1. Introduction

2. Retrograde Pyelogram

3. Ureteroscopy and Visualization of the Stone

4. Laser Lithotripsy

5. Renoscopy

6. Exit Ureteroscopy

7. Repeat Retrograde Pyelogram

8. Stent Replacement

9. Post-op Remarks

Androgen insensitivity syndrome (AIS) is a rare condition caused by an X-linked mutation of the androgen receptor with an estimated incidence of 1–5 per 100,000 individuals. Varying degrees of presentation exist for complete, partial, or mild depending on the severity of androgen resistance. Patients with complete AIS (CAIS) are born phenotypically female but have male XY chromosomes and testes instead of ovaries. They exhibit normal secondary female sex characteristics such as breast development and external female genitalia, but lack a uterus and other Müllerian duct structures due to testicular production of Müllerian-inhibiting factor (MIF). Due to androgen-resistance, androgen-dependent Wolffian duct products fail to develop such as the epididymis, vas deferens, and the seminal vesicles. These patients often present either during infancy with inguinal hernias or sublabial masses, or during adolescence with primary amenorrhea. On physical exam, they will typically have normal breast development, lack pubic or axillary hair, and will have a blind-ending vaginal pouch of varying vaginal lengths. Diagnostic work-up is often conducted using ultrasound or MRI, serum hormone levels, and karyotype analysis. 
For patients with CAIS, their testes can be located within the inguinal canal, sublabially or intra-abdominally. Following puberty, patients with intra-abdominal testes are at a 15% increased risk (range 0–22%) of developing germ cell tumors (GCT). Management consists of prophylactic gonadectomy with subsequent hormone replacement therapy (HRT) to maintain normal pubertal development and promote adequate bone health. Debate regarding the timing of prophylactic gonadectomy is ongoing with some patient support groups arguing against gonadectomy citing concerns with long-term hormone therapy and the desire to preserve fertility. Current convention promotes delaying gonadectomy until after physiologic puberty has been achieved as the risk of developing prepubertal GCT is relatively low (0.8–2%). We outline the presentation, diagnosis, intraoperative techniques, and postoperative considerations for managing CAIS via bilateral laparoscopic gonadectomy. 

<h4>CHAPTER 1</h4>

<p>My name's Ryan Hankins, one of the attending urologists at MedStar Georgetown University Hospital. Today, we have a ureteroscopy laser lithotripsy, and stent replacement. This is a 76-year-old gentleman that was admitted through the emergency room two weeks ago, found to have a 1-cm left proximal ureteral stone, obstructing, with a forniceal rupture and fevers. He was taken two weeks ago for left ureteral stent placement. After he was decompressed with the internal stent for two weeks and was on antibiotics, he's here today for definitive management with a left ureteroscopy, laser lithotripsy, and stent replacement. We'll begin the procedure after a timeout by performing cystoscopy, indwelling stent removal, passing a wire to the kidney, performing a retrograde pyelogram to delineate a roadmap. And after that is performed, we will pass a flexible ureteroscope to the level of the stone and perform laser lithotripsy. After complete fragmentation of the stone, we will place a new indwelling ureteral stent, which will stay in for three to seven days after the procedure to allow his kidney to drain due to expected edema after this type of procedure.</p>

<h4>CHAPTER 2</h4>

<p>Okay. We have one wire up. The previously internal stent is out. We will use the double lumen catheter, for a retrograde pyelogram. What size stent are you putting in at the end? 6 French. This is the pendulous urethra, the membranous urethra, the prostatic urethra. We'll advance the double lumen into the - ureteral orifice. You have your pedal for the retrograde? Mm hmm. Go ahead. Retrograde pyelogram. Good. Good, and… Two wires, one spot. Excellent. Push, pull. Now we'll break down the cystoscope. Grab both - nice. We'll take the flexible ureteroscope, please.</p>

<h4>CHAPTER 3</h4>

<p>We will pass the flexible ureteroscope over a wire to the level of the stone. Right there. All right, so we're performing ureteroscopy. We've visualized the stone within the ureter. We'll take the 273-micron laser fiber, please.</p>

<h4>CHAPTER 4</h4>

<p>Let's dust this stone. Can we please do this? Don't worry about that anymore. Okay. All right, so we'll go to 0.6. And 15. Then you can hit ready. So we will use the holmium laser fiber to fragment the stone. Can you get on the leading edge? Much better. We try to paint this and dust it without fragmenting it into large pieces so that the majority of the small sand and gravel will pass down on its own and won't need to be extracted. Excellent, keep it up. You're doing perfectly. Take your time. During this procedure, while we laser the stone, the assistant controls the inflow of fluid to help with visualization. Yeah, get a little closer. It's looking good. Yep, work on the leading edge again, nice. You can - I'll take the stent. I'll take the stent. Are you ready? I didn't know you were going to be so speedy today. We're not done just yet, but it's going well. Anytime things go well, I'm extremely happy, Ryan. Good, I love it. That's what I like to hear. I just want to make you happy. I know, it's an important thing. Trying not to flush it back up into the kidney. We're now in the kidney. We'll follow the stone down into one of these calyces and we'll continue fragmenting the stone here into small dust and gravel. Yep. Go for it, yep. It'll just flush around to you. Let me give you a little bit more. You just stay right there. We'll just - we'll let the laser fiber do its work, just stay in there. Yep. The retropulsion of the fiber and the flushing move it around so that you can just stay there and… The camera will move with the kidney rather than with the patient's breathing. We're getting close, we are almost there. A couple fragments that are a little bit large that we want to make just slightly smaller, but most of them are fragmented. Back up, back up, back up, good. All right, start coming back, let's see. These things are tiny. Come on back. Come on back. Come on back. Come on back. Yep. Tiny. All right, come on back. No, you can keep coming back. Those are good. They're not going to go anywhere. A lot of dust.</p>

<h4>CHAPTER 5</h4>

<p>Good, let's take a quick look around the kidney. Let's perform a renoscopy. Nope, nope. Just take your foot off the pedal and go for it. Touch the wall. I'll hold the penis. Touch the wall somewhere. Yep, good. Clear. Back it up. Down there - that's where you were. That's where you were. Okay, go back up to the other one. Clear the top. Okay, go in there. Good, come back. Lowest poles, yep. Get that. Clear. Good, next. Good. Okay. And that's everything. Okay, let's come down the ureter.</p>

<h4>CHAPTER 6</h4>

<p>We'll perform exit ureteroscopy to make sure there are no more stones within the ureter. This is where the stone had been impacted. The rest of the ureter appears healthy. That's fine. Excellent, okay. That's exit ureteroscopy. We'll now switch back to our regular cystoscope.</p>

<h4>CHAPTER 7</h4>

<p>All right, nice. All right. All right, there you go. Let's get a little bit of contrast. Perform a retrograde pyelogram to delineate where our stent needs to go. If you've got the pedal, go for it. Yep. And one more. Excellent. That shows us our roadmap. Nice, one moment.</p>

<h4>CHAPTER 8</h4>

<p>Our flexible stent - to allow the kidney to drain for a few days. All right. There's your stent. Where the...? What dropped? Did something drop? Do you have your pusher up there? No. No? I have no idea where it is. Turn on some fluid, please. One line. Keep coming, yep. One line, take a spot, please. Very good. You got a good curl up there. Oh, so close. How close are you? Push in. I can give you the other one. Sure. If we can pull it out. Yup, there you… Hang on then. There you go. There you go. All right, good curl. Empty the bladder once, and then make sure that the curl is still excellent. Where is - I don't have the pusher, the orange pusher. I don't know where it is. It doesn't make any sense. No worries. We made do without it. We're good. Okay. All right, we're emptying the bladder. The case is done. It's in excellent position.</p>

<h4>CHAPTER 9</h4>

<p>After completion of his ureteroscopy, laser lithotripsy, and stent replacement, he will have his ureteral stent remain in place for the next week or so. He will be discharged home on narcotic pain medication for a few days. Typically these patients are discharged home with five to seven Percocet in my practice, Colace, Flomax, which has been shown to help with some stent discomfort. The - typically we'll have an anticholinergic prescribed for a few days, such as Vesicare. And then, they will have their stent removed in the office with cystoscopy and ureteral stent removal, which takes about 30 to 45 seconds in the office a week from now. I do give patients two tablets of an antibiotic to be taken as a periprocedural antibiotic on the day of their stent removal as well. As you saw with this case, the ureteroscope was advanced to the stone. We fragmented the stone completely. And the small, sub-millimeter fragments will pass down the ureter without complication. Typically, stones only cause patients pain when they obstruct the flow of urine, that's how they can form up in the kidney without causing significant discomfort. And patients oftentimes don't know that they're even there until they pass into the ureter and obstruct the flow of urine. So, I expect that he will do very well after this procedure today.</p>


Watch this full-length, narrated surgical video of a ureteroscopy, laser lithotripsy, and stent replacement for a ureteral stone performed by Dr. Ryan Hankins at MedStar Georgetown University Hospital.

MedStar Georgetown University Hospital