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Prophylactic Laparoscopic Bilateral Gonadectomy for Complete Androgen Insensitivity Syndrome

J. Corbin Norton1; Amrit Singh, MD2; Laura L. Hollenbach, MD3; Georgia Gamble, MD3; Laura A. Gonzalez-Krellwitz, MD2; Stephen J. Canon, MD4

1Department of Urology, University of Arkansas for Medical Sciences
2Department of Pathology, University of Arkansas for Medical Sciences / Arkansas Children’s Hospital
3Department of Obstetrics and Gynecology, University of Arkansas for Medical Sciences
4Department of Pediatric Urology, Arkansas Children’s Hospital

Transcription

CHAPTER 1

So our case today is a case of a 15-year-old young lady who presented with primary amenorrhea. Family and she reported that she's otherwise healthy, no significant urologic history, but she'd never had a menstrual cycle. So we evaluated her, first did a physical exam, and then did some labs. On exam, she was a normal 15-year-old young lady with the exception of her genital exam. We noted normal breast development for a 15-year-old young lady, but on her genital exam, she had a lack of any pubic hair present and also had normal labia noted. So we then proceed with obtaining labs and an ultrasound. Our initial findings in her labs included a low estradiol level for a 15-year-old female, and a markedly-elevated testosterone level. She also had elevated LH and FSH as well. And her karyotype type returned 46,XY. Her ultrasound was noted to have a lack of a uterus, but also intra-abdominal gonads consistent with that of testicles rather than ovaries. So our assessment is complete androgen insensitivity syndrome. So we met with the family and discussed this with the family and the patient at length. First, outlining the reasons for the diagnosis, and then ultimately her increased risk for intra-abdominal testicular malignancies in light of her complete androgen insensitivity syndrome. We also discussed the implications for future fertility and also sexual function and whatnot. Ultimately, these patients are at increased risk for intra-abdominal testicular malignancies. And since she's had adequate time for breast development and bone development, which is the standard of care for these patients, we then recommended bilateral gonadectomy. Our preference is to perform this with a laparoscopic approach. So the subsequent procedure, or video, will outline the steps of the procedure. Also, the intraoperative findings, and then subsequently the pathologic findings in this case.

CHAPTER 2

So first of all, we're going to inject the umbilicus here with local anesthetic. Just to minimize her pain post-op and during the procedure. All right.

CHAPTER 3

So you grab about like so. Okay. Like that. So we're incising at the umbilicus on the inferior aspect. Pick up right here, if you would. Now we're just going to take the incision down to the fascia. Okay, can I have a hemostat? We use a needle point Bovie. I don't know if you guys use that very much, but it's pretty sharp. Just so you know. Okay. Army-Navy. Now I'll hold this one just for a sec. Okay, just making a plane down to the fascia. Can I have another one of those? Hold like that. Now let me see the Army-Navy again. Come out with that. DeBakeys. I think we're down to the fascia there. Hemostat. Now we're just going to get control of the fascia with some hemostats - another one - in order to help facilitate - and switch out that one, just let that be, and then hold that right there. DeBakeys. Just so we've got good control on the fascia. Then we pass the Veress in. Now we're passing the Veress into the abdominal cavity. Okay, go ahead and go for it. Okay. Let's go ahead and insufflate there. Can we - what's that? You did the water drop test? We did the water drop test. Can we insufflate now? Can we turn the gas on? All right, so we're insufflating now. Wait until our pressure gets up to 15 mmHg before we put in the sheath. We're almost there, pressure's at 12, 13. In just a minute, we're going to… I think she's a little bit more full. In a minute, we're going to put in the port. Yeah, she's definitely tympanitic now. I think we're there. Okay. And you got the 12 mm? Yep. Can we get the Army-Navy again? Hold on a second. I got a little bit of return right there, let me… Hold right here. Let me see another hemostat. And let me see another Army-Navy. The other side. DeBakeys first. I think we were right there before. That should be it. Yep. All right, so just keep some back pressure for me. I'll tell you what, drop that one and hold the other one over here. Now we're putting in our port. All right, that's good. Now we're going to insufflate through our port. You can take those off. Now, we're going to look in with our camera.

CHAPTER 4

Here we go, white balancing. Okay, now we're good. Now we're looking inside the peritoneal cavity. Now… She has had a history of bilateral hernia repairs. And so now we're looking down in the pelvic area. We can see the bladder here, for the urachus, and the [medial] umbilical ligaments. First of all, let me look straight back, and… Her intestines look fine. Now we're going to need a Trendelenburg, please. We have a Foley catheter in the bladder currently to decompress the bladder. There's the testicle. So here we have a right testicle. That's kind of being hidden by the intestines. We're going to need to move those out of the way in just a minute. I'll tell you what, let's go ahead and put in our other working ports, so we can work around. And now we'll just use the 5 mm. Yep, with the Veress in it. All right, so now we're putting some local in, and identifying our port site. Look a little bit further south. We may just not be through, try that one more time. Okay. Back to you. Make an incision in the skin. Now I'm going to dilate down to the fascia, or at least close to it. Can you pull the camera back just a touch and see? No, okay. There it is. All right. Putting in a working port on the left side, along the midclavicular line or just lateral to it. All right. I need you to just - along the Langer's lines, just cut current. Placing the right working port now. I think you're getting close now. Going through the side. I think it might have popped back. Can we get another one of those? Sure. I think there's a testicle on the right, but on the left, I'm not really sure that there is one, but we're gonna look up underneath that omentum right there, to get to the bottom of it. But with her having a prior hernia repair, it is possible that we're going to, if we can't find a testicle, then we'll call and talk to the dad. Here you go. There you go, you got it now. All right, good deal. Here you go. Thank you. We're switching to a 0-degree lens. I think this will optimize our visualization. Okay. She's going to have to calibrate here. All right. Now… All right, you got it? So I have a LigaSure in my right hand and Marylands in my left. I may need a longer Maryland. Do you have a longer one? So, can you look down a little bit? I'm definitely going to need longer instruments on the left. Okay, so it looks kind of like a fallopian tube to me. That's really big. Clearly a gonadal structure, right there. It's not showing itself to us really readily yet. I'm going to have to dissect that around - from the surrounding structures. Let's look over on the left side, and see if we can identify anything over here. Okay, there we go. I think it's hiding down in the pelvis.

CHAPTER 5

All right. So this - is a gonadal structure of some sort right here with some cysts on it. Okay. I'm just going to develop a plane behind it, through the peritoneum. Bluntly. So, got to be careful, her ureter will be in that location, also, not far away from the iliacs as well, which should be right here. Can we get any more Trendelenburg? A little bit of scar tissue, I think from the prior surgery. Do you need me to move? Okay, this is good. Okay. Getting a little bit of plane behind the spermatic vessels to what appears to be a small testicle with a little bit of surrounding scar tissue, presumably from the history of inguinal hernia repair. All right. Let me see if I can sweep these anymore. All right. Without retracting it up like that, we really don't have much exposure at all. That seems a little bit better, don't you think? Can't see behind it super well. And so I want to be really cautious about doing anything underneath here that we can't see. Nothing else in the area that this could be other than gonad. We're right, just superficial to, or superior to, the internal ring. It looks like an epididymis there, on the lateral aspect. All right, so we have a plane behind there. All right, look up north, just a little bit right above it. Right above the testicle, right here. Okay, so I'm just going to spread it with my right hand a little bit. Do you have a hook? Can I have a hook? Okay, so we're going to use a hook to try to dissect off the peritoneum, just above where we're working in our - in this window in the peritoneum. I'm going to pull it up away from the other structures. All right, go ahead and try to incise the peritoneum a little more medial. So, all right, pretty good visualization right here. Kind of like to use the LigaSure on that though. Okay, so it looks like we have a little plane just anterior to the spermatic vessels on the peritoneum. I'd kind of like to see it a little bit better. And there we see the cystic structure on the gonad again, lateral, doing its best to get in our way. A little more peritoneum. All right, let's see if we can get a good picture of the internal spermatic vessels here, away from everything else. Okay, so what I'm going to do when we get through here, is I'm going to take this in about three different bunches right next to each other to make sure we get it ligated really well. Okay. May take it a little bit more broadly. And then before I cut, I'm just going to go a little more distal. And again. One more time. And then I'm going to go back to the middle or a little closer. And then cut that. Look down real quick. Before we lose that, I'd like to get another, I'd like to fulgurate it again. Now look just a little bit to your right. I want to make sure that I see that's intestine right there, maybe appendix. Away from where we are. Okay. Peritoneum on the backside here. I'm just going to go ahead and release that as well. Okay, so now I just want to demonstrate right here. Look down, first. All right, so we see - that looks like small intestine, and that is away from where we cauterized, and it looks fine. Okay. So now, that gives us a little more mobility in here to take down what we have here. I'm going to grab a little more north on the testicle. Again, pulling up and away from everything else in the pelvis, such as the ureter or the iliac vessels. Okay, I think we're about ready to switch the LigaSure to the other hand. So your ureter is more caudal from where we are, right? Yes, yeah. It'd be like right here. Right in there. But it'll - especially in a little kid, it can really sneak up on you. And so I think you have to be really cognizant of it. If you go a little bit further, like right in here, you'll get into the obturator foramen, and, you know, the obturator nerve potentially. Normally, these testicles are a little more mobile, but because of the prior surgery, it was stuck down near the intestines there, and on the posterior aspect of the peritoneum. So it wasn't as easy as a lot of times it is to dissect out. But now - and you can see also, we have a closed internal ring - this is where it would be, right here. Can you scoot in a little bit more? Why don't we clean off our camera too? I think it's a little bit… All right. So now, that's the peritoneum on the - just lateral to the testicle that we're taking down. Let me get a better purchase of the testicle here. Okay, now we're coming upon the vasal structure, which actually looks a little broader than I would imagine it to be. Of note, you can see it going toward - slide on down to the left. I'll tell you what, let me run it for just a second, you got that? Yeah, sure. So, bladder. If there was a uterus, we would see it right in this location. And there's no obvious uterine structure, although that is where the… You know, this also is where the prostate and the posterior urethra are. So, what appears to be a - kind of a broad vas deferens heading down toward the prostatic urethra, which is where the ejaculatory ducts would be. So, with that being said, let me give you this. And then I'm going to take back over, and then we're going to transect this vas deferens.

Clearly an abnormal looking testicle as well. Okay, so just to verify that we have proper orientation. So you can see we're outside the pelvis, we're away from the bladder. We're away from the rectum, we're anterior to the rectum. There is a, you know, gonadal structure that appears to be consistent with a testicle, with what appears to be a lateral sulcus. And so, what we need to do here is just transect this. I'm going to do it in a few - adjacent fulgurations - before transecting it. All right, again. And now we're going to transect it. Okay. So the right one is no longer attached, so we're going to leave it. Let's leave it right over here on the right side, in the right pericolic gutter.

Okay? Also, I don't see any bleeding there where we were working before. Let's look over on the right side, a little bit where the testicle originally was. Okay. It looks dry. You know, what might be easier is - for the other side, is let's switch sides. You think that looked like a fallopian tube, or are you thinking it looks like a...? I mean those - I don't know if you get gonadal cysts, but those look like paratubal cysts. Yeah.

CHAPTER 6

Some more of those cystic structures there. I'm going to switch that to my left hand. You know, being a laparoscopist, you get a little bit of practice with contorting your body, like you're playing twister. So, the added benefit is we get our ab workout in. Surgery is a full-contact sport. That's right. It feels like it after a day in. Okay. So I feel more confident now that we're in the right spot and that we see what looks like a gonadal structure. You know, I'm going to - I hate to do it - actually, we'll take this down first. You can see also, if you would look at the internal ring - left and up - also closed, no hernia. So they successfully fixed the hernias before. You can also see the iliacs. Look down just a little bit, right in there. So we want to stay away from those. So I'm going to incise the peritoneum distal to the testicle - since it's showing itself to us. So, if you looked real close, you can see the iliacs down there, through - and just medially, that would be where the ureter is. So, you know, it's important to - back up just a little bit - to always maintain your awareness because that could definitely change the feel of the case. I think traction, counter traction, exposure, and patience are key. I'm just going to get the peritoneum right in front of us. So this is gonad number two. You can see some cystic changes to it, but it appears to be a vas going up to it. If you would look over at the right side. And you can see where the other one was located. And there it is. We just took down the spermatics and the vas with the LigaSure in a couple different sections, right adjacent to each other. So, let's go ahead and get back to this. Okay, so I'm going to switch hands again. I just like the angle relative to the iliacs a little bit better from here. Especially with regard to the - internal spermatic vessels. So you can see, I can get above the iliacs here and spread without having to worry about past-pointing into the iliacs, which are right there. Okay. So just verifying our orientation again - to the gonadal structure here with the cysts, iliacs here, vas would be down here. There's no way that - sorry, the ureter would be down here. So there's no way that this can be ureter, it's too superficial, too anterior. Okay, so we're going to go ahead and come across this. I'll tell you what I am going to do, I'm going to just take a little bit of this down first - just so I can see it a little bit better. Almost making it bleed again. Taking the internal spermatics to the left gonad. Again, with the LigaSure. Just fulgurating adjacent - to the last fulguration to be able to ensure that we have good hemostasis before dividing it. And then, I'm going to go back to near the middle. Fulgurate and divide. No bleeding. Let's get that again. Just inspecting the posterior attachments here. I think I'm going to switch back again with the instruments in order to have a better angle with the LigaSure to take down what's left and also the vas. All right. Picking up on the vas. Making another little window on the distal attachments. I have it retracted up well. Okay, and now we're down to the vas on the left.

Just trying to dissect down to just - all we have left is the vas with one more fulguration. And now we're doing it. All right. And now the vas is transected on the left. I'm going to set this down over in the left also. You want to clear out the gas and then get a good view of the…

CHAPTER 7

All right, so here you see the fulgurated and ligated left vas deferens. Can we look on the right side? And there you see the fulgurated and ligated right vas difference. And then if you would show - okay. So there is the site where the testicle was previously - on the right, and then also on the left. All right, so I think we're ready to remove them. Since we have a 12-mm port, I think we probably can remove them through the port. We need a 5-mm camera. Do we have one? And then what I'm going to suggest we do is you look in through the left, and then I'll reach in and grab one at a time. While you have that out, I'm going to go ahead and take the gas down too, just to make sure that there's no bleeding. So just desufflate for a minute. Just taking the gas down, so that when we look back in, if there's any bleeding, then we should be able to see. It'll prevent tamponade of any small venous bleeders. Okay, so let's look back in again. Insufflating again. All right. Okay, so I'm going to try to grab it on an end and see if… Now importantly, we don't see any bleeding either. Okay. Little bit tight. Let me see if I can grab it right on the end. You have a Marylands? We'll give one more try with this, and then if I can't get it… It's not going to fit. I think we're going to have to reach back in, yeah. Well, let me just bring it out of the port. If we need to replace the port, we can. So we'll take them both out together. Are you ready? Watch your eyes. I'll tell you what, would you keep an eye on what it looks like in there? There we go. Got it. That is the right side. Here you go. Good. Thank you. All right. Oh no, it looks a little bigger, doesn't it? Let me grab it on its end and make sure that we have the skinniest part of it. Will it come through the port at all? Doesn't look like it. Do you have an endo bag, Endo Catch? I'll tell you what, just have it available, don't open it yet, I'm going to try to get it with - can I get a hemostat? I think I might be able to just... Oh, it's going up. Yeah. Army-Navy. Yeah, thank you. Almost there. Here we go. There's the left side, left gonad. All right, thank you. All right, let's look in and just make sure that we don't have any bleeding. Okay, so just to do a debrief. So we've done a cystoscopy, vaginoscopy, EUA. And then laparoscopic bilateral gonadectomy. We have two specimens. One's the right, one's the left gonad for gross and histology. I wonder if we need DNA extraction from that. We'll call and find out. Okay. Look right, down left, one more time. Good, and then on the right. And then down. All right, now let's look around at the intestines. Good. All right. And straight down. Okay, good. Well that looks - maybe over on your side. All right. So no intra-abdominal injuries, no bleeding. At this point we're going to close. We'll desufflate and close.

CHAPTER 8

I just want to make sure we have it all - all that gas out. I don't want it getting intestines coming up through there. Okay, do you want to go ahead and pull that? I'm just trying to close the fascia for the 12-mm port. I'm going to need a 2-0 Vicryl and a UR-6. Okay, if you can hold that. Sure. And then DeBakeys. There is the hole right there. I'll tell you what, I'll hold this one. Can you hold that for just a second? Another Kelly. I think this one I could actually reposition. Now, that's fascia. Could you hold this Constance? And then 2-0 Vicryl. Thank you. That's all right. Empty needle driver. She's not quite as deep as I thought she would be. Okay. And now I'm just going to put one right by it. Great. Okay. Needle driver, again. Okay. Now I'm going to tie this. All right, scissors. Let's see what this feels like before we let go of any of these. Needle down, I think I might put one more right over here. Just come off of this. All right, we almost have the fascia closed at the 12-mm port site. Since the others were step ports and they were small, we're going to leave them to close. You can let go of that now, and let's feel it. You know, I don't like the way to belly button is tethered down there. Another Adson. Would you hold this one right here? And Bovie. Can we get that Army-Navy. You're good. It's not a button hole. There's a little tissue that I think got incorporated in the closure. Go ahead and Bovie that. Yep. Mm hmm. Yeah. All right. I'm happy - hello. You happy? All right. Let's see, 5-0 monocryl. Can we get two of them? So, I usually just do subcuticular interrupteds. Okay. Pickups, Adsons. Okay, good, you got it. All right, so let me just show you how I like to do them. 5-0 Monocryl. And maybe you could do one over there on that side, and then if you finish up quicker than me, I'll let you do over here too. But just, can I get some other Adsons? I'm not sure those meet very well. Or I can use the, whatever the big facial closures are. Okay. We're going to use Dermabond here too. Okay, so we're just going to do some subcuticular interrupteds to close the - take two on that one. Okay, so you got that for there? Yes, sir. Can I have another one? Any reason why you like using interrupteds? Well on the umbilicus, with a kind of a semilunar incision, it's easier to close. It's hard to make a running suture straight. And I also like the fact that it doesn't dehisce. So we got the fascia closed. We got the specimen out. We checked to make sure that the fascia was closed well. And now we're closing the skin. The incision in the umbilicus will be really well hidden. It'll be hard to see. These others you'll see for about six months, and then they'll be hard to see as well. We're going to put Dermabond over each of the incisions, and that's it, as far as dressings go. All in all, I think that the case has gone well. I think the family will be very relieved, and the patient, that she won't be at risk for testicular malignancies anymore. It would be interesting to see what the final path shows because there were definitely some cystic changes on the gonad, which were - did not look completely normal. Well it didn't look normal at all actually. We did not see - importantly, we did not see any evidence of a uterus either. And again, we couldn't feel a cervix. And what would you guys estimate was her vaginal length? 7 cm. 7 cm for the vaginal length. Okay. So she's going to come back in about two to four weeks. We'll review the final path, we'll evaluate her incisions. We also have Dr. Hollenbach, our fantastic adolescent gynecologist, who's going to start hormone replacement therapy. So she'll need to be on hormone replacement therapy until the natural age of menopause in order to support bone and cardiac health. So we're going to give some Toradol for postoperative analgesia. So we are going to be admitting her, and that's because of social and language reasons more than anything. They live in northwest Arkansas and speak Hmong. But normally if you do this procedure, you would send them home the same day? Normally, we would send them home - yeah. Now we're just going to clean the incisions. In a second, we're going to put the Dermabond bond on. How much local did we inject? 7, sir. Okay, 7 ml of local. Could I have an Adsons, as well? Okay. I'm ready for the Dermabond. That side just got a little bit traumatized from the Bovie, I think. Here you go, you want to do that? I think you got enough.

CHAPTER 9

So our young lady had a stable, immediate postoperative recovery in the recovery room. And she was admitted for observation since the family lived a long ways away from the hospital where our tertiary referral center's located. She had good pain control, resumption of diet, and was ready for discharge to home the next day. We did initiate estrogen replacement hormone therapy with her gynecologist since patients who have had a prophylactic gonadectomy with complete androgen insensitivity syndrome require long-term hormone replacement. And she tolerated this well, and - which will also allow her to maintain normal bone development and also breast development.

So, she returned later for discussion of her pathology findings, which were very unusual and rare, even in this instance. She was found to have a bilateral germ cell carcinoma in situ in both specimens, as well as bilateral paratesticular leiomyomas. So we discussed these findings with the family, and ultimately also referred her to oncology, who will be following her for monitoring her tumor markers, as well as her general progress. So needless to say, we were very happy with the result, that we were able to intervene early in order to remove her gonads prior to conversion to formal germ cell tumors. And therefore, we're very optimistic about her long-term recovery. Thank you.