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Complex Craniopharyngiomas: Pearls and Pitfalls

Juan Fernandez-Miranda

July 06, 2020

Transcript

- Welcome, ladies and gentlemen and thank you for joining us for another session of the Virtual Operating Room from the Neurosurgical Atlas. I'm very excited tonight. Our guest is a dear friend, Juan Carlos Fernandez-Miranda. Truly a pioneer in endoscopic skull base surgery, and has really pushed the boundaries and has taught us so much about specially endoscopic transnasal cavernous sinus surgery and complex surgery in the region. Juan Carlos will talk to us about resection of complex craniopharyngiomas. I believe Juan Carlos, if you agree with me that craniopharyngiomas besides pituitary adenomas, is the only other tumor that has truly established itself as something that is quite amenable to resection via endoscopic routes. It's really exciting to learn from your journey for resection of these complex tumors and please go ahead.

- Thanks Aaron. It is truly an honor to be here with you again, saving time and learning with you along. I wanna thank you for your pioneering efforts with the neurosurgical education. This is admirable and you were a visionary doing these webinars long ago before they were done all the time in this pandemic time, but congratulations for that Aaron and for your great work.

- Thank you.

- I also wanna welcome and thank you all the friends and colleagues from all over the world, South America, Europe, North America, China, Japan, Africa. All places that are motivated to learn and I hope we are up to that. I wanna start the webinar acknowledging my mentors and the mentors of many of us, Professor Evandro de Oliveira, Professor Rhoton. They taught me and they taught us that surgical neuroanatomy is the most important knowledge that we need to have as neurosurgeons. In particular, I wanna acknowledge Professor Evandro de Oliveira as many of you surely know, he's suffering with terrible disease ailing, and all our love goes to him and to his family in these difficult times for them. But we will always be thankful for everything he's done for me in particular and for neurosurgery in general to expand the beauty, what we call the supreme art of microneurosurgery, and we hope to be up to your standards Evandro. Thanks to Evandro, I was able to join Professor Rhoton and I spent two years with him in the lab. This was already 15 years ago, it was a unique time for me and key for my training. I would encourage everybody in the audience to go to the lab, do dissection of neuroanatomy because that is the key to become a better surgeon. After my time with Professor Rhoton, a bit after I joined the Pittsburgh team, phenomenal group of surgeons that I trained with for a two year fellowship and after that I joined my dear colleagues at UPMC with whom we develop a large skull base experience and also in particularly, endoscopic endonasal surgery. Also, we tried to expand the knowledge all over the world to facilitate the establishment of endonasal surgery as a safe and effective route for many of the lesions. As Aaron said, pituitary adenomas craniopharyngiomas are definitely ideal for endonasal surgery, I would add some other tumors like chordomas, chondrosarcomas are also clearly my opinion better for endonasal. For others still we need to determine, but craniopharyngiomas is one of those topics that is key for endonasal surgery. After my time in Pittsburgh for about almost two years ago now, I joined the Stanford University that recruited me and to lead the skull base pituitary program here, and it's been a phenomenal journey. Is key to have when you do a skull base with your great partners. Great partners that you share with cases, you are called for use with them. I have three rhinologist that were with me, phenomenal team, world class lateral skull base surgeon, endocrinology and I also wanna acknowledge my dear fellow who has helped me putting this talk together, Dr. Margolin. It was three years ago already Aaron that you and I together we sit to review technical nuances and anatomical concepts for endonasal resection of craniopharyngiomas. At that time, I think it was a great webinar that I truly enjoyed. We reviewed key concepts for craniopharyngioma surgery. But I wanna take this three years later, another step further and what I wanna share is my experience over the last year and a 1/2 here at Stanford doing craniopharyngioma surgery, in particular difficult craniopharyngiomas. Because there is no easy craniopharyngioma but some of them are particularly challenging. I faced that during my time at Stanford and I wanna share that with you. I've done 18 craniopharyngiomas since I started here. This is about 10% of the sellar/parasellar tumor practice or is a significant part of my practice and one that I particularly enjoyed very much. I recently reviewed just to show what the practice been like, or cases here with the 18 adults, eight pediatric cases. If you see in red, you see inoperable cases that were treated multiple times before and got radiation sometimes even twice. About 40% of adult patients, 35% of pediatric patients. There are very few patients that we actually operate as first presentation and that is a problem because the best chance to cure a craniopharyngioma is in the first operation and then it becomes more difficult. It still can be cured but it becomes more of a challenge. The resection rates for this tumor has been excellent in my experience and this is again an evolution of a long learning curve. But we removed all growth of a resection in all tumors except one adult and one pediatric craniopharyngioma. On the two we did not, we just left a small residual tumor, usually in case in the carotid artery and therefore not safe for a complete tumor resection. If you look at the complications and when you do challenging craniopharyngioma, you're gonna have complications. In the adult series, the main one is a patient with a stroke, which I'm gonna share with you. Was most an asymptomatic stroke, a small vessel, thankfully, not very symptomatic. On the pediatric population, we had two patients with CSF leaks. That's always a challenge, one of them I'm gonna share with you. It was really difficult to solve this problem but we did and I wanna share that experience with you too. If you look at the rate of panhypopituitarism is very high and this is expected for craniopharyngiomas. The natural history of these tumors, it means that they will injure at some point the function of the pituitary gland. But I always try to preserve the stalk whenever possible. I did that in six out of 10 adult craniopharyngiomas. The stalk was preserved, but that doesn't mean the stalk works. Only two of those six the stalk was working after the operation, but I still try. In pediatric patients, as you see, is less common that we can perform a good preservation of the stalk. Perhaps because of the nature of this tumor, sometimes they are larger, more difficult to really preserve any meaningful pituitary stalk. But if you look at the function of the pituitary before and after surgery, a good number of these patients have always before the operation, either partial or complete pituitary dysfunction. The rate of new deficit is about 30 to 40% in adults, maybe 25 to 35% in pediatric patients. This is an example of a pre and post on a 15 year old with a really large tumor stuck to the hypothalamus. But in spite of that, we were able to perform a complete tumor resection. Patient had low growth hormone before the operation, he was panhypopit after the operation, impossible to save the stalk in this particular case. But we got a complete resection and patient had excellent recovery with visual outcome, intact vision after the operation. I'm hoping for that long cure in this case. That's usually the goal for many of these operations. I'm gonna share with you five cases and these five were to some extent, described as inoperable craniopharyngiomas when they were refer or consulted with me, for a number of reasons which I'll review for each particular case. As we said Harvey Cushing said, "Craniopharyngiomas are the most formidable of all intracranial tumors," and I would add that we're gonna be seeing here some of the most difficult of this formidable intracranial tumors. He's taking the challenge a bit further. I'm gonna review just very few key concepts. We reviewed these three years ago, just as an overview, is key to perform the right exposure. Means is mostly a wide exposure when we expose the optic canal and all the beginning right here, and we need to expose this bone right here. Remove the bone of the curvaceous colloidal artery. Usually I expose the whole carotid, paraclinoidal carotid because that is gonna allow me to then open the dura here and then as I fold the dura, I'm gonna be able to see the supraclinoidal carotid here and the supra hypoxia arteries. Also I can see the optic nerve early on and then the Pcom as it goes posteriorly. These wide exposure is key to perform clear... To perform a good dissection as you go intradural in the cistern. At the same time, the anterior exposure of the craniopharyngioma is relatively short especially for tubular infundibular craniopharyngiomas as they go into the third ventricle. We do not need a very long exposure. Sometimes I see surgeons drill all this plan on bone and most of them you don't need to do that. Or at least if you drill it, there is no need to open the dura here. The only exception is for some of these pre-infundibular craniopharyngiomas that extend towards the planum. But those are not that common. The majority grow up and posterior towards the third ventricle. The key landmark for me is this one right here. This is the dura fold at the limbus of the sphenoid. This is the limbus of the sphenoid right here on the bone and it separates the planum sphenoidale here from the prechiasmatic sulcus right here. This dura fold which you see as you remove the bone, as you can see here in this operation right there or in this dissection here, is a very important landmark. Because it also goes laterally and becomes the roof, the dura of the roof of the optic canal or the falciform ligament. It's a key landmark to find the location of the optic nerve. As you know, sometimes this optic nerves are very displaced by the tumor and it's good to have an area where the optic nerve sits. Then you can follow it in this direction and dissect a tumor off the optic apparatus. The anatomy of the superior hypophyseal artery is very important and we studied this very carefully in the laboratory. There are many variations and this is the classic configuration right here. There is a common trunk and then this trunk continues straight in what is called the infundibular branch that has a universal anastomosis. Almost all of this is anastomosis with the other side and that's an important point. Then there is a branch going to the optic nerve, which of course you have to preserve and then there is a descending branch that goes to the dura of the diaphragm or to the pituitary gland upper surface. This artery, we often sacrifice because it allows us to mobilize this trunk superiorly and then release the superior hypophyseal artery and preserve the key branches, which again are the optic nerve and the infundibular branches. Using this dissection, this is the area where I would, often if I see this artery, would sacrifice it and this artery often, is providing vascular supply to the tumor. You have to get rid of it. Sometimes the infundibular artery also gives branches to the tumor. But in this case, you for example, when I coagulate this one right here, but preserve the one going to the stalk and we're gonna see this in our cases. Now, another important trick is how to mobilize the pituitary as we try to increase the space or work in a space for craniopharyngiomas is always between the optical apparatus here and the pituitary gland down here. This window can be narrow, but a way to make it wider is not by of course, mobilizing the optic apparatus, but it's by mobilizing the pituitary gland. You can displace it down. I often would remove some of the floor of the sellar here. Simply because that will allow me to mobilize the pituitary gland inferiorly and get better access to get direct visualization of the origin of the pituitary stalk and from there follow the stalk superiorly and then find the origin of the tumor and start pulling off the pituitary stalk. There are times where tumors extend into the retrosellar space behind the pituitary gland and those are difficult because you don't have enough exposure through the usual suprasellar approach. In those cases, we have to transpose the pituitary gland. Usually for the craniopharyngiomas, I do that in an intradural fashion, which means we're opening the dura at the pituitary and we just mobilize the pituitary gland to one place or the other or superiorly. I'm gonna show you a case doing that. When you do that, you have all these dorsum sellae exposed which I can drill and then get direct access posteriorly. There is another way of doing a pituitary gland mobilization which is the interdural transposition here. This one is done through the cavernous sinus and this one I usually employ for all the type of tumors, chordomas, meningiomas, tumors that are, let's say extrinsic, not intrinsic. In craniopharyngiomas, I have to identify the pituitary gland and the stalk above and that's why I have to do an interdural pituitary transposition. I need to have control of those structures as I'm doing the mobilization and the tumor resection. Luke, will you move my window down? Thank you, because I cannot clear the screen. Thank you so much. Now the retroinfundibular retrochiasmatic space is this space behind the optic chiasm here, right? Is all this area right here. Retroinfundibular behind the infundibulum. It's a space that has a lot of important vascular structures. The most important to remember is the Pcom right here and is the most important not because of the vessel itself, because as you know the Pcom can actually be sacrificed if you are careful with the peripheral branches, but because the tumor often attaches to the Pcom, and it becomes risky. You could avoid the artery or you can injure the artery. I'm gonna show you two examples of this and you gonna have the ability to fix it. Always be very careful with that Pcom and of course, all the periphery branches that you see here come coming from the carotid directly or from the Pcom itself. As you all know the Pcom of course joins the PCAs and the basilar bifurcation here, and the tumor often is stuck to the basilar bifurcation in this area. We need to be very careful and have very good control of the basilar proximally and then follow it up and then follow it towards one P1 and follow it towards the other P1. Having excellent vascular control and visualization of the basilar bifurcation is very important in this case. For that, as we said, we need to go behind the dorsum sellae, remove the dorsum sellae posterior clinoids and open the dura and see behind the pituitary gland towards the interpeduncular cistern. The last tip is, and this is one that took me time to develop, is the ability to identify this plane of dissection at the level of the hypothalamus. As you follow the tumor up from the stalk, you can get an idea of the plane between the pituitary stalk that continues with the hypothalamus and the mammillary body, which is a key landmark right here and the tumor, and you develop a subpial plane of dissection. You separate the tumor from what appears to be brain tissue gray matter of the hypothalamus and is a plane of dissection that is key to perform complete tumor resection, but at the same time to preserve the integrity of the hypothalamus. As you know, hypothalamic injury is always a major concern with craniopharyngioma surgery but in my experience is very rare, because we have very good visualization, and we are careful in developing this plane of dissection. Let's go with the first video. This was the third case I did here at Stanford, when I was just starting. This patient had been around for about eight years with very poor vision, as you see here and the cyst having continued to grow. The patient had been told that this tumor was inoperable and I'll show you why. Because of the very large calcification this patient has right here. This is not a micro calcification, is a very large rock solid calcification that is stuck behind the anterior circulation, behind the optic chiasm, towards the third ventricle and moving this it really is a challenge. The problem is that to really remove the cyst effectively, you have to remove the calcification. Now this patient also, this is very interesting, the sellae basilar expanded and what we had in the sellar was a pituitary adenoma. Imagine my surprise my third case at Stanford, I sent tissue and it comes back like adenoma and I said, "What is this," and this patient had an adenoma and a craniopharyngioma and both, very unique. But in any case, we are now opening the dura and dissecting the arachnoid. You see the wide exposure I told you here, I can see the supracranial carotid and I can also see the superior hypophyseal arteries, and that's because of my wide exposure. Then I can selectively coagulate and transect the vessels going to the tumor, but preserve this vessel here, for example, that's going to the optic apparatus. We can selectively coagulate vessels and we need to choose which one to coagulate. What I'm doing here is I'm opening the cyst posteriorly. I'm aiming to detach the cysts from the calcified portion. That is another branch, going to the tumor, get another one. You see the Pcom posterior and this one, for example, is tricky because it has two branches, one goes to the tumor and the other one is going to the optic tract. We need to preserve the one go to the optic tract. You can see how thin is this optic tract right here, really poor looking optic tract. I continue dissecting the calcification from the structures around, detaching it from the optic chiasm. Now I'm looking at the posterior circulation here, basilar bifurcation, detaching the capsule. You see both posterior cerebral arteries and you start seeing the mammillary body. I can finally start rotating this calcification. You see the optical apparatus above. I'm not putting any pressure on the optic apparatus. Finally, I can transect the capsular on the calcification and this classification can be rolled out. See the size almost does not fit on the opening. But finally, we can move this calcification. This I tried doesn't show and we had tried to fit it up in pieces, but it was not possible. It would bounce against the optic apparatus and it was not safe so hard to remove in block. Now we're looking at the hypothalamus right here. This is hypothalamus right there, here, and this is the membrane of the cyst that I'm trimming and I continue trimming until I have a clean hypothalamic interface. In this patient I preserved the pituitary stalk, part of the pituitary stalk, patient did not have pituitary function, Just MALDI but he needed hydrocodone after the operation. This is the post-op, you can see the fat graft in the reconstruction and some enhancement but that often is just post-surgical change, you need to follow that up. This patient is now almost two years out and has a clean MRI, no recurrence. As we said, pituitary dysfunction, but otherwise a very good outcome with improved vision although not complete improvement because of the long term damage on the visual apparatus. That was a good case to start here my experience at Stanford. Shortly after, they also refer this patient to me. This was a true challenge that I actually thought twice whether to operate or not. This 48 year old multiple recurrent craniopharyngioma, seven previous operations, five transcranial, two endonasal, the last two in the United States by a good surgeon. He did transcranial and endonasal and it still left quite a bit of tumor and the tumor grew back quite rapidly. He had also radiation in the past. At this point... There's tumor you cannot cure, but this patient is blind chronically because of this tumor. There is no vision to worry about in this patient. But he's having cognitive decline. He's having severe ataxia. Even to have some weakness because of the compression that you see here at the cerebral peduncle. This was a really challenging case that I decided to tackle. Let's pull the second video, please Luke and then you'll see what I'm doing here is pulling the flap that was use originally down and then expanding the approach. All the way to the carotid down here as the carotid enters the cavernous sinus. I need to have that control extended multilateral until I see the cranial colloid, which you see right here. I was using a doppler to confirm. This is the other carotid and cavernous sinus on the other side, extending the drilling superiorly to expose the limbus of the sphenoid all the way there. I'm cutting the dura and the tumor is plastered against the dura. As I'm detaching the tumor as I open the dura. You see the basilar is down here and you see this as a double superior cerebellar artery, P1, the other P1. You can see the stem of the Pcom right there which was sacrificed at one of the previous operations. This was a tumor with ugly plains, really difficult to work but patiently we're dissecting here the tumor from the anterior circulation. This is a tumor on one side and on the other side. This is an A1 right here on the patient's right side and I'm carefully trimming the tumor off the vascular structures. I'm using an angular scope to look superiorly and I would worry about an optic chiasm, but in this patient, as you know, the patient is blind. This is what is remains of the optic chiasm, is just a very thin layer of tissue. It's been difficult to differentiate. This is the Pcom and remember how we mentioned the Pcom is always at risk and here I'm trying to dissect this tumor which is encasing the carotid and the origin of the Pcom from the carotid. Here I'm trying to do this dissection and this progress and aggressive move on my part, a mistake in retrospect, as I was trying to dissect. Next time, what I would do is I would probably just coagulate the Pcom and that way, I will not get an injury like this. This little pinhole in the PCA, probably because of the traction I was doing. But this injury was very easy to control. Just a small hole with a bipolar I can just seal it. I just seal the pinhole, but at the same time, preserve the integrity of the P1 in this case. As you will see this patient will get a stroke as you see with post-op from a small peripheral vessel. I'm not sure exactly what it is but I guess somewhere in this area of the P1. At the end of the surgery we have basilar bifurcation, superior cerebellar artery, to the whole third ventricle, open the hypothalamic interface right here and then this anterior commissure, this is anterior circulation right here with Acom Beautiful Massa Intermedia right here all plastered because the Mass Effect. Just to reinforce the vessel right here, I put muscle patch some to seal, and then reconstruction which was also a challenging one. I use fascia lata and one of the few cases, I've ever used to flaps. Because the flap from the previous operation was not enough, it had retracted. We put two flaps, one parallel to the other. They should not overlap , they need to touch with, not overlap because they don't heal well if they overlap. This patient amazingly did not leak after the operation. As you will see, he woke up with some asymmetry, some mild weakness, but recovered just in a period of a few days and had a very good recovery and good outcome. He's still alive more than a year later. There is some cystic recurrence happening. This is the mere post-op. The mere post-op shows that there is some residual right here around the carotid and also around this carotid. This I could not clean. It's just encasing the carotid arteries. This is the stroke he got in the anterior thalamus, but internal capsule here is intact or is anterior part of the capsule, so it would not cause motor symptoms. Just transient hemiparesis because of the swelling perhaps but recovered completely, which was great and the patient is now doing quite well. You see the complex reconstruction here with a large fascia lata at the two flaps. This again, the post-op, you see all the brainstem has been nicely decompressed, very large tumor resection, but he got some tumor progression, which is tumor growth here. Then he got fractionated radiation and he's starting to grow a cyst here that we are watching and hopefully, he won't need another operation in the near future. Because it's quite a difficult case.

- You know what Juan Carlos, this is an extremely difficult case. I think you should give yourself a lot of credit. These kind of cases with so much previous surgery and radiation, the risk of a small stroke is very high and for you to get such a good result is very impressive. Very impressive Juan Carlos.

- Thanks Aaron. Sometimes we need and you do it all the time, all right, I know that. We need to push the envelope, we need to try to... There are some patients that they run out of options and sometimes we can give them some hope. This patient had no hope and now he's a year after this operation and he's doing relatively well.

- Beautiful work.

- Thanks Aaron. All right, let's go to another case. This was also an interesting case. This patient had five previous operations. All of them transsphenoidal, microscopic transsphenoidal surgery. This is the last picture right here. This is the tumor right here. This patient was told to be inoperable. He was offered palliative chemotherapy, experimental chemotherapy. But this is a sellar type of craniopharyngioma that is invading the cavernous sinus. The calcification you saw on the CT scan is very important. Luke, perhaps you can go back to the CTA, would you mind? You see this... Right there please. You see this classification here and here is on the other side of the tumor. But I think it's key to remove this calcification in this case. If you don't, the tumor is gonna keep recurring forming new cyst, new growth. Please Luke, run it right again and let's look at the next one. You see this the carotid artery right here, is very stenotic. We could have sacrifice it. Go to the next Luke please. But we decided to just do BTO. You see that is a very narrow carotid right here and I decided not to sacrifice the carotid and go for surgery to see what we can do and you will see what happened. Let's go look for the video. As always the first thing is expand the approach. Before the approach was only this, but we're expanding in every direction all the way down to V2. What you saw round there is V2. This is the other cavernous sinus on the right side. V2 down here, so wide exposure. Enter into the tumor in the sellar that's easy, but then we open and there is a lot of a scar, the optic nerve and you see they are scarred towards the... This the pituitary gland here. What is left is this patient has pituitary function, is still intact, amazingly. We need to carefully dissect this peripherian vessels. This is the pituitary gland up here and this is the calcification we were talking about before. First I have to detach it by drilling the clivus and then yeah, I can take the calcification. This is the Pcom, is stuck to the dura. This on the other side the supra hypoxia artery which I carefully preserve as it goes through the optic apparatus and then I can start taking tumor out, using a doppler all the time. Now I'm opening lateral to the carotid. The carotid runs right here and what I'm doing is now directly going into the cavernous sinus. I don't know if you know, but this patient has a complete cerebellum palsy that is chronic, he's not gonna recover. It happened after radiation. I don't have to worry about going aggressive into the cavernous sinus because there is no cranial nerve to preserve. Those are gone already, which makes our job much easier. But here I'm now dissecting towards the supraclinoidal space, that's the origin of the Pcom right there, and I'm working on both sides of the carotid within the cavernous sinus, trying to clean complete the cavernous sinus until they get a clean wall and you will see there are... I would even see some nerves in the wall of the cavernous sinus. This is clinoidal space all the way to the distal ring. We're basically pulling off tumor off the carotid. Yeah, it was scary, because of the amount of... Oh, perfect. Amount of scarring around the carotid. I could see the carotid becoming more prominent as it were in every section, thanking us from taking the tumor out from it, and that second is the distal ring, and pulling it off, and working on both sides of the carotid. You see that some nerve either I believe, but again, I don't care and then just coagulating the remaining. You see all that is left is the distal ring attached to the carotid and nothing else, all clean. The pituitary gland up and this patient had pituitary function preserved. You see a lot of fat in there, I had to put out a lot of fat, which I usually don't do because this patient didn't have a good flap, because of the previous operation. We use a lateral nasal wall flap to do this repair and it worked really well. Everything you see in the cavernous sinus is fat. This patient is now 18 months from surgery with a clean MRI, which I'm very happy about because, again, it's a patient that was sent for experiment of chemotherapy at that point. Thanks Luke, let's go to the next one. The next two cases are gonna be pediatric cases. This was a very challenging one. You see a huge tumor. Luke, could you mind going back and stop at the first slide? This 11 year old kid had an Ommaya place with multiple cysts operations and he was developing more and more cysts and this would happen with some of these patients. His pituitary function was partially compromised, not entirely but partially. You see the amount of growth and also the amount of disease behind the sellar behind the clivus. This is a very difficult. You have to do a transclival approach in combination with a suprasellar approach. In this case, I'm gonna show you how I did a pituitary transposition. Now at the end, it was probably futile because the plan didn't work, it still doesn't work. But I tried, I tried to dissect the pituitary gland and I was able to preserve a meaningful stalk but again with no function. Please Luke go ahead and run it. You can see the stretch on the optical apparatus and this person also partially is calcified, but different than the first case. These are micro calcification you can usually work through and remove in a piecemeal fashion, not like the other first really solid calcification. You can see the Ommaya that is going directly into the tumor. The challenge with pediatric craniopharyngiomas of course, is that they don't have a pneumatosis phenitinus for the most part. You need you need to recreate anatomy. We are creating here a clival recess, as you see around there and drilling on top of the carotid and on optic nerve. This is a middle clinoid process that remains there but because is osseous ring with the anterior clinoid and now doing a transclival approach down here. I'm now finally opening the dura. Here's the pituitary gland, which I'm mobilizing because I need to get access behind. We need to dissect the pituitary gland tissue from the walls of the cavernous sinus or walls of the sellar. You saw a small vessel going to the pituitary gland and now we're cutting the dura of the floor and of the posterior wall of the pituitary fossa. These are combined transclival and transsellar approach with the pituitary just hanging in there. We see the basila approximately six nerves down there. Now we start doing some debulking. This is third nerve right here on the patient's left side. I believe is the ACA, anterior cerebral artery that is stuck to the tumor. Always be careful and don't pull the tumor. Always try to do careful dissection, only pull when you have everything under control. This is the Pcom again, major challenge to work around this Pcom right here. But now we're working on the other side and we see the other third nerve right here. If you remember the imaging, the patient had a cyst going on top of a third nerve, will tackle later. Now finding this key plane, this is the peduncle, cerebral peduncle and from here, if I keep going up, I'm gonna find the mammillary bodies. This is my key landmark to then developing a plane and preserve the hypothalamus. This is this is the cyst going on top of the third nerve. I'm doing this dissection and I was able to pull this out. That's the membrane of the cyst and this goes on top of the third nerve towards the uncus. What you see right here, this is the uncus of the temporal lobe right here, above the third nerve. Now we're looking superiorly. This is the third ventricle and I have developed already a nice plane of dissection between hypothalamic wall and tumor. This is the optical apparatus here, another cyst, some perforating branches and I'm trying to develop a plane here between the optic apparatus and the tumor. This is the pituitary gland here. Then we trim this directly off of the hypothalamus. The beauty of this is, you can very nicely see the interface between tumor and hypothalamus right here. That's the origin of the tumor. We can see the catheter that was placed there originally and then this is the, I will say, the origin of the tumor right here. The attachment that we need to carefully transect and find a plane between hypothalamus and tumor. That is a key part of the operation where you really need to slow down and think what you're retinal dissection is in this area. At this point, I stop. I see soft tissue, grayish looking, even if there are micro calcification doesn't mean that's tumor. I believe you are entering to hypothalamic territory, you wanna be a bit more conservative at that point. This is the pituitary gland and all the basilar bifurcation. Again, these are always telling you reconstruction, this is a layer of DuraGen, in this case, fascia lata, a fat graft that fills up the clival defect. Then a good extended nasal flap that covers everything very nicely. And with strategy this patient did very well. No CSF leak, you can see the resection we have intraoperative MRI that we can do right after the operation to assure that we have a complete resection and this patient is now more than a year post-op. Is still with a clean MRI and no complications other than complete pituitary dysfunction at this point, which was partially compromised before. In spite of preserving the gland, the stalk, no function I tried. In retrospect, maybe it's more practical to just transact the stalk and in a case like this. But we were giving it a trial. You know Luke, we can go for the next video. This is a very special case. If you can stop here Luke that will be great. Is a very special case because this is two year old kid. One of the great pleasures of what I do is operate on children because you can make a very long term impact in these patients. It's very gratifying when things go well. This is a two year old patient that presented with seizures. At a different hospital they treat these with transfrontal cyst fenestration. The cyst grew and developed other two cysts, became more complex. This is a very intelligent family looking for multiple options. What to do at this young age with this very complex tumor. The patient has no pituitary function at this point, which makes things a bit easier for us. But gather multiple opinions. Some people even recommend radiation, which I think is crazy for a tumor of this size and at this age. Endonasal approach for a two year old is really a challenge because as you see here there is no sinus. This is just a old bone, but it's a bone you can drill all the way from here to here and get excellent access to the tumor, perfect access to the long axis of the tumor. Luke please keep going. This case took a lot of preparation and with this VR which is always nice to get a three dimensional idea of the relationship of the tumor with surrounding vessels. My main concern, I have never done a patient was this young with an endonasal approach for such a difficult tumor. My main concern was being able to have enough space to work. We did the 3D printed model in this case which I actually took to the lab and this is real size and I was able to practice on it and get comfortable with the space, and surprisingly the space in the nasal cavity of this kid was good enough to work and to go after the craniopharyngioma. That's the key data on the operation. You see a small nostrils but the nostrils are flexible and you can work through them. But as we go in the flap has been done. You see the SFEE-noyd SY-nus. There is no SFEE-noyd SY-nus This is the tergal of one flap on one side, the tergal on the other side. What I have to do here is to drill everything and everything means from the orbit here to the other orbit, to the of the anterior skull base down to the clivus and nasal down here. It's a complete drill out of the sphenoid sinus and a skull base here. This takes some time but you can do and you can see the same landmark we were saying. You see, this is the limbus of the sphenoid right here. And cutting the limbus as we typically do, opening the dura, we entering to the tumor. This is perhaps residual pituitary tissue. This patient also had a large calcification but again you can break it into pieces. This ultrasonic aspirator bone curator is ideal for these cases I believe. Once we go into the tumor we can start debulking in a piecemeal fashion. You see this is tumor going up and that is the stalk itself. This is a case where is no stalk to save and there is no function to save either. But using this as arachnoidal plane, you can dissect the tumor from the surrounding neurovascular structures which is key. Once you get to the floor of the third ventricle, is not an arachnoid plane, is a subpial plane which we need to develop. This is a cyst that was going inferiorly and now I'm mobilizing the cyst up and you see a beautiful basilar bifurcation and Pcom on both sides. Beautiful view. You can see the supra hypoxia arteries and now this is the key step. What you need to do is subpial dissection to differentiate tumor and that is the subpial plane right here. You can tell, I use the mammillary bodies as a very good landmark to identify where the floor is and from there I try to do accurate dissection of the tumor off the neural tissue. Looking superior towards the third ventricle and bringing the tumor down that usually is easier because it has no attachment superiorly. The attachment is at the floor for the most part, but in this case we had a difficult scenario. Suddenly we had some arterial bleeding which we didn't know where it was coming from. Then I inspected it and I saw that the Pcom right here had been a valse from the PCA at this area. That's a problem. I tried to seal it with the carotid as you saw me doing in that other case, but that only make it worse and this is bleeding more and it's whether you carry the video that Luke. In that scenario, you have arterial bleeding from the PCA on a long corridor in this narrow cavity in this pituital patient, so what to do about this bleeding? First thing is, of course, to stay calm and to try to identify the exact side of the injury. We put a patty there and see what is the hole. I tried to coagulate as you saw but that's not working. You need to change your strategy and think what to do next. One option is to just pull a muscle patch and keep pressure. There you go. But as you see also, we have this aneurism clips available that have single shaft and I've used them a few times for situations like this and they come very handy. Again the bipolar is written up making it better but worse, so you put a patty and you can control the bleeding. I came with this angle clip and I carefully try clip just the side of injury while at the same time preserving the integrity of the lumen of the PCA. The clip went right in and then we take the doppler and you see the PCA still intact and there is flow, and we're clipping just the side of the injury. Can be unnecessary but I just put a muscle patch just to reinforce the reconstruction and some and that solved the situation, solved the problem and things remain stable. I continue the resection for a little bit longer just chasing bits of tumor in the third ventricle and finally reconstruction. This patient did initially very well. They had no stroke. This is the immediate post-op MRI, intra-op MRI with what appears to be a complete resection. There is a DWI showing there is stroke and the patient was progressing really well. He was almost ready to be discharged. It was day eight day nine post-operative . Luke, go ahead and pull the next video because I wanna share complications with you because this is a great learning point for me and as for others. This patient got... Luke I'm sorry, would you go back please to the first slide of this video? This patient developed sudden deterioration. Neurological decline, even more sleepy and you see here a lot of air into the ventricles. This of course, are a problem of the reconstruction. The patient was... We tried to do a repair with the flap, what happened is the simple flap had necrosed did not survive perhaps because of the narrow space, the tergal was compromised. We tried lateral nasal wall flap and it also necrosed, did not work. We ran out of options to seal this opening on the skull base. Then I relied on this temporo-parietal fascia flap technique which I had used just a few times, but basically is based on the STA just like you were doing STA dissection and then you take pec ring along with it. Then you transpose it from the infratemporal fossa to the maxillary sinus pterygopalatine fossa. Run the video please Luke. Of course, you require an incision in the scalp to dissect the flap. This is how the reconstruction, the scalp with defects looks. This is the pterygopalatine fossa. This is the opening transposing the TP flap. This is just a pituitary and you can use a dilator as we're using here. This is from a tracheostomy set. This dilator allows you to tunnel this vascular flap. This is a beautiful flap because is large and is heavily vascularized because is based on the STA. That is a fascia lata graft and this is our TP flap that covers the skull base very nicely. This made a trick, you can see the STA right here and you can see how well it enhances in the post-op MRI and the patient had and excellent recovery. It took him some time to recover but he healed completely. He did not require a VP shunt. This is the kid after the operation and the happy family with a very good outcome at that point. But this is not the end of the story for him and for his family unfortunately. He's been doing really well but he presented a year and 1/2 later with this recurrence. This is just a cyst as you see right there and recently was bit worse I took him back very recently like ten days ago. This is the TP flap that the first time I've ever went back into a patient into a TP flap and I'm elevating the TP flap. That's a doppler to check where the carotids are and I'm just dissecting and pulling down this TP flap because I need to use it for reconstruction. We discussed radiation options and we all agreed that the best option given for this client was to go back and try to remove the cyst completely. This is the exposure, the dura that is bulging. We cut through this thick tissue which is the first sellar that will repair we're using the past and that is the cyst right in there. This wasn't easy although its just a cyst. It wasn't easy because of the again, narrow space, the flap on one side, this thickening dura, but we went into the cyst capsule, emptied the cyst and I start dissecting the cyst off the surrounding structures, the optic apparatus. There were significant scars from the previous operation and you can start seeing the vascular clip we had placed before, the aneurism clip and this thick tissue right here, I realized that was the muscle patch we had placed, so I had to dissect it off. This is the optic apparatus and starting the hypothalamus right there. Doing the acri dissection at that level. I'm trying to find my again my subpial plane at this point to remove the tumor from there. Now dissecting from the other optic apparatus and then slowly as you progress from the optic nerve medially, you're gonna get into the hypothalamus. Medially and posteriorly and that is what you need to develop your plane. Now again, the clip, the basilar bifurcation down there entering the third ventricle and trimming the tumor capsule. This is the end for resection but we still see some tumor right here perhaps, whether this is tumor so I keep trimming it carefully and I send some of these pieces for biopsy and the last two I got came back negative so we finish our resection. I was very worried about reconstruction of course, given the past history of this patient. In this case dura was so thick in that I was able to just put a few stitches and these made the reconstruction act more effective. Because it's not water type but I can close it very well, minimize the CS of leakage, put a fascia lata on top and then these TP flap can be reused and this is the post-op intra-op MRI. It was a very nice resection and you wanna put voice, this is a lovely thing to see. This a kid the day after surgery saying he's been really well and this is the kid saying that he wanna be a surgeon when he grows up. He makes all efforts worth it because we really can have an impact in these patients. I really hope that this is his last operation and he has a long term remission of cure for this tumor. I just finalized with some conclusions. As you've seen endonasal endoscopic surgery provides an excellent corridor for even the most complex craniopharyngiomas these days. It provides this unique view of the chiasmatic and hypothalamic dissection plane because you come from below, you can do this good arachnoidal and then subpial dissection plane. There are key anatomy and nuances to remember, medial OCR, exposure of the artery of the carotid, limbus of the sphenoid, anatomy of the hypoxia arteries, the Pcom, the third ventricle interface and how to mobilize the pituitary to gain better access. At the same time we need to be very aware of how to prevent and how to manage vascular injuries in particular the Pcom, which is the most posterior circulation which are the most common. You see the surgeon's training, the experience and the skills of the team is what determines the operability of a sector area for a particular tumor. With enough experience and training, you can tackle very complex cases as the one I have shared with you. Thanks so much.

- Excellent master work. There was a challenging cases Juan Carlos. Very difficult especially the last case to dare to go back in there is therefore worthy of a Nobel prize.

- I've been sober. Thanks so much, Aaron I really appreciate. This, yeah, it was a struggle but I'm very happy with the outcome.

- Oh yeah, it's an excellent outcome. Some of the questions that was brought up during your excellent lecture are on review. One of the is, do you remove every micro calcification or are you comfortable leaving some of them alone if it is difficult to get out?

- Yeah, sometimes you can't. Yes, sometimes I do leave micro calcifications behind. Sometimes you can see them all along the wall of the third ventricle and those are impossible to remove. It's important to remove those that are in the custody of the tumor, but once they are embedded in the optic nerves or in the wall of the hypothalamus, I don't think there is a point of removing those.

- What kind of endoscopes to use? I saw that you are using different angles. There was a question what kind of endoscopes to use. It appears you use to do 30 degrees and try to stick with zero as much as you can. Is that correct Juan Carlos?

- Yeah, but I do most of the operations with zero and then when I need to see more, then 30 or 45.

- I see.

- 70 degree with you also have but that's for real extreme lateral cases.

- Rolando is asking, "What are these obvious severe craniopharyngiomas that you feel are good candidates for endoscopic versus those that aren't?" I assume where the floor of the third ventricle is intact, that's not a good choice. Can you elaborate please on what craniopharyngiomas do you consider inoperable via the nose.

- You got it, I think you nailed it. I think the one that I consider not ideal for endonasal is the one that is purely interventricular. As you know, those are rare. Those are 1 or 2% of all craniopharyngiomas. Because there is no tumor in the cistern at all and there is a floor in third ventricular that is displaced down and in those cases if you come endonasaly, you're gonna have to cross or go through noma hypothalamic tissue. In those cases I think it's better to go through a transventricular approach, lamina or transcallosal. Now there is another group you perhaps that those that had extension towards for example, sylvian fissure or very emergent transcranial fossa, which may be easier to do through a transcranial approach. But often if it's just a cyst that extends laterally, you just pop into cyst and the membranes will come down. You can bring them down.

- I agree. The other question is, how do you manage carotid injury Juan Carlos?

- I manage it mainly trying to avoid it. That's the best way to manage them. I've been very fortunate. As long as I've had a number of Pcom injuries especially in craniopharyngiomas, I haven't had a carotid injury in a long time and I touch wood right now. 10 years without a carotid injury, but I'm all the time working around it especially for cavernous sinus tumors. First by being very careful and not get it. If you get it then there is a protocol that you need to follow and there are multiple tips. One you should not forget is the use of a muscle patch, cross muscle patch, put pressure on the carotid and it can be very effective to stop the uncontrolled bleeding from the carotid.

- I think the interface between the craniopharyngioma and hypothalamus is over straighted. I think you can really be safe as long as you take whatever the part that is very infiltrated with the tumor, the patients do very well. To be honest with you, I have been very aggressive, not too aggressive obviously, of removing the part of the hypothalamus that's very very much discolored.

- Yeah.

- I have not had any major hypothalamic syndrome. I think, although everybody really emphasizes the point, we do wanna overemphasize as to cause subtotal tumor resection. Don't you agree with me Juan Carlos?

- I completely agree because I think that the hypothalamic injury comes mostly from the time where transcranial approaches were being done, and in those cases the hypothalamus is not seen and is the last thing you get access to resort to pulling and I think that's when the hypothalamic injuries is happening. Endonasaly is not severe. I think is very rare and I agree with you. You can be very aggressive in that area.

- Right, I really do believe you are right, chordomas and chondrosarcomas are essentially only endoscopic. I've done many of them the non-endoscopic approach because they are extradural. Those tumors are really 100% territory of endoscopy but intradural tumors, I think craniopharyngioma is one tumor type that truly has proven itself very, very nicely approachable via endoscopic approach and I would really say that its, and I know that's a lot of a dogmatic and I don't like dogma, that most of them can be very effectively removed via endoscopic routes. Very effective results, better preservation of vision, articles responding increasing the rate of CSF leaks multiple studies have demonstrated that and therefore I think craniopharyngioma is a beautiful tumor for endoscopic approach. With that in mind, I wanna congratulate you again, Juan Carlos for a spectacular career. I have no doubt we're gonna hear a lot from your great successes in the upcoming years and look forward to having you with us as another guest in the near future. Thank you.

- Thank you so much Aaron and again congratulations for a phenomenal work. Your pioneering visionary work with Webinar neurosurgical education. You are doing this way before anybody else was doing it. Kudos to you my friend.

- Thanks Carlos. God bless you and God bless all our viewers that joined from all over the world. We really thank you for supporting us. More than 250 viewers hanged around for you which is really a demonstration of your excellence and we're honored to have all of you with us. With that, good night. Again Juan Carlos, thank you. Thank you, thank you for being tonight. That's officially the end of our discussion. I will be in touch with you when the recording is available Juan Carlos and look forward to having you again.

- Thank you very much, bye bye.

- All right, take care.

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