Vaginal Birth Defects and Buccal Mucosa Vaginoplasty – PediaCast 544

Show Notes

Description

  • Dr Linda Baker visits the studio as we consider birth defects of the vagina… and the buccal mucosa vaginoplasty. Around 1 in 4,000 girls experience these birth defects, and using tissue from inside the mouth is less invasive than traditional repairs. We hope you can join us!

Topics

  • Birth Defects of the Vagina
  • Buccal Mucosa Vaginoplasty

Guest

Links

Transcription:

Announcer 1: This is PediaCast.

 

Announcer 2: Welcome to PediaCast, a pediatric podcast for parents. And now, direct from the campus of Nationwide Children's, here is your host, Dr. Mike.

 

Dr. Mike Patrick: Hello everyone and welcome. Once again to PediaCast. It is a pediatric podcast for moms and dads. This is Dr. Mike coming to you from the campus of Nationwide Children's Hospital. We're in Columbus, Ohio. It's episode 544 for September 6th, 2023. We're calling this one "Vaginal Birth Defects and Buccal Mucosa Vaginoplasty". I want to welcome all of you to the program.

 

0:00:59

 

So, as you can tell by the title of this episode, we have a technical topic for you this week. But there's good news, it is not too difficult to comprehend. Vaginal birth defects, that terminology is pretty easy to understand. But what in the world is buccal mucosa vaginoplasty?

 

0:01:18

 

Well, let's break down those words right off the bat. Buccal mucosa is the medical term for the lining of the cheeks inside the mouth. And vaginoplasty refers to construction or reconstruction of the vagina. So buccal mucosa vaginoplasty simply refers to constructing or reconstructing birth defects of the vagina with tissue from the inside of the cheek.

 

Now you may be asking how often is this even a consideration? Well, it turns out about one in four thousand girls are born with absence or incomplete development of the vagina. So, it certainly is not a rare condition. But you may not have heard about this because it's not really something people talk about in mixed company.

 

0:02:08

 

And yet it is important to raise awareness because traditional approaches to treating the birth defects of the vagina were quite a bit more invasive than the buccal mucosa vaginoplasty is. Also, there are not many surgeons who have experience with this newer and less invasive technique.

 

But we are in luck here in central Ohio because Dr. Linda Baker is a pediatric urologist at Nationwide Children's Hospital, who pioneered the buccal mucosa vaginoplasty. And as luck would have it, she is our guest today. She'll help us walk through the birth defects that lead to the need for vaginoplasty.

 

We'll talk about the traditional and more invasive procedures and explore the benefits of buccal mucosa vaginoplasty compared to those traditional approaches. And if you are a parent of a young girl with this condition, even if you are not living in Central Ohio, we'll describe how you can consult with our team here in Columbus and provide resources for making the connection.

 

0:03:05

 

So, a lot's coming your way this week as we consider birth defects of the vagina and the buccal mucosa vaginoplasty.

 

Before we get to our interview with Dr. Baker, let's run through our usual quick reminders. Don't forget, you can find us wherever you get your podcasts. Also, please consider leaving a review wherever you listen so that others who come along looking for evidence-based child health and parenting information will know what to expect.

 

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0:04:05

 

So, let's take a quick break. We'll get Dr. Linda Baker connected to the studio and then we will be back to talk about vaginal birth defects and the buccal mucosa vaginoplasty. It's coming up right after this.

 

[Music]

 

Dr. Mike Patrick: Dr. Linda Baker is a pediatric urologist at Nationwide Children's Hospital and a clinical professor of Urology at the Ohio State University College of Medicine. She also serves as co-director and principal investigator with the Kidney and Urinary Tract Center at the Abigail Wexner Research Institute at Nationwide Children's.

 

0:05:03

 

Dr. Baker has a passion for helping children impacted by birth defects of the female genitourinary system and is a pioneer in the use of buccal mucosa vaginoplasty as a treatment for absence or defects of the vagina that are present at birth.

 

That's what she's here to talk about, but first let's give a warm PediaCast welcome to our guest, Dr. Linda Baker. Thank you so much for visiting us again today.

 

Dr. Linda Baker: Thank you so much, Dr. Mike. It's my pleasure to join you again.

 

Dr. Mike Patrick: Yeah, I really appreciate it. I say again, because you were here a few weeks back when we covered Prune Belly Syndrome. And that's a really interesting disease process and I'm going to put a link to that episode that we did together in the show notes for this one, so folks can find it easily, if you're interested in learning more about that.

 

But today, we're going to turn our attention to a procedure called a buccal mucosa vaginoplasty. And that's a lot of big words. So, if you could just break that down for us and explain exactly what that is.

 

0:06:04

 

Dr. Linda Baker: Yeah, great. So, the first word that I'll start with is vaginoplasty. The vagina is the female reproductive part in the genital area which attaches from the outside world deep inside to the uterus and cervix and fallopian tubes. And so, a vaginoplasty is a plastic operation on the vagina.

 

And so, you can think of it like if you don't have a vagina at all or if you have a vagina that's not formed properly, then you might need a plastic operation to make it functional or make it usable as a female might use of the vagina. So that might include wearing tampons if you're a young woman. Or it might include having sexual relationships like a penetrative intercourse. So that's the vaginoplasty word.

 

0:07:03

 

So, if you kind of look back at how have surgeons made or corrected vaginal problem, there's been many different methods. And so back in 2004, I started using what's called buccal mucosa to repair or make a vagina.

 

And buccal mucosa is the medical name for the skin that's inside of your cheek of your mouth. So, if you open your mouth and you stick your finger in it and you rub it on the inside of your cheeks, you're going to feel this really slippery, slick skin. And if you accidentally bite your cheek, then you have bit your buccal mucosa.

 

And so, I had the idea of if a female had a birth defect or had a problem where the vagina was not large enough or there wasn't enough tissue to make a vagina, that maybe I could take some of the skin from the inside of the mouth, the buccal mucosa and then transplant it or move it and use it in the reconstruction of building a vagina.

 

0:08:14

 

Dr. Mike Patrick: And it's not really that unusual to use connective tissue from other parts of the body in plastic surgery procedures, right? So, you were really using techniques that have been known but doing it in a novel way.

 

Dr. Linda Baker: Yeah. So as a pediatric urologist, most, and even an adult urologist, many times, were short on supply of a tissue to build something. And so like, for example, the urethra, which is the body part that you pee out through, that releases the urine out of your bladder, so we urologists, adult and pediatric, have been known to go to the inside of the mouth and take buccal mucosa to build a urethra. So, it wasn't too far of a stretch for me to think of it. I was comfortable with doing that type of surgery in the mouth.

 

0:09:02

 

Dr. Mike Patrick: And I know in the dental profession, they use buccal mucosa also when they're doing some grafting, gum grafting, and those sorts of things. So that tissue must be very hospitable to growing in lots of different places. It must have a good blood supply, right?

 

Dr. Linda Baker: Yeah. It's a very interesting tissue because number one, there's no hair on it. Number two, it's not dry. It's actually self-lubricating in a way because there's these miniature little mucous glands that are underneath it. And so that helps to keep it lubricated.

Number three, It's really stretchy but yet strong. I mean, think about how many marshmallows you can stuff inside of your cheeks when you eat. It's really pretty stretchy but yet it's incredibly strong. There's a really elastic layer inside of the buccal mucosa.

 

So, all of these properties, as well as the fact that blood vessels can grow into it really quickly and help it to survive being moved, all of those properties really help to make it really a useful piece of tissue to move to another part of your body to surgically implant.

 

0:10:15

 

Dr. Mike Patrick: Now, you were mentioning that some of the indications for this procedure may be babies who are born with a birth defect of the vagina or born without a vagina. Is this something that happens very commonly?

 

Dr. Linda Baker: So, they say that about 3.2% of females have a birth defect of the entire female reproductive tract. Now, of course, that includes like fallopian tubes and uterus and cervix. And we're not really talking about that today, up to 3.3% and they say that maybe is 1 in 4,000 will have, again, a female reproductive tract birth defect.

 

Dr. Mike Patrick: Yeah. So, when we say 1 in 4,000, I think that's probably more common than people would think it is. And I guess one of the reasons is that's not something you talk about at family get-togethers. And it's more of a private issue. But I think we need to be aware that it is out there and there are lots of folks who are impacted by it to the tune of like 1 in 4,000 females.

 

0:11:20

 

Dr. Linda Baker: Yeah, you're exactly right. Exactly right. Just to say the word vagina in the home that I grew up in, it was very not a commonly used word and there was, everybody's worried about how much sexual information do you disclose to your children? And so many of the times when these types of birth defects become obvious, it's happening either in a newborn baby or it's happening right around the time that the child is transitioning through puberty, which is already a very kind of sensitive time in their life to learn about their own sexuality and their own sexual aspects of their bodies. So yeah, it's a difficult thing to talk about in many ways.

 

0:12:02

 

Dr. Mike Patrick: Yeah, absolutely. Now, what is the ideal age to perform this procedure? So, let's say a baby is noted not to have a vagina or there is a defect that's caught early, is this something that you treat early or do you wait a little while?

 

Dr. Linda Baker: That's a complicated question only because of the fact that there are some conditions that babies have that have to be operated on early in life. And it's typically because there's some blockages that are occurring or they're getting urinary tract infections because there is some vagina but it's not connected to the outside. And instead, it's connected to the inside of the body.

 

It's like connected to their bladder or their urethra, or actually combined that, and the intestine. So those type of issues warrant surgical management early in life. But in general, if you had to ask me when do you do most of the work of let's say buccal mucosa vaginoplasty, it's in the teenager, young adult.

 

0:13:11

 

So, most of the patients that I have operated on who received this particular operation would be somewhere around the age of 13 to 35. Although I must say the oldest patient that I did was a 62-year-old woman who had to have surgery for cancer and she needed reconstruction.

 

Dr. Mike Patrick: Is this a fairly difficult procedure technically or is it something that once you learn to do it, it's easy and goes faster? Is this like several hours? Tell us a little bit about the procedure itself.

 

Dr. Linda Baker: It depends again on the actual birth defect that's there. So, it can range from an operation that might take about 4 hours to an all-day reconstruction, like 12 hours. And it somewhat depends on how many other body parts we have to disconnect or move or rearrange to get the functional outcome that we want.

 

0:14:15

 

Dr. Mike Patrick: Yeah. So really, it's a kind of a case which each individual person is unique in terms of their defect in the approach. And so, I would imagine this takes a lot of planning before the actual procedure on your part to figure out exactly what you're going to do.

 

Dr. Linda Baker: Yes, you're right. I started using this technique in 2004 and then I had the opportunity to talk about this method of reconstruction at several medical conventions and venues. And I've been able to do videos and things like that.

 

So, I think the word has kind of gotten around about it. And thus, there are other doctors that are starting to do it and have been doing it. So, I think that the fact remains though, the number of physicians, the number of surgeons that are doing this are still limited in the US.

 

0:15:11

 

Dr. Mike Patrick: And we are very fortunate to have the pioneer of this procedure, Dr. Linda Baker at Nationwide Children's Hospital. And we'll talk more about you seeing folks who might need this procedure in a little bit.

 

As we talk about this being kind of a new way of approaching when there is an absent vagina or there's a birth defect there, that would indicate that there were maybe some treatments that were done before the buccal mucosa was used. And tell us a little bit about the traditional treatments of these conditions. And especially if there are limited folks who do the buccal mucosa procedure, what other options are out there that folks may be impacted by?

 

0:15:54

 

Dr. Linda Baker: That’s, again, a kind of complicated question, a good one. Thus, if there is no vagina at all, there are a series of methods that have been used. And always, the first one that even I suggest also is to do what's called progressive dilation, where if there's no vagina present, you can use a dilator and push in the genital area to a stretch and in many cases create a very excellent functioning vagina.

 

The challenge with that is you need a coach to teach you how to do it because the urethra is down there and the anus is down there. They're all really close together and what you don't want to do is take off and try this at home without being taught how to not harm yourself. So, dilation is really the only non-surgical method to try to create a vagina.

 

But then when it comes to, again, a female that has no vagina at all and has tried dilation or doesn't want to try that option, there are several surgeries. People have used a segment of intestine to make the vagina. People have used skin flaps, where they take skin from the inside of the thigh and rotate it in.

 

0:17:19

 

You can do what are called grafts, which is buccal mucosa is a graft. It's where you take a piece of skin from somewhere else on the body. Like oftentimes, the skin on the buttock, the bottom, is grafted and used to make a vagina.

 

There's several other methods and some of these can be done laparoscopic and involve going into the belly. So, there's several methods to make a vagina when there is absolutely no vagina present at all.

 

They all have their pros and cons. There is, I would say, no single one that is the absolute best. But you have to kind of take the rest of the patient's context into play to decide which operation is maybe the best for that particular patient.

 

0:18:12

 

In contrast to that, which is for patients that don't have any vagina at all, when it comes to trying to create or lengthen, let's say, a short vagina or a vagina that is up high inside of the body and has a long way to try to get it down to the outside world, then I think that buccal mucosa is a very, very nice option.

 

People also use bowel or flaps or grafts as well. But I think that the problem with using skin for the vagina is that skin as a graft has the issues of it's dry and it has hair on it. Even a little bit of pubic hair if it's grafted inside the vagina will grow very, very long. And so, some people do not like the idea of having hair growth inside of the vagina.

 

0:19:08

 

There are several issues that go with using skin. Using the bowels many times works well, but sometimes the bowel can get inflamed and irritated because it's used to having stool go through it. And there's some small reports of cancer forming in the bowel or cancer forming on skin. So, there's several, not super common but known risks that go with using all of these different parts to create a vagina.

 

Dr. Mike Patrick:  In terms of the buccal mucosa approach, what are the risks of that particular procedure?

 

Dr. Linda Baker: So, because it is being taken from the inside of the mouth, there is a chance that the mouth can become tight. So, I talk to my patients and I say I measure how wide they can open their mouth literally. And so, there's a few birth defects that actually affect the jaw and the neck. And if they are present, then that might make those particular patients not a candidate for using buccal mucosa.

 

0:20:16

 

So, I screen patients to figure out who is it safe on and who is it not safe. But then, even if their mouth is really nice and wide, I always teach patients to do what I call mouth exercises to stretch their jaw immediately after surgery, to try to prevent the tightening. And the medical name of that is a contracture where it tightens down so much that you can't get your mouth around a big stack of hamburger anymore because your mouth won't open as much as it should.

 

Dr. Mike Patrick: And I would imagine that that's a pretty small risk compared to the benefits over some of the other procedures if a patient is a candidate for the buccal mucosal approach, correct?

 

0:21:02

 

Dr. Linda Baker: Yes. So, I have done around 80, 85 patients using this method. And out of those patients, I've had about 4% that have developed a mouth contracture. And so fortunately, that's quite rare.

 

And I too, because I kind of, as you mentioned, I started this method, I too have learned along the way. And somewhere along the way, I started implementing mouth exercises as I call them. And I think that that has really made a huge difference in preventing the tightening of the mouth.

 

Dr. Mike Patrick: Now, in terms of long-term outcomes with this, it's, of course, not been around as long as other procedures. Do we have an idea of a couple decades out how these will hold up?

 

Dr. Linda Baker: Yeah, that's a great. Unfortunately, we don't. So, in 2004 was the very first patient that I used buccal mucosa on. So, we're soon to have a 20-year anniversary, if you want to call it that for the first patient.

 

0:22:15

 

But I think that the majority of the patients, as people have around the US and world has become more aware of this method, the numbers of patients that I have done it has increased over the last 10 years. So, the majority of the data that we have is more around a 10 or a 12-year outcome. So, we don't have 20-plus year outcome data.

 

Dr. Mike Patrick: But that's been pretty good, correct?

 

Dr. Linda Baker: Yes. Some of the patients that have come to me are patients that have had surgery already to try to make a vagina or to correct a vaginal birth defect.

 

0:23:00

 

And some of the times, just like any operation, you can have a complication. And unfortunately, probably the most common complication that we see from any vaginal surgery is also stenosis or narrowing of the vagina itself. And a young woman might be able to tolerate some narrowing of the vagina if she's still able to pass the menstrual flow, right? The blood can come out.

 

I'll worry if she can put a tampon in and she wants to wear a tampon. But at some point, if she wants to have sexual intercourse, then the vaginal size, the diameter of it needs to be adequate for penetration. And so, the vagina diameter is too tight, then at some point, she wants to change her life, then it might require surgery.

 

Now, people will try to dilate the vagina and that has been used for many years and oftentimes, it works. But many of the patients that have been sent to me have very severe stenosis, like the size of a pencil. And there's no way that you're ever going to be able to stretch that sufficiently to be able to get it of the size that's needed for intercourse.

 

0:24:18

 

So, instead of trying to stretch it and tear and hurt and maybe make more scar tissue, we instead recommend just going straight to that type of surgery where it's repaired.

 

Dr. Mike Patrick: If a family, maybe not in central Ohio, is hearing about this and it's something that their family is impacted by, are they able to come to see you? Do you see folks outside of Central Ohio who might need this procedure?

 

Dr. Linda Baker: Yes. I have seen patients from Pakistan and Ecuador. And I've gone to several European countries and perform the surgery there. So, yes, it's not a Central Ohio limited thing.

 

0:25:09

 

In addition, I practiced medicine for about 23 years in Texas. And so, this is really where I was when I started doing buccal mucosa vaginoplasty. So, I have a large number of patients that are there.

 

Dr. Mike Patrick: And then how can families get connected with your team, in particular if they're not in Central Ohio? Their pediatrician may not know how to get in touch with you. Can families just do that directly?

 

Dr. Linda Baker: Probably the easiest way to reach our Urology Clinic is to call 614-722-6630. And that will help to direct to you to getting an appointment in the Urology Clinic, and you can specify that you'd like to see me specifically.

 

Dr. Mike Patrick: And we'll put that phone number in the show notes so folks can find it easily. We also have a handy request and appointment page at the website at nationwidechildrens.org. And you just put your contact information in and they forward that onto the correct department. And then, someone will reach out and get back to you. So, a couple of different ways that you can connect with the Urology team at Nationwide Children's Hospital.

 

0:26:12

 

And then, you are a pediatric urologist. I'm sure there are lots of other things that you guys do. What are some of the other services that you provide?

 

Dr. Linda Baker: Pediatric urologists take care of the genitourinary tract and so that includes males and females. And it starts at the adrenal glands and kidneys goes down the ureters, includes the bladder the urethra, the penis, the vagina, the external male and female genitalia, testicles. We cover all of these body parts.

 

So we see unfortunately fair number of children that are born with birth defects of all of these. Probably the most common things that we see are a birth defect of the penis called the hypospadias where the urethra is on the low side, the hole where the urine comes out is on the low side of the penis instead of at the tip of the penis.

 

And we also probably second most common see undescended testicles. About 3% of newborns are born with an undescended testicle.

 

0:27:14

 

So those are probably the most too common things that we see. We also see acquired conditions like urinary tract infections. We see kidney stones. We see a lot of different disorders that affects the kidneys and the bladder.

 

Other birth defects that we see commonly are spina bifida, which is a birth defect of the spine and the nerves that come out of your spinal cord. And so it disturbs the way that the bladder works. So, we deal with urinary incontinence with those patients, as well as other patients that have urinary incontinence.

 

Dr. Mike Patrick: Great. And we'll put a link to the Pediatric Urology at Nationwide Children's Hospital in the show notes so folks can find that easily if you'd like to know more information about all the services that you provide.

 

0:28:02

 

Also, in the show notes again, we'll have a link to the request and appointment page and that previous episode that we did on Prune Belly Syndrome. That was PediaCast 534. And we'll put a link to that in the show notes as well.

 

So once again, Dr. Linda Baker with Pediatric Urology at Nationwide Children's Hospital. Thank you so much for spending some time with us today.

 

Dr. Linda Baker: You're welcome, it's been my pleasure. Thank you so much.

 

[Music]

 

Dr. Mike Patrick: We are back with just enough time to say thanks once again to all of you for taking time out of your day and making PediaCast a part of it. Really do appreciate that.

 

0:29:01

 

Also, thanks to Dr. Linda Baker with Pediatric Urology at Nationwide Children's Hospital.

 

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0:30:03

 

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[Music]

 

Announcer 2: This program is a production of Nationwide Children's. Thanks for listening. We'll see you next time on PediaCast.

 

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