What is PFV?

Our Latest Adventures, Retina & Cataract Removal, Strabismus Surgery

Persistent Fetal Vasculature or PFV is a developmental disorder that occurs at birth. In utero, the eye is built by the body using different structures – called stalks. In a typical child, the stalk will regress or wither away when it is done building the eye. In people with PFV, the stalk fails to regress causing structural abnormalities that usually lead to impairment in their vision (ASRS). In most cases, PFV will only affect one eye.

When looking at the eye, you will first see the cornea, followed by the Pupil, Iris and lens. But, the structure doctors look at first when dealing with a PFV case is the Retina. The retina is a thin layer of nerve tissue that is very light-sensitive that sits at the back of the eye. When a person sees light, the light enters the eye through the Iris and heads to the back of the eye where the light is processed into images which are focused and reported up to the brain, producing sight. Since the connecting the retina to the front of the eye never retreated in people presenting with PFV, the light is unable to filter properly through the Iris and its trip to the retina is disrupted. This causes poor to no vision in these individuals. This disorder usually does not progress after birth but as the eye continues to grow, the stalk and its components will not grow with it. As the stalk holds back other structures of the eye, it may cause other issues moving forward. The stalk may cause the retina to detach or the person’s eye to develop cataracts.

As you can see from many discovery stories on our blog, diagnosis of PFV is hard to come by. Some are lucky enough to spot it at birth but in others, it may present itself in other ways that lead to misdiagnosis. For example, PFV can present itself as the child being cross eyed (Stabismus), having abnormal eye movements (nystagmus) and as having a lazy eye (amblyopia) (ASRS). In many people with PFV, the affected eye will appear smaller than the other, however this isn’t the case for everyone.  When a doctor believes the person may have PFV, they will look at the back of the eye to see if the stalk is present. Ultrasounds or MRI scans may be required to rule out other disease like Retinoblastoma – a rare tumor that may present itself on the back of the eye. 

When Retinoblastoma is ruled out, the doctor may suggest surgery to remove the lens, remove the stalk and in some cases, reattach the retina.  The surgery may not completely restore the childs’ vision to a state of “normal” but, without the stalk holding back the structures of the eye, the function of the eye can be improved and the affected eye may grow at a typical rate post surgery (ASRS). 

For most children and families, post surgery starts an entirely new journey. A contact lens is usually prescribed to children who had their lens removed during surgery. They may also get glasses to use instead of or in addition to the contact lens. Soon after surgery, your child may begin patching therapy to help with their strabismus or amblyopia. 

Unfortunately, peer reviewed studies of people with PFV are far and few between. When you do a general search of PFV, you will be directed to articles published that are full are very medical terms with a lot of conjecture and not many reliable numbers. The truth is, PFV is rare and doctors simply have not had the opportunity to study this disorder to its fullest potential. Until more research is done, we will continue to share what we have learned from these various medical journals and will continue to hope for more research moving forward!

The aftermath of strabismus surgery

Strabismus Surgery

When we were first told Olivia would need Strabismus surgery, the doctor filled us with a lot of ‘worst case scenarios’. They said that unlike the cataract surgery, Olivia would be in a lot of pain for quite a while after this one. We were told she could not touch or rub her Nemo eye at all during those first few days postop which is so daunting for the mother of a one year old to hear! We prepared the house for every single scenario – had all her favorite movies ready, her favorite snacks and macaroni on hand and every medicine she could possibly need. We were even ready to put an air mattress in her room next to the crib in order to watch her all night to be sure she didn’t wake up and start rubbing her eyes.

I am so happy to report that in our experience, the best case scenarios played out! When Olivia woke up from anesthesia, she was groggy but smiled right away. Her eye did secrete a few bloody tears which is completely normal for the type of surgery she had done. We could see a lot swelling and her eye was a beet, deep red color. She kept it somewhat closed due to the swelling but we could see her eye at all times so we know she kept it open – at least a little. Despite the look of her eye, she seemed to be in little to no pain and she as happy to see everyone, including the nurses and was even chatting up a storm.

Our drive home was rough trying to get her to leave her eye alone since it was night time and she was pretty done with the car seat. But when we got home, Olivia played with all her toys without skipping a beat. Olivia did her normal bed time routine with the introduction of her perscribed Tylenol dosage and her parents watching her like hawks in the camera. She ended up waking up a couple of times that night but got through without needing more medicine – but did need quite a bit of night time nursing sessions. In the morning we gave her children’s tylenol as soon as she woke up and every 4-6 hours after that. We did that very consistently for about 5 days. On day 6, we just watched her for signs of discomfort and if I felt she was hurting I would give her the Tylenol. By the time a week post op was through, Olivia was no longer taking Tylenol and was back to sleeping through the night without constant nursing and comfort. We continued to put the goopy eye ointment in her Nemo eye every morning and every night until the tube was completely gone – which took about 3 weeks.

After just one week, we could see a huge difference in the orientation of Olivia’s eye! It would look directly at us. Our surgeon did such an amazing job that her eye was right in the center. The redness slowly dissipated and revealed her gorgeous baby blue iris looking straight ahead. Now this surgery did nothing to change how much she can currently see. She still can’t see very much through her Nemo eye (which soon became painfully clear when we started patching again) but having her pupil forward gives her the best chance to gain more vision. In theory, more light will go through the front of her eye which will create more opportunities for more vision.

We are over the moon with how well Olivia handled this surgery and of the results so far. We are looking forward to getting back to our routine and to just keep patching.

Strabismus Surgergy

Strabismus Surgery

Well, surgery day came out of nowhere! We got the call that Olivia was scheduled for surgery at 10:30am at Yale New Haven hospital. We were advised that she couldn’t eat anything after midnight, breast milk needed to be done 4 hours before her procedure and apple juice or clear liquid needed to be finished 2 hours before. It’s funny how yes, I was of course nervous about Olivia being put under anesthesia and to have this operation but that morning, I was most concerned about how my one year old daughter was going to understand that momma couldn’t give her milk! Luckily for us, Olivia did really well in the car that morning on our way to New Haven! We missed all the traffic and got there super early just to be safe. We checked in and we were brought to a small doctors office where we set up Olivia’s favorite monkey – Pinkberry the monkey to be precise – and Moana on the tablet. Olivia was given a miniature check up which included listening to her lungs, checking her blood pressure and asking mom and dad a ton of questions about how we feel she’s been doing. Olivia LOVED the nurse and would reach for big hugs every time she came in. The nurse even filled us in on what to expect while she bounced Olivia on her side. When Olivia started to get ansy and ask for “milkies” we started walking the halls and asking the other nurses for stickers. This was the day I was introduced to what a life safer stickers are! Those stickers adorned with Mickey and Poppie the troll were enough to keep her mind and fingers busy until it was time to get all dressed up in our hospital gowns.

When it was time, the anesthesiologist came in and explained to my husband and I, what to expect. I decided to take Olivia in for the procedure so we both got all dressed and headed out on our walk – for this part we took “Patches” the giraffe for morale support. Now, be mentally prepared if you decide to take your little back to the operating room. I’m SO glad I took her but it did shake me a bit. You walk into a big, cold medical room. I was told to put my sweet girl on the bed and they put strabnwery scented gas up to her mouth – we picked that scent :). Having such a strong willed little girl meant I had to hold her still while the anesthesiologist got the gas on her mouth. For a little reassurance, I was told the harder they fight, the more breaths they take and the faster the gas should take effect. Unfortunately, that reassurance dissipated as I watched my princess stare into what seemed to be my soul and cry momma. I looked at her and kept telling her I was right there and that it would all be okay. I reminded myself, while holding back tears, to be strong for her and that she needed this procedure. That it was going to improve her quality of life. After about 60 seconds of crying and fighting, she drifted to sleep. The nurses covered her up, I gave her a kiss and I was whisked back to my husband in the waiting room. Now, I was warned that Olivia could have reacted to the medicine by convulsing and twitching. She didn’t thank goodness but that could have happened – and it would have been totally normal.

Patches and I took the long cold walk back to Dan where I understandably collapsed into his arms and let out the strength I tried so hard to keep together in front of my girl. We both took a deep breath and went to the waiting room to watch the screen that would eventually give us the green light to smile. The procedure took about 2 hours – long enough for my teacher husband to write his lesson plans and for me to explore Yale – which in case anyone is heading there, is beautiful and has the BEST cafeteria! After doing school work and eating my last bite of pork roast – yes, pork roast! – we saw the light next to Olivia turn green and say “in recovery”. We both broke down, breathed a sigh of relief and knew we had made it through another one.

Our amazing surgeon came out to see us and told us that Olivia did wonderful! Both sides of the eye responded really well to the manipulation of the muscle. She warned us that her eye would be very “Santa clause suit” red and her tears may have blood in them but not to worry. We were given an ointment to apply twice a day in her Nemo eye and little arm restraints in case we needed them to prevent her from rubbing her eye – lucky for us, we didn’t need them :). We were then led back to recovery where we saw this little tiny peanut in a giant hospital bed, still asleep from the anesthesia. We waited there as paitently as can be expected for an hour before she finally woke up. Waiting there for a loved one to wake up has got to be one of the hardest parts of surgery. You do everything in your power to not let your mind go to that dark place that says “what if” but until you see those baby blues look at you, it’s just the scariest part. She woke up very drowsy but aware and went right for her “milkies”. After eating, she was smiling and waving at all the nurses. She didn’t skip a beat! And despite how badly I just wanted to hold my baby, she just wanted to be with all the nurses! The nurses loved her and took her to say hi to all the doctors – which Olivia absolutely loved.

Our experience at Yale and with Stabismhs surgery was one I would recommend to any family that is looking to have such a procedure done. Next week, I will post Olivia’s post-op procedures and how she did healing that little Nemo eye. We may be on a mandatory patch break but to all our followers – just keep patching!