PHPV (Persistent Hyperplastic Primary Vitreous)

My daughter, Madison, was born with a condition called PHPV.  My wife and I were writing a blog about her condition, but, like most blogs (including this one), it didn’t get updated as much as it should.  As such, we have taken down the blog, but I wanted to document her story so far so that other parents with children with PHPV can read about our experience.

Madison was born in late December 2009. My wife noticed within the first few weeks that her left eye seemed smaller than her right eye. Her left pupil did not seem to dilate with light and seemed constricted for most of the time.

We brought this up with her physician at the time, who did not think there was anything abnormal with the eye.  My wife was not convinced, and, so, I asked one of my colleagues who was an ophthalmology resident at the time to take a look at some pictures of her. She suggested that I bring her by the hospital that weekend so she could take a better look.

When she was checked out by my colleague, she made the diagnosis of PHPV.  She referred us to a pediatric ophthalmologist, who we saw the next day after she was diagnosed.  He gave us information on the condition, and essentially we had two choices: do nothing and let Madison develop normally with the use of one eye, or, the second option, to do surgery to remove the cataract caused by PHPV and then go through 7 years of daily patching with Madison, as well as the possibilities of complications, like glaucoma and retinal detachment. There was also little hope of having useful vision in her affected eye, even if we followed through with the surgery and daily patching.

My wife and I ultimately decided to go with the more aggressive approach. She underwent surgery to remove the cataract.  She tolerated the operation well and only had to wear an eye shield overnight.

Over the next month or so my wife noticed that her affected eye seemed to be getting bigger.  When we attended our followup visit from the surgery, her ophthalmologist noted that her eye pressure was elevated and made the diagnosis of glaucoma.  We were aware that this was a possibility after the first surgery, and that outcomes were typically poor if glaucoma developed.  She was started on oral medications to try and lower the eye pressure.  We were referred to a pediatric glaucoma specialist, who arranged for an EUA.  The pressure continued to be elevated despite the medication, so she was booked to have a tube inserted in her eye to relieve the pressure.  She underwent a second operation to do this, and it seemed to be successful.

Since the tube (Ahmed valve) was inserted, Madison has undergone 3 EUAs and everything seems to be developing normally in her affected eye.  Her optic nerve remains healthy.

We put in a hard contact lens into her eye everyday.  She wears a patch for 6 awake hours.  She generally tolerates the patching well, except when it is bright out, as her pupil does not constrict in her affected eye.  Her vision seems to be pretty good in her affected eye.  She can do everything with the patch on that she can do with the patch off.  So in that respect, I think the outcome is better than what we hoped for.

Patching at the beginning was very difficult.  We started with doing 1 hour a day, which was challenging.  We usually had to split it up into 15 minutes at a time.  It progressively became easier as time went on.  The biggest issue now is that she can take the patch off herself.  We generally go through 2-3 patches a day.

We found that it was difficult to find good patches locally in Edmonton.  We end up ordering patches from the USA and from a supplier in Toronto.  Our first favourite is the Ortopad Elite. They are available in Canada through InnovaMed.  They are a little pricey, but they seem to be the most comfortable for Madison, and they seem to stick on her skin the best. They do not have online ordering — you have to call them and they will ship them to you.

In the USA you can purchase them through Amazon, which gives you a lot of options for free shipping. Here is a current list of their products:

Our other favourite patch is the MYI Occlusion patches.  They ship directly from the USA to Canada and they have online ordering available.  They are quite a bit cheaper that the Ortopads, seem to stick well, but don’t fit as well as the Ortopads.  They are available here.

I will continue to update this blog as Madison gets older to keep everyone informed of her progress.

UPDATE- May 6, 2012 – Madison now has a pair of bifocal glasses to help her focus on far away distances.  She’s done very well with the glasses.  She wears her patch, contact lens, and glasses for most of the day, and then just her glasses and contact lens.

UPDATE – November 29, 2012 – Madison got a new pair of glasses last month.  They are bifocals, but the actual frames and lenses are much bigger.  She tolerates them very well, and isn’t able to cheat and look over the lenses like her last pair of glasses.  She continues to progress well and is tolerating her patching without any difficulties.

UPDATE – February 27, 2013 – Madison continues to do well.  Her pressures have measured equal with her other eye at her last appointment.  She wears her patch 6 hours a day and wears her glasses at the same time.

UPDATE – December 28, 2013 – Madison turned 4 today.  She continues to wear her patch for 6 hours a day.  She often wears it to pre-school, and she seems to be able to function fine with it.  She has a new ophthalmologist and her last examination was good, with no worrisome findings.

UPDATE – April 27, 2017 – Wow, four years between updates!  Madison is almost done grade 1.  We stopped patching her when she turned 7.  Her vision in her affected eye isn’t great, but she is able to see with it when she has her contact lens in.  The most difficult thing over the past four years is controlling her glaucoma.  She still has the tube in place in the eye and we use two different eye drops to control the pressure.  We take her for a check up every 6 months.

19 Replies to “PHPV (Persistent Hyperplastic Primary Vitreous)”

  1. Dear Brock!I read your daughter story. My son was born with PHPV too, unfortunatly on both eye. We live in Hungary (Europe), and I am searching for a medical center, or a doctor who can operate him. Could you tell me where were Madison’s operations taken?Thank you in advance.

    1. Dear Zsuzi
      OUR daughter had phpv operation on her right eye at istanbul retina web site http://www.istanbulretina.com They have patients from many other countries and a very well known prof.who is specialist in pediatric surgery prof.m.karacorlu did the operation with success.if you like just check the web site .Best wishes for your son.rds A.ertan

  2. Dear Brock,

    While desparetely searching for parents with hspv affected babies i found your story about Madison..First of all thank you for sharing your experiences with your daughter..great help. Our daugher Lara was born with hspv in her right eye.Thank God the doctor was able to see that the day she was born.She had an operation on her eye when she was only 5 days old.The operation was succesfull and we give her eye drops 4 times a day.She was today checked by another doctor who will guide us from now on about the contact lens ,patching and so on.Tomorrow she will have a contact lens and one hour patching will start as you did for Madison.The opth.prof. told us we have to wait until she reaches the age of 1 and so to find out how much the affected eye is cured.He says sometimes even %100 vision is obtained if no complications arise.
    We dont even want to think about the other possible risks which he also mentioned.We live in Istanbul in Turkey and Lara is my third child.Could you kindly keep on updating news regarding Madison and tell us about the things we should particularly pay attention to at this stage( as parents).Many thanks again best wishes and kisses to Madison.

    1. Dear Ahmet,

      Thank you for your kind comments. The first few months will be difficult, but it is important to keep up with the patching, even if you have to do it only 15 minutes at a time to begin with. Please keep us updated on how your daughter is doing.

      Brock

  3. Dear Brock,

    Thank you for your reply.Lara now wears a contact lens and we started patching slowly (although we cant cover the healthy eye completely without any light getting inside but will find a way) she is today 2 weeks old and grows healthy.i would like to ask you which type of phpv was present in Madison (anterior or posterior) and how well she can see with the affected eye now?we watched with my wife some videos of Madison and can tell you that she has some admirers living thousands of kilometers far away..she is such a lovely kid.:))Thank you again for your kind reply and best wishes for your daughter.

    1. Hi Ahmet,

      I’m not sure what type Madison had. She can see quite well from what we can tell, she can see a 1mm bead on her hand during her last testing at the doctor. She functions quite well with her patch on, it doesn’t seem to affect what she can do.

      Thank you for the comments on the videos. We are glad that we can help you in any way possible.

      Brock

  4. Hi Brock,
    How nice to hear that Madison can see well and doing fine with the patch on.This was the best thing i’ve heard in the last 10 days.We are also very happy for her and will pray for her all the time..we hope our little Lara will also do fine as your daughter.i need some more advice from you if you dont mind..as i told you yesterday we are having difficulties in patching -as she is very little and the patch wont fix on her nose side..we use pediatric 3M patches .we looked at Madison photos with the patch on which was totally fine..but she was older in those photos ..(over 1 month).Do you know of any patch brand which is suitable for babies less then 1 month old?Did you have the same difficulties in patching when you first started?(just in case you found an practical way at that time)Although her eyes orientation will start from 6 weeks onwards we want to do the patching as required from the beginning.Thank you again for your kind help .we are really greatfull for yor advices.Kisses to Madison:))

    1. Hi Ahmet,

      We found that ortopad worked the best with sticking to their noses. I’m not sure who ships to Turkey, but I believe there are distributors in the EU, which I suspect would probably ship them to you! They have ones made specifically for children less than 2. They will probably still be a little big on Lara because she is so young, but she’ll eventually grow into them. We found that the 3M patches didn’t work well, they would fall off all the time.

      Let me know if we could be of any more help to you.

      1. Hi Brock how is Madison? We have managed to find ortopad eye patches thro a colleuge in germany.he will ship them to us next week.hope they will work.in the meantime we are using other brands and a large rollfix to secure it on her face(as you did for Madison when she was 5-7 weeks).Regarding the contact lens that she wears now on weekly basis we will take her to doctor next wednesday.How did you manage with the contact lens when Madison was very young?Did you do this on your own or asked for assistance each time? this is not a easy work at this age and a lot of care needed.Do you know also until which age Madison will do the patching?Sorry about millions of questions but we give great importance to your advices and so far you have been very helpfull.Thank you so much.Ahmet

        1. Hi Ahmet,

          We put in her contact lens in the morning and take it out at night. We generally have to hold her down when we do either (hold her arms). We also have a little stick with a suction cup on the end of it that lets us get the lens out pretty easily, but we also do it with our fingers sometimes if we can’t get it with the stick.

          We have been told that Madison will have to keep patching until she is seven years old. So only 4 and a half more to go! 🙂

          Please let us know if we can help with anything else.

          Brock

          1. Hi Brock,

            Thank you for your reply – we will keep them in mind..we would like to send some photos of Lara to you .. (maybe you would like see who are you helping to:)) ) is it ok.for you and if yes pls let me know thro which way.? you havent mentioned in your reply how is Madison doing these days:)) Awaiting your reply and thanks again for your kind assistance.Best regards .

          2. Hi Ahmet,

            Madison is doing well, having lots of fun outside now that it is nice out here in Canada. You can email them to debenham (at) ualberta.ca

            Best regards,
            Brock

  5. Hi – my 10 yr old son has phvp – we also had surgery – vision is poor – the eye is small – 5 months ago his retina detached -may loss his eye. Retinal detachment is a complication around 8-10 – be wise to floaters/dark spots. very scary.

  6. Thank you for taking the time to write this blog. Yesterday my husband and I found out our three-year-old son has this and have been scouring the Internet for information. Your blog has been helpful. Thank you again.

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