PHPV: my perfect deformity

I’ve been meaning to write this post for months—since September of last year, really. I’m only getting to it now because I’ve had some horror dentist experiences in the past few weeks (there is nothing wrong with my teeth, I might add, other than one front tooth was busted in my youth and poorly fixed. I do mean to get around to fixing that, but it’s just not a top priority). But because I’m all too quick to rant about our (american) health care system, I’m going to start by relating a few good experiences.

Last fall I noticed floaters in my vision. I only have sight, for the most part, in my right eye because of a birth defect called persistent hyperplastic primary vitreous (PHPV) in my left. It’s fairly rare. It happens in utero, beginning around the third month. The eye forms with a gel that turns into vitreous as it develops. With this condition, for some unknown reason, the hyaloid artery does not recede and the gel instead keeps growing and becomes scarred. My doctor explained that it hardens into a stalk, which causes all sorts of structural problems. A few include the stalk pulling on the retina, elevated eye pressure, a smaller size (microphthalmia) and orientation of the affected eye, band keratopathy (calcium deposits on the cornea), cataracts, and extreme light sensitivity, though the scarring prevents enough light from getting to the back of the retina. Because the eye is so malformed, there isn’t much that can be done for it other than to protect the vision of the good eye.

I saw a lot of ophthalmologists in my childhood, and wore a patch for awhile in hopes of strengthening my left eye. It didn’t work. Now, I can see a bit in the left eye, but it’s mostly peripheral vision on the far left. Because of the calcium deposits on the right side of the left eye (which appear to be on the left side of the eye on the right in the photo), I see more out of the left side. The right is just fuzzy. My brain has trained itself to see the periphery with this eye. If I focus hard and try to get full vision with both eyes, I see double, as the left eye is tilted up a bit, as you can see in the picture.

I adored my childhood ophthalmologist, or my adolescent ophthalmologist I should say (Dr. Todd Woodruff MD), who looks quite a bit like David Letterman. I was transferred to him when I was about 11 to be treated for elevated eye pressure, which was occasionally called glaucoma. I was always the only patient in the office under 70, as it’s not a young person’s condition. Regardless, he was much more patient with me than the pediatric ophthalmologist I’d suffered for my first decade of life. After a horror experience with an eye doctor in Oakland during my college years who told me I needed surgery because of a detached retina (I didn’t), and suggested cosmetic work to make my eyes look more alike (it’s fairly risky to even wear a contact lens when one has vision in only one eye), I didn’t trust anyone else with my eyes for a long time. Though inconvenient, I would try to make appointments with Woody, as we called him, when I visited my mother.

This got a bit tiresome scheduling wise, especially as my insurance didn’t cover him. And when my mother started visiting me, I stopped going to the eye doctor. Bad. So, in September, when I started seeing floaters, and coincidentally read in an unrelated blog that it can signal retinal detachment and potential blindness if untreated, I logged on my health insurer’s website to find an ophthalmologist I might trust. Oh dear.

This is getting a bit long. I’ve not even gotten to my point. Even so, I’m going to continue tomorrow.

other posts on phpv:
my perfect deformity
my perfect deformity, part ii
PHPV: the eye, vision, and how I see


8 thoughts on “PHPV: my perfect deformity

  1. Hey, Im 24 and have PHPV… I notice lately I have been getting red eye and increased pressure… I have recently had a successful stabsimus surgery and have eyes that are fairly close in size and color but not perfect by anymeans

    It would be nice to talk more over email about what you are currently doing for your PHPV… Also did you say its not good to go upside-down? im confused about that… hmm

  2. Hi Corey, thanks for the comment. I don’t do anything for my PHPV short of getting an annual exam. I could arguably use stabsimus surgery, but it’s never been suggested and prefer the least invasive treatment possible. Being upside-down increases eye pressure. I will drop you an email. ~A

  3. I’m Sasha, I’m fourteen and I have PHPV. Because this case is very rare it’s difficult to find much about it on the internet but can any of you tell me any options I have? I’ve heard about an op I could get which joins the muscles in the eye so they move together and in the same direction. I’ve also heard about mordern day glass eyes which look and feel great but I’m still slightly confused by all this. Any help?

    1. Hi Sasha, Thanks for the comment. I know Corey had the stabsimus surgery. I’ve never heard of someone with PHPV getting a glass eye, but really don’t know. Are you on facebook? There is a PHPV group there which might have something to say, though I find that most activity on the internet is from parents who have babies with PHPV and are upset and looking for info. It’s much more rare to hear from someone who has it. It would be interesting to know how different the treatment is in the UK as well. Very different medical system. Best, A

  4. Thanks yeah I am on facebook so I’ll check that out. I lived in the states for about three years and one thing they said to me far more than the docter in England was that they highly reccomend getting poly carbonate glasses to protect my good eye. I have got some after recently having an accident with my bad eye where I got struck. I got a mild femur and the pressure is still high but I’m OK. I just thought that if I accidently got caught in my good eye I could of been in a bit of trouble with my sight. I’m still going to the docters to get the eye looked at so next time I go I’ll ask about options with them and give you an update. 🙂

    1. Yes, my eye doctor always insisted on polycarbonate lenses, especially for playing tennis, etc. Now these are the norm for lenses, most usually. I tend to prefer less treatment than more, but my eye isn’t that dramatic, I don’t think. Some docs go the cautious route, and insist that glasses are safer because they protect the eye and there’s no risk for infection as with contacts. Other doctors go the cosmetic route, not least because there is money to be made in it in the states.
      :-/ Yes, keep us up on your options. Great to hear from you!!

  5. So the docter described an operation where they loosen the muscles on the outer side of the eye and tighten the ones on the inner of it. Basicaly bringing the affected eye more central. She said it was just one, simple op so it’s likely I’ll get that in a few months time. The docter then also said that if I want it to look even more similar they could put a lens in it (a bit more practical than a glass eye). Thanks very much for all the advice and info!

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