PHPV: the eye, vision, and how I see

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This is part iii of my perfect deformity. It stands alone, but part i and part ii are informative as well.

PHPV (Persistent Hyperplastic Primary Vitreous) is a rare, congenital eye disease that begins around the third month in utero. I have it in my left eye (right to you) and have written about it before. In short, the primary vitreous and hyaloid artery of the developing eye do not become clear and recede (they’re persistent), but instead grow even more (hyperplastic), scar, and form a stalk. Sometimes this is in the front of the eye. Sometimes the back. Mine runs from the cornea in the front all the way back to the retina. This, my ophthalmologist calls “classic,” “amazing,” “beautiful,” and “textbook” when describing it to her residents, whom she will pull off lunch break to view because it’s so rare to see such a case. Also rare because the cataracts and calcium deposits that can develop on the cornea often make it impossible to see into the eye. Not so for me. Mine fog up only the right side of my eye, so you can see straight in.

It is oddly comforting to have my deformity so appreciated. And since I’m a huge advocate of real world education, I’m happy to let the apprenticing doctors take a look, painful as it may be.

When I was little, as in the photo above, the deposits gave the eye more of a blue cast, so I appeared to have one brown eye and one blue (no, not like your cat). Now the coloration isn’t as extreme, but the eye is smaller (microphthalmia) and doesn’t track with the right. Other side effects are the retina peeling off a bit and elevated eye pressure (glaucoma). I have both, though both are pretty stable.

adult-phpvPhoto: Far right, adult eye with PHPV doesn’t track with normal eye.

I have yet to meet someone with PHPV. There’s a facebook group called “People with Persistent Hyperplastic Vitreous Unite” but it should be called “Parents of Babies & Toddlers with PHPV Support and Discuss.” I’ve chatted online with someone upstate (we’re FB friends now), and a few people here who have read my other posts, but I have never met another person with this disease. And before the internet (most of my life), the only information I got was from my ophthalmologist. There’s only so much one can absorb in a visit.

That’s why I write this. There’s very little info out there, and nothing about what it’s like to have PHPV.

Even so, I’ve known I see differently since I was young. My pediatric ophthalmologist (he was mean. Parents of Small Children with PHPV, please do not send your child to a mean eye doctor. Traumatown) gave me a slew of tests. One was a fly coming off a board, and I was meant to say if it was 3-D or not. It was the 70s, and this was the “Titmus Fly Stereotest.” Oh, I found a picture. What a horror.

I knew there was a correct answer to the question and I was pretty sure it was not what I saw. So instead of answering as such, I guessed. I don’t remember if I guessed right. I remember the doctor, the scariness, the stress, the tests, and trying to guess what I was supposed to see and say. I was perhaps four or five, and my dad was there in the dark doctor’s office, so I knew it was serious business.

Titmus Fly StereotestI do not have stereopsis, or, what most people take for granted as three-dimensional vision. Stereopsis requires that both eyes track together, so that the brain can use the perfect disparity between the right and left eyes to judge depth. A few inches apart, they see a slightly different image and the visual cortex uses that difference to create the third dimension. It is a trick of the mind. The cells in the visual cortex of the brain that do this develop quite early, and they rely on sight from two properly aligned eyes.

What does this mean to a kid? I sucked at ball games, because judging the distance of a ball moving through the blue sky is pretty much the pinnacle of three-dimensional sight. I loved photography since before I can remember, and got my first camera for Christmas at age ten. I first thought I was trying to freeze and memorize images, just in case I went blind. Later I realized that using one eye to make two-dimensional images is my reality, so of course it comes naturally. Though I do wonder how others see photographs. While your two-dimension vision is no different than mine, it differs from your regular, three-dimensional vision. Mine does not. All the tricks my brain uses to judge depth are pretty much there in a photograph. So perhaps I’m good at relaying the third-dimension in only two. I can’t know.

I also realized in high school that I could play tennis, as long as there were no lobs, because my brain used the lines on the court to judge where the ball was. I liked that. I did not like 3-D movies, because they didn’t work. I saw a lot of lines. I didn’t and don’t like many movies because the brightness hurts my eyes, which are ultra-sensitive to light. Especially in a pitch black room.

These things I had figured out on my own. In the last few years, I’ve noticed even more. Partly due to technology, and perhaps partly due to yoga and meditation, and simply being more aware of my experience. This is getting a bit long, so I’ll save more on how I actually see for the next post.

other posts on phpv:
my perfect deformity
my perfect deformity, part ii

15 thoughts on “PHPV: the eye, vision, and how I see

  1. Hi there, thank you so much for allowing me to read your post, very informative. My daughter rachel has PHPV in her left eye also, she is nine years of age now. Over the last 9 years there has been some very daunting times for my husband and I feeling useless not really understanding what exactly rachel was able to see. Rachel doesn’t lie under it, but I have noticed as she is getting older and becoming more aware of her condition it does bother her. Rachel’s eye is very very light sensitive, so much so that even with perscription sunglasses she still puts her hand up to cover her eye when out in sunshine or under very bright lights. To be able to hear what it is like from a person living with PHPV is really great. So thanks again and I look forward to your next post.

    • Hi Tina, thanks for your thoughts. Yeah, prescription sunglasses are with me at all times, even in winter. I also wear a hat with a brim in summer, as the shade really helps block the light. Maybe Rachel can find some cute hats to make life more fun? :) I’m sure it’s hard not to worry, and it’s also hard for a kid to explain because they don’t know what ‘normal’ sight is. It’s great you keep up on it though. I’m sure she appreciates and benefits from your concern. More soon! ~A

  2. Hey I too have phpv. I am 38 years old and have always been told that I am among one of the first documented cases. My picture is in medical books. I would love to talk to you. You can contact me via Facebook or email. I live in Georgia. I have never known anyone else with PHPV.

  3. Hi Sallie,

    Wow, really? That’s amazing. I’d love to see the books.

    This makes me think. We are about the same age. Okay, fine, I have one year on you, so my case would have been earlier. I’ve never considered when PHPV was first documented–I assumed it had been around. And looking back, in my childhood I was always told that I had band keratopathy (one of the many complications of PHPV), not PHPV. A few years ago my mother asked me what PHPV was and at the time, I just thought it was, you know, my mother being my mother. But maybe I wasn’t really diagnosed with PHPV until much later on. Interesting.

    A friend and I are playing with the idea of a road trip, returning up the eastern seaboard. Maybe we could meet then! I’ll be in touch. Thanks for writing!

    ~Anastasia

  4. thanks for your time to tell this story. I am in research mode with a 2 week old granddaughter diagnosed in her left eye. She will have cataract operation at 4 weeks and I am scared for her.
    I am trying to learn all I can to help her parents and my granddaughter as life goes on.
    I am in search of the perfect doctor and technology at todays time frame. Yes a nice Dr is important in a small child. The scariness of it all can make it a apprehensive awareness to the child which I wish to keep as normal as possible.
    I will research the facebook site for young mothers with infants and this condition in various stages of development so my daughter in law can contact.

  5. Hi Gabe,

    Thanks for sharing this. You’re an amazing grandfather for being so involved! I must say that I’m a bit alarmed by what is being done to babies with PHPV these days. Most usually, there isn’t much to be done for an eye that stopped forming properly in only the 3rd month in utero. Not much sight can be saved, and doctors do profit immensely off of painful, stressful interventions that have no benefit. I’m also saddened to see that babies have their PHPV eyes removed completely and replaced with glass. For cosmetic reasons? The eye isn’t a threat. I have considerable peripheral vision on my left side with my PHPV eye. Had it been removed, I would lose about 70degrees of vision to my left. To what benefit? I think having a doctor who is willing to have this conversation and be honest about all options is extremely important.

    People with PHPV can live totally normal lives, with a unique way of seeing the world. Good luck with everything! ~Anastasia

    • Hi Samantha, Sorry for the late reply, I was out of town. I assume you’ve always had it and were just diagnosed now? Makes sense, as it wasn’t really defined, I think, until the early-mid 70s. Has anything changed since diagnosis? Best, A

  6. Thank you for sharing this story. I am findng comfort in knowing there are others out there that also have PHPV. I was born with it effeting my left eye. I am now 26 years old. I too was told this condition is very rare and born in the 80s they said not many doctors would see that in their time practicing. The more research I do, as an adult, to become more informed, the more I am finding that others have it as well. I am curious what your doctors say can be done as you age? They say to me, not much can be done, but only continue to have surgeries to correct the laziness. Is this the same things you hear? Thanks for sharing.

    • Hi Ashley, Thanks for your comment. Yeah, it’s strange in this world of so many to have something that you don’t know anyone else with, right? I’ve still yet to meet someone with PHPV. Yes, the eye is so malformed from the beginning that it really can’t be corrected, especially as it ages. Frankly, I am glad I was born before all of the surgeries they are doing now, which is probably traumatic for the infant and if the eye is removed, causes loss of vision. I know I value the peripheral vision I have in my PHPV eye. I suppose there are always cosmetic corrections that can be made, but I prefer doctors who are interested in keeping my eyes as healthy as possible. Thanks for being in touch!

  7. Hi – I am 58 and have PHPV in my left eye. I started seeing an eye dr. when I was 2, and my parents were told there was nothing they could do. The hyaloid tissue is still there at the retina, but does not go all the way to the lens, so my eye looks normal but started crossing when I was 1. My parents didn’t tell me much when I was growing up, probably because they didn’t know much, so I never realized I saw differently from everyone else. I always just thought I was clumsy, and I sucked at ball games and cartwheels and tree climbing. I had surgery when I was 15 to straighten my eye and was so happy to look normal!

    Then in my 40s I started to develop high pressure and a cataract in that eye. I’ve been managing with eyedrops and put off cataract surgery because the specialist was cautious about doing it if it wasn’t necessary. But the pressure’s getting harder to control and the cataract is getting dense enough that the bit of peripheral vision I DO have is getting cloudy. If I don’t have this surgery, pretty soon I won’t be able to see through the cataract anyway.

    So, I’m looking at cataract surgery this summer, hoping that will clear up the cloudiness and maybe relieve some of the pressure. Just had an ultrasound of my eye and actually saw the “fibrous stalk” attached to my retina – pretty cool to see. The surgery is riskier than average because of the PHPV, but they will have their retina specialist ready in case of complications. I just want to keep the peripheral vision because it’s useful when I drive. Also, while I can’t read or drive without my good eye, I can navigate my house without it. (Found that out when I scratched the cornea of my good eye and had to have it patched for 24 hours.)

    I appreciate reading your blog and how you’ve encouraged worried parents. PHPV has not kept me from leading a normal life – I’ve just had a few manageable limitations. (Jet pilot, brain surgeon, or Nascar driver were not options for me, among others.) My kids used to toss things to me, just to be tickled at how I couldn’t catch them, but it was good to teach them that no one’s perfect. I’ve also never met anyone else with this. My specialist told me he’d seen only a handful of people with it over the years, some in Boston and some here in the Philadelphia area. I grew up in Nebraska and saw an eye dr. at a teaching hospital, so every time I came in he’d round up all of the students to come and look into my eye. It got a little old over time. Hooray for connecting over the internet!

  8. hi Diane,

    Thanks for your comment! It’s interesting to hear from someone >40 with PHPV. Most people seem to be younger (because it’s more prevalent, more frequently diagnosed, or there are more young people on the internet? Who knows).

    Yeah, I don’t think they really knew much until the 70s, and then more and more. Even in the early 90s I went to a group of specialists in the bay area who didn’t know it and told me I needed surgery for a detached retina (terror. I didn’t). It can be hard to find a good ophthalmologist (mine dropped my insurance so I’m without again).

    I’m not sure I really realized I see differently until my 20s. It’s in retrospect I understood the tennis, etc. Though I took all the 3-D tests, no one explained it to me, unfortunately. I don’t think docs really knew that much, or they didn’t explain it. And now, seeing as they are so willing to remove the PHPV eye, I wonder if they understand much now. The sight in that eye, as you say, is helpful for many things.

    I’m sorry your eye is troubling you now. Stressful. It’s good your specialist is cautious and is well informed. The ultrasound sounds awesome. Send it on if you have it! Thanks so much for dropping by, and let me know how your surgery goes. And if I’m ever in Phil., we must have coffee!

    Best,
    Anastasia

  9. Hello
    i have two month old son and he have diagnosed phpv hes left eye.this eye is smaller than right eye.he has also catarac This eye.we live in Finland.i hope that if someone have This same diagnosed please send me email fverholmmiia@gmail.com

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