible I was having to take morphine in hospital, even days after the surgery. You can expect to be on dihydrocodeine for weeks upon release. The constipation from these is terrible. I was glad to get out of hospital because of it in the end. No bowel movement for 4 days!! If the hospital staff tell you, you'll be out the same day or next day, take it as an optimistic assessment and prepare yourself for a longer stay.. you can expect to be in 4-5 days probably, so plan accordingly. Remember PVNS is a rare condition and treating it is not straightforward. Each person is different.
PAIN MANAGEMENT
The post-surgery pain is the main problem.. its excruciatingly bad when you're moving, standing, but ok if you're resting lying down. If you keep taking the opiate painkillers you have all the discomfort and unpleasantnesss of constipation.. the laxatives they give take days to work and have only minor effect in my case.
SELF-ADMINSTERING BLOOD CLOT INJECTIONS
Then there's the injections to prevent blood clotting.. these have to be done daily in stomach, either administered yourself, or a relative/friend can do it. They are not bad though. I was terrified to do it and insisted the District Nurse come and do them, but she coaxed me into trying one injection and I honestly was surprised. It was dead easy to do and I didn't feel any pain at all. It's just getting past the initial fear of sticking the needle in yourself..but it's such a slight tiny needle you won't even notice it. So have no fear about the injections if you're on your own. Just try one with the nurse, and you'll see it's nothing. Don't expect the nurse to do these for you for 2 weeks. As I found out, they will not, not matter how much you argue!! The alternative is to go to local practice nurse at drs surgery (trust me, the pain of going anywhere in first week is not worth the hassle..try it yourself at home).
PHYSIOTHERAPY
You will get an assessment from the hospitals physiotherapists a day or so after surgery and they will see how able you are to manage by yourself at home.. if you can get on and off the bed, go to toilet unaided, walk up and down stairs, they will most likely let you do the physiotherapy exercises at home, (as in my case)...
This may sound a better option, but some words of caution here.. its not easy to do these exercises alone and takes A LOT of time and mental discipline. If you live alone, as I do, it is very challenging to find the time and to get into a routine. However you absolutely must do all the exercises, daily, three times a day in fact (and not stand on leg or sit for more than 30-60 mins, nor lie in bed for more than 1-2 hrs without moving your leg).. else you will have problems with the joint and end up having to go into hospital for physiotherapy.
ADAPTING TO LIMITED MOBILITY
After surgery, you can expect massively reduced mobility. Even walking across a room is a major effort on two crutches..carrying meals/drinks/equipment etc, all very difficult if you live alone.. you will definitely need help preparing meals and someone to act as a carer for the first week or two, unless you're lucky enough to have relatives or a partner living with you. If you live alone, you will certainly need to make a lot of adjustments to your living environment. In my case, it's even worse because not only do I live alone, but I'm also in a 2nd floor flat which has 3 flights of stairs!
Funny irony.. before I had my surgery, I had stiffness and pain from PVNS and would walk with a stick but could walk briskly and would pass these old people with zimmer frames and two sticks, crippled up from arthritis moving slowly and think, my god.. how terrible. Now, these same old people are moving quicker than me, and looking at me in sympathy!
DON'T OVER DO IT!
Even just walking a few yards is a huge effort outside..I went to the shops just 2 minute walk away from my flat and was physically exhausted and in a lot of pain by the time I returned.. If you overdo it and don't do exercise gradually (as I did by throwing myself straight into a load of business work, with lots of standing around) you will get massive swelling and that causes further muscle/joint strength loss and increased pain. All you can do then is put ice on it and rest it up elevated.
PLAN YOUR LIFE AROUND RECOVERY
I've been told it will take at least 6 weeks of physiotherapy for my own recovery due to open surgery and having more of my knee lining removed (but even so, I had the lesser localised version, not diffused). If you're fortunate to have the keyhole surgery and very minor joint lining removal, you may get away with much less mobility problems and physiotherapy. I was told by the surgeon that in diffused/worse PVNS, where they remove the entire knee lining, it can take 6-12 months of physiotherapy. So I consider myself lucky it's only 6 weeks..even if it is hell right now in the first week!
I've been told it does get easier as you regain strength, but it's vital you do the exercises and rest. Don't be tempted to rush back into work or lots of moving around, until you have your strength back, else you will just prolong the agony.
So, in summary, here's some key points and advice...
- If you feel much improved after the initial biospy and fluid draining (pre-surgery) don't assume it will last long if you go straight back into using the knee/joint and doing strenuous physical activities. If you need to postpone surgery for any reason and want to retain mobility, my advice is to avoid doing anything strenuous that uses the joint.. even light jogging. Else you risk requiring surgery in a few short weeks, as the PVNS symptoms may return fairly quickly. Make the most of the improvement after the biospy by not exercising the joint strenuously and I think you could maybe postpone surgery (if you need to) for many months or even a year if you're lucky.
- If having open surgery, prepare yourself for major lifestyle change for several weeks/months, and realise you won't be the same for a while or able to do much beyond physiotherapy and recovering.
- Plan ahead, adjust your living space and make sure you have a carer available.. you will need easy access to toilet (bed bottles/pans else), meals cooked by someone or easy to prepare meals... everything you need should be in easy reach of the bed.
- Be prepared for major constipation from daily pain relief. Drink plenty of fluid/water constantly to hydrate.. get plenty of fibre and take laxatives with senna tablets.
- Use your crutches properly at all times to keep weight off the bad leg and don't be tempted to hop around or walk on the bad leg without support. Definitely do not undertake any strenuous physical activity like climbing or lifting things in first week from hospital. The pain relief may give you a false sense you can, but remember the joint is fragile and you will end up with massive swelling, inflammation over the joint and severe pain. Once the painkillers wear off the pain is 10x worse then and they no longer work at normal dosage!!! And the more you take, the more constipation/nausea you get.
- Do all your physiotherapy daily as instructed and don't be tempted to skip them or think you can. You can't. If you don't do it, you will be sorry the next day as you feel stiffness and pain set in. The exercises help the strength return to joint and ensure you retain mobility. It's vital you do them as instructed.
- Don't worry about the anti-blood clot injections. They're nothing to be scared of. Once you try one you will see it's nothing at all, you won't feel a thing most likely as the needle is hair line thin and the pain killers block any sensation.
Hope that helps and hasn't scared anyone! I will post further updates on my progress in the coming weeks...
Paul
zaza02279 morphix
Posted
Â
fadi_48579 morphix
Posted
ashley12698 morphix
Posted
Shaq64 morphix
Posted
Dear Paul,
Thank you for sharing your story. it really is great help. l know its been 5 years ago that you shared this post and it was just after your surgery.
l am a 14 year PVNS sufferer and l am 24 years old this year. The diagnosis was made this January 2018 and l have been scheduled for synovectomy for the 28th of Mar 2018.Frankly, l am terrified as hell. l am also a full-time employee and part time Bachelor of Science student. Ohh and did l forget to mention; l am from South Africa ... 3rd world country - Medical Care is beyond "good" compared to the 1st world countries.
You have clearly outlined what to look out for from pre to post Op - thank you once again. Now its 5 year later, l would like to know how you are managing? Has the condition returned? How are your day-to-day activities, are you better now or you'd have preferred to stay without the Op done?
My condition is the diffuse type by the way and my surgeon proposed and dual surgery. He will open my knee from the front ... remove all he can; in 2 months time he will open the back part and take out the remainder. l should be ok by Sep 2018 so he says and follow up checks will be done to see if the tumor will reoccur.
l hope to hear from you soon. Hoping that you are free from this rare painful condition.
Regards
Shaqueal.
Oldfatguy1 Shaq64
Posted
Apparently every ortho has his own way of doing this. Unfortunately, I was ill advised to have radiation to start with so 30 rounds of 75% strength radiation and my knee was nuked to medium well done. Then 8 months later a very skilled orthopedic oncologist scoped my joint for the synovectomy. I really wish I would have avoided the nuking as it really made the tissue difficult to rehab. Even with all of that, I still have a little of the tumor left in a nerve bundle and it can't be removed for fear I might lose the leg. I was much older than you(65) when this thing broke loose. I never had a bit of knee pain until it broke loose and started bleeding. I had 3 surgeries in less than 2 months to have it drained and cleaned up. They took 200 cc each time and they also drained it with a needle and syringe 5 times taking 65 to 75 cc. Eventually I had a tkr and 9 years layet a revision. With all of that I still have the same spot in the nerves. I will be 81 next week and was jokingly told I would probably finally get rid of this in the oven of the crematorium. ( sort of the ultimate heat treatment). For those that know nothing of the disease that's more than likely a little more info than they want. My surgeon for the last5 years was a associate professor (instructor) at a major university medical center a teaching hospital. Consequently he had seen a number of cases and has been very close and interested in my situation. He moved to another facility recently but I was able to follow him. I am in the middle of the US and my Dr is David Anderson now practicing at menorah medical center in overland park KS. I'm sure your surgeon is well versed in PVNS but I know Dave has published some papers on the disease in case you guy would like another view point.
As far as the synovectomy, don't be alarmed if you experience a lot of bleeding for a week or so. I had a drain and a cyrocuff (a modified ice chest with a motor and a bladder that wrapped around the knee.) It tools a little time for the bleeding to be under control. Eventually they sent me to physical therapy to help get my leg working properly. Due to the damage from all this, I wound up with a tkr about a year and half later. I'm sure you are too young for th a t and if you havent had all the bleeding and damage they wouldn't consider anything that drastic.
Just from a personal study I've done concerning who has this bedamnnable disease I would like to ask you a personal question if you don't mind answering. Are you Caucasian or a person of color. Of all the people that I have come in contact through this forum and through my Dr, I have only run into one person of color. All the rest are of northern European background. Good luck, this is not a disease one would wish on an enemy. As I've told a number of friends, its just my luck that instead of being a 1 in 2 million lottery winner I wound up with a rare tumor.
Shaq64 Oldfatguy1
Posted
Hi,
Its great to get a response so fast. But again it is very sad to be discussing such an unresolving condition. Before l start bubbling around let me answer your questions:
Yes l am a "person of color" l prefer being called Black but l again understand why you phrased it that away
l am getting treated at a government public hospital in South Africa. Spending more than 10 mins with a a physician in such facilities is almost impossible. l learnt more about this condition from "Dr Google" because our med care is poor. A special thanks to people like you who are willing to share their stories with people like me.
Honestly, as l was reading down these threads, l literally started avoiding some of your texts because my heart would literally sink and my world turn black everytime l would read about your experiences. l still can't bring myself to think how one person manages to go through all that you have gone through. l am a 24 year old, people tell me that there is a whole life ahead of me but if l am to relate to what you have gone through could be my future, l feel like my life has just ended. Please Don't get, me wrong; this is too much to bare and l really feel what you mean when you say you can't wish for such for your enemy. One cannot just embrace this condition and live normally as we have seen with some of the "now managed and controllable" chronic conditions. l am so sorry that you had to go through all that, and looking at your age, goodness Lord its unfair for you to be going through so much pain and discomfort. On a happy note - (Happy 81st birthday in Advance). Wish you a better life going further without all this hell pain.
Coming back to me; Yes, the condition is not a clone across us all as suffers, we all reporting different signs and sypmtom, management methods, treatment methods etc. Like l said above, l am not sure but l believe l could be the only one from Africa on this platform or the only African who is getting treated in Africa for this condition. FYI, 3rd world countries are never your best bet when it comes to seeking medical attention; l belief y'all see it when your fellow country-women go abroad to go have those "extra huge implant surgeries" inserted, the results are mostly disastraus... if not fatal. Here l am having to make a life changing decision, either to have the op done or not.
Skeleton breakdown:
they want to open the front part of the knee 28 Mar 2018 and remove most of the tumor.
l doubt l will have half of the care y'all got but l pray and believe that God will take me through all this.
l saw you talked about staph infections coming up due to the metal plate (l hope l do not come to that) in one wayor the other cause l could as well start digging my grave.
Morphine is not a drug they readily have on this part of the world so l might need to brace myself to rely on Tramadol and buy a stiff pillow that l would have to bite hard provided the pain gets unbearable.
Meds to stop blood clots - thrombosis ( l think they will give me aspirin tablets) maybe injections during my hospital stay.
Then will have to go home for self management.
2 months later, l will be scheduled for another Op, from the back this time because my PVNS is the diffuse type.
l really do not know if l will survive this ordeal, Lord knows.
l am not being negative but l am trying to brace myself for the worst. l can not afford, surgeries after surgeries. With people mentioning that this thing can grow back within 12 months; Lord l am at the edge right now.
lt has also come to my attention that clinical trials are running for this condition in America, Australia, UK and China. Have you guys thought of trying these out.
l know there is a lot of controvesy when it come to clinical trails; ethics and staff like that but please read on the PILOT trial (plx 3397). it didnot cure PVNS/TGCT but it helped retain the leg function, lessened the pain and the participants were able to do much more than before. Its a pity that they do not have these for PVNS anywhere in Africa
and it hurts me to say that l would have done anything to qualify for one because trials are less invasive, lots of examinations are done during the course and medical care is way better than my current standard of care. l just find myself not having the means to wake up in any of these countries. If y'all know who l can write to, please share with me.
l hope that they find a solution for PVNS in the nearby future.
cryogen Oldfatguy1
Posted
cryogen Shaq64
Posted
Hi Shaq. Don't be depressed. I had the diffused PVNS too but am fully cured at the moment (fingers crossed), except I do not run or do any heavy lifting. I do workout normally otherwise. Please read my old posts for details
Shaq64 cryogen
Posted
And it goes without saying... CURED?. Thats a blessing hey, l have read for weeks and the stories were nothing other than depressing sad news. Will relate more. Thanks for the support, encouragement and ray of hope. Stay Blessed
Â
fadi_48579 Shaq64
Posted
Oldfatguy1 cryogen
Posted
Oldfatguy1 Shaq64
Posted
Thanks for your kind and poignant response. I honestly believe there are so many directions that this disease takes that you just have to wait and see. One of my original issues was medical attention. I was going to a practice of 8 orthopedic surgeons with over 100 yrs of experience and none had ever seen the disease. It really put me behind in treatment. As far as the staph, it had nothing to do with PVNS. It was all related to the broken femur and subsequent repair. Other things become so much more important in the scheme of things. Being caregiver to a spouse with Parkinson's doesn't give me much time to worry about myself any more. Pain is still an issue but so much less than years ago.
Best of luck. Will pray for your recovery. Keep us advised.
Shaq64 fadi_48579
Posted
Hey Fadi. thank you so hey. l am feeling more comfortable discussing with people like y'all.
l too am so happy for you. Like OFG said, you guys are very fortunate. l hope that all remains well.