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!

Retina Update!

Retina & Cataract Removal

Today, we took our 7th trip to Great Neck, New York with our little warrior! Olivia HATES the car – I know, what baby hates the car?! – but we take the three hour drive every three months because our retina surgeon is the best doctor we have ever met. He alone is worth the screaming baby, Moana soundtrack on repeat and endless amounts of gas. When we first went to this office back in January of this year, we were crushed. This was the place where we found out our little, beautiful girl would need to go under anesthesia. We were told that Olivia’s retina had detached – yes its as scary as it sounds! Like many others with Olivia’s condition, children with PFV run at a very high risk for retinal detachment due to the ‘stalk’ that was not severed in utero. He looked at our tear soaked, terrified faces and explained that he would put Olivia under, go into her tiny eye and sever the stalk and reattach the retina. This was the scariest information we had been told but I am going to be really honest, our doctor was so un-phased, calm and confident, we left the appointment with a sense of confidence ourselves.

By the time surgery day came, we trusted this doctor with our most precious cargo and he did not disappoint. Not only did our doctor successfully remove the stalk and begin the process of reattaching her retina, he removed the cataract that was blocking her vision and left what he called a ‘capsule’ making Olivia a candidate to one day receive an Intra Ocular Lens (this would be a contact lens that is surgically implanted). Not only did he give us our daughter back in the condition that we left her but rather, he went above and beyond!

So needless to say, we take the three-hour drive, every three months to see the man that saved our daughter’s eye. We wait in the waiting room for the chance to see our favorite doctor for five minutes – just enough time for him to check out Olivia’s little nemo eye and give us the latest on how its doing. And the most amazing thing about these appointments is Olivia’s demeanor with this doctor. With others, Olivia is a bit fussy – who wouldn’t be with a bright light shining in their eye? – She gets irritated snuggles into my arms to avoid the doctor poking at her eye. With this doctor, Olivia sits on my lap, still and silent, looking into the eyes of this amazing man. She doesn’t move but rather listens to the direction of his voice and moves her gaze in the way the doctor intends. We are in and out of that room in record time because just like us, Olivia feels most comfortable with this amazing doctor.

Today we went to New York for a retina check up before Olivia’s strabismus surgery next week. Not only was Olivia cleared for her surgery, but we were informed that her retina has FULLY reattached! Her eye is healthy and pressures were in the normal range. With the year we’ve had, this good news couldn’t have come at a better time. We will still be driving up to see our favorite doctor (now every four months – yay!) but for now, we will just keep patching.

Surgery Day

Retina & Cataract Removal

The day we had been waiting for was finally here! Today was the day Olivia would have her retina re-attached and the stalk caused by her PFV would be clipped. The doctor would also check to see how big Olivia’s cataract was in order to determine whether to remove it during this procedure or to wait and do that at a later date.

For babies as young as Olivia (3 months at the time) they are not allowed to eat 5 hours before the procedure. Of course as a new momma that is nursing her baby, I was extremely anxious that Olivia would not only be scared to be in a new place with a bunch of doctors but that she would also be so hungry with no way to soothe. So I ended up setting my alarm for 2:30am in order to get her one last feeding before her 8am appointment. It worked out really well and there were no last minute hungry cries to be heard J. We also had to continue our vigorous drops schedule for Olivia’s eye.

When we arrived at the hospital, they led us into waiting room after waiting room until we were ushered into her final patient room. She was given the cutest little hospital gown that she swam in – but that only increased her adorable factor. We stood in that room and hugged our baby girl tighter than we ever had before. My husband and I were each other’s rock when our minds would wonder to that negative headspace. Our biggest fear – as it is whenever anesthesia is present – was what if she doesn’t wake up? We were afraid of a number of complications we were told could arise when doing such a precise surgery on a very small eyeball. We read horror stories but to every one-horror story were five success stories and that’s what we tried to remember. So we kept hugging our baby, sending each other reassuring smiles and playing a singing Pooh bear her favorite song over and over and over again.

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When the doctor came in to take Olivia in for surgery he made sure we understood everything that was about to happen and what his expected result was. He also informed us that he would be measuring her cataract once he was in her eye. If the cataract exceeded a certain number, then he would attempt to remove it. This would mean removing her lens but would give her the best chance to gain vision in her nemo eye. There are few moments in life that are burned into your memory. The second the nurse took my baby back for surgery and I had to walk away and into the waiting room was the hardest moment of my entire life. We waited in the center of the hospital for what felt like an eternity. About 2 hours later, the doctor came out and gave us the news – Olivia was a ROCKSTAR. She did amazing! She took the anesthesia really well and he was able to sever the stalk in order to reattach her retina and ended up removing the cataract and lens without complication. After taking a couple huge deep breaths and signs of relief, we walked back to see our gorgeous girl wake up.

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As soon as Olivia woke up, I was able to hold and nurse her. She had lots of bandages on her eye and a shield to protect it. She kept both eyes closed the majority of the time but I would feel her squeeze me ever so often as if to remind my husband and I that she was okay. We were released from the hospital and headed back to the hotel for some much needed rest. For anyone going through a similar situation, don’t panic if your baby doesn’t open their good eye right away. We were terrified and so confused as to why she wouldn’t open her good eye after surgery. In fact, it took her about 5 hours to open her good eye while the nemo was guarded. Looking back, it makes sense that she was tired, her eyes were most likely sore and it was probably odd to be unable to open one of her eyes and easier to just keep both of them closed. We stayed over night one more time because after surgery the patient is required to be seen in post op by the surgeon the next day to be sure that everything looks good! We got through the night with Olivia on my chest and very little sleep but received a clean bill of health the next morning. We made it through the hardest day of our lives, together. I couldn’t have gotten through this without my amazing, supportive husband and strong warrior of a daughter. This experience showed me how strong we truly are. We had no idea that new eye adventure we were about to embark on but after getting through this, we knew we could take on anything.