Article

CVI: past, present and future with Al Lantzy

This interview with Dr. Alan Lantzy explores medical aspects of Cortical Vision Impairment (CVI).

Dr. Al Lantzy, a former neonatologist and chair of pediatrics at Western Penn Hospital, is interviewed about CVI (cortical visual impairment) by Robin Sitten. His last name is very familiar, and he is the husband of Dr. Christine Roman-Lantzy.

What are the medical causes of cortical visual impairment (CVI)?

Robin Sitten: Well, let’s just start with you explaining to us something that I think many people know, but many people are still very confused about. But what are the medical causes of cortical visual impairment?

Dr. Al Lantzy: Medical causes of cortical visual impairment are many. Anything that can damage the brain or cause abnormalities to the brain is a potential culprit in medical conditions associated with cortical visual impairment. Most of the time, we have some kind of history, like a known episode of low oxygen or low blood flow. And that would be called asphyxia. Sometimes there is a stroke. Sometimes there is something called periventricular leukomalacia, which is damage to the white matter. And that occurs 99% in premature babies.

There can be infections. And sometimes the brain just isn’t put together in the appropriate manner from the beginning, a genetic cause. The majority of kids have asphyxia, which means that they either had low blood flow or low oxygen to their brain for a period of time either before delivery, during the delivery process, or even after if they’ve had an episode, a medical condition that caused them to have low blood flow and low oxygen.

Are premature infants more at risk than full-term infants?

Robin Sitten: Is it true that preemies are more affected than full-term infants?

Dr. Al Lantzy: Actually, no, it’s not true. When we looked at the data– and by we, I mean Dr. Roman and myself– of over 450 children with cortical visual impairment that came to the Pediatric VIEW Program at West Penn, the majority of the babies that had cortical visual impairment were term. Now, premature babies are at higher percentage risk, but there are many more term babies born that are at risk of having a congenital malformation of the brain, a stroke, asphyxia. About 2/3 of the children that we have studied were term, and only one third were preterm.

Preterm does set you up for a higher risk of intraventricular hemorrhage, a higher risk of periventricular leukomalacia. But because there are more term babies being born, term infants have a higher– there’s more number of term infants with CVI than preterm infants. That’s something that not everybody pays attention to, I think, because you think, oh, prematurity, born only at a pound or a pound and a couple ounces, and they’re much more likely to have other problems. They are more likely to have cerebral palsy, some learning disabilities, but CVI is more common in term infants than preterm infants.

Now, you’ve been a neonatologist during some important changes in how– certainly, premature babies have been born prematurely for a long time through human history. But it seems to me that in the lifetime of your practice, there’s been a lot of changes in survivability, health of the mother, different responses to those little ones in the NICU. Can you reflect a little bit on the changes that you’ve seen over the years?

There have been dramatic changes in the survivability of infants born at lower and lower gestational age. A full-term baby is considered 37 to 40 weeks. Anything less than 37 is premature. And when I first started into practice as an attending physician, 1979, there were no babies less than 2 pounds that were born with lung disease who did very well. That gestational age has now changed to 24, 25, and in some places even 23 weeks gestation from the 28, 29 weeks. And those babies now can survive, not at a great percentage, but can survive down to a pound at birth.

Now, they are at risk of other problems– higher risk of infection, higher risk of another cause of visual impairment, retinopathy of prematurity– that we do not see in term infants. But if you’re born at 24, 25, 26 weeks gestation, you are still at risk of developing ROP. In the United States, we’ve been able to control that with appropriate equipment to monitor the oxygen levels. But in developing countries where some of those babies can survive, ROP is still a major cause of visual impairment. But in the United States, in developed countries, we now all know that it is cortical visual impairment.

Why do you think that CVI is often diagnosed later than ocular visual impairments?

Robin Sitten: And yet CVI is diagnosed much later than any of these ocular impairments. Why do you think that is?

Dr. Al Lantzy: Oh, when I started into practice a long time ago, almost 40 years, CVI was considered Roman numerals for 106. Nobody knew what CVI was. Now, because of the work of very few people, but Perkins included, Dr. Roman, others in the field, Jim Jan, CVI has become a hot topic. And I think physicians, both ophthalmologists, optometrists, as well as pediatricians, developmental follow-up, are starting to pay attention to cortical visual impairment.

It’s only been 20 years that that has become a hot topic and changed the education. I know Perkins has changed many of their programs and has made environmental adaptations because of this CVI. Bridge School near San Francisco has done the same thing. And it is gradually catching on that CVI is now the leading cause of visual impairment in not just our NICU graduates but people from the PICU, Pediatric Intensive Care Unit, and it can be a problem from birth, or it can be acquired from trauma, infection, et cetera.

I’ll go back 10 years ago. A very prominent pediatric ophthalmologist at a very prestigious university in Maryland, but I won’t mention its name, saw a family whose child had CVI. And he told this family, there is nothing we can do. The eyes are fine. The brain just got damaged. And they said, well, what can we do?

And his answer 10 years ago was, I think you should just go home and not put the baby in a closet. Now, that same very famous pediatric ophthalmologist recently told another family that was there for an opinion that they should see somebody who has experience with cortical visual impairment, somebody that is Roman-Perkins endorsed, and their child’s vision can improve. Now, that’s a big change in 10 years.

Robin Sitten: That’s a big change.

Dr. Al Lantzy: And it’s something that is slowly sweeping and helping. There is a person, Sharon Lehman, who is a pediatric ophthalmologist in Delaware, who has made inroads in the pediatric ophthalmology community with information about cortical visual impairment. She’s also spoken to a group of a thousand neonatologists who see these kids in the NICU but don’t really know what to do with them. So hopefully, in the next couple years, the diagnosis will be made earlier, still not as early as obvious ocular impairment.

The problem is many of these kids are just trying to survive. And if you have seizures, a gastrostomy tube, a tracheostomy, you’re still on oxygen, it’s very tricky. Your vision sometimes can get missed until later. But we all know how important it is to start interventions as soon as we can when there’s still the maximum plasticity of the brain, the maximum time for the baby to make responses and really start to, hopefully, improve their vision. And ultimately, that will be great improvement in their life if we can get that. And the earlier, the sooner, the better.

I tell this story all the time about take her home and hope someday she smiles, that that was what she was told. And you see that child now and how much the intervention has really helped her participate in her family and her community, in her school.

Yes, they’re great. I mean, the kids that are in this moms’ group– they have made so much progress. And I don’t know, but it’s a lot because of the families. Perkins has had a very nice situation. And I can name five people who are really great at interventions and teaching for CVI. But it’s just in the last 15, 20 years that this has really become a subject. And I’m not going to brag about her too much, but there’s one person that is primarily responsible for that revolution.

Robin Sitten: Well said. Well said. And I think it is important, your point about how the families need to have each other. They need to have that support and know that they’re not alone. And you can see the difference when someone signs into these parent groups and says, we got this diagnosis today, and I didn’t know what it was until I did an internet search, and then I found you guys, and immediately has 3,000 new best friends.

How has the NICU experience changed for the infant?

Robin Sitten: You mentioned just talking about how we’ve now become able to recognize it, whereas before maybe it wasn’t recognized for a while. And I just wonder how it compares to other trends that you see in those NICU graduates, particularly related to their vision but in other kinds of trends. How has that NICU experience changed, particularly for the infant?

Dr. Al Lantzy: Yeah, neonatologists, even back 40 years ago, were aware of breathing conditions, which are most commonly what happens and why premature babies get in the NICU in the first place, poor development of their lungs. We paid attention to nutrition. We have improved the way that we have ventilated babies, the way we have fed these premature babies. What we didn’t pay attention to, probably, until the last 10 or 12 years was how was their brain doing, their brain, which would include visual impairment if things didn’t go right.

But we just thought, oh, well, they’re going to do fine. But it’s a very different environment between being in an incubator when you weigh one pound six ounces versus remaining in your mother’s nice, warm uterus with appropriate nutrition, lack of overstimulation, lack of getting your foot stuck for blood work. And it’s really only been about 15 years that we started to pay attention to have these babies not just survive but actually thrive. And we have diminished the overstimulation.

We continue to make improvements with respiratory status, with surfactant, which is used to help the baby’s lungs, with the use of antenatal steroids for the mother. But the brain has– we wanted him to survive. Now, in addition to that, there’s much more attention to thriving. And I think that when that trend continues– and I expect it to continue– that we will have further adaptations to the environment to try to minimize the overstimulation, to try to minimize damage to the brain that is being done just by virtue of being out of this perfect, idyllic in-utero environment. And we just can’t replace in-utero life versus extra-utero life.

Can you tell us a little bit about your own experience as a neonatologist? How did you get into that field of study?

Dr. Al Lantzy: Well, way back in medical school, I was lucky enough to spend a rotation during my third year in the NICU. And the NICU was at the university hospital. And I was a student. I was fascinated by how small the babies were. I had a lot of time to talk to parents, and I actually bonded with one particular family. And they eventually asked my opinion. I’m a third-year medical student, but I had spent so much time talking with them that they wanted my opinion when it came to making a decision, even though they’ve talked to the attendings. So external gratification was very big for me.

I met a physician by the name of Dr. Hugh Macdonald, who became my mentor. He was at the university for 13 years at that time. And he just was the person that most inspired me to go into neonatology. So to do neonatology, you have to do pediatrician time, three years for me, and then go back and do a fellowship. And that’s specific time in the NICU. I was lucky enough to get credit for some of my work, so I only had to do one year of a fellowship. Right now, it’s three years.

In addition to that, neonatology gave me a chance to do something with my hands. In the general office pediatrician practice that most people take their kids to, there’s very little– you examine the patient maybe, maybe. You stitch a cut every once in a while. But in the NICU, you get to put in umbilical arterial, umbilical venous catheters, intubate patients, put in central lines. And so it was a combination of not surgery but some procedures in addition to the medical care that goes along with being a pediatrician. So I enjoyed that aspect.

I think, by having had a pediatric practice for about 20 years, in addition to neonatal we had a follow-up practice. And many of the graduates from the NICU came to our follow-up practice. I developed, and my partners, a unique perspective that let us see not just the kids in the unit, not just the families in the unit but, when they went home, how their family life was affected by having a premature baby. Now, most premature babies did perfectly fine.

But there were kids that had long-term problems and long-term disabilities, a small percentage, but how that affected family life. I’m not sure many neonatologists have that. They may see kids back once or twice in a follow-up center. But to actually see them come in for their ear infections, for their fevers, for their well-child care and immunizations and see the effects of a kid that had developmental problems on the family, on the siblings– I think that was unique to myself and my partners. And I think it gave me a little bit of different perspective when talking to families in the intensive care unit and afterward.

That’s a big part of why they have the interns do those rotations, honestly, because you explore some field that you might not even have considered.

Yeah, that was a student. And then I did a fourth-year rotation, and then I was hooked.

June 18, 2019

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