Medical Minute 4-1: Helping Babies With Birth Defects

By: Vanessa Welch Email
By: Vanessa Welch Email

The first thing you notice about Gavin Azzopardi is his unstoppable energy. He's all boy with a big heart, but what you don't notice -- and what Gavin barely notices himself -- are his hands.

"We didn't know until he was born that his hands were like that," said Laura Azzopardi.

Born without his index, middle and ring fingers on each hand.

"It was never caught in the ultrasound because when he was sucking his thumb, he did that, it looked like he was like this, so it didn't show."
Now, Cleveland Clinic Surgeon Doctor William Seitz is creating four new fingers for Gavin.

"We took his index metacarpal bone -- that was there -- and moved it over to add on to his thumb. We took some toe bones and added to some other fingers," said William Seitz, M.D., Orthopedic Surgeon at
Cleveland Clinic.

The bones are then attached to a temporary metal lengthening device which helps them grow one millimeter a day. At the end of a month: 30 millimeters, which is a little more than an inch.

"Then, we went in and separated the fingers out so that he could use the little muscles in his hand."

So far, Gavin's had four surgeries. The fingers will continue to grow, but there may be several more surgeries, depending on the function of his fingers.

"So that he could use the little muscles in his hand so he could move his little fingers around."

And what his patients are able to do surprises most. This little girl needed three fingers on one hand. Now, look what she can do. This is Gavin when he was born…this is now.

"They amaze me. I try to give them something down here to make it easier, but they've got it here and here to make it work."

"When he was first born, of course, he didn't have anything and to see the fingers Dr. Seitz created, hold the cup and move to grasp whatever he is holding, it's amazing!"

As for Gavin, he's planning to take his game out on the field…
And hopes to be a baseball player when he grows up.

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THE FOLLOWING IS AN IN-DEPTH INTERVIEW WITH THE DOCTOR FROM THE STORY ABOVE:

William Seitz, M.D., and orthopedic surgeon from the Cleveland Clinic, discusses how he is giving a helping hand to children born without fingers and hands.

Are these children that you care for ultimately born with these deficiencies in their hands?

Dr. William Seitz: Yes. Something goes wrong in the development of the hand. The limb bud starts to form at about the sixth week of gestation (this is about the same time as the heart, the spinal column and the intestinal tract). If there is a global problem – a syndrome that someone might have, a defect in their hand – they may also have a difference in one of these organ systems. That is certainly what we look for when a child is born . . . a difference. But the vast majority of time, it is not something that occurs either genetically or tele-genetically (from an outside noxious stimulus). It is more often than not a random event, but we still do all of the appropriate genetic testing and so forth.

In the past year, how many children have you seen this occur in?

Dr. William Seitz: I see anywhere from 1 to 3 children a week.

Do these children have their knuckles or any part of their hand?

Dr. William Seitz: It may be that they have nothing. They may have no hand at all. They might not even have a complete forearm. Sometimes, they have a portion of an upper arm with a couple of little fingers sticking off it. Sometimes, they have just two forearm bones. It really can be all over the map if you will.

What is your goal for these children in creating fingers for them?

Dr. William Seitz: Ideally, if there is a foundation to give them a thumb and four fingers that is what we do or at least try to do. The hand will always be a little bit smaller. It will furthermore always be a little bit less mobile depending on the number of inherit joints that are there. There may be some stiffness in the fingers. Our goal no matter what is to give the child a hand (or two hands), which can provide some form of prehension or function. Our goal is not cosmesis. Although, if you can use your hand pretty normally, and you don’t bring a lot of attention to it . . . a lot of times children with substantial differences get along without people even noticing that they in fact have a difference.

Can you explain Gavin and the situation that he specifically was born with?

Dr. William Seitz: Gavin was born with sort of a partial base of the hand. He had the basis of the metacarpals – the bones that sit in the palm, but not the actual fingers. So, in Gavin’s case, because he had some muscles here at the base of his thumb, but he didn’t have really functional fingers, what we did was we took his index metacarpal bone that was there, and moved it over to add to the base of his thumb, in addition to taking some toe bones and adding it to the other fingers. That created a much bigger web space here between the thumb and the palm. It gave him a mitten hand. And then from that, after that bone that we transplanted got its own healthy blood supply again (6 months), we went back in and lengthened the fingers so that he had independent functional fingers. Subsequently, we went in and separated the fingers out, and put some skin grafts in-between where we had the defect, so that he could use the little muscles in his hand to move the fingers around and in due course have prehension and hold items.

Were you able to do that for both hands?

Dr. William Seitz: Yes.

In Gavin’s case, sense you took bones from his feet, will that cause problems for that area?

Dr. William Seitz: No. The way that we do this is that we make a little incision in the top of the toe, open up the tendon that makes the toe extend, and then carefully dissect the first 3 toe bones and remove them. Nevertheless, we remove them in the soft tissue envelope so that envelope (periosteum) is where the bone gets its blood supply. It also surrounds the growth plate so that the bone has a potential for growing. It may not grow at the same rate as it would normally but sometimes it does.

What happens to the toes then?

Dr. William Seitz: The toe bone is very small and has the potential for growth but at that point it is small, so what happens is the toe shortens up a little bit – telescopes down – and when everything heals it is just a little bit shorter than it would have been before, but no deficiency whatsoever in terms of structure and strength. I have performed this on children who have grown up to be high school and college athletes and are quite excellent performers.

So for Gavin, with the aid of the tow bones, his fingers will continue to grow but at a smaller pace than a normal child of his age. Am I correct in saying that?

Dr. William Seitz: Well, what he has in each finger is the native metacarpal – the one that was there that has a little growth plate – and the toe bone that we transferred, which has a growth plate as well. Ultimately, Gavin has 2 growth plates. A normal hand has 4 growth plates per digital ray – one in the metacarpal and one in each of the three finger bones. What that means is that he will not have the full number of growth plates there but he doesn’t have it in his thumb either, so there will be a relative proportion between the fingers as they grow; his fingers will always be on the small side. What will happen is that his muscles will grow, and we will watch them grow, and if he needs any fine-tuning in terms of releasing some scar tissue or potential secondary lengthening, which we have already done once, we can do that.

What do you mean by secondary lengthening?

Dr. William Seitz: Basically, it means to go back a second time and add more length if some of the fingers are not keeping up with growth.

Do you add length for the patient using additional tow bones?

Dr. William Seitz: No. We use a device like this, which is a little lengthening apparatus that I helped design. What this does is that it goes into the hand (these little pins get threaded into the bone), and we make an incision in the bone itself. We take a fine scalpel and cut the bone, and then I repair that layer of soft tissue around it where it gets its blood supply in which we begin to let it heal. After it heals for about a week and it has some new potent tissue in there, we then begin the lengthening process by simply turning this little screw. Each time that we take half of a turn, we lengthen a quarter of a millimeter. We do that 4 times a day, so that at the end of the day we have a millimeter; end of a week, we have 7 millimeters; end of the month, we have thirty millimeters in length.

Is it painful?

Dr. William Seitz: No. Here is another device that has been turned and rotated out, and you can see the difference in the length.

Does this come out then?

Dr. William Seitz: Yes. This basically stays temporarily. What this does is it slowly stretches out the healing bone, and it provides a mechanical signal to the body to make more bone. Compared to other methods of lengthening that were implemented in the past (rapid stretching), this is far less painful. This device actually very slowly stretches that bone out. In addition, by going slowly, it actually stimulates the surrounding soft tissue – the blood vessels, the nerves, the skin, etc. – so that entire tube of soft tissue actually hypertrophies as we get out. And what we see after we have done the lengthening and separated the fingers, and these devices are off, as kids grow they have pretty normal sensation in skin function.

What is the future in terms of this procedure and what can be done for these children?

Dr. William Seitz: What we have encountered in a couple of situations has been that when we do a lengthening and haven’t quite gotten a complete fill of bone in the tube of new tissue that we have created, if for some reason the device comes loose and we have to remove it prematurely, we often find that we can actually create a little joint in there. It is not a real controlled way of doing it, but as I approach the years I want to spend more time in research and cut back on my 90 hours weeks, I think I would like to get back to doing some basic science research – not only can we make bones but we can make joints.

Are you amazed by some of your patients and how they handle all of this?

Dr. William Seitz: They are all amazing. There is something special about these children. They have some sort of alternative power that makes them very successful, determined and a tremendous amount of ingenuity in finding ways to excel . . . and they do. They tend to be remarkably bright. They amaze me. All I try to do is give them a little better function down here [hand], but truth be told they have plenty of function here [brain] and here [heart]. They can make it work.

How have you seen Gavin transition into having functional fingers and thumbs?

Dr. William Seitz: Gavin is amazing. He is an absolute delight to be around. He can do anything. I’ve seen him hit a baseball in addition to throwing one. That is the other thing, you give a kid with no prehension the ability to use him hands . . . it’s amazing what they can do . . . often times even ahead of normal milestones. Watch him draw and you will be able to see that he is way ahead of his developmental milestones.


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