Dr. Daniel Vo and Dr. David Shaye - Noma: The Face of Poverty

Good morning, everybody. Uh, I think we'll get started here. Uh, my name is Daniel Vaux. I'm one of the members of the pediatric anesthesia department here at Boston Children's. Uh, I'm not gonna bother introducing myself very much because you're all aware of my list of achievements, and we just don't have enough time to cover all of them. But, uh, we, today we're talking about um a trip I did with Doctors Without Borders in August of this year, um, which occurred in Nigeria and was specifically with this pathology, Noma. Um, I didn't know who else was going to be on the trip at that time, but it just randomly occurred that I was paired with, uh, Doctor David Shea, who's one of our facial plastics and reconstructive surgeons at Mass Pioneer. So we're able to do a 2-week mission, um, on this process, which we'll talk about in greater detail. And after completing the two weeks, we thought it was uh very interesting from both an anesthetic and a surgical perspective, and uh thought that our departments might benefit from sharing our experience. So we actually, uh, were able to give this talk as well at Mass Pioneer about a month ago and um we're grateful that we got time on the schedule to be able to share it with you guys today. So we'll be kind of trading off, uh, different portions of the talk and they will be talking about the background and uh project uh structure. All right. So, can you hear me? Can you hear me? OK. So thank you very much. Um, thanks, Daniel. And um I work over at Mass Pioneer, but I work about 3 or 4 months a year overseas in Africa. And Daniel also didn't mention, but he, he worked for uh 6 months with Doctors Without Borders in South Sudan. So with that kind of background, although we didn't know each other, we did meet in Nigeria, which is what um the background to this project is. So, um, so we'll be talking about NOMA. Um, does anyone, just by a show of hands, has anyone, um, seen a case of NMA? We have one person back there, OK. Has anyone heard of, what about show of hands, anyone heard of Noma? OK, so very few. So that's usual when we present it over at Mass Pioneer and other venues um similar because it, it pretty much, um, it's a disease of that occurs in areas of extreme poverty and also um of severe malnutrition. Um, there's about 100, somewhere between 40 and 140 cases per year, um, primarily occurring in sub-Saharan Africa and parts of Asia. But, um, the, but in, in, um, this part of the world, in Europe, there are sporadic cases, but they occur, um, very, very rarely and oftentimes are, are, um, missed. So, Noma is, um, the, a common way to describe it would be a flesh-eating disease of the face. It's an opportunist opportunistic infection. Usually is preceded by something that depresses the immune system, most commonly measles, and always is in the, in the setting of severe malnutrition. Primarily affects children in the ages of 1 to 4, um, oftentimes when they're just being, coming off of breastfeeding. Um, so other names for Noma, which I thought would kind of help the description of it, orofacial gangrerene, this is other names in the literature, um, a necrotizing ulcerative gingivitis, starts out as a small infection, um, on the gingiva and balloons into a larger, um, uh, necrotizing gingivitis, and then, um, parts of the soft and hard tissues disintegrate. Cankra Morris is a little bit in the older literature, it's referred to as Kara Morris. In the Nigerian um language of Hausa, it's called Siwaniska. This refers to the, um, this refers to disease of the wind. It refers to the speed at which it develops and progresses. And in say the Vietnamese wordkanma is also it refers to galloping, like galloping like a horse. Again, referring to the speed with with which it develops. And there are some social kind of references to the social aspect of it because it occurs in settings of extreme poverty. The face of poverty is what we entitled the talk that's been referred to in the literature. But, um, I'm gonna show a photo. There, there are a couple of photos in here. Some of they're, they're not, um, they're, they could be disturbing, but they're also important for just showing the, the type of disease. So this is acute noma in a, um, in a 4 year old girl, Fatima. Um, and about 90% of, 90% of children who do get noma um uh die from it. So it's 90% mortality, 10% survival. Um, as you can see, it's, it's eaten away at the lips, but can go into the hard tissues of the face as well. And then if, but if this child can get basic treatment, so if this child can just get to a district hospital, not anything fancy, just gets IV fluids, nutrition. Um, antibiotics and some very basic wound care, just, you know, dressings, um, that can invert and you have a 90% survival. So, nothing too fancy on this, it's mostly identification and kind of rapid transport to the nearest outreach center, the district hospital level. Um, when that child heals, when Fatima healed after, um, after somewhere between 6 to 9 months, you see you're left with a, a, a very difficult surgical problem, which is, you know, missing a large portion of the lips are missing. Um, and you're also in a setting where, uh, you know, you don't have necessarily, um, the ability to do like large like multi-stage procedures or free flap, a free tissue transfer, um, for, for these types of cases. So, there's a setting on sort of what's available and there's a cha or there's a challenge on what's available. There's a challenge of the type of disease you have and also how to recognize the disease in the same. This is another child. I showed this picture just because you can see the hard tissue, um, the maxilla has sequestered off and disintegrated. So this would be even more complex because you have to think of how to put bone in to replace. So, um, I referenced NOMA. Um, one thing that's interesting about NOMA is you can't, um, the, the statistics are very poor on it because, um, like another author in the literature had noted, you, you have, um, Once a country reaches a certain point where it can feed the majority of its population, it also can then start to measure statistics. But without that, without that ability to feed a large portion of its population, it can't always get accurate statistics. So a lot of times these are, we estimate that probably a lot of the cases are lost or it's just unknown how many cases. That's why the estimate's so wide, somewhere between 400,000 and 140,000 cases a year, probably around 200,000 survivors at any one point, um, who live with that, with that type of disease. This is the NMA belt, so it shows this area, mostly sub-Saharan Africa, but these are also sporadic cases throughout. Um, also, the reason why, um, in here is just because of the level of mal. also, um, instability in that region of the world. The only other time in this, um, this past century where there's been any concentration like that was in the, uh, was in Auschwitz actually, after World War 2. So, or during World War 2. So, um, that was an article in The Lancet that, that came out right after a doctor who had been, um, a, uh, in the concentration camps. So, um, what's, what's being done about NOMA, uh, now, this is, um, the setting for what we're speaking about is the NOMA Children's Hospital. This is in northern Nigeria. It's in Sokoto State. Um, and this is a, these are just some of the patients in the program that runs. In the background, so we're, we're talking about surgical interventions where there's 4 to 5 surgical interventions a year, but in the background of all that, there's, there's outreach programs, epidemiologists, there's a, there's a very robust background, kind of, uh, uh, outreach and, and awareness program. This hospital is the largest NOMA hospital in the world. It was started by a Dutch NOMA organization, um, a few decades ago, about 30 years ago, and they've been really doing the prim primarily the majority of the work, um. Up to up to a few years ago when the situation in northern Nigeria deteriorated mostly because of Boko Haram and security issues, so that organization stepped back based on security issues and then Doctors Without Borders stepped in and those, which is an many of you probably already know, that's an organization that works in conflict zones or difficult areas with difficult security. Um, so this is the, the area, this Sokoto is the area where a lot of the people with NMA in this NOMA belt can get to. Um, So I'm going to pause and give it over to Daniel. He's going to talk about evaluation and project planning. Thanks, Steve. So, I'll take a few moments to talk about um perioperative evaluation and then kind of structuring the mission as a whole, uh, in order to kind of maximize the efficiency and safety of the procedures. Usually, when you talk about risk navigation in a Doctors Without Borders project, naturally, your primary thoughts are to your own personal risk, you know, what's the security situation? What's my mobility? Is it safe to drink the water? The risk navigation that I'm talking about is the kind of that we do on a daily basis here, perioperative risk navigation. And I've shown this slide in a few talks that I've given to my department where I like to think of a model where risk is the interplay of these components, the patient. The team, the environment, and the procedure. And either through years of training or uh study or experience, we all come up with our own algorithms for navigating the risks that uh all of our patients undergo every day. I just like to take a moment to think about extrapolating this to an international health uh intervention. So, for example, the team, um, Dave and I basically met on the flight over and we happen to work in sister organizations, but oftentimes you have various levels of experience, expertise, um, language barriers that can occur in the team. So there's a lot of team dynamics that have to grow up very quickly in order to take care of these complex patients. The environment, Dave mentioned, a few of the things that you naturally think of, security and infrastructure, but even more simply, what kind of structure are you working inside of? Is it an inflatable tent? Is it a permanent structure? Is it uh constructed by the Ministry of Health or something that uh MSF has put up? Uh, we were kind of lucky in the procedure realm in that our procedures were specifically defined, which is kind of rare for a Doctors Without Borders mission. But even though that these were restricted to facial reconstructive surgeries, uh, as we'll go, we'll cover in a few of the cases that we show, there's a lot of variety within that domain. And I'll spend a few slides talking about assessing the patient, which is oftentimes the hardest thing to do, because You have to remember that many of these patients, this is their first encounter with a Western healthcare system. If they've seen any kind of healthcare provider, it's typically a traditional healer who might have been doing something that's counterproductive to what you're trying to do um in your setting, like packing wounds with dung or ash or other faith healing methods. But how do you come up with uh a risk assessment or your ability to assess how a patient's going to tolerate an anesthetic in a place that's devoid of a medical context, a place where children don't have birth histories or allergies or med lists um that they're coming with. This is the first time you're encountering them. And just to show, as contrast, um, I'll carry you through an exercise. So this is the ACS NIA Pediatric Surgical Risk Calculator. Uh, I show it because I do some, um, Of my non-clinical work on risk assessment and because Shawn Rangel is one of the surgical captains here and helped develop this risk calculator. But basically, on the basis of over 200,000 procedures that have been performed in over 60 children's hospitals in the country, they've come up with this calculator where you can input a procedure and any of these kind of 17 risk factors, and it will give you a 30-day predicted morbidity and mortality. And the reason I show this other than like introducing it to people is that if you were to put our patient population that we had in Sokoto into this calculator, it would grossly underestimate their perioperative risk, and that just makes sense because it was derived from a completely different data set or population for different purposes. More likely, the risk calculator needed in the developing world setting has a lot fewer variables, but I would argue that many of us are less familiar with these variables and their interactions with anesthesia. And there are things that don't necessarily fit as well into a calculator, but I would argue have probably more impact on health outcomes than things we typically think of as perioperative risk factors. And that's for uh an international health mission in general, but for the NOMA uh perioperative assessment, obviously, we're spending a lot of our time focusing on an airway risk assessment. When we're thinking about our goals as anesthesiologists, we're trying to balance them with the surgical goals and risks. And as Dave will talk more about when he goes through some of the cases, what they're thinking is, how do I improve this child's function? How do I improve their eating, their speaking, their ability to open their mouth by recreating these structures that have been eroded by this process, their cheeks, their nose, and their lips. So they're thinking, what tissue do I have available to myself? And given that tissue, how do I create inner and outer linings for these structures. From our point of view, we're trying to accomplish a lot of the same things that we accomplish here on a daily basis anxiolysis, uh, securing the airway safely, hemodynamic stability, optimizing the surgical field, and postoperative pain management. So when we do a NOMA um airway assessment, we're using the same exact assessment that we use here. We're looking at thyromental distance, uh, mouth opening, neck extension, subluxation, assigning a mala patty class. But I would argue, even though that we're doing all those and they're helpful, they don't quite give you an exact picture of what it's going to be like to manage that airway under real-time conditions. And a lot of that has to do with the heterogeneity of lesions. Obviously, Noma is not following any kind of textbook when it decides to um start to erode these facial features. So you can take a patient like this, and I think you and I would both agree this might be a difficult laryngoscopy, you know, a short chin could be an anterior airway. But there are patients that have perfectly normal mouth openings but could pose other problems like diff difficult mask ventilation. This woman also had a normal mouth opening, but because of her inner incisor distance was actually a difficult laryngoscopy and the tube actually had to fit, you know, between her teeth. And this gentleman, who doesn't have much of a cosmetic, uh, deficit, but if you've had any experience, uh, with Noma patients, you might notice that over his left cheek, there's this hyperpigmented area which could be some fibrous scarring, uh, indicative of trismus, and he actually had severe trismus, and this was, uh, his mouth opening. He had had Noma as a child and, you know, it's been so long standing that his gingiva had started to grow together at that point. So during the year, patients were recruited to the Noma Children's Hospital, and on our arrival, we screened 25 patients that had been recruited, and then the surgeons assessed them and came up with their surgical plans, and then I and another anesthesiologist performed HMPs and came up with our airway assessments. And in terms of our airway planning, Of course you can choose from a lot of different algorithms. This is the Difficult Airway Society from the UK. I think most of us are familiar with the ASA's Difficult Airway algorithm, and some people prefer to use the airway vortex. All of them are basically the same thing, right? So moving between mask ventilation, a supraglottic airway device, or an endotracheal tube in kind of a controlled, methodical fashion. Some people like the airway vortex cause it uh gives you a sense that as you move from one modality to another, oftentimes your conditions are changing or getting worse between different attempts as you move towards tracheostomy. Our situation was a little bit different because these were not unknown difficult airways, but assumed or known difficult airways. So we had time to kind of ask these six questions with each patient. How much time do we have? And for the initial intervention, uh, these were under elective and very controlled conditions. We had to ask ourselves what was available. You didn't always have um both nares or availability to the mouth. Um, we asked ourselves how compromised was the airway, which tissues were involved, and then came up with a series of options uh to meet their needs. And importantly, the last question was, how can I make my situation worse? Specifically, in our planning, we found ourselves asking these questions repeatedly. Do the, the surgeons need an oral or a nasal tube? Does the patient have trismus? Cause that automatically put them in a kind of a different risk category. Uh, due to cheek defects, were they maskable? Uh, due to age, uh, or development, were they cooperative? Uh, and importantly, as we'll talk about at the end, what is the postoperative airway look like? So for each patient, uh, I and the other anesthesiologists came up with a plan A, B, C, and D. Typically, the plan C was some kind of rescue, oxygenation, ventilation strategy and plan D was, uh, surgical or percutaneous airway. In terms of ordering or scheduling the procedures, there were a few principles that were applied, namely that, uh, as Dave mentioned, some of these procedures, procedures required to be staged as opposed to what we would do here, which is raise a free flap and then inset it with microvascular techniques that obviously wasn't. Able to be done in this low resource setting. So what we were relying on were these petticled flaps which had their own blood supply. So the first stage was to raise the flap, allow it to revascularize itself, and then about a week later insert it into the defect. From an anesthesia point of view, um, our considerations were that we wanted to give ourselves time to get familiar with the team as well as each other and the equipment before taking on our most challenging airways. So, we scheduled some of our simplest airways first, um, and then the challenging ones, and then the stage procedure. So our two-week intervention ended up looking something like this. We wanted to leave time at the end of the intervention for complications or emergency walk-ins. Um, so we try to keep the last few days free as well. Uh, any talk about airway planning wouldn't be complete without telling you what equipment we had available. Obviously, we had laryngoscopes and endotracheal tubes. I brought a set of, uh, intubating LMAs with me that ended up being less useful than I thought they would be. Um, but we actually had access to a disposable fiber optic bronchoscope. So I don't know if anyone's ever seen one of these, but Ambu makes a disposable fiber optic bronchoscope. They're about $300 apiece, and I know it's kind of hard to call it a low resource setting when you have uh disposable fiber optic bronchoscopes, but it's one of the benefits of working with a robust organization like MSF. It's actually, um, terrific to use. It's a very stripped-down system, just a fiber optic camera and a cable to, um, the screen. Uh, there's an injection port for local anesthetics and a suction port as well. Um, It comes in two sizes, adult and pediatric. The pediatric size could accommodate a 30 cuff tube. Luckily, on our particular mission, we didn't have any patients smaller than that. In terms of ventilator, we were using a Glostevent Helix. This is a drawover system. And the way that differs from what we use here in the hospitals is that we have plenum-based ventilators, which means that they use compressed 02 sources like a wall source or 02 tanks to drive 02 through or room air through a um high resistance uh variable bypass vaporizer. Um, In limited resource settings, they typically can't afford to use 02 tanks and don't have well source 02, so they rely on these things which are oxygen concentrators, which essentially pressurize room air through some filters and take out the additional gasses in room air to concentrate oxygen, and they can usually achieve concentrations of greater than 90% at flows of about 8 to 10 L. The drawback of these, uh, even though they're very cost-effective, is that they are, uh, reliant on electricity. So if you're in a setting that doesn't have a reliable power source, um, those will go out. So, the draw, the benefit of the drawover is that it's a low resistance halothane vaporizer that a patient's own spontaneous ventilation or diaphragmatic movements can pull vapor or room air, um. Through the vaporizer into the patient, uh, with a pretty consistent concentration dependent slightly on the patient's minute ventilation. People like these systems because they're simple, they're very robust. Uh, you don't have to be a biomedical engineer to service them cause they're very straightforward and as I said, they can run in the absence of oxygen. Or, uh, yes, of electricity and oxygen. So, I'm gonna pause there and move on now to talking about a few cases. So I'm gonna present our most difficult airway, which is this gentleman here, Mr. Abdullai Liman, and, um, you know, we have permission from our patients to uh use their information and MSF chooses to keep them identified so that you, they're not just a, a normal lesion to you, but they're actually a patient with this deficit. But as I described, this is a 42-year-old gentleman who suffered from Noma as a child, and for 35 years had had basically very severe trismus to the point that his mouth was closed. He spoke like this, and he survived on a liquid diet and mashed up food and pushed them retromolar, and that's how he existed. He worked as a farmer and otherwise was very active. This is not an uncommon, uh, complication of pnoma because of this kind of, uh, progressive, vicious cycle of initial infection, pain with use, so they keep their mouth closed, which causes them to use their joint less, which leads to more disuse, atrophy, and joint degeneration. Um, so, what you'll see in the children's hospital is children running around the playground with these popsicle sticks stacked in their mouth, and this is their physiotherapy to try to prevent this vicious cycle from occurring. Now, when we think of um trismus, we typically are thinking of either TMJ arthritis or some kind of master spasm with malignant hypothermia. Um, the temporomandibular joint is actually a very interesting one because this is the condyle of the mandible and it fits into this mandibular fossa, and it's articulating on top of this intraarticular disc and the motion is both a translation and a rotation as the muscles of mastication kind of contract and relax. Um, When we see problems with TMJ, uh, disorders here, it's typically because there's locking or clicking because this disc has been anterior displaced or, uh, is inflamed. It, it can be caused by much more than that in the setting of noma. So, Uh, trismus is kind of just a generic word for being unable to open the mouth, but they reserved the term ankylosis for fusion that occurs, and that can either be bony or fibrous. If it's occurring in the temporomandibular joint, they call it true ankylosis. If it's extra-articular, they call it false, uh, ankylosis. You can imagine that these, as opposed to some of the um trismas that we see, uh, is not alleviated by general anesthetic or muscle relaxant, OK. Um, another process that occurs is hypertrophy of the coronoid process. And the coronoid process is kind of this horn that is on the mandible that sits right behind the back molars. And if the mandible is closed for a prolonged period of time, this horn elongates and can actually fuse into the zygomatic arch, causing a bony fusion there. And this is um a CT of a patient, not our patient, but uh from a research article which shows that fusion of the coronoid process and the zygoma. And the contralateral side is shown to be normal. So, uh, I think most of the anesthesiologists here have done an awake fiber optic intubation, so I'm not gonna belabor the point. I will say that some of the challenges here is that we had no access to the intraoral, um, cavity, so we couldn't do glossopharyngeal blocks or have access to the tonsillar pillars. So what we did was start with a, uh, nebulized lidocaine solution, 4%. And this just shows you his preoperative mouth opening. And then then we did superficial uh airway blocks. So we blocked the superior laryngeal nerve by identifying the cornea of the hyoid bone. Injecting some local anesthetic. He's under some mild sedation at this point. And we also performed a transtracheal block. And we elicited a nice cough reflex to help distribute the local anesthetic. What I'll say was different about this mission was that, you know, as opposed to a lot of other settings where you're a solo anesthesia provider, we actually had a lot of help in the operating theater. So this is our OT nurse who was from Norway. We actually had uh essentially CRNA equivalents that were national staff that worked for the Ministry of Health. This is the other anesthesiologist who was from Ireland. So, we had a lot of trained hands to help with uh managing these complex airways. And kind of the final puzzle piece to difficult airway management in this setting is making sure that the plan is stated to everyone explicitly, assigning roles that are commensurate with their level of training, and then Uh, explaining what the trigger points are for moving from plans A to Plan B or from Plan B to plan C. I will say that it might have been luck of the draw, but we did 22 cases over these two weeks and we actually were able to stick to our plan A for all of them. Um, all right, so Dave is gonna continue this case and tell you about the surgical management of Mr. Lehman. All right. So, um, with, so we, we were very fortunate cause we had an excellent anesthesia team. So the cases all, we, we were always ready for a, a tracheostomy in the background, but we never had to actually even, um, go down that route. So we were, uh, we were very fortunate as surgeons to be able to not be as concerned about the airway and some very challenging airway cases and really dedicate some, um, some additional thought to the surgical plan, um, and, and dedicate our energy to that. So, I'm gonna run through a couple of cases, um, Continuing with uh Mr. Abdullahi. Um, so, he, his main problem was was charisma. It's not really an aesthetic problem, but it was, it was trying to get his mouth open so he didn't have to spend the majority of his day, um, eating in that way that, that was taking up so much time. So, um, when we, but now he had had, um, we didn't have a CT scan, and we also don't have like, you know, uh, we didn't, it was more difficult to get, say, blood transfusions or we, there was no ICU. So for him, when we, we did a transfacial approach, his facial nerve on that side had already been damaged from the original infection, from the no infection, and we were, our plan was to go down, most likely because the coronoid fuses to the maxilla there and we were going to do an osteotomy there. This is a very common approach to, to this type of trismus. When we got in there, we, we did realize it had extended. The fusion had extended. It wasn't just the coronoid process here, but it's actually his entire mandible had fused to his maxilla, so it was bigger than what we. Thoughts. So, a little bit of a game-time decision to change things based on the, uh, the, well, somewhat because of the lack of imaging there. So, we ended up doing a, um, a mandibuectomy or a segmental mandibectomy, a very small one, just to release the mandible and to free it up. And then we're left with a, a, a soft tissue defect. So, these defects, I'll, like the others I'll show, have to have lining. They have to have inner lining and outer lining. Um, to be able to, to not, so they don't scar down again, so you have to think of how we, how we'll go about doing that in a setting where we don't have free tissue transfer. So here in, in, in Boston, we would, we would put a free flap here, put a large amount of, uh, free tissue in, and that would, that would do a good job. We don't have that luxury there, so you have to think a little bit how are we gonna do that in this setting, um. So we go back in time, that helps tremendously. So, here is a, this is mapped out in a diagram called the delta pectoral flap. So, um, described in 1918, 1st for nasal reconstruction, but then primarily used a lot for oesophageal reconstruction in the, in the 1950s. In the 1950s, so, um, it's a regional flap, it's a fascio cutaneous flap, the, the blood supply comes from the medial part of the chest. This is all in size down to underlying muscle, and this is lifted up and, and then put into defects primarily on the neck. Um, or the esophagus in previous times. And then this area can be skin grafted, and this is left pedicled for a period of time and then divided about 6 weeks later. So it can be done. There's really low bleeding risk, and it's the patient does need to be pedicled for 6 weeks, but these patients, when they're coming to the hospital, they often will come for a few months at a time, so acceptable to that patient population. Um Here, because we're going up onto the face, not the, not onto the neck where this is, this is primarily been used. We, we really need a longer flap. It has to come way out onto the, um, onto the shoulder. So when we were taking a longer and longer flap, we do what Dan what Daniel spoke about, which is delay. So we, uh, we'll use the delay phenomenon. We will do the first surgery for this gentleman. Um, it has nothing to do with the face. Actually, we just incise this distal part, lift it up, skin graft it, and then put it back down like this shows here. We just skin graft the under surface, put it back down, let it sit for a week, let the skin graft heal, but also. We're depriving this part of the flap from any surrounding blood supply, so that it's recruiting additional blood in here, and that makes it a safer, more and more likely to live when we take a really long flap like this. Um, so, then the second surgery, about a week later, is when, um, sorry, some of the pictures are so messy, there's only so many things we could do at the time. So, um, but the, but the pedicle flap is then lifted up and inset, so there's skin graft on the outside actually, and the, and the skin from his chest on the inside. And this is, and you can see he's got mouth opening, and this is reconstructed, and then this will be divided, um, six weeks later. So, in the end, he's able to have mouth opening, he needs to have aggressive physiotherapy. Um, it's, it's not an ideal reconstruction, but it's, it's ideal for that. It's the best we can do for that setting. Here's another, um, another case, um, I wanted to share. So, this is, her name is Aisha Bellow. She's, um, 14 years old. She had Noma uh 6 years previously. So, she, she through the Noma process lost the majority of her lower lip, as you can see, and it's already gone through a failed reconstruction with an attempted delta pectoral flap, um, from one side. So, um, Again, trying to think of how here in, in Boston, we might do um another probably also use free tissue transfer, use a radial forearm free flap for this type of surgery. Um. So again, trying to think of how to, how to do this. Again, going to this workhorse, this sort of workhorse Noma uh Noma flap, which is very safe for this setting, um, and also using the delay phenomenon. So this has been delayed a week earlier, so this goes into the staging of the surgeries. And then again, looking at this defect and thinking, you know, how are, how are we going to address this? For a lower lip, we have to reconstruct the inner lining, the outer lining, and then you have to have something that's going to hold it up, some suspensatory mechanism. So, the existing um failure, actually, we decide to incise along here and turn it up. So now this skin is, is the inner lining of her lip. That's not gonna stay there unless you have some sort of way to hold it up. So we um We utilize fascia from her legs, so that's readily available. We don't have Gore-Tex or something else, but, but fascia does work. Um, and we need to fixate that somewhere. We need to, it's like a belt. We're going to hold that up, but we need to fixate it to something on the face. We don't have plates and screws, but we look for an area where you could fixate this. That could be the. Form aperture. So we made little incisions underneath the lip and here's the, the nose, the piriform aperture right here, this edge, um, we have a hand drill we could put a little hole through there and then with that we can suture through these tunnels we can create these little tunnels that connect down to this area where the flap will be picked up and we can suture the fascia here, run it through this tunnel, run it around the lip here, and then we can tighten it up to however we want to hold that lip up. So that's the suspensatory mechanism. Then on top of that goes our flap, OK, and that's situated, if you look here, this is the, the lower lip vermilion. So this is that it, the part that's been turned in and it comes right out. You want to put that seam of this juncture of the seam of this flap to this flap right along the vermilion. So this is her on the table at the end of that, um, this is the second stage procedure, right, because the first we just delayed the flap. The second one we inset it. So she's got a lower lip vermilion cutaneous border, and this will sit for 6 weeks. So. We're not there for the full 6 weeks, but we work with a local surgeon, Doctor Addy, and he, he's divided probably more more pedicles than anyone in the world. He's probably divided 20,000 pedicles in his life just cause he's, he's worked in this hospital for 20 years. He worked in this hospital for 20 years and seen so many of these surgeries, but it took him 20 years to get a plastic surgery, um, residency spot. He just got one recently. Um, but that goes into a different problem of the surgical workforce. There's not enough education programs to train someone. So, although he's very experienced in this, and, and again, I, it's funny to me that he's divided more pedicles than anyone that I'll ever divide in my life. So, um, and he communicates with us back and forth afterward on, on, by phone on some of these patients. So, uh, so this is how she would leave, Aisha leaves the um operating room and, and this is her, you know, maybe a week out. Um, so, she's happy, mostly she's happy because, um, functionally, she can eat a little bit better. The food's not gonna, it, it's not a mobile lip, but it can, it, it will hold food in. She doesn't drool just at rest, but most importantly, she said she could get married. So it, this is, again, this is, it's for patients who have survived, this isn't life saving. It's just sort of life-changing for quality of life afterwards. So the, the, the next patient, um, I wanted to mention, he's, this is a 15-year-old boy. His name is Adamu. He's, uh, he looks, if you looked at him in our exam room, I probably would have guessed he's around 8 or 9. That's also malnutrition and, and stunting of his growth of his growth. Um, he had noma a few years back. He had had a, a major lip defect which was reconstructed with an Eslander flap, and then he has, you see, total, total loss of his nose. So this is obviously a very complex problem. I just had. Someone come to the, my clinic, uh Monday, who had his nose bitten off and I was talking to him about, you know, probably, I was prepping him for the next 2 years of surgery. It's gonna be, you know, maybe 10 surgeries and, you know, I, I didn't tell him the cost, but it's probably somewhere around like $500 million or a million dollars of cost here in this setting. So how are we gonna even start to think about that when someone has no structure at all, no bone or anything like that. Um. So again, when we think about a problem like this, you need inner lining, you need the structure of the nose to hold it out, and then you need some cover, some skin to replace the outer. You can't just put skin down, um, otherwise that'll collapse. So what we use here frequently and we can easily harvest is ribs. We can take two ribs, um, and that can be done safely in this setting, and this is a district hospital, um, which is a joint Ministry of Health venture and Doctors Without Borders. And then, but for lining, which is a difficult part to line the inside of the nose, um, here, we would probably use again free tissue transfer. We'd use a radial forearm free flap and prep this, um, with, with and give a whole lot of new tissue to the area, but it, it, we don't have that ability, we don't have an ICU setting, and so here we do lining flaps from the face. So we'll take these and we'll fold them in so that the inner, inner nose has lining. Then, once that's lined, I can, you can split. The rib and form a tripod here um and form some structure to the nose that's sutured together. We don't have wires there. We had suture we could use. Develop a little pocket, slip it into there, and then the last step would be how do you cover it? How do you provide some sort of nasal cover, um, going back, actually this is still used today, but this developed this forehead flap, which you see is a large flap from the, from the scalp skin in the forehead, developed in the 6th century BC in in India, then like refined in the Renaissance time and then um. Uh, it's still used today, actually. Um, so this is a pedicle flat. The blood vessel runs up through here. Um, you can take this large defect here, this granulates in over time, and then that's flipped down. So many of you have seen that before. So here's the, here's the, um, the, uh, structure, the tripod structure, and then you have someone who's, who's like that, and then on the table from a one-stage procedure can, can leave the table like that. So he's then. Petticled, that'll probably stay for 2 months. Doctor Addy would take that down later, and you could go through further refinements. He probably won't because he has to go back to his village and there's planting seasons. He probably won't come back for the multiple refinements, but, um, he has a nose, he can breathe through it. His, his, um, and mainly the, the main purpose of this is he's not ostracized from, from the community. So he's happy, his dad is thrilled, um, and, you know, we don't really know. You can't, if you, if you just measured this on disability adjusted life years, he probably, you know, he's not going to die from not having a nose, so that might not come up as much, but in terms of like hope or changing his life, it's a significant difference. Also, you don't really, can't quantify it, but if he goes back to his village, people may come for other reasons to seek medical care based on what they, what they see. I'm gonna turn it back over to Daniel. All right. So, after spending two weeks seeing some really impressive reconstructions um and amazing surgeries, you know, uh, at the end of each of these cases, we were thinking, we were hoping to preserve these and uh keep extubation as smooth and gentle as possible. So the considerations we had when extubating these patients and the challenges were You know, a lot of these patients are adolescents or pediatric patients. So what to do with the delirious patients, we didn't have Presodex. Uh, we only had a limited arsenal of analgesics. We actually, uh, didn't even have morphine. Our shipment hadn't come in, uh, of morphine, so we were stuck with fentanyl. Uh, with the draw of a vaporizer, there was no inital gas monitoring for titrating wake-ups. Um, even though the volume of cases obviously is much lower than what we have here, there was still a time pressure because we needed to finish our scheduled, uh, surgeries before sundown because MSF didn't want to transport us from the hospital back to our hotel after sun, um, sundown for security reasons. As Dave mentioned, there's no ICU and although we had a very good PACU staff, um, we needed to have the patients pretty much packaged in terms of pain control and respiratory drive, uh, before leaving them overnight in the PACU. And I'll spend a few slides talking about when the planned difficult airway becomes the unplanned, more difficult airway. So, a lot of the postoperative Uh, changes actually made these airways more complex and more difficult, um, in the circumstances of, uh, takebacks or, uh, uh, or complications. So you've seen the DP flap several times here now, and you can imagine that if this young adolescent boy wakes up, uh, thrashing or um delirious, that he could potentially injure himself or the flap. You can imagine if he has any kind of airway complication. Somebody who was previously a grade one view on laryngoscopy now actually doesn't have as much neck extension as they did prior and could be more difficult to um intubate. Similarly, uh, Dave showed one example of the flap that we use for reconstructing lips, and this is the Abbe-Eslander flap. So you basically take the good lip and rotate, uh, tissue from the good lip to cover the defect, but what's left is a pedicle that kind of sews the mouth shut. So if this patient had any kind of airway bleeding or airway compromise postoperatively, Uh, your options are either to cut through the pedicle or take the flap down, neither of which is desirable. So a lot of these, um, reconstructions were actually made our situation worse, and you can imagine a patient like this is gonna remain like this for 3 to 4 weeks postoperatively until the pedicle is taken down. Um, so oftentimes we wanted a very awake patient that was also very calm, so we would take our time with their emergence. We had the luxury of having two anesthesiologists, so a handful of patients were taken to the PACU, and one of us watched the patient till they emerged and extubated in the PACU while the other one continued to do cases in the operating theater. Um, I won't spend a whole lot of time talking about postoperative pain management. I think the bulk of the pain management is actually relying on the fact that these patients are incredibly stoic, and you know that they've just had a mandibectomy and they must be in incredible pain, but they will tell you they're not having any pain. You must have to force analgesics on them. But we had to rely mostly on PO analgesics and the PACU, Tylenol, tramadol, diclofenac. We had morphine sulfate PO but it had uh expired, so it leads to interesting ethical questions. A lot of people would argue that if you're not going to use expired medications in your home setting, it would be unethical to use them. Uh, in a low resource setting. And while that theoretically, um, makes a lot of sense, at the end of the day, you're stuck with a patient who's suffering, uh, and we did in fact use some of our expired stock to treat their pain. So I'll conclude here. I'll just give a few thoughts and give Dave a chance to give some thoughts, but um we presented this and uh because we thought it was a really interesting From both a technical and intellectual, uh, standpoint for both the anesthesia and the, uh, surgical approach. Um, what hopefully has happened over the course of the talk and what happened certainly to us over the course of two weeks is that You know, you don't just see the lesion, but you begin to realize that the, there are patients associated with these lesions. And the Noma Children's Hospital is actually a really special place where you see children and their siblings and their friends' siblings kind of playing in the courtyard and um you kind of stop doing double takes when you see um either active infections or um pretty significant disfigurations. Uh, so it's a very humanizing place. Uh, it was a It's a really great opportunity to work there. I'll let to conclude. Um, so, again, thanks for your attention. This is, um, this is, I think was one, a unique opportunity for surgery and, and, and a surgical challenge and an anesthetic challenge in a low resource setting. Um, but by no means is surgery the answer for NMA. Um, I think it's not a surgical cure. It's not even probably a medical cure, although there's so much outreach and there's so much research on it, which probably starts to get to it, but it's, it's probably more in the realm outside of medicine, which is, um, Uh, you know, food security and how governments might provide security for the people and how, um, you know, we treat each other as human beings more in that realm than, than medicine. But one of, um, um, uh, Doctors Without Borders' tenets is, is bearing witness. So I think from a medical standpoint, you can just sort of, you know, see it, treat it, offer people who have survived that hope, and then, um, and then sort of report to the wider community and, and be aware of it. So, um, thanks for your attention. We're happy to entertain any questions or comments. Start by saying it's remarkable what you've been able to accomplish in the cases are really demonstrative. I'm sure of the whole work volume that you took care of there. And it's, it's hard to believe that that what you've been able to accomplish for these children and adults hasn't been totally transformative of their life, and as you stressed, they weren't life threatening issues that they had, but clearly, you know, their function and quality of life was, was uh. Markedly changed. I think it's interesting that when you were showing the flaps that you utilized, you went back to very historic flaps that obviously didn't require um The microvascular free flaps that you weren't able to do there, so you went to old methods that were very functional for these, for these patients. Um, is there I presume there are major efforts at trying to Decrease the incidence of noma now to try to avoid these disastrous long-term complications. Um, yeah, most of that falls along the lines of, um, addressing malnutrition and, uh, immunizations, because most of it, most noma is preceded by measles, um, or malaria, so I'm guessing again addressing malaria. And then the programs of information in the most remote in the district settings where there's little signs that just show if you see acuteoma, like if you see any sore on the, on the mouth, you know, refer on or or treat with in a, in a setting and with severe malnutrition, you know, be aware of that. It's not a small thing because usually it erupts as as a larger infection and, and then sort of the tissue starts to disintegrate, but once it gets into that florid state, it's pretty. Uh, you know, the mortality is, is high, 90%. NEMISF is also collecting epidemiologic data, um, as well as trying to look at, uh, underlying mechanisms for actually how it occurs as well, so that, uh, research is currently being written and hopefully published soon. Um, I, I suspect I speak for everyone in the audience that, um, you both have our admiration and respect for what you did and, um, this presentation. Um, could I ask you this, and David, you alluded to this, um, Uh, people who do medical missions are all well intended. Um, the, uh, some of the criticism, David, you alluded to is, uh, uh, do these well-intended missions really get to the root of the problem? And you pointed out the root of the problem is political and economic. But my question to you both is more at the individual level. Um, lots of people think about doing it. Uh, only a few go out and really do it. Um, some people sign up for it and then do it, but, um. You know, candidly, probably aren't as suited for it as others. What are the individual traits that you both think allow someone to be suitable to go into a resource limited environment and do a lot of workarounds, but still get the job done? Um, what are those traits? You wanna go first. Um, I think, uh, from a You know, from a, from a, just a surgical standpoint, it would be having um You know, having a broad training background and then also, no training will necessarily, like you just have building pieces, building blocks, some of what you've seen, uh, some of what you've read from like, you know, World War One textbooks or something. And then you can, you can piece all those together like little Lego pieces that you could come up with, cause that's never gonna be in a textbook, um, but you can piece those together. So having that kind of background and then, uh, you know, with a close, always, anywhere we've worked, it's always been with a close uh collaboration with local surgeons cause Um, again, like Doctor Addy has seen more NMA than I'll ever see just living there. So, you're having that close collaboration with someone locally, then, then together, I think it, it elevates the level of care you can provide. Um, and also the education, so like, you know, I, I also get just as much education when I'm there that, you know, I might offer as well. Um, if I had to add anything, I would say, I mean, obviously, flexibility helps uh in these situations. I think in my experience, the better generalist you are, um, is also very useful there. Uh we tend to really hone in on a specialty or a niche when we're in our home settings here, but being there, you, you need to be an emergency medicine physician, you need to be an intensivist, you need to be a pediatrician, you need to be an internal medicine doctor. Um, you also have to have family members that are OK with it, which I think is important. Um, so you need buy-in from the people you love, um, and it's not fair to put them through undue stress going through this. So, uh, it's maybe an external factor, but it's something you should think about, and, um, Yeah, that's all I have. The, um, I was, I want more, the, some interventions have been kind of described either vertical, like this would be sort of a, um, vertical intervention. You go somewhere, you do cert, you do a certain amount of care and then you might leave, um, and some might be, uh, described as like horizontal, like you're there for long term, you really build partnerships with local institutions to teaching, it's really, uh, usually it's a bigger, uh, investment. And then, um, diagonal would sort of be like any blend in between. So, but it's not that a, a, a vertical intervention isn't, isn't bad necessarily. Sometimes it's appropriate, especially say like a disaster setting. You don't have time to sort of work on sustainability. You just need to plug the gap. Um, so, so it's not that that's wrong, it's just, I guess every intervention, we always would think, you know, how could we, it might be like some small amount of teaching or it might be like some longer plan to develop teaching or something more sustainable. So basically converting vertical to some diagonal intervention. So I was intrigued by the, uh, epidemiological map you showed at the beginning, which showed a few red dots in the United States, Japan, Italy, the UK. And I was wondering, I mean, food insecurity is an issue in the United States. Uh, do you know if those cases are patients who, uh, that, that, you know, the cases of Noma arose in those countries, or were they refugees coming from another country? Those are mostly, so almost all of them are case reports, and they're usually someone who's either like, like elder abuse that's really neglected or someone with florid HIV that never took care of it. But, but frequently here it wouldn't be recognized. Like I, I, it would probably go unrecognized cause people just, you wouldn't, you wouldn't know about this. Um, whereas, you know, somewhere in Nigeria where people see it more, more frequently, they would recognize it. So, the few cases you've seen are, are, uh, that are sporadic on that, on that map are from, um, from mostly HIV related or, um, like abuse situations where someone's neglected. Uh, that was extraordinary. Question for you. There, there's certainly a lot of situations where the choice is to bring patients here for complex care and Many of the decisions you made about how to treat were based on local resources and all. In what would have been considerations for some of those of coming here for contemporary free flat-based micro approaches with an ICU and all that? Was the limiting factor culture, expense? What, what were the trade-offs? All probably funding. It's just that's not the, that doesn't fall in line with the um with that, with the mission or the intervention principles because I guess like the first step in any sustainability would be to, to do the surgery there and also just to think of it as a sustainable program, you know, Doctor Addy, when he completes his, his residency program, could probably take over the large majority of these types of, um, or at least work in collaboration with us, so. Yeah, so that's not considered, although there are groups that do that. A lot of the case reports and the case series have been, you know, from patients who have been brought to Europe or the US. But it would be funding and putting people up for extended periods of time and all that a sustainable solution to it. Additional questions for Daniel and David? So, quick question, were the majority of cases in the rural or urban setting? Um, you were in a district hospital, I believe you mentioned. That's the number one question. The second question I have is, how would you rate the local physicians, um, working in the area? Um, and do they do this sort of plastic surgery on their own? Do we have a plastic surgeon there at all, or? The, the, um, this is a, this is a city in a sort of a more rural state, so the north which is affected primarily from malnutrition, not only in that Sokoto state, but it kind of serves as a draw, a catchment area for going up into ***. And going over towards Chad and, and this whole area that's been affected by Boko Haram specifically and also just has underlying uh malnutrition that's just rampant throughout the area. So it serves as a catchment area. Sokoto, where we were, is a city, but it's a smaller, more remote city from, from Nigeria stand standards and um. So, but most of the cases are coming from rural areas. And then, um, That we worked with, you know, fantastic surgeons. There's oral surgeons, plastic surgeons. I think we can't say enough about the physiotherapy and the support staff in the hospital. They were fantastic. Really, really, I mean, it couldn't, what, what we did is we just came for, there's 4 or 5 interventions a year. Um, they leaned all the groundwork, did all the work. Patients were prepped for it. I mean, they did all the organization. It was for us, it was, you know, fun. We got to come in and do it, but they, they did all, all year, they did this tremendous amount of work. The Specific question was, are there any physicians there who do these surgeries? Is there a plastic surgeon there? Um, no, I don't know, not in that, not that, in that setting. I mean, so dividing a flap, yes, and assisting, but not at that level. But there's no reason, you know, he or she couldn't, no. Daniel and David, thank you for what you've done and the lessons that you've shared with us this morning. Thank you. Mhm that's fantastic uh nice group really supporting. I forget it's a