Facial Defects - Jason Toranto, MD
Pierre Robin sequence is a condition present at birth in which the infant has a facial abnormality that causes difficulty in breathing. Dr. Jason Toranto from Children's Hospital of Orange County explains this condition and the subsequent problems related to it. The condition that we are going to talk today is called Pierre Robin sequence and that's a fancy way of saying that there are three things that happen at the same time: one is that a baby is born with a cleft palate, another is that they have small jaw and finally they have a tongue that actually falls into the back of their throat and that's really the biggest problem because it blocks the baby's ability to breathe. This is a condition that is decently rare. However, it happens somewhere between 1 in every 2,000 to 1 in every 30,000 births.
When it does happen however, it's very problematic because the baby can't breathe, this is an acute emergency from the moment the baby is born and these babies are immediately taken to the intensive care unit the neonatal intensive care unit. In order to do this surgery, it takes an entire team of people together. So it takes the ICU team, it takes the anesthesia team, in my situation it takes somebody who's trained in plastic surgery or cranial facial surgery, who's trained over many years to be able to accomplish this surgery. It's a very high level surgery but what's so great about it is that it's life saving.
The operation takes the better part of 3 to 4 hours because of all the safey things that have to be done to make sure that everything happens in the operating room in the safest manner possible. We do the same surgery on both sides and we actually cut the jaw bone and when we cut it, we put this device on and this device, there's a fancy name for it and it's called a distractor. But essentially what it is, is it's a screw and you put this on and as you twist it, the 2 plates that you can see here actually move apart and as they move apart it creates a gap in the bone.
Because babies are so amazing, they actually fill in that gap in the bone and they will make their own bone in there. So we can wait, their airway gets opened up after turning this enough that the jaw actually moves and the tongue is attached to the jaw. So as you move the jaw it pulls the tongue out of the airway and lo and behold they can breathe and they can go home. This is the distractor that I was explaining to you and I spin it, you can see that what happens is the two plates or these two sides actually separate from each other and so the gap that I was talking about that's created in the bone you can see it happening here between these two plates. The baby will be in the ICU for a period of time and once the airway is open because the device has done it's job and they'll go home and then of course, they follow-up with me again so that I can take the distractor off and then the other part of it is as I said, a lot of these babies are born with a cleft palate and I also do the cleft surgery that's also part of my practice. So they come back and see me later and I get to take care of their cleft palate as well.
So they really are patients that I know throughout their childhood, not just, this is the first time that I get to meet them but we really become friends over their entire childhood. The babies have very good outcomes, the technology to be able to do this hasn't always been here. This has been evolving over time and I'm very fortunate to be sitting here now at a time when the technology has developed to where it's small enough that we can actually do this procedure and do it on a newborn level. People ask me all the time, how can you work at a children's hospital isn't it sad? And the truth is, if you walk through the halls of CHOC, what you see are kids smiling they're happy, they've turtle talk, they have child life, they have all sorts of resourses and it's just a wonderful place to work. On the surgeons' side, the hospital has provided me all of the resources that I need which includes not just the physical resourses, the actual screws and plates and things that I need to do my work but also the other people to collaborate with that I've been able to build these teams with them so that we can take care of children like this. We also now have a jaw clinic, a jaw team that I've been putting together to take care of these children and other children who have jaw abnormalities throughout their childhood.
I'm Dr. Cory Resnick, and I'm a surgeon in the craniofacial center at Boston Children's Hospital. Today, I'd like to talk to you about a condition known as Robin Sequence,…By: Boston Children's Hospital
Pierre Robin sequence is a condition present at birth in which the infant has a facial abnormality that causes difficulty in breathing. Dr. Jason Toranto from Children's Hospital…By: DocsTalk