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SCORE School Spleen
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SCORE School Spleen
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Language: EN.
Segment:0 .
ALBERT SHAHEEN: OK, everyone. We're going to start in just a few seconds after it hits 8 o'clock. OK, it's 8:00. Good evening, everyone, and welcome to SCORE School. My name's Albert Shaheen. I'm the assistant editor of SCORE, and I would be your moderator tonight.
ALBERT SHAHEEN: Before we get started, a few housekeeping items, we encourage you to use the chat box to ask questions and make comments. Everyone has been muted to avoid unnecessary noise feedback. You will see the QR codes for attendance tracking displayed a few times throughout the presentation. You only need to submit it once, and you will receive an email confirming your attendance. All of these SCORE School sessions are being recorded for later viewing on the SCORE website.
ALBERT SHAHEEN: You should be aware that the names in the chat will be recorded as well. We encourage you to view these recorded sessions as your schedule allows. Later on, this month, we will be moving the time of these live broadcasts to the week preceding the corresponding twist week. So we'll make an announcement about that change. Please check the SCORE website and Twitter for those specific times and instructions.
ALBERT SHAHEEN: Blog and information, as of now, will remain the same. For tonight's SCORE school session, we'll be discussing three modules-- Hematological Diseases of the Spleen by Dr. Ralph Edwards and [? Schlecta, ?] Splenic Abscesses and Neoplasms by the same authors, and splenectory by Dr. Joshi and [? Boulos. ?] It is now my pleasure, my great pleasure to introduce our faculty presenter, Doctor Elizabeth Hechenbleikner, who is an Assistant Professor of Surgery at Emory University Dr.
ALBERT SHAHEEN: Hechenbleikner obtained her undergraduate degree at Wake Forrest, where she lettered all four years in Division 1 field hockey. After obtaining her medical degree at the University of North Carolina at Chapel Hill, she completed general surgery residency at Georgetown University Hospital, where I had the privilege to work with her and help train her.
ALBERT SHAHEEN: During her training, she spent three years as a research fellow at the Johns Hopkins Hospital during which time she conducted studies related to colon cancer as well as clinical outcomes research among colorectal surgery patients with a focus on surgical site infections. She was part of the Armstrong Institute resident scholar training program in patient safety and quality while she was there at Hopkins.
ALBERT SHAHEEN: After finishing her residency, she went on to complete a fellowship in advance laparoscopic and GI surgery with a focus on bariatric surgery at the Icon School of Medicine at Mount Sinai in Manhattan. Dr. Hechenbleikner is a board certified surgeon with multiple manuscripts and book chapters published. She serves on the publications and resident and fellow education committee of Sages and is an ad hoc reviewer for surgical endoscopy and surgery for obesity and related diseases.
ALBERT SHAHEEN: She is actively involved in quality improvement and research projects among bariatric surgery patients. Focusing on protocols for VTE prophylaxis, ERAS, and the use of pharmacists for assistance with in-patient discharge medication, reconciliation, in addition to mentoring Emory general surgery resident and fellows and research projects. Her research interests include investigations and patient outcomes, including reduction of surgical site infections and 30-day re-admissions, as well as patient safety and quality initiatives in laparoscopic GI surgery.
ALBERT SHAHEEN: She's married to a wonderful husband, Dan, who is her coauthor on multiple of her papers. And she's a statistics wizard, and they have two adorable daughters. Lizzie loves her family, Peloton, field hockey, and soccer. So thank you so much, Dr. Hechenbleikner, for lending your expertise to us tonight. Take it away.
ELIZABETH HECHENBLEIKNER: Can everybody hear me, first, before I start? Yup, we can hear you.
ELIZABETH HECHENBLEIKNER: Great. Thank you very much for the introduction. I am very excited to be participating in the SCORE School this evening. I have long been a supporter of SCORE at the Twiss curriculum, and certainly, this newer initiative. I am particularly honored to have been introduced by Dr. Shaheen, who as he indicated with my former program director at Georgetown, and who is one of my favorite surgical educators of all time.
ELIZABETH HECHENBLEIKNER: So you can advance the next slide. I have no disclosures. And on the next slide, it just shows off some of my references. So we can skip to the next slide, for, my outline. As Dr. Shaheen indicated, I have the core modules listed here, as well as the advanced module for the Twiss curriculum sequence covering spleen.
ELIZABETH HECHENBLEIKNER: I'm going to start by giving you all an overview of splenic anatomy, histology and function. And then I will shift the discussion to a more problem-based format where, I will go over diagnosis, preoperative management, non-operative management, and some operative treatment, based on some the different splenic pathologies that we're going to encounter during this talk and also as surgeons.
ELIZABETH HECHENBLEIKNER: And toward that end, I will try to shift back to going over more of the technical aspects of splenectomy with a focus on minimally invasive surgery, because that's one of my favorite things to do. And then we will end up discussing some of the complications and issues, long-term follow-up and then some questions. We'll go to the next slide.
ELIZABETH HECHENBLEIKNER: So operative anatomy is very important for the spleen. I'm going to start with a broad overview of some of the key concepts that you see listed here. You can go ahead and advance to the next slide. So the spleen, as most of everyone knows here, is an intra-peritoneal structure. It's located in the left upper quadrant under the diaphragm, and tucked up on the ribs 9 to 11.
ELIZABETH HECHENBLEIKNER: I don't know if you guys can see this picture any well at all, but for anybody that is attending, does anybody know what the dots in this picture refer to? Feel free to answer in the chat or to shout out the answer.
ALBERT SHAHEEN: They can't shout it out, they're muted but the chat is perfect. Come on, guys. Let's hear it.
ELIZABETH HECHENBLEIKNER: Derek. So Derek is correct. Derek answered accessory spleen location, so nice job. So, yes. These are the potential locations for accessory spleens, which you will find in about 10% of patients. And you can pluck that in the back of your mind for a second, because we're going to talk about that in the latter part of the presentation.
ELIZABETH HECHENBLEIKNER: In terms of some basic dimensions, an average spleen weighs about 530 grams, which is roughly on the 300 grams side, 7 to 8 ounces. It's roughly 12 centimeters long, 7 centimeters wide, and 3 centimeters thick. Obviously, the size can vary dramatically, depending on what underlying splenic pathology you're dealing with. You can go to the next slide.
ELIZABETH HECHENBLEIKNER: So surrounding structures-- the spleen has visceral relationships with many organs and structures. More and purely, this includes the greater curve of the stomach and the splenic structure of the colon, as you can see, on the right side of the screen, here this picture shows those structures removed-- more posteriorly, the associated retroperitoneal structures include the left kidney, as well as the pancreas. The tail of the pancreas does affect the hilum of the spleen pretty closely in most patients.
ELIZABETH HECHENBLEIKNER: The superior surface is roofed by the diaphragm, which separates the [INAUDIBLE] from thoral cavity. The ligaments that suspend the spleen are really reflections of the peritoneum, and they essentially mirror, and case the structures that are in close contact with the spleen. And those include the gastrosplenic, splenocolic, splenorenal, and splenophrenic ligaments. The main blood vessels enter and exit the spleen via the hilum, which is also shown here through the picture that's on the right side of the screen.
ELIZABETH HECHENBLEIKNER: The splendic artery is one of the main branches of the celiac trunk. It is a tortuous vessel that travels behind the pancreas, giving off multiple branches as it heads toward the hilum. The main venous drainage is through the splenic vein, which is not a tortuous vessel, also travels behind the pancreas, and typically joins the SMB along with the IMB, somewhere behind the pancreas to converge into the portal vein, heading back to the liver.
ELIZABETH HECHENBLEIKNER: Let's head to the next slide. So in terms of the ligaments, the important part of operative anatomy here, you need to know these ligaments because this is where you're going to encounter blood vessels. The gastrosplenic ligament carries the short gastrics vessels, and its superior portion and in the left gastroepiploic and it's inferior portion.
ELIZABETH HECHENBLEIKNER: The splinorenal ligament houses the splenic artery and vein, as well as the tail of the pancreas. And then the splenocolic and splenophrenic ligaments finally are relatively avascular structures. However, you may encounter some large collateral vessels if you're dealing with patients of portal hypertension. For our next slide, I am going to delve into some more detailed aspects of the blood supply.
ELIZABETH HECHENBLEIKNER: So regarding the main afferent blood supply to the spleen, there are two commonly described variants or types that are found in most surgical textbooks. So the magistral type of blood supply refers to when the splenic artery divides terminally, very close to the spleen. So this is going to be about 1 to 2 centimeters from the hilum, and this occurs in about 30% of individuals. When a division of this splenic artery however occurs earlier, on the pathway toward the hilum this is usually greater than 2 centimeters away from this area, away from the hilum and then the pre-pancreatic segment.
ELIZABETH HECHENBLEIKNER: This is called a distributive blood supply and this is the more common variant that you're going to see in about 70% of individuals. It's important as surgeons to recognize that there are a lot of variations and branching patterns of this splenic artery, and it can give rise to branches like the left gastric, middle colic, and the left hepatic. So you want to be prepared for this when you are dissecting in the hilum in this area and other structures that are close by to make sure that you're avoiding injury to these vessels and other surrounding structures.
ELIZABETH HECHENBLEIKNER: For our next slide, I'm going to jump in to some of the histology. So as most of you know, spleen is covered by a fibroelastic capsule. And this capsule further compartmentalizes the spleen via the trabeculae. As the spleen splenic artery comes in through the hilum, and starts to head toward the trabeculae, it branches into these terminal branches and continues dividing to create these central arterials that go in between the trabeculae.
ELIZABETH HECHENBLEIKNER: These arterioles continue to branch into even smaller vessels and then the adventisha ultimately gets replaced by a covering of lymphatic tissue that's known as the periarteriolar lymphoid sheaths or PALS. And this continues until the vessels thin to capillaries. Another layer of lymphoid tissue that's surrounding the PALS is an area that's called the marginal zone. And interspersed in the marginal zone is even more lymphoid tissue, called primary follicles.
ELIZABETH HECHENBLEIKNER: The white pulp, which you'll commonly read about and histology textbooks about the spleen, makes up about 25% of the tissue. It does comprise both the PALS, which is a T cell and macrophage-rich area, as well as the marginal zones, which have a lot of macrophages and then the interspersed follicles which are B cells. Those are the three components of the white pulp. So it's important-- the reason why we always talk about the spleen is this large lymph node in the left upper quarter is because of this white pulp and all of the lymphoid tissue.
ELIZABETH HECHENBLEIKNER: It's very important for fighting infection and producing antibodies, which are important for both viral and bacterial infections. So the white pulp also interfaces with the red pulp. And you can see an arrow pointing to the interface here, between the red and the white pulp. In this particular area, interface is near the marginal zone.
ELIZABETH HECHENBLEIKNER: In the marginal zone, the arterioles and the capillaries ultimately lose their lymphatic tissue, and they evolve into these very thin walls splenic sinuses and sinusoids. These sinusoids ultimately merge in the venules, which then drain and to collecting then to travel along the trabeculae to form the splenic veins that mirror their arterial counterparts. And then they finally drain as the main splenic vein.
ELIZABETH HECHENBLEIKNER: Between these end vessels and all these splenic sinuses and sinusoids, there is additional tissue called the cords or the cords of Billroth. and the cords of Billroth are really in the red area that's featured in the picture on the right side of the screen where the red pulp is. Together the red pulp is comprised of both the venous sinuses and sinusoids as well these cords of Billroth.
ELIZABETH HECHENBLEIKNER: And that makes up about 75% of your splenic tissue. So again, a very mildly rich lymphoid structure but very rich in blood supply. So let's move to the next slide, where we'll discuss some function. So the spleen has the main role for hematopoietic function for the fetus until about five months in-utero, where the bone marrow takes over. In adulthood, the spleen can resume some of this hematopoietic function and certain pathologic conditions, particularly those that cause hypersplenism and destruction of cells.
ELIZABETH HECHENBLEIKNER: We talked about the spleen is this the big lymph node and filtering system. It's important sort of delve into this a little bit more, because the spleen has an important role in removing microorganisms and literally cleaning our blood. There's a couple of different theories of how blood flows through the spleen and around the arterials to end up creating a filtration process. And I think one of the more interesting theories is the theory of an open system.
ELIZABETH HECHENBLEIKNER: In the open system, the way that this is described as essentially blood is being fed into the spleen through these tiny little arterials and capillaries, and then it's trickling through this sub-like parenchyma of lymphoid tissue that's rich and reticuloendothelial cells. And as such, it then flows through all of this and then ultimately empties into this splenic sinuses and a portion of venous system.
ELIZABETH HECHENBLEIKNER: And as it's flowing through this open system with the [INAUDIBLE],, the thought is that these reticuloendothelial cells assist with the cellular cleansing processes. That involves removing pathogens like parasites but also sequestering RBC for maturation and serving as a reservoir for platelets as well. Importantly, as plasma is directed toward the lymphoid tissue, soluble antigens are exposed to this area, and it stimulates the production of antibodies.
ELIZABETH HECHENBLEIKNER: The spleen in a similar fashion also makes opsonins, which classically, the two are prepared in intestine, which you guys will frequently be tested on in the ab side. Prepared and actually initiates one of the pathways of complement activation, which helps destroy bacteria and otherwise abnormal cells and [INAUDIBLE] enhances basically the phagocytic activity as phagocyles and other leukocytes.
ELIZABETH HECHENBLEIKNER: So it's important to realize that when you lose the spleen, and you lose these opsonins and other splenic functions that you're going to be particularly at risk for infection. And that's particularly encapsulated bacteria's, which we will talk about a little bit later on, in this presentation. Another important aspect of splenic function is maintaining RBC morphology and function.
ELIZABETH HECHENBLEIKNER: As we all know, RBCs basically have a lifespan, and that's roughly 120 days. After this lifespan goes away and RBCs get old, they have decreased plasticity and they don't work as well. So it's important that the spleen is there to remove them. In addition, RBCs are not always perfect. A lot of times, they will accumulate nucleoli, Howell-Jolly bodies, Heinz bodies, et cetera, and the spleen actually needs to remove these and the reason being is that if it doesn't, then you won't have normal RBC function.
ELIZABETH HECHENBLEIKNER: So under normal circumstances, these are all the functions that the spleen is actually doing, and they're very important. And as you see, we'll talk about pathologic states. The spleen is sort of doing similar things, trying to clean the system of pathologic conditions, but it ends up causing problems. So let's move the next slide. So over here, this is just a kind of an overview table to show you guys the different biologic substances that are removed by the spleen.
ELIZABETH HECHENBLEIKNER: So the top part of the table actually describes the non-pathologic states or normal conditions and what the spleen does. And the lower half of the slide describes what the spleen does, and what it's removing under pathologic or disease started. And we will get into some of the specific disease states that are listed here on the slide in the upcoming slides. Let's move to the next slide.
ELIZABETH HECHENBLEIKNER: So in terms of thinking about the spleen and its presentation to surgeons, on a high level, we can really break it down into emergent, very emergent, and the trauma setting, of course, and usually, very elective conditions. So these are kind of on opposite ends of the spectrum, but these are very different circumstances and situations, where we are going to be forced with potentially needing to do a splenectomy.
ELIZABETH HECHENBLEIKNER: On the left side of the screen, the emergent setting really entails our trauma patients, and we're rarely and usually not emergent, sometimes, splenic absences, which are a very rare condition that I'll briefly touch on. And the elective setting, there are a host of pathologic conditions that may require splenectomy, which includes everything from hematologic conditions like ITP to primary tumors and even cysts. So in the next slide, we will delve into what happens and the trauma setting.
ELIZABETH HECHENBLEIKNER: The thing about diagnosis in the emergent setting, obviously very different from what you're doing electively. Whenever you're thinking about trauma patients, it's always about your ABCDEs, primary survey and mechanism of injury, especially when you are trying to think about splenic trauma. The spleen is certainly on the top of the list of organs that are going to be most injured in blunt trauma.
ELIZABETH HECHENBLEIKNER: So if you're worried about a splenic injury in this setting, it's important to factor in very quickly your patients hemodynamics, physical exam findings, and then your diagnostic add-ons like FAST and CT. And these are usually, going to help you quickly determine whether you're going to the OR or not. Obviously for any unstable patient with a positive FAST, you're going to be going to the operating room, but for those who are stable and have a concerning mechanism of injury for abnormal trauma, we usually evaluate all these patients with the CT scan.
ELIZABETH HECHENBLEIKNER: So when evaluating CTs, and specifically looking at the spleen, you were looking for one key finding, which is a blush of IV contrast or active extrapolation indicating that there's an active injury. You also want to assess the grade of injury, and you also want to think about volume of hemoperitoneum, other injuries, et cetera. Active extravasation should prompt either embolization by IR or going to the operating room.
ELIZABETH HECHENBLEIKNER: In terms of the grade of injury, you can see here on the right side of the screen that there is a high-level presentation of the splenic injury scale that was developed by the AAST. There are five grades of injury, going from grade 1, which is the most simple injury where you can have either a subcapsular hematoma that's less than 10% of surface area of spleen, or a laceration that's less than 1 centimeter deep, all the way to a grade 5 injury, which is essentially completely devascularized and shattered spleen.
ELIZABETH HECHENBLEIKNER: It's important when you are working up these patients in evaluating CT scan you know that they are notorious for underestimating the injury grade, so this has to be guided by your splenical judgment, and of course, how the patient is doing. So moving on to our next slide, you don't have a lot of time for preoperative management when it comes to your trauma patients. You're going to be quickly stabilizing patients, placing large bore IVs, giving blood products, hemostatic adjuncts, considering the massive transfusion protocol-- MTP, and getting people and patients to the operating room as fast as possible.
ELIZABETH HECHENBLEIKNER: These patients are going to be placed quickly, supine, on the OR table. You're going to be warming them at table and performing an exploratory laparatomy, via midline LAP, and trying to get the spleen out in a few minutes, which can be accomplished. From there, your post-op care is obviously going to be in the ICU. And we will talk about vaccines little bit more through the latter part of the presentation, but just know that in this particular setting, vaccines are going to happen hopefully prior to discharge of this patient population.
ELIZABETH HECHENBLEIKNER: So moving on to the next slide here, this focus is on non-operative management, because this is used very commonly for splenic injuries, particularly in blunt abdominal trauma. Over 90% of splenic injuries, certainly in the pediatric population, are going to be successfully managed, non-operatively. Dr. Shaheen might be able to speak more to these numbers, given that he is a pediatric surgeon.
ELIZABETH HECHENBLEIKNER: Certainly, in, adults the numbers are substantially lower and closer to 67%, using these pathways. The decision to employ nonoperative management is not necessarily predicated by the grade of injury, but more how the patient is doing clinically. So it does require that patients meet certain criteria. First and foremost, that they are hemodynamically stable and the absence of other suspected injuries.
ELIZABETH HECHENBLEIKNER: The remaining factors in the decision-tree here really rely on sound splenical judgment and using good surgical common sense. Anyone that's having significant drops in hemoglobin, increasing transfusion requirements, or obviously anyone that becomes hemodynamically unstable, you'd do there an IR and then operative intervention. And again, just keep in mind that this is not necessarily mandated by the grade of injury.
ELIZABETH HECHENBLEIKNER: Featured here, you see a relatively complicated nonoperative pathway that is used at a particular institution, and it can help guide you in terms of how to think about an hour non-operative management. So moving on, we are going to-- this here is a quick slide that just shows you how you can log your attendance. However, moving on to our next slide, we're going to get into some more--
ALBERT SHAHEEN: Maybe, can I stop you for a second?
ELIZABETH HECHENBLEIKNER: Yeah. Absolutely.
ALBERT SHAHEEN: Feel free to ask questions. This is a good spot for you to ask questions and if I could just say one thing one little thing about the non-operative management, it started with pediatrics. We started doing it in Toronto in the '70s and '80s and then it expanded to adults. And your point about the blush on the CT scan, we always have a debate in the pediatric world about whether that would necessitate action like embolization or operations.
ALBERT SHAHEEN: But I know that in adults, it's a little different. Can you speak to that that If you do see a blush, you do have to act, right, in adults?
ELIZABETH HECHENBLEIKNER: Yeah. I mean, I am not-- obviously not in the trauma world much anymore, but yes, in my experience, when there is active extravasation but a stable patient, that there's a minimum, a trip to interventional radiology.
ALBERT SHAHEEN: Yes. Thanks.
ELIZABETH HECHENBLEIKNER: But other people, obviously, feel free to chime in, if anybody else has a different institutional experience that they would like to share with the group.
ALBERT SHAHEEN: You've got a client tonight. Come on. [INAUDIBLE]
ELIZABETH HECHENBLEIKNER: Well, moving on to the next topic to hit on, which is one that's incredibly rare. So kind of the opposite end of splenic trauma, which is going to be something very common that everybody in this chat is going to see. Splenic abscesses here-- this is one of the other conditions that we may encounter as surgeons where we do have to intervene, but more in the urgent setting. Splenic abscess is a very rare clinical entity that's got a very non-specific presentation.
ELIZABETH HECHENBLEIKNER: Vague abdominal pain, fevers, and even sporadic chest pain. It's rare enough to where it's been reported largely an autopsy series, with only an incidence about 0.1% to 0.7%. Usually, this condition is secondary to hemategenous spread from infective foci elsewhere in the body. It's commonly found in patients with underlying immunocompromised state. The spleen can be seated in the setting of bacteremia, UTI, pneumonia, you name it.
ELIZABETH HECHENBLEIKNER: And they can even have rare conditions like tuberculosis. The ideology here is not always clear. However, when it comes to the bugs that typically are the causative agents and found in splenic abscesses is the gram-positive cocci, so commonly staph, strep, or enterococci, and then also gram-negative enterics. In terms of diagnosing this condition, CTs are really the preferred methodology.
ELIZABETH HECHENBLEIKNER: However, the diagnosis can be made with ultrasound. And splenomegaly as expected, not really common in this condition. So in terms of how to manage these conditions, we're going to the next slide, first and foremost, just in terms of reviewing what to look for on cross-sectional imaging, these typically appear as hypodense lesions, sometimes with septations, sometimes with peripheral enhancement.
ELIZABETH HECHENBLEIKNER: In terms of management, splenectomy previously was the gold standard for this type of condition. However, in patients whose co-morbidities really preclude this or people who otherwise have favorable abscesses, CT guided percutaneous drainage and a combination with appropriate antibiotics is really where a lot of treatment strategies are moving. And I personally feel like this non-operative management is a very reasonable approach to go with, with any patient out of the gate.
ELIZABETH HECHENBLEIKNER: So as you can see here, on the left side, this is what's called a unilocular abscess. It's very well-defined, as you can see, there are no septations, and there's no necrotic debris. So these are people that are more likely going to have successful outcomes from percutaneous drainage. Success rates can be up to 75% to 90%, and some are for a series and any failure here would require a splenectomy.
ELIZABETH HECHENBLEIKNER: And featured here on the right side of the slide, you guys can see that this is a more ill-defined splenic abscess with septations and a little on the cystic looking areas. And really, this is not going to do well for drainage, so you're talking about a splenectomy in this setting. laparoscopic splenectomy has been described for this condition, and it can be an option in the right splenical circumstances.
ELIZABETH HECHENBLEIKNER: And otherwise, you would be doing these cases open. So moving on to the next slide, we're going to now shift gears and start talking about more the common elective clinical situations and pathologic states that commonly require splenectomy. So we'll review some of the hematologic conditions, both benign and malignant. We'll talk to them about the non-hematologic tumors that can affect the spleen, and then cysts and pseudocysts.
ELIZABETH HECHENBLEIKNER: There is a very bizarre diagnosis of something called wandering spleen, which is actually mentioned in Sabiston textbook chapter associated with this Twiss series and modules, but it's not something that I've ever encountered, and just going to skip it for this particular talk. So moving on to the next slide, we're going to jump in to one of the really big topics that you guys are going to hear about and potentially even encounter on tests, and that's ITP.
ELIZABETH HECHENBLEIKNER: So that the immune thrombocytopenic purpura. so this is a very complex autoimmune condition, where you typically find isolated platelet lab values that are under 100. It's characterized predominantly by the binding of anti-platelets auto-antibodies to platelets followed by their phagocytosis by the reticuloendothelial cells that are mostly in the spleen. The spleen also serves as a site of reproduction of these antibodies and they're mainly IgG antibodies.
ELIZABETH HECHENBLEIKNER: There's also apparently some abnormal T cell activity toward platelets as well as some impaired enzyme production that impedes megakaryocyte functions that are also involved in this disease process. When you're talking about ITP, it's important to realize you've got to rule out secondary causes. There are a lot of things that we all know clinically that cause thrombocytopenia. So nobody know any common causes of thrombocytopenia in the clinical world?
ELIZABETH HECHENBLEIKNER:
ALBERT SHAHEEN: We have HIT Austin says, H-I-T.
ELIZABETH HECHENBLEIKNER: Yes, Austin. HIT, very good. That's one of the key drug-induced ones, probably the most common drug-induced one, I would say, that we'll see in our surgical patients. So other things that can cause thrombocytopenia are pregnancy, viral infections, cirrhosis. So those are other issues are other things to think about. So when you think about secondary ITP, it's triggered by inherited or acquired predisposing diseases like chronic infections.
ELIZABETH HECHENBLEIKNER: And some of the key ones are HIV, autoimmune diseases like SLE, so like lupus and then rheumatoid arthritis. It can also be caused by Helicobacter pylori infection, which I did not realize until rereading the [INAUDIBLE] chapter. It can also, in infectious cases, what's really thought to be going on is that the viral and bacterial antigens are sort of recognized as platelet antigens, and then they give rise to anti-platelet auto-bodies.
ELIZABETH HECHENBLEIKNER: So it's this term called molecular mimicry, where the body is sort of confusing viral antigens or bacterial antigens with platelets. So moving on to our next slide, again, just to discuss a little bit in more detail what I mentioned here on the first slide, it's important that when you are presented with someone with presumed ITPS as a surgeon that you're thoughtful, and that you realize that this is a diagnosis of exclusion.
ELIZABETH HECHENBLEIKNER: Obviously, you don't to be working somebody up for this all by yourself. If you're noticing this in your patients, and you want to refer all these patients to hematology, but you need to make sure that this is not a situation where you have lab error that's causing from the thrombocytopenia. There could be competing issues and platelets related to EPA, which helps actually keep blood from clotting.
ELIZABETH HECHENBLEIKNER: And other common causes of thrombocytopenia, we already mentioned on the last slide. But you guys need to keep those in mind, and you also need to think about other causes that can be mistaken for ITP. So you've got myelodisplasia, some of your congenital thrombocytopenia, TTP, and chronic DIC. So you obviously don't want to be taking spleens out of people that have any of these conditions.
ELIZABETH HECHENBLEIKNER: So that's why it's important for you as the surgeons to know that. Moving on to the next slide, we're going to talk about some of the phases of ITP. For the first phase is called the newly diagnosed phase, and this is within the first three months post-diagnosis. Your second phase is known as persistent ITP, and this refers to symptoms that are lasting between 3 to 12 months.
ELIZABETH HECHENBLEIKNER: And then the third phase is called chronic ITP, and this is where symptoms are persisting beyond 12 months. The majority of adult patients are going to progress to this chronic stage. In terms of presentation, on the next slide, this is typically a disease of young women. The typical presentation is going to be TKI, and then bleeding from gums and from your nose.
ELIZABETH HECHENBLEIKNER: You guys, this picture here, what do you think this is? Should be relatively easy but want to make sure everyone is paying attention. Close. I don't see well. It's not purpura. Let's see if I can see the chat. Petechiae, yeah.
ELIZABETH HECHENBLEIKNER: Yes, Brian. Good job. So petechiea are very common in the presentation. Atypical things that can happen-- GI bleed, hematuria, and then intracranial hemorrhage. The intracranial hemorrhage can be life-threatening, and it's very serious. In terms of this being disease, as I indicated, mostly of young women, about 70% of affected women, just so everyone knows, are younger than 40 years.
ELIZABETH HECHENBLEIKNER: And primary ITP in adults, this constitutes about 80% of diagnosed patients in the adult population. So the rest of your 20% of adults that you'll encounter are going to have some type of secondary ITP. As you can see, here both for the prevalence and incidence numbers, this is a very rare disease. And it's also important to know that the spleen is not normally enlarged in this disease process. If it is, you need to think about another diagnosis, because the patient really should not have a ITP.
ELIZABETH HECHENBLEIKNER: This right here really focuses on the adult presentation of this disease process. It's very different in children. And again, I'll go through some of the key concepts here, but Dr. Shaheen might be able to chime in, given his experience, but in children, it's very different. It's a much more acute and severe presentation, with the thrombocytopenia.
ELIZABETH HECHENBLEIKNER: Both genders tend to be affected equally. And the good news in children is that it tends to have more complete and spontaneous remissions, and this can be seen in up to 80% of affected children. And so really the rule of thumb with children is it should be treated conservatively. And it's typically a trend adults and unfortunately persistent as a chronic disease process. Does anybody have any questions that they want to ask about ITP ?
ELIZABETH HECHENBLEIKNER: OK, well, we'll move on to the next slide, but if you have any questions, feel free to jump in. So in terms of the non-operative management of ITP, really the severity of this process predicates how you are going to treat it. In terms of the severity and the symptoms, that's all listed here on the left side.
ELIZABETH HECHENBLEIKNER: And that, again, it's going to help guide us in terms of what treatment strategies we go after. So for patients that typically have a platelet count over 50 and are asymptomatic, we can observe them safely without any other follow-up or necessary treatment. For those who have platelets that are 30 to 50 and are asymptomatic, we can observe them, but they usually need more routine follow with a hematologist.
ELIZABETH HECHENBLEIKNER: In patients who have a platelet count less than 50, and who are bleeding, and it doesn't have to be life-threatening bleeding, it just can be persistent nose-bleeding or gum-bleeding or an active lifestyle that would put them at risk for a serious injury, these are patients that we need to be treating medically. And then on patients who have platelets that are less than 20 to 30, and regardless of whether they're bleeding or not, we need to treat these patients.
ELIZABETH HECHENBLEIKNER: We can observe they need some type of medical intervention. In terms of the treatments, overall, I will talk about those. And they're listed here, as you can see, on the right side of the slide, but importantly, for this disease process, platelet transfusions are only indicated for severe hemorrhage, because they're just going to get destroyed by all these antibodies, and they're not going to be really effective.
ELIZABETH HECHENBLEIKNER: So again, only for people that have life-threatening bleeding. In terms of chronic ITP and thinking about treatment strategies, you really need to go with the least toxic treatment, at the lowest dose. That's really the thought-process. And when it comes to emergency treatment of this disease process, just know that it's not going to be a single agent that you're using. It typically requires combination therapy.
ELIZABETH HECHENBLEIKNER: So glucocorticoids, as you can see here on the top right side of the table for ITP treatments, on the right side of the screen, those are your first-line. And the dose is typically a 1 milligram per kilogram, per day dose. IV immunoglobulin is not first-line but it's a second-line agent that's frequently used when you're going to the operating room, and it can also be used in acute bleeding, and also in pregnancy.
ELIZABETH HECHENBLEIKNER: The reason why IVIg works is because it decreases the rate at which macrophages are consuming these antibody type platelets. And so as you can imagine, when you're taking somebody to the OP for a splenectomy, it's an ITP patient, they already have low platelets, and by definition they've failed responses to other treatments. And so the great thing about IVIg, despite it being incredibly costly, it rapidly increases your platelet account and that can help get you hopefully out of needing large amounts of transfusions when you're in the OR.
ELIZABETH HECHENBLEIKNER: Going on to additional second-line agents, RhoGAM is one that can be used in Rh positive people who also have a functional spleen. And then for refractory ITP, Decadron, methylpred, Rituximab, and then TPO receptor agonists can also be used. The thromboprotein receptor agonist works by stimulating the platelet production in the bone marrow. The other treatment strategies that I've talked about, the glucocorticcoids, the IVIg, they work by actually curtailing platelet destruction.
ELIZABETH HECHENBLEIKNER: So the only one that actually stimulates platelet production is going to be your TPO receptor agonists. So moving on to the next slide, when you think about operative management for ITP, the basic things we need to know as surgeons, what are our indications? So typically, as someone who's in practice, a lot of these referrals are coming in to hematologists, and hematologist are going to help guide you about failures of medical treatments for these patients.
ELIZABETH HECHENBLEIKNER: So anyone who is refractory to steroid treatment, and usually this is people that have failed for six weeks of medical therapy, patients who have frequent relapses or having incomplete responses, despite actual medical therapy, people who have severe allergies or just toxic doses from these high-dose steroids, these are people that you really need to consider doing a splenectomy. In terms of preoperative preparation, as I previously discussed, in people who are bleeding and people that are high-risk, you're going to want to consider IVIg, going to the OR, imaging, if you have the luxury of having that, which most people should, mostly CT scans.
ELIZABETH HECHENBLEIKNER: You should think about looking at the size of your spleen, because again, it should be relatively normal, and you want to think about looking for accessory tissue or accessory spleen. And then vaccines too, and in the elective setting, they're given preoperatively. And I'll delve into the vaccines a little bit later on in the talk. In terms of operative considerations, really these are all MIS cases, the minimally invasive cases, that can be done laproscopicaly or robotically.
ELIZABETH HECHENBLEIKNER: Other key concepts, don't give platelets until your artery is clamped. Why do you guys think that you don't give platelets until the artery is clamped? Yes, exactly. So, Robert answered correctly. If you give platelets to somebody who has ITP before you clamp the splenic artery, they're going to be tagged with the antibodies, and the spleen is going to destroy them.
ELIZABETH HECHENBLEIKNER: So you can clamp your artery first, and then you can give the product. And then, Derek, I saw that you had a question. For accessories for ITP, yes, you always want to look for accessory spleen. And I'll explain why in the next slide.
ALBERT SHAHEEN: If you'd like to know, do you have to look for the accesory spleen pro-op, with imaging? How good is imaging for that?
ELIZABETH HECHENBLEIKNER: Honestly, it's not great. In most of the imaging studies, or most of the studies that actually talk about what you're imaging modalities are, and what the sensitivity and specificity is, fo finding accessories spleens is very poor. And it's usually for people who have failed splenectomy, but it's not very reliable. So I honestly think that CT scans, they're probably less than 50%, even lower numbers, for being able to help you identify accessory spleens.
ELIZABETH HECHENBLEIKNER: But there is also [INAUDIBLE] that you can use, so [INAUDIBLE] suspects [INAUDIBLE],, which is actually supposed to be a little bit better. I think the key thing is looking intra-operatively and systematically, especially, when you're doing cases where it applies. And for ITP cases, looking for an accessory spleen is definitely a key stuff.
ALBERT SHAHEEN: And Dr. Joshi asked a question about, can you explain exactly where and how you isolate the splenic artery? Are you going to discuss that later on with the [INAUDIBLE]??
ELIZABETH HECHENBLEIKNER: Yeah. I do discuss that a little bit later on, and I, not so much an open splenectromy, but more so, just some basic strategies for isolating the artery and vein in the latter part of the presentation.
ALBERT SHAHEEN: OK, thanks.
ELIZABETH HECHENBLEIKNER: So I'll address that then. And then, obviously, anybody else can feel free to chime in there if they have any other thoughts or considerations. So we'll move on to the next slide here. In terms of more long-term fall for ITP. When you look at some of the earlier systematic reviews that discuss long-term follow-up, the overall failure rate of splenectomy is just over 25% in five years, and in 2009 Systematic review.
ELIZABETH HECHENBLEIKNER: And then in 2004, there was another Systematic review that basically quoted relatively similar numbers that they had a 72% complete response rate with splenectom, and the relapse occurred in the median of 15% of patients with a follow-up of 33 months. And to Derek's question previously, causes of failure and the reason why we look for accessory spleens in these patients in cases is because it's one of the big causes of failure.
ELIZABETH HECHENBLEIKNER: So typically, accessory splenic tissue is either missed at the initial operation. It spilled at the initial operation, when you're trying to get the spleen out if you use a finger-fracture morselating or sort of in a piecemeal removal that could happen. And then the last reason why a failure will happen is compensatory hypertrophy of splenic rest.
ELIZABETH HECHENBLEIKNER: So let's move on to the next topic. Hereditary spherocytosis is another frequently tested on topic, on the ab site, and something that we encounter more in the pediatric surgery population. So hereditary spherocytosis is an autosomal dominant disease that affects the production of spectrin, and which is one of the red blood cells cytoskeletal proteins.
ELIZABETH HECHENBLEIKNER: And the loss of this protein basically causes the red blood cells, which have a nice deformable concave or biconcave shape to become these small, fragile sphere-shaped structures. And they have increased osmotic fragility, and they're basically more susceptible to destruction in the spleen. So as such, patients will present with anemia, jaundice sometimes, as well as splenomegaly.
ELIZABETH HECHENBLEIKNER: In terms of your work-up, I always want to look at the peripheral blood smear. Patients will have an increased reticulocyte count, as well as increased osmotic fragility, and they will have a negative Coombs test as well. A negative Coombs test is basically looking for antibodies, whether they're directly attached or free-floating that will destroy red blood cells, and that needs to be negative.
ELIZABETH HECHENBLEIKNER: In terms of preoperative preparation and treatment, of course, everyone gets supportive care. And we really only operate on people for severe cases, and we prefer, especially, in the little kiddos to delay this until age-5 due to the immune function and role of the spleen. In terms of preoperative imaging work-up, you do want to get a sense of the size of the spleen, because it's going to help tell you whether you can do cases laparoscopically versus open.
ELIZABETH HECHENBLEIKNER: So that's one important thing. And you also want to assess for gallstones. Do you guys know why we want to assess for gallstones in this patient population? That's correct. So you can get these bilirubin and do stones from all the breakdown of bilirubin, so that's great.
ELIZABETH HECHENBLEIKNER: So moving on to the next slide, another similar, somewhat similar condition that you may encounter, and more, again, in the pediatric surgery population is a pyruvate kinase deficiency. This is in contrast to hereditary spherocytosis and autosomal recessive disease that basically causes RBC to have an abnormal spiculated shape, and therefore, they cannot deform very well, and can get stuck in the microvasculature, and they get destroyed in the spleen.
ELIZABETH HECHENBLEIKNER: So it has a very similar presentation in terms of anemia and splenomegaly in jaundice as HS, but really the only difference in work-up is that you're going to see a normal osmotic fragility whereas in hereditary sphereocytosis, it will be increased. Preoperative preparation treatment, very similar to the HS population in terms of needing to look both for the size of the spleen, as well as assess for gallstones.
ELIZABETH HECHENBLEIKNER: And again, surgery is really going to be reserved for severe cases. So moving on to sickle cell anemia, this is something that we see quite commonly in patients. You might see it more in the pediatric surgery or repeat population, but this is another autosomal recessive disease that we all know has a single amino acid substitution, that basically causes the hemoglobin molecules to get stuck in this sickle shape, particularly where there's low oxygen levels.
ELIZABETH HECHENBLEIKNER: And it can lead to anemia and splenomegally, vaso-occlusive pain crises and other problems. Here, the only key difference is work-up is really adding a hemoglobin or electrophoresis, and there, of course, is a lot more supportive care in this patient population with other treatment strategies, including hydroxyurea, fluids, opioids, NSAIDs, et cetera. And really, in terms of when you want to operate on these patients, it's only for a few indications.
ELIZABETH HECHENBLEIKNER: It's either a massive spleen that's affecting somebody's quality of life, and children, gross delay is an important thing to think about. Kids that can't really eat or drink, because they're so uncomfortable from this disease process or having frequent bouts of bad splenomegaly or pain crises. And then acute sequestration-- that's the only sort of different thing with sickle cell disease compared to some of these other benign hematologic conditions is that acute splenic sequestration is a potential crisis that can occur where sickle cells essentially gets stuck within the red pulp of the spleen, and they obstruct large draining veins.
ELIZABETH HECHENBLEIKNER: And this propagates very quickly causing a very quick drop in hemoglobin, rapidly emerging spleen, and also even cardiovascular collapse. So in these patients, you have to resuscitate and hydrate them and transfuse them, and it's usually followed by splenectomy. So moving on, for malignant diseases, I'll just sort of briefly touch on this, because this is not something that we really commonly have to intervene in for splenectomy and patients with malignant disease.
ELIZABETH HECHENBLEIKNER: So typically, it's sort of your lymphomas and leukemias, and I would say most commonly non-Hodgkin's lymphoma. So on a historical note, one of the standard staging treatments for Hodgkin's was a splenectomy, which was used for staging, and ultimately guiding treatment strategy but that's not the case anymore. For non-Hodgkin's lymphoma, some of the leukemia is listed here. It's really for massive a splenomegaly hypersplenism or in some rare cases of NHL isolated splenic disease, and then with your other primary tumors and metastatic tumors.
ELIZABETH HECHENBLEIKNER: Vascular tumors are really the most common etiology that you'll see in the spleen, things like lymphangiomas, angiosarcs. And those are really doing splenectomy for diagnosis treatment of palliation. And the same thing for, if you think about metastatic tumors, splenectomy is really only for a symptomatic palliation for metastatic disease to this area. And some of the cancers that commonly metastasized to the spleen are breast, lung, and melanoma.
ELIZABETH HECHENBLEIKNER: So jumping on to the next slide, this is a little break here. If anybody has any questions, feel free to chime in.
ALBERT SHAHEEN: Any questions about any of these diseases before we move into the operative section? OK.
ELIZABETH HECHENBLEIKNER: All right, let's move to the next slide. I'm going to, since cysts, they're a relatively rare thing, I think that we can basically jump over this section, and then moved to the operative treatment, because I think this is important, and I want to get to this. So again, operative treatment-- the same thing as the different pathologies. This is really opposite ends of the spectrum, emergent disease, processes are elective ones.
ELIZABETH HECHENBLEIKNER: For our trauma patients, these are all of some cases typically, midline laparatomies. And then for these other elective cases, it's really all about the various MIS approaches and strategies that we have now. So it's either a straight laparoscopic case, straight robotic case, or even lab hand-assisted cases, depending on what you're dealing with. So theme of preoperative preparation-- moving onto the next slide, especially, in the elective setting, you do want to look at your imaging, and you want to make sure you get imaging, especially for cases where you're dealing with splenomegaly.
ELIZABETH HECHENBLEIKNER: Because the spleen, once it gets about 19 or 20 centimeters, it's very hard to take out laparoscopically, not only technically, because of the-- just sort of the dangers and the difficulties and dealing with the splenic hilum in these cases, but also, you need to be able to fit it in bags laparoscopically so that when you take the spleen out, you're not spilling splenic tissue everywhere. So we have the luxury, look at your imaging.
ELIZABETH HECHENBLEIKNER: Thinking about vaccinations, the reason why we--
ALBERT SHAHEEN: Can I ask a quetion here, [INAUDIBLE]??
ELIZABETH HECHENBLEIKNER: Sure. Yep.
ALBERT SHAHEEN: So what's the largest bag that we have laparoscopically, guys? What's the diameter of the largest bag? 15, right. Well, that's Dr. Joshi. I know you know that. OK. Yeah, 15 or is it?
ALBERT SHAHEEN: The Endo Catch [? too, ?] I think, it's 20 centimeters, but I'm not 100% sure, but that size range, 15 to 20 is tough. After that, it gets tough, yeah.
ELIZABETH HECHENBLEIKNER: Additional pearls of preoperative preparation, vaccines are really important. And I'll jump into those and tell you guys exactly what you need to give, but we have to give these vaccines, because they help protect against infection from encapsulated organisms. Again, for elective cases, it's going to be standard, just two weeks before. In some pathologies and cases, especially when you're dealing with a really big spleen, surgeons will do preoperative splenic artery emobolization.
ELIZABETH HECHENBLEIKNER: So that's an important adjunct to think about. You want to think about having blood products available. Anyone that's on for prolonged steroids, like your ITP cases, you also practically need to be thinking about things like stress-dose steroids intra-operatively, and then of course, your standard prophylactic IV antibiotics. Moving on to the next slide, just for delving in a little bit more detail, for vaccines, this is an important topic that everyone needs to know practically, but certainly, for test-taking as well.
ELIZABETH HECHENBLEIKNER: Elective cases, preoperatively, it's supposed to be greater than or equal to 2 weeks before. And then for emergent urgent cases, there is some debate in the literature about the timing of when you should be giving vaccines, but of course, you have to be given post-operatively. If you're going to be dealing with an unreliable trauma patient, it's probably better just to give them vaccines before they leave the hospital.
ELIZABETH HECHENBLEIKNER: But in other circumstances, two to four weeks, post-operatively, has been reported as acceptable. So for the vaccine types that we give, you guys are probably all familiar with them, but just to reiterate, in adults, it's usually the pneumococcal vaccine, PPV23, which protects against strep pneumonia. It's the H influenzae type b conjugate vaccine, which protects against HIV, and then the meningococcal polysaccharide, which of course, protects against Neisseria meningitidis.
ELIZABETH HECHENBLEIKNER: So moving on to our next slide, this is just a nice flowchart here, that discusses in terms of peri-operative preparation how you want to think about vaccines for your elective and emergency cases. Again, reiterating what I said before, at least two weeks preoperatively for elective cases. And then it just provides some subtle nuances between the vaccines that you want to give for children under the age of two, as well as other patients.
ELIZABETH HECHENBLEIKNER: And then in terms of the emergency setting, it's OK immediately post-operatively to give your pneumococcal vaccine. Again, for children under two, it's going to be the pneumococcal 7-valent vaccine and then, for everyone else it's the 23-valent vaccine. And then in this particular flowchart here, they're recommending at least two weeks post-operatively giving the Haemophilus influenza and then the meningococcal vaccine.
ELIZABETH HECHENBLEIKNER: So if you can follow this in your practice, , great but more importantly, you just need to make sure that they get all these vaccines before or after. So moving on to the next slide, quickly, I'll touch on open splenectomy. This is an interesting article that I found for patients undergoing open splenectomy. Apparently, there are four different incisions that these authors described.
ELIZABETH HECHENBLEIKNER: I am most familiar with the midline life laparatomy incision. Again, this is really going to be your trauma patients. People are in the supine position, but apparently, you can also use [INAUDIBLE] incisions. This is more for cancer cases. And then there are some upper abdominal transverse incisions, as well as this long oblique incision for giant [INAUDIBLE] here, that can also be used.
ELIZABETH HECHENBLEIKNER: I am most familiar with midline laparatomies. Moving on, to get into more the MIS splenectomy, for this position is obviously very different. It's a right or semi-right lateral decubitus position. The MIS splenectomy is now really the preferred method for taking the spleen out. Mostly, it's been described laparoscopically, but you can also do it robotically, in a hybrid hand-assist approach.
ELIZABETH HECHENBLEIKNER: This technique was first described in 1991, and it certainly has evolved a lot since then. Some of the advantages are decreased pain, more rapid recovery. People get out of the hospital faster and disadvantages are, of course, longer operating times, and it's sometimes really difficult to remove these large organs.
ELIZABETH HECHENBLEIKNER: In terms of just basic setup for this laparoscopic case here, in this picture featured from the Sabiston textbook, as you can see, this person is on an OR bed that's flexed, such that there is appropriate distance between the iliac crest, as well as the costal margin. It's important when people are in this decubitus position that they are usually on a beanbag or some type of a kidney rest to help maximize exposure and stability.
ELIZABETH HECHENBLEIKNER: It's important that you're taping people very safely, and making sure that the legs and the arms and all the pressure points are padded well. So lots of taping, and be careful, don't take shortcuts here. As you can see here, there's a four-fork technique that's featured in this picture. You usually have your liver retractor in ports 1 or 4, and you can have the camera either in 2 or 3, and then you can sort of hop around the ports.
ELIZABETH HECHENBLEIKNER: And the surgeon can work through 1 and 3, or through 2 and 4, depending on what side of the spleen they're working on and what type of exposure you need. So typically, you can do this with the four-port technique. And then when you think about your incisions, most of these can be done with 5 millimeters, but ultimately, you are going to have to up-size a port at the end to get your specimen out. So you just have to think about using one of the sites that you've already created, or making another incision to get the specimen out through the back.
ELIZABETH HECHENBLEIKNER: I'm moving on to the next slide. Again, this is just a sort of reviewing what we discussed earlier-- sites for accessory spleen. And again, this is very important for your ITP and HS cases. The most common sites that we typically see are the gastrosplenic ligament for accessory tissue, splenic hilum, the tail of your pancreas, your splenocolic ligament, your transverse mesocolon, and your greater omentum.
ELIZABETH HECHENBLEIKNER: So we can, I think, go ahead and skip to the next slide, just because I want to get to some of the-- An overview of the important operative steps for the MIS splenectomy. So this is just sort of a review of nine key steps for this procedure. The first step is usually mobilizing your splenic flexure. You're going to get it down off its lateral attachments and sort of medialize it as much as possible.
ELIZABETH HECHENBLEIKNER: You're going to divide the splenocolic ligament. Then you're going to divide some of the lateral peritoneal attachments and splenic attachments on the spleen, leaving a rim, so that you can move the spleen around. You're going to enter the lesser sac, divide your short gastric vessels. So remember, you'll be mobilizing the gastric splenic ligament when you're doing that. In terms of dissecting up at the splenic pedicle, which is your splenorenal area, you obviously need to take caution as you're working around the pancreas.
ELIZABETH HECHENBLEIKNER: You don't want to injure it. Basically, in terms of some of the basic techniques that I have reviewed for both the magistral and the distributive types, or in the magistral technique, since the main splenic artery and vein are dividing really close the spleen and hilum, you can typically take those vessels and block together with a vascular stapler. However, in a distributive technique since things are branching further away from the capsule, you typically need to completely divide out, and ligate and divide your vessels separately.
ELIZABETH HECHENBLEIKNER: And you also just have to be mindful when you're doing this that any other little isolated branches will need to be ligated and divided separately for this particular blood supply. Ultimately, then once you've mobilized all of your spleen and divided the hilum and the vascular structures, your spleen should be attached to the superior-- Superior pole should be attached to the diaphragm. And then when it's sort of hanging down like that, it's really ideal to get your bag in and then place your specimen in the bag, and then divide the spinal splenophrenic attachments so it's ready for you to remove it right away.
ELIZABETH HECHENBLEIKNER: And then the next step is to figure out where your extraction side is going to be, and then to remove the spleen. And a piecemeal fashion, of course, being, careful on those hematologic cases where you don't want to spill any splenic tissue. So moving onto the next slide, complications. So we'll go over this pretty quickly, so I can just talk about OPSI for a little bit.
ELIZABETH HECHENBLEIKNER: One thing that you can see in the acute-- [INAUDIBLE] is post-splenectomy thrombocytosis. This is particularly in patients who have milely proliferative disorders. This can result in thrombosis of mesenteric portal and renal veins. It can be severe and life-threatening because it can not only lead to bleeding but also can lead to bad complications from thromboembolism.
ELIZABETH HECHENBLEIKNER: More importantly, and what's commonly tested on, and what you need to practically speaking as well, and also for educating your patients is OPSI or overwhelming post-splenectomy sepsis. This is the most common fatal complication that happens after splenectomy in terms of late-timing. It's especially high-risk for this in the first two years after splenectomy. However, you can really get it at any time.
ELIZABETH HECHENBLEIKNER: The risk is higher for anyone undergoing splenectomy for malignant processes, hematologic conditions, especially children who are under the age of four. It's very important that you educate all your patients as well as their providers on taking the appropriate precautions, not only for recognizing symptoms early, but also for making sure that the kids and adults are getting appropriate vaccines, and just staying on top of what the symptoms are.
ELIZABETH HECHENBLEIKNER: Moving on to the next slide, and discussing OPSI in a little bit more detail, this overwhelming post-splenectomy sepsis is honestly something that I have never seen in splenical practice, but it's very scary from its description. It typically has a prodromal phase with fevers, rigors, and other non-specific symptoms. Patients can actually present with focal sites of infection, like pneumonia and meningitis, but they can also sometimes only present with bacteremia.
ELIZABETH HECHENBLEIKNER: But the hallmark of this disease process is a rapid splenical progression in hours to hypotension, DIC, respiratory failure, and coma. And the mortality here, as you can see, and some serious, it's very scary. It's up to 50% to 70%. So early recognition is important. Going to the next slide here, this is just-- I'm sorry.
ELIZABETH HECHENBLEIKNER: Here, I see that we have a little pause. We're almost finished with the topic, and we're going to jump to the questions.
ALBERT SHAHEEN: Actually, at this point, let me just ask-- Dr. Joshi, ask about morcellization as an extraction tool, would it be appropriate for non-oncological cases like ITP, for example?
ELIZABETH HECHENBLEIKNER: My understanding, [? morcellazation ?] is, it is OK as long as you're not-- if you want to, like, for example, minimize your [INAUDIBLE] site. But it's fine as long as you're not spilling any tissue.
ALBERT SHAHEEN: And not using the morcellator? [INAUDIBLE] ELIZABETH
HECHENBLEIKNER: Oh, no.
HECHENBLEIKNER: No, not an actual morcellator. It's really, I don't know anybody that does that in splenical practice, but more like either finger-fracture technique or moving it with reinforcers, but not an actual morcellator.
ALBERT SHAHEEN: OK. Thank you.
ELIZABETH HECHENBLEIKNER: So for the next two slides, you can skip over the next slide on vaccines, and then the next slide too. Let's just skip over that. It's just talking-- Has a brief primer on antibiotic prophylaxis. So why don't we just jump in to our questions as we are nearing completion of the hour? So for this first question, I'll go ahead and read it and then open it up for the chat for responses.
ELIZABETH HECHENBLEIKNER: So the first patient is a 20-year-old healthy female who presented with recurrent nosebleeds. A dark red rash and new onset bleeding of her guns. She takes her medications. Has normal vital signs and has labs that are remarkable for platelets of 46. The rest of her work-up is completely negative, as you can see here, on the question. And her physical exam just demonstrates no hepatosplenomegaly.
ELIZABETH HECHENBLEIKNER: So what is the best initial treatment strategy for this patient? Yes, I see a lot of responses. Everyone is saying A, and that is correct. So this is a patient who is symptomatic, and who has platelet levels that are less than 50.
ELIZABETH HECHENBLEIKNER: And based on what we discussed for ITP treatment, this man needs some type of medical intervention. And you guys are right. The first-line of therapy is going to be glucocorticoids, prednisone. And as you can see, the correct doses are listed here. So moving on to the next question, so we got a 10-year-old boy with hereditary spherocytosis, who is presenting with worsening fatigue and upper abdominal pain with eating.
ELIZABETH HECHENBLEIKNER: He had a viral upper respiratory illness three weeks ago, blood transfusions, three years ago for a similar episode of fatigue. Takes the medications and has all appropriate vaccines up to date. He's afebrile. His heart rate is a 110. His blood pressure is 90 over 60. He's a little bit low for this age range.
ELIZABETH HECHENBLEIKNER: So you know, he's pale with mild scleral icterus, and on exam, has a firm mildly tender mass in his left upper quadrant. His lab show hematocrit at 24%, platelets at 105. A total bili of 6.4 and a direct bili of 1.2. The abdominal ultrasound shows a very enlarged spleen, he's got multiple mobile gallstones with no evidence of cholecystitis. The extrahepatic billiary tree is otherwise normal. And after stabilization, what would you guys recommend as the next best step in management of this patient?
ELIZABETH HECHENBLEIKNER: So we go to the next slide, it'll show everyone the answers. All right, I see some answers for C, which are splenectomy with cholecystectomy. I see an answer for B, splenectomy alone. Seems like right now, two people are favoring splenectomy with cholecystectomy.
ELIZABETH HECHENBLEIKNER: So the answer C, is the correct one for this particular question. First and foremost, this child has significant splenomegally, and life-limiting symptoms, and the setting of hereditary spherocytosis. So that alone mandates splenectomy. However, his history of abdominal pain with eating is a bit concerning for biliary colic. And he has an ultrasound that's showing gallstones that are presumably pigmented gallstones.
ELIZABETH HECHENBLEIKNER: And so this patient should have a concurrent cholecystectomy. So for our final question, we've got a 56-year-old female, who presents to the ED. Status-- post a high speed motor vehicle collision. Her primary survey is intact upon presentation. Her secondary survey revealed left flank ecchymosis, as well as right lower extremity open fractures.
ELIZABETH HECHENBLEIKNER: She has a CAT scan of her abdomen, which is a grade-III splenic injury. She's hemodynamically stable and is going to the ER with ortho for her lower extremity fractures. What is the most appropriate management of splenic injury in this patient? So far, we've got a couple of E's.
ELIZABETH HECHENBLEIKNER: So you guys are right. And this is a grave splenic injury and a person who is presenting with hemodynamic stability, and no reports of any active extravasation. So non-operative management would be very appropriate in this patient. What this will typically entail after going to the OR for fracture management is going to the ICU bed-rest, serial hemoglobin levels, and staying NPO, to make sure that the patient stays stable.
ELIZABETH HECHENBLEIKNER:
ALBERT SHAHEEN: All right. Any final questions for Dr. Hechenbleikner?
ELIZABETH HECHENBLEIKNER: Thank you, guys, very much.
ALBERT SHAHEEN: Thank you so much. We really appreciate your time. This was lovely and very helpful. Thank you so much, and good night, everybody. We'll see you next week.
Segment:0 .
ALBERT SHAHEEN: OK, everyone. We're going to start in just a few seconds after it hits 8 o'clock. OK, it's 8:00. Good evening, everyone, and welcome to SCORE School. My name's Albert Shaheen. I'm the assistant editor of SCORE, and I would be your moderator tonight.
ALBERT SHAHEEN: Before we get started, a few housekeeping items, we encourage you to use the chat box to ask questions and make comments. Everyone has been muted to avoid unnecessary noise feedback. You will see the QR codes for attendance tracking displayed a few times throughout the presentation. You only need to submit it once, and you will receive an email confirming your attendance. All of these SCORE School sessions are being recorded for later viewing on the SCORE website.
ALBERT SHAHEEN: You should be aware that the names in the chat will be recorded as well. We encourage you to view these recorded sessions as your schedule allows. Later on, this month, we will be moving the time of these live broadcasts to the week preceding the corresponding twist week. So we'll make an announcement about that change. Please check the SCORE website and Twitter for those specific times and instructions.
ALBERT SHAHEEN: Blog and information, as of now, will remain the same. For tonight's SCORE school session, we'll be discussing three modules-- Hematological Diseases of the Spleen by Dr. Ralph Edwards and [? Schlecta, ?] Splenic Abscesses and Neoplasms by the same authors, and splenectory by Dr. Joshi and [? Boulos. ?] It is now my pleasure, my great pleasure to introduce our faculty presenter, Doctor Elizabeth Hechenbleikner, who is an Assistant Professor of Surgery at Emory University Dr.
ALBERT SHAHEEN: Hechenbleikner obtained her undergraduate degree at Wake Forrest, where she lettered all four years in Division 1 field hockey. After obtaining her medical degree at the University of North Carolina at Chapel Hill, she completed general surgery residency at Georgetown University Hospital, where I had the privilege to work with her and help train her.
ALBERT SHAHEEN: During her training, she spent three years as a research fellow at the Johns Hopkins Hospital during which time she conducted studies related to colon cancer as well as clinical outcomes research among colorectal surgery patients with a focus on surgical site infections. She was part of the Armstrong Institute resident scholar training program in patient safety and quality while she was there at Hopkins.
ALBERT SHAHEEN: After finishing her residency, she went on to complete a fellowship in advance laparoscopic and GI surgery with a focus on bariatric surgery at the Icon School of Medicine at Mount Sinai in Manhattan. Dr. Hechenbleikner is a board certified surgeon with multiple manuscripts and book chapters published. She serves on the publications and resident and fellow education committee of Sages and is an ad hoc reviewer for surgical endoscopy and surgery for obesity and related diseases.
ALBERT SHAHEEN: She is actively involved in quality improvement and research projects among bariatric surgery patients. Focusing on protocols for VTE prophylaxis, ERAS, and the use of pharmacists for assistance with in-patient discharge medication, reconciliation, in addition to mentoring Emory general surgery resident and fellows and research projects. Her research interests include investigations and patient outcomes, including reduction of surgical site infections and 30-day re-admissions, as well as patient safety and quality initiatives in laparoscopic GI surgery.
ALBERT SHAHEEN: She's married to a wonderful husband, Dan, who is her coauthor on multiple of her papers. And she's a statistics wizard, and they have two adorable daughters. Lizzie loves her family, Peloton, field hockey, and soccer. So thank you so much, Dr. Hechenbleikner, for lending your expertise to us tonight. Take it away.
ELIZABETH HECHENBLEIKNER: Can everybody hear me, first, before I start? Yup, we can hear you.
ELIZABETH HECHENBLEIKNER: Great. Thank you very much for the introduction. I am very excited to be participating in the SCORE School this evening. I have long been a supporter of SCORE at the Twiss curriculum, and certainly, this newer initiative. I am particularly honored to have been introduced by Dr. Shaheen, who as he indicated with my former program director at Georgetown, and who is one of my favorite surgical educators of all time.
ELIZABETH HECHENBLEIKNER: So you can advance the next slide. I have no disclosures. And on the next slide, it just shows off some of my references. So we can skip to the next slide, for, my outline. As Dr. Shaheen indicated, I have the core modules listed here, as well as the advanced module for the Twiss curriculum sequence covering spleen.
ELIZABETH HECHENBLEIKNER: I'm going to start by giving you all an overview of splenic anatomy, histology and function. And then I will shift the discussion to a more problem-based format where, I will go over diagnosis, preoperative management, non-operative management, and some operative treatment, based on some the different splenic pathologies that we're going to encounter during this talk and also as surgeons.
ELIZABETH HECHENBLEIKNER: And toward that end, I will try to shift back to going over more of the technical aspects of splenectomy with a focus on minimally invasive surgery, because that's one of my favorite things to do. And then we will end up discussing some of the complications and issues, long-term follow-up and then some questions. We'll go to the next slide.
ELIZABETH HECHENBLEIKNER: So operative anatomy is very important for the spleen. I'm going to start with a broad overview of some of the key concepts that you see listed here. You can go ahead and advance to the next slide. So the spleen, as most of everyone knows here, is an intra-peritoneal structure. It's located in the left upper quadrant under the diaphragm, and tucked up on the ribs 9 to 11.
ELIZABETH HECHENBLEIKNER: I don't know if you guys can see this picture any well at all, but for anybody that is attending, does anybody know what the dots in this picture refer to? Feel free to answer in the chat or to shout out the answer.
ALBERT SHAHEEN: They can't shout it out, they're muted but the chat is perfect. Come on, guys. Let's hear it.
ELIZABETH HECHENBLEIKNER: Derek. So Derek is correct. Derek answered accessory spleen location, so nice job. So, yes. These are the potential locations for accessory spleens, which you will find in about 10% of patients. And you can pluck that in the back of your mind for a second, because we're going to talk about that in the latter part of the presentation.
ELIZABETH HECHENBLEIKNER: In terms of some basic dimensions, an average spleen weighs about 530 grams, which is roughly on the 300 grams side, 7 to 8 ounces. It's roughly 12 centimeters long, 7 centimeters wide, and 3 centimeters thick. Obviously, the size can vary dramatically, depending on what underlying splenic pathology you're dealing with. You can go to the next slide.
ELIZABETH HECHENBLEIKNER: So surrounding structures-- the spleen has visceral relationships with many organs and structures. More and purely, this includes the greater curve of the stomach and the splenic structure of the colon, as you can see, on the right side of the screen, here this picture shows those structures removed-- more posteriorly, the associated retroperitoneal structures include the left kidney, as well as the pancreas. The tail of the pancreas does affect the hilum of the spleen pretty closely in most patients.
ELIZABETH HECHENBLEIKNER: The superior surface is roofed by the diaphragm, which separates the [INAUDIBLE] from thoral cavity. The ligaments that suspend the spleen are really reflections of the peritoneum, and they essentially mirror, and case the structures that are in close contact with the spleen. And those include the gastrosplenic, splenocolic, splenorenal, and splenophrenic ligaments. The main blood vessels enter and exit the spleen via the hilum, which is also shown here through the picture that's on the right side of the screen.
ELIZABETH HECHENBLEIKNER: The splendic artery is one of the main branches of the celiac trunk. It is a tortuous vessel that travels behind the pancreas, giving off multiple branches as it heads toward the hilum. The main venous drainage is through the splenic vein, which is not a tortuous vessel, also travels behind the pancreas, and typically joins the SMB along with the IMB, somewhere behind the pancreas to converge into the portal vein, heading back to the liver.
ELIZABETH HECHENBLEIKNER: Let's head to the next slide. So in terms of the ligaments, the important part of operative anatomy here, you need to know these ligaments because this is where you're going to encounter blood vessels. The gastrosplenic ligament carries the short gastrics vessels, and its superior portion and in the left gastroepiploic and it's inferior portion.
ELIZABETH HECHENBLEIKNER: The splinorenal ligament houses the splenic artery and vein, as well as the tail of the pancreas. And then the splenocolic and splenophrenic ligaments finally are relatively avascular structures. However, you may encounter some large collateral vessels if you're dealing with patients of portal hypertension. For our next slide, I am going to delve into some more detailed aspects of the blood supply.
ELIZABETH HECHENBLEIKNER: So regarding the main afferent blood supply to the spleen, there are two commonly described variants or types that are found in most surgical textbooks. So the magistral type of blood supply refers to when the splenic artery divides terminally, very close to the spleen. So this is going to be about 1 to 2 centimeters from the hilum, and this occurs in about 30% of individuals. When a division of this splenic artery however occurs earlier, on the pathway toward the hilum this is usually greater than 2 centimeters away from this area, away from the hilum and then the pre-pancreatic segment.
ELIZABETH HECHENBLEIKNER: This is called a distributive blood supply and this is the more common variant that you're going to see in about 70% of individuals. It's important as surgeons to recognize that there are a lot of variations and branching patterns of this splenic artery, and it can give rise to branches like the left gastric, middle colic, and the left hepatic. So you want to be prepared for this when you are dissecting in the hilum in this area and other structures that are close by to make sure that you're avoiding injury to these vessels and other surrounding structures.
ELIZABETH HECHENBLEIKNER: For our next slide, I'm going to jump in to some of the histology. So as most of you know, spleen is covered by a fibroelastic capsule. And this capsule further compartmentalizes the spleen via the trabeculae. As the spleen splenic artery comes in through the hilum, and starts to head toward the trabeculae, it branches into these terminal branches and continues dividing to create these central arterials that go in between the trabeculae.
ELIZABETH HECHENBLEIKNER: These arterioles continue to branch into even smaller vessels and then the adventisha ultimately gets replaced by a covering of lymphatic tissue that's known as the periarteriolar lymphoid sheaths or PALS. And this continues until the vessels thin to capillaries. Another layer of lymphoid tissue that's surrounding the PALS is an area that's called the marginal zone. And interspersed in the marginal zone is even more lymphoid tissue, called primary follicles.
ELIZABETH HECHENBLEIKNER: The white pulp, which you'll commonly read about and histology textbooks about the spleen, makes up about 25% of the tissue. It does comprise both the PALS, which is a T cell and macrophage-rich area, as well as the marginal zones, which have a lot of macrophages and then the interspersed follicles which are B cells. Those are the three components of the white pulp. So it's important-- the reason why we always talk about the spleen is this large lymph node in the left upper quarter is because of this white pulp and all of the lymphoid tissue.
ELIZABETH HECHENBLEIKNER: It's very important for fighting infection and producing antibodies, which are important for both viral and bacterial infections. So the white pulp also interfaces with the red pulp. And you can see an arrow pointing to the interface here, between the red and the white pulp. In this particular area, interface is near the marginal zone.
ELIZABETH HECHENBLEIKNER: In the marginal zone, the arterioles and the capillaries ultimately lose their lymphatic tissue, and they evolve into these very thin walls splenic sinuses and sinusoids. These sinusoids ultimately merge in the venules, which then drain and to collecting then to travel along the trabeculae to form the splenic veins that mirror their arterial counterparts. And then they finally drain as the main splenic vein.
ELIZABETH HECHENBLEIKNER: Between these end vessels and all these splenic sinuses and sinusoids, there is additional tissue called the cords or the cords of Billroth. and the cords of Billroth are really in the red area that's featured in the picture on the right side of the screen where the red pulp is. Together the red pulp is comprised of both the venous sinuses and sinusoids as well these cords of Billroth.
ELIZABETH HECHENBLEIKNER: And that makes up about 75% of your splenic tissue. So again, a very mildly rich lymphoid structure but very rich in blood supply. So let's move to the next slide, where we'll discuss some function. So the spleen has the main role for hematopoietic function for the fetus until about five months in-utero, where the bone marrow takes over. In adulthood, the spleen can resume some of this hematopoietic function and certain pathologic conditions, particularly those that cause hypersplenism and destruction of cells.
ELIZABETH HECHENBLEIKNER: We talked about the spleen is this the big lymph node and filtering system. It's important sort of delve into this a little bit more, because the spleen has an important role in removing microorganisms and literally cleaning our blood. There's a couple of different theories of how blood flows through the spleen and around the arterials to end up creating a filtration process. And I think one of the more interesting theories is the theory of an open system.
ELIZABETH HECHENBLEIKNER: In the open system, the way that this is described as essentially blood is being fed into the spleen through these tiny little arterials and capillaries, and then it's trickling through this sub-like parenchyma of lymphoid tissue that's rich and reticuloendothelial cells. And as such, it then flows through all of this and then ultimately empties into this splenic sinuses and a portion of venous system.
ELIZABETH HECHENBLEIKNER: And as it's flowing through this open system with the [INAUDIBLE],, the thought is that these reticuloendothelial cells assist with the cellular cleansing processes. That involves removing pathogens like parasites but also sequestering RBC for maturation and serving as a reservoir for platelets as well. Importantly, as plasma is directed toward the lymphoid tissue, soluble antigens are exposed to this area, and it stimulates the production of antibodies.
ELIZABETH HECHENBLEIKNER: The spleen in a similar fashion also makes opsonins, which classically, the two are prepared in intestine, which you guys will frequently be tested on in the ab side. Prepared and actually initiates one of the pathways of complement activation, which helps destroy bacteria and otherwise abnormal cells and [INAUDIBLE] enhances basically the phagocytic activity as phagocyles and other leukocytes.
ELIZABETH HECHENBLEIKNER: So it's important to realize that when you lose the spleen, and you lose these opsonins and other splenic functions that you're going to be particularly at risk for infection. And that's particularly encapsulated bacteria's, which we will talk about a little bit later on, in this presentation. Another important aspect of splenic function is maintaining RBC morphology and function.
ELIZABETH HECHENBLEIKNER: As we all know, RBCs basically have a lifespan, and that's roughly 120 days. After this lifespan goes away and RBCs get old, they have decreased plasticity and they don't work as well. So it's important that the spleen is there to remove them. In addition, RBCs are not always perfect. A lot of times, they will accumulate nucleoli, Howell-Jolly bodies, Heinz bodies, et cetera, and the spleen actually needs to remove these and the reason being is that if it doesn't, then you won't have normal RBC function.
ELIZABETH HECHENBLEIKNER: So under normal circumstances, these are all the functions that the spleen is actually doing, and they're very important. And as you see, we'll talk about pathologic states. The spleen is sort of doing similar things, trying to clean the system of pathologic conditions, but it ends up causing problems. So let's move the next slide. So over here, this is just a kind of an overview table to show you guys the different biologic substances that are removed by the spleen.
ELIZABETH HECHENBLEIKNER: So the top part of the table actually describes the non-pathologic states or normal conditions and what the spleen does. And the lower half of the slide describes what the spleen does, and what it's removing under pathologic or disease started. And we will get into some of the specific disease states that are listed here on the slide in the upcoming slides. Let's move to the next slide.
ELIZABETH HECHENBLEIKNER: So in terms of thinking about the spleen and its presentation to surgeons, on a high level, we can really break it down into emergent, very emergent, and the trauma setting, of course, and usually, very elective conditions. So these are kind of on opposite ends of the spectrum, but these are very different circumstances and situations, where we are going to be forced with potentially needing to do a splenectomy.
ELIZABETH HECHENBLEIKNER: On the left side of the screen, the emergent setting really entails our trauma patients, and we're rarely and usually not emergent, sometimes, splenic absences, which are a very rare condition that I'll briefly touch on. And the elective setting, there are a host of pathologic conditions that may require splenectomy, which includes everything from hematologic conditions like ITP to primary tumors and even cysts. So in the next slide, we will delve into what happens and the trauma setting.
ELIZABETH HECHENBLEIKNER: The thing about diagnosis in the emergent setting, obviously very different from what you're doing electively. Whenever you're thinking about trauma patients, it's always about your ABCDEs, primary survey and mechanism of injury, especially when you are trying to think about splenic trauma. The spleen is certainly on the top of the list of organs that are going to be most injured in blunt trauma.
ELIZABETH HECHENBLEIKNER: So if you're worried about a splenic injury in this setting, it's important to factor in very quickly your patients hemodynamics, physical exam findings, and then your diagnostic add-ons like FAST and CT. And these are usually, going to help you quickly determine whether you're going to the OR or not. Obviously for any unstable patient with a positive FAST, you're going to be going to the operating room, but for those who are stable and have a concerning mechanism of injury for abnormal trauma, we usually evaluate all these patients with the CT scan.
ELIZABETH HECHENBLEIKNER: So when evaluating CTs, and specifically looking at the spleen, you were looking for one key finding, which is a blush of IV contrast or active extrapolation indicating that there's an active injury. You also want to assess the grade of injury, and you also want to think about volume of hemoperitoneum, other injuries, et cetera. Active extravasation should prompt either embolization by IR or going to the operating room.
ELIZABETH HECHENBLEIKNER: In terms of the grade of injury, you can see here on the right side of the screen that there is a high-level presentation of the splenic injury scale that was developed by the AAST. There are five grades of injury, going from grade 1, which is the most simple injury where you can have either a subcapsular hematoma that's less than 10% of surface area of spleen, or a laceration that's less than 1 centimeter deep, all the way to a grade 5 injury, which is essentially completely devascularized and shattered spleen.
ELIZABETH HECHENBLEIKNER: It's important when you are working up these patients in evaluating CT scan you know that they are notorious for underestimating the injury grade, so this has to be guided by your splenical judgment, and of course, how the patient is doing. So moving on to our next slide, you don't have a lot of time for preoperative management when it comes to your trauma patients. You're going to be quickly stabilizing patients, placing large bore IVs, giving blood products, hemostatic adjuncts, considering the massive transfusion protocol-- MTP, and getting people and patients to the operating room as fast as possible.
ELIZABETH HECHENBLEIKNER: These patients are going to be placed quickly, supine, on the OR table. You're going to be warming them at table and performing an exploratory laparatomy, via midline LAP, and trying to get the spleen out in a few minutes, which can be accomplished. From there, your post-op care is obviously going to be in the ICU. And we will talk about vaccines little bit more through the latter part of the presentation, but just know that in this particular setting, vaccines are going to happen hopefully prior to discharge of this patient population.
ELIZABETH HECHENBLEIKNER: So moving on to the next slide here, this focus is on non-operative management, because this is used very commonly for splenic injuries, particularly in blunt abdominal trauma. Over 90% of splenic injuries, certainly in the pediatric population, are going to be successfully managed, non-operatively. Dr. Shaheen might be able to speak more to these numbers, given that he is a pediatric surgeon.
ELIZABETH HECHENBLEIKNER: Certainly, in, adults the numbers are substantially lower and closer to 67%, using these pathways. The decision to employ nonoperative management is not necessarily predicated by the grade of injury, but more how the patient is doing clinically. So it does require that patients meet certain criteria. First and foremost, that they are hemodynamically stable and the absence of other suspected injuries.
ELIZABETH HECHENBLEIKNER: The remaining factors in the decision-tree here really rely on sound splenical judgment and using good surgical common sense. Anyone that's having significant drops in hemoglobin, increasing transfusion requirements, or obviously anyone that becomes hemodynamically unstable, you'd do there an IR and then operative intervention. And again, just keep in mind that this is not necessarily mandated by the grade of injury.
ELIZABETH HECHENBLEIKNER: Featured here, you see a relatively complicated nonoperative pathway that is used at a particular institution, and it can help guide you in terms of how to think about an hour non-operative management. So moving on, we are going to-- this here is a quick slide that just shows you how you can log your attendance. However, moving on to our next slide, we're going to get into some more--
ALBERT SHAHEEN: Maybe, can I stop you for a second?
ELIZABETH HECHENBLEIKNER: Yeah. Absolutely.
ALBERT SHAHEEN: Feel free to ask questions. This is a good spot for you to ask questions and if I could just say one thing one little thing about the non-operative management, it started with pediatrics. We started doing it in Toronto in the '70s and '80s and then it expanded to adults. And your point about the blush on the CT scan, we always have a debate in the pediatric world about whether that would necessitate action like embolization or operations.
ALBERT SHAHEEN: But I know that in adults, it's a little different. Can you speak to that that If you do see a blush, you do have to act, right, in adults?
ELIZABETH HECHENBLEIKNER: Yeah. I mean, I am not-- obviously not in the trauma world much anymore, but yes, in my experience, when there is active extravasation but a stable patient, that there's a minimum, a trip to interventional radiology.
ALBERT SHAHEEN: Yes. Thanks.
ELIZABETH HECHENBLEIKNER: But other people, obviously, feel free to chime in, if anybody else has a different institutional experience that they would like to share with the group.
ALBERT SHAHEEN: You've got a client tonight. Come on. [INAUDIBLE]
ELIZABETH HECHENBLEIKNER: Well, moving on to the next topic to hit on, which is one that's incredibly rare. So kind of the opposite end of splenic trauma, which is going to be something very common that everybody in this chat is going to see. Splenic abscesses here-- this is one of the other conditions that we may encounter as surgeons where we do have to intervene, but more in the urgent setting. Splenic abscess is a very rare clinical entity that's got a very non-specific presentation.
ELIZABETH HECHENBLEIKNER: Vague abdominal pain, fevers, and even sporadic chest pain. It's rare enough to where it's been reported largely an autopsy series, with only an incidence about 0.1% to 0.7%. Usually, this condition is secondary to hemategenous spread from infective foci elsewhere in the body. It's commonly found in patients with underlying immunocompromised state. The spleen can be seated in the setting of bacteremia, UTI, pneumonia, you name it.
ELIZABETH HECHENBLEIKNER: And they can even have rare conditions like tuberculosis. The ideology here is not always clear. However, when it comes to the bugs that typically are the causative agents and found in splenic abscesses is the gram-positive cocci, so commonly staph, strep, or enterococci, and then also gram-negative enterics. In terms of diagnosing this condition, CTs are really the preferred methodology.
ELIZABETH HECHENBLEIKNER: However, the diagnosis can be made with ultrasound. And splenomegaly as expected, not really common in this condition. So in terms of how to manage these conditions, we're going to the next slide, first and foremost, just in terms of reviewing what to look for on cross-sectional imaging, these typically appear as hypodense lesions, sometimes with septations, sometimes with peripheral enhancement.
ELIZABETH HECHENBLEIKNER: In terms of management, splenectomy previously was the gold standard for this type of condition. However, in patients whose co-morbidities really preclude this or people who otherwise have favorable abscesses, CT guided percutaneous drainage and a combination with appropriate antibiotics is really where a lot of treatment strategies are moving. And I personally feel like this non-operative management is a very reasonable approach to go with, with any patient out of the gate.
ELIZABETH HECHENBLEIKNER: So as you can see here, on the left side, this is what's called a unilocular abscess. It's very well-defined, as you can see, there are no septations, and there's no necrotic debris. So these are people that are more likely going to have successful outcomes from percutaneous drainage. Success rates can be up to 75% to 90%, and some are for a series and any failure here would require a splenectomy.
ELIZABETH HECHENBLEIKNER: And featured here on the right side of the slide, you guys can see that this is a more ill-defined splenic abscess with septations and a little on the cystic looking areas. And really, this is not going to do well for drainage, so you're talking about a splenectomy in this setting. laparoscopic splenectomy has been described for this condition, and it can be an option in the right splenical circumstances.
ELIZABETH HECHENBLEIKNER: And otherwise, you would be doing these cases open. So moving on to the next slide, we're going to now shift gears and start talking about more the common elective clinical situations and pathologic states that commonly require splenectomy. So we'll review some of the hematologic conditions, both benign and malignant. We'll talk to them about the non-hematologic tumors that can affect the spleen, and then cysts and pseudocysts.
ELIZABETH HECHENBLEIKNER: There is a very bizarre diagnosis of something called wandering spleen, which is actually mentioned in Sabiston textbook chapter associated with this Twiss series and modules, but it's not something that I've ever encountered, and just going to skip it for this particular talk. So moving on to the next slide, we're going to jump in to one of the really big topics that you guys are going to hear about and potentially even encounter on tests, and that's ITP.
ELIZABETH HECHENBLEIKNER: So that the immune thrombocytopenic purpura. so this is a very complex autoimmune condition, where you typically find isolated platelet lab values that are under 100. It's characterized predominantly by the binding of anti-platelets auto-antibodies to platelets followed by their phagocytosis by the reticuloendothelial cells that are mostly in the spleen. The spleen also serves as a site of reproduction of these antibodies and they're mainly IgG antibodies.
ELIZABETH HECHENBLEIKNER: There's also apparently some abnormal T cell activity toward platelets as well as some impaired enzyme production that impedes megakaryocyte functions that are also involved in this disease process. When you're talking about ITP, it's important to realize you've got to rule out secondary causes. There are a lot of things that we all know clinically that cause thrombocytopenia. So nobody know any common causes of thrombocytopenia in the clinical world?
ELIZABETH HECHENBLEIKNER:
ALBERT SHAHEEN: We have HIT Austin says, H-I-T.
ELIZABETH HECHENBLEIKNER: Yes, Austin. HIT, very good. That's one of the key drug-induced ones, probably the most common drug-induced one, I would say, that we'll see in our surgical patients. So other things that can cause thrombocytopenia are pregnancy, viral infections, cirrhosis. So those are other issues are other things to think about. So when you think about secondary ITP, it's triggered by inherited or acquired predisposing diseases like chronic infections.
ELIZABETH HECHENBLEIKNER: And some of the key ones are HIV, autoimmune diseases like SLE, so like lupus and then rheumatoid arthritis. It can also be caused by Helicobacter pylori infection, which I did not realize until rereading the [INAUDIBLE] chapter. It can also, in infectious cases, what's really thought to be going on is that the viral and bacterial antigens are sort of recognized as platelet antigens, and then they give rise to anti-platelet auto-bodies.
ELIZABETH HECHENBLEIKNER: So it's this term called molecular mimicry, where the body is sort of confusing viral antigens or bacterial antigens with platelets. So moving on to our next slide, again, just to discuss a little bit in more detail what I mentioned here on the first slide, it's important that when you are presented with someone with presumed ITPS as a surgeon that you're thoughtful, and that you realize that this is a diagnosis of exclusion.
ELIZABETH HECHENBLEIKNER: Obviously, you don't to be working somebody up for this all by yourself. If you're noticing this in your patients, and you want to refer all these patients to hematology, but you need to make sure that this is not a situation where you have lab error that's causing from the thrombocytopenia. There could be competing issues and platelets related to EPA, which helps actually keep blood from clotting.
ELIZABETH HECHENBLEIKNER: And other common causes of thrombocytopenia, we already mentioned on the last slide. But you guys need to keep those in mind, and you also need to think about other causes that can be mistaken for ITP. So you've got myelodisplasia, some of your congenital thrombocytopenia, TTP, and chronic DIC. So you obviously don't want to be taking spleens out of people that have any of these conditions.
ELIZABETH HECHENBLEIKNER: So that's why it's important for you as the surgeons to know that. Moving on to the next slide, we're going to talk about some of the phases of ITP. For the first phase is called the newly diagnosed phase, and this is within the first three months post-diagnosis. Your second phase is known as persistent ITP, and this refers to symptoms that are lasting between 3 to 12 months.
ELIZABETH HECHENBLEIKNER: And then the third phase is called chronic ITP, and this is where symptoms are persisting beyond 12 months. The majority of adult patients are going to progress to this chronic stage. In terms of presentation, on the next slide, this is typically a disease of young women. The typical presentation is going to be TKI, and then bleeding from gums and from your nose.
ELIZABETH HECHENBLEIKNER: You guys, this picture here, what do you think this is? Should be relatively easy but want to make sure everyone is paying attention. Close. I don't see well. It's not purpura. Let's see if I can see the chat. Petechiae, yeah.
ELIZABETH HECHENBLEIKNER: Yes, Brian. Good job. So petechiea are very common in the presentation. Atypical things that can happen-- GI bleed, hematuria, and then intracranial hemorrhage. The intracranial hemorrhage can be life-threatening, and it's very serious. In terms of this being disease, as I indicated, mostly of young women, about 70% of affected women, just so everyone knows, are younger than 40 years.
ELIZABETH HECHENBLEIKNER: And primary ITP in adults, this constitutes about 80% of diagnosed patients in the adult population. So the rest of your 20% of adults that you'll encounter are going to have some type of secondary ITP. As you can see, here both for the prevalence and incidence numbers, this is a very rare disease. And it's also important to know that the spleen is not normally enlarged in this disease process. If it is, you need to think about another diagnosis, because the patient really should not have a ITP.
ELIZABETH HECHENBLEIKNER: This right here really focuses on the adult presentation of this disease process. It's very different in children. And again, I'll go through some of the key concepts here, but Dr. Shaheen might be able to chime in, given his experience, but in children, it's very different. It's a much more acute and severe presentation, with the thrombocytopenia.
ELIZABETH HECHENBLEIKNER: Both genders tend to be affected equally. And the good news in children is that it tends to have more complete and spontaneous remissions, and this can be seen in up to 80% of affected children. And so really the rule of thumb with children is it should be treated conservatively. And it's typically a trend adults and unfortunately persistent as a chronic disease process. Does anybody have any questions that they want to ask about ITP ?
ELIZABETH HECHENBLEIKNER: OK, well, we'll move on to the next slide, but if you have any questions, feel free to jump in. So in terms of the non-operative management of ITP, really the severity of this process predicates how you are going to treat it. In terms of the severity and the symptoms, that's all listed here on the left side.
ELIZABETH HECHENBLEIKNER: And that, again, it's going to help guide us in terms of what treatment strategies we go after. So for patients that typically have a platelet count over 50 and are asymptomatic, we can observe them safely without any other follow-up or necessary treatment. For those who have platelets that are 30 to 50 and are asymptomatic, we can observe them, but they usually need more routine follow with a hematologist.
ELIZABETH HECHENBLEIKNER: In patients who have a platelet count less than 50, and who are bleeding, and it doesn't have to be life-threatening bleeding, it just can be persistent nose-bleeding or gum-bleeding or an active lifestyle that would put them at risk for a serious injury, these are patients that we need to be treating medically. And then on patients who have platelets that are less than 20 to 30, and regardless of whether they're bleeding or not, we need to treat these patients.
ELIZABETH HECHENBLEIKNER: We can observe they need some type of medical intervention. In terms of the treatments, overall, I will talk about those. And they're listed here, as you can see, on the right side of the slide, but importantly, for this disease process, platelet transfusions are only indicated for severe hemorrhage, because they're just going to get destroyed by all these antibodies, and they're not going to be really effective.
ELIZABETH HECHENBLEIKNER: So again, only for people that have life-threatening bleeding. In terms of chronic ITP and thinking about treatment strategies, you really need to go with the least toxic treatment, at the lowest dose. That's really the thought-process. And when it comes to emergency treatment of this disease process, just know that it's not going to be a single agent that you're using. It typically requires combination therapy.
ELIZABETH HECHENBLEIKNER: So glucocorticoids, as you can see here on the top right side of the table for ITP treatments, on the right side of the screen, those are your first-line. And the dose is typically a 1 milligram per kilogram, per day dose. IV immunoglobulin is not first-line but it's a second-line agent that's frequently used when you're going to the operating room, and it can also be used in acute bleeding, and also in pregnancy.
ELIZABETH HECHENBLEIKNER: The reason why IVIg works is because it decreases the rate at which macrophages are consuming these antibody type platelets. And so as you can imagine, when you're taking somebody to the OP for a splenectomy, it's an ITP patient, they already have low platelets, and by definition they've failed responses to other treatments. And so the great thing about IVIg, despite it being incredibly costly, it rapidly increases your platelet account and that can help get you hopefully out of needing large amounts of transfusions when you're in the OR.
ELIZABETH HECHENBLEIKNER: Going on to additional second-line agents, RhoGAM is one that can be used in Rh positive people who also have a functional spleen. And then for refractory ITP, Decadron, methylpred, Rituximab, and then TPO receptor agonists can also be used. The thromboprotein receptor agonist works by stimulating the platelet production in the bone marrow. The other treatment strategies that I've talked about, the glucocorticcoids, the IVIg, they work by actually curtailing platelet destruction.
ELIZABETH HECHENBLEIKNER: So the only one that actually stimulates platelet production is going to be your TPO receptor agonists. So moving on to the next slide, when you think about operative management for ITP, the basic things we need to know as surgeons, what are our indications? So typically, as someone who's in practice, a lot of these referrals are coming in to hematologists, and hematologist are going to help guide you about failures of medical treatments for these patients.
ELIZABETH HECHENBLEIKNER: So anyone who is refractory to steroid treatment, and usually this is people that have failed for six weeks of medical therapy, patients who have frequent relapses or having incomplete responses, despite actual medical therapy, people who have severe allergies or just toxic doses from these high-dose steroids, these are people that you really need to consider doing a splenectomy. In terms of preoperative preparation, as I previously discussed, in people who are bleeding and people that are high-risk, you're going to want to consider IVIg, going to the OR, imaging, if you have the luxury of having that, which most people should, mostly CT scans.
ELIZABETH HECHENBLEIKNER: You should think about looking at the size of your spleen, because again, it should be relatively normal, and you want to think about looking for accessory tissue or accessory spleen. And then vaccines too, and in the elective setting, they're given preoperatively. And I'll delve into the vaccines a little bit later on in the talk. In terms of operative considerations, really these are all MIS cases, the minimally invasive cases, that can be done laproscopicaly or robotically.
ELIZABETH HECHENBLEIKNER: Other key concepts, don't give platelets until your artery is clamped. Why do you guys think that you don't give platelets until the artery is clamped? Yes, exactly. So, Robert answered correctly. If you give platelets to somebody who has ITP before you clamp the splenic artery, they're going to be tagged with the antibodies, and the spleen is going to destroy them.
ELIZABETH HECHENBLEIKNER: So you can clamp your artery first, and then you can give the product. And then, Derek, I saw that you had a question. For accessories for ITP, yes, you always want to look for accessory spleen. And I'll explain why in the next slide.
ALBERT SHAHEEN: If you'd like to know, do you have to look for the accesory spleen pro-op, with imaging? How good is imaging for that?
ELIZABETH HECHENBLEIKNER: Honestly, it's not great. In most of the imaging studies, or most of the studies that actually talk about what you're imaging modalities are, and what the sensitivity and specificity is, fo finding accessories spleens is very poor. And it's usually for people who have failed splenectomy, but it's not very reliable. So I honestly think that CT scans, they're probably less than 50%, even lower numbers, for being able to help you identify accessory spleens.
ELIZABETH HECHENBLEIKNER: But there is also [INAUDIBLE] that you can use, so [INAUDIBLE] suspects [INAUDIBLE],, which is actually supposed to be a little bit better. I think the key thing is looking intra-operatively and systematically, especially, when you're doing cases where it applies. And for ITP cases, looking for an accessory spleen is definitely a key stuff.
ALBERT SHAHEEN: And Dr. Joshi asked a question about, can you explain exactly where and how you isolate the splenic artery? Are you going to discuss that later on with the [INAUDIBLE]??
ELIZABETH HECHENBLEIKNER: Yeah. I do discuss that a little bit later on, and I, not so much an open splenectromy, but more so, just some basic strategies for isolating the artery and vein in the latter part of the presentation.
ALBERT SHAHEEN: OK, thanks.
ELIZABETH HECHENBLEIKNER: So I'll address that then. And then, obviously, anybody else can feel free to chime in there if they have any other thoughts or considerations. So we'll move on to the next slide here. In terms of more long-term fall for ITP. When you look at some of the earlier systematic reviews that discuss long-term follow-up, the overall failure rate of splenectomy is just over 25% in five years, and in 2009 Systematic review.
ELIZABETH HECHENBLEIKNER: And then in 2004, there was another Systematic review that basically quoted relatively similar numbers that they had a 72% complete response rate with splenectom, and the relapse occurred in the median of 15% of patients with a follow-up of 33 months. And to Derek's question previously, causes of failure and the reason why we look for accessory spleens in these patients in cases is because it's one of the big causes of failure.
ELIZABETH HECHENBLEIKNER: So typically, accessory splenic tissue is either missed at the initial operation. It spilled at the initial operation, when you're trying to get the spleen out if you use a finger-fracture morselating or sort of in a piecemeal removal that could happen. And then the last reason why a failure will happen is compensatory hypertrophy of splenic rest.
ELIZABETH HECHENBLEIKNER: So let's move on to the next topic. Hereditary spherocytosis is another frequently tested on topic, on the ab site, and something that we encounter more in the pediatric surgery population. So hereditary spherocytosis is an autosomal dominant disease that affects the production of spectrin, and which is one of the red blood cells cytoskeletal proteins.
ELIZABETH HECHENBLEIKNER: And the loss of this protein basically causes the red blood cells, which have a nice deformable concave or biconcave shape to become these small, fragile sphere-shaped structures. And they have increased osmotic fragility, and they're basically more susceptible to destruction in the spleen. So as such, patients will present with anemia, jaundice sometimes, as well as splenomegaly.
ELIZABETH HECHENBLEIKNER: In terms of your work-up, I always want to look at the peripheral blood smear. Patients will have an increased reticulocyte count, as well as increased osmotic fragility, and they will have a negative Coombs test as well. A negative Coombs test is basically looking for antibodies, whether they're directly attached or free-floating that will destroy red blood cells, and that needs to be negative.
ELIZABETH HECHENBLEIKNER: In terms of preoperative preparation and treatment, of course, everyone gets supportive care. And we really only operate on people for severe cases, and we prefer, especially, in the little kiddos to delay this until age-5 due to the immune function and role of the spleen. In terms of preoperative imaging work-up, you do want to get a sense of the size of the spleen, because it's going to help tell you whether you can do cases laparoscopically versus open.
ELIZABETH HECHENBLEIKNER: So that's one important thing. And you also want to assess for gallstones. Do you guys know why we want to assess for gallstones in this patient population? That's correct. So you can get these bilirubin and do stones from all the breakdown of bilirubin, so that's great.
ELIZABETH HECHENBLEIKNER: So moving on to the next slide, another similar, somewhat similar condition that you may encounter, and more, again, in the pediatric surgery population is a pyruvate kinase deficiency. This is in contrast to hereditary spherocytosis and autosomal recessive disease that basically causes RBC to have an abnormal spiculated shape, and therefore, they cannot deform very well, and can get stuck in the microvasculature, and they get destroyed in the spleen.
ELIZABETH HECHENBLEIKNER: So it has a very similar presentation in terms of anemia and splenomegaly in jaundice as HS, but really the only difference in work-up is that you're going to see a normal osmotic fragility whereas in hereditary sphereocytosis, it will be increased. Preoperative preparation treatment, very similar to the HS population in terms of needing to look both for the size of the spleen, as well as assess for gallstones.
ELIZABETH HECHENBLEIKNER: And again, surgery is really going to be reserved for severe cases. So moving on to sickle cell anemia, this is something that we see quite commonly in patients. You might see it more in the pediatric surgery or repeat population, but this is another autosomal recessive disease that we all know has a single amino acid substitution, that basically causes the hemoglobin molecules to get stuck in this sickle shape, particularly where there's low oxygen levels.
ELIZABETH HECHENBLEIKNER: And it can lead to anemia and splenomegally, vaso-occlusive pain crises and other problems. Here, the only key difference is work-up is really adding a hemoglobin or electrophoresis, and there, of course, is a lot more supportive care in this patient population with other treatment strategies, including hydroxyurea, fluids, opioids, NSAIDs, et cetera. And really, in terms of when you want to operate on these patients, it's only for a few indications.
ELIZABETH HECHENBLEIKNER: It's either a massive spleen that's affecting somebody's quality of life, and children, gross delay is an important thing to think about. Kids that can't really eat or drink, because they're so uncomfortable from this disease process or having frequent bouts of bad splenomegaly or pain crises. And then acute sequestration-- that's the only sort of different thing with sickle cell disease compared to some of these other benign hematologic conditions is that acute splenic sequestration is a potential crisis that can occur where sickle cells essentially gets stuck within the red pulp of the spleen, and they obstruct large draining veins.
ELIZABETH HECHENBLEIKNER: And this propagates very quickly causing a very quick drop in hemoglobin, rapidly emerging spleen, and also even cardiovascular collapse. So in these patients, you have to resuscitate and hydrate them and transfuse them, and it's usually followed by splenectomy. So moving on, for malignant diseases, I'll just sort of briefly touch on this, because this is not something that we really commonly have to intervene in for splenectomy and patients with malignant disease.
ELIZABETH HECHENBLEIKNER: So typically, it's sort of your lymphomas and leukemias, and I would say most commonly non-Hodgkin's lymphoma. So on a historical note, one of the standard staging treatments for Hodgkin's was a splenectomy, which was used for staging, and ultimately guiding treatment strategy but that's not the case anymore. For non-Hodgkin's lymphoma, some of the leukemia is listed here. It's really for massive a splenomegaly hypersplenism or in some rare cases of NHL isolated splenic disease, and then with your other primary tumors and metastatic tumors.
ELIZABETH HECHENBLEIKNER: Vascular tumors are really the most common etiology that you'll see in the spleen, things like lymphangiomas, angiosarcs. And those are really doing splenectomy for diagnosis treatment of palliation. And the same thing for, if you think about metastatic tumors, splenectomy is really only for a symptomatic palliation for metastatic disease to this area. And some of the cancers that commonly metastasized to the spleen are breast, lung, and melanoma.
ELIZABETH HECHENBLEIKNER: So jumping on to the next slide, this is a little break here. If anybody has any questions, feel free to chime in.
ALBERT SHAHEEN: Any questions about any of these diseases before we move into the operative section? OK.
ELIZABETH HECHENBLEIKNER: All right, let's move to the next slide. I'm going to, since cysts, they're a relatively rare thing, I think that we can basically jump over this section, and then moved to the operative treatment, because I think this is important, and I want to get to this. So again, operative treatment-- the same thing as the different pathologies. This is really opposite ends of the spectrum, emergent disease, processes are elective ones.
ELIZABETH HECHENBLEIKNER: For our trauma patients, these are all of some cases typically, midline laparatomies. And then for these other elective cases, it's really all about the various MIS approaches and strategies that we have now. So it's either a straight laparoscopic case, straight robotic case, or even lab hand-assisted cases, depending on what you're dealing with. So theme of preoperative preparation-- moving onto the next slide, especially, in the elective setting, you do want to look at your imaging, and you want to make sure you get imaging, especially for cases where you're dealing with splenomegaly.
ELIZABETH HECHENBLEIKNER: Because the spleen, once it gets about 19 or 20 centimeters, it's very hard to take out laparoscopically, not only technically, because of the-- just sort of the dangers and the difficulties and dealing with the splenic hilum in these cases, but also, you need to be able to fit it in bags laparoscopically so that when you take the spleen out, you're not spilling splenic tissue everywhere. So we have the luxury, look at your imaging.
ELIZABETH HECHENBLEIKNER: Thinking about vaccinations, the reason why we--
ALBERT SHAHEEN: Can I ask a quetion here, [INAUDIBLE]??
ELIZABETH HECHENBLEIKNER: Sure. Yep.
ALBERT SHAHEEN: So what's the largest bag that we have laparoscopically, guys? What's the diameter of the largest bag? 15, right. Well, that's Dr. Joshi. I know you know that. OK. Yeah, 15 or is it?
ALBERT SHAHEEN: The Endo Catch [? too, ?] I think, it's 20 centimeters, but I'm not 100% sure, but that size range, 15 to 20 is tough. After that, it gets tough, yeah.
ELIZABETH HECHENBLEIKNER: Additional pearls of preoperative preparation, vaccines are really important. And I'll jump into those and tell you guys exactly what you need to give, but we have to give these vaccines, because they help protect against infection from encapsulated organisms. Again, for elective cases, it's going to be standard, just two weeks before. In some pathologies and cases, especially when you're dealing with a really big spleen, surgeons will do preoperative splenic artery emobolization.
ELIZABETH HECHENBLEIKNER: So that's an important adjunct to think about. You want to think about having blood products available. Anyone that's on for prolonged steroids, like your ITP cases, you also practically need to be thinking about things like stress-dose steroids intra-operatively, and then of course, your standard prophylactic IV antibiotics. Moving on to the next slide, just for delving in a little bit more detail, for vaccines, this is an important topic that everyone needs to know practically, but certainly, for test-taking as well.
ELIZABETH HECHENBLEIKNER: Elective cases, preoperatively, it's supposed to be greater than or equal to 2 weeks before. And then for emergent urgent cases, there is some debate in the literature about the timing of when you should be giving vaccines, but of course, you have to be given post-operatively. If you're going to be dealing with an unreliable trauma patient, it's probably better just to give them vaccines before they leave the hospital.
ELIZABETH HECHENBLEIKNER: But in other circumstances, two to four weeks, post-operatively, has been reported as acceptable. So for the vaccine types that we give, you guys are probably all familiar with them, but just to reiterate, in adults, it's usually the pneumococcal vaccine, PPV23, which protects against strep pneumonia. It's the H influenzae type b conjugate vaccine, which protects against HIV, and then the meningococcal polysaccharide, which of course, protects against Neisseria meningitidis.
ELIZABETH HECHENBLEIKNER: So moving on to our next slide, this is just a nice flowchart here, that discusses in terms of peri-operative preparation how you want to think about vaccines for your elective and emergency cases. Again, reiterating what I said before, at least two weeks preoperatively for elective cases. And then it just provides some subtle nuances between the vaccines that you want to give for children under the age of two, as well as other patients.
ELIZABETH HECHENBLEIKNER: And then in terms of the emergency setting, it's OK immediately post-operatively to give your pneumococcal vaccine. Again, for children under two, it's going to be the pneumococcal 7-valent vaccine and then, for everyone else it's the 23-valent vaccine. And then in this particular flowchart here, they're recommending at least two weeks post-operatively giving the Haemophilus influenza and then the meningococcal vaccine.
ELIZABETH HECHENBLEIKNER: So if you can follow this in your practice, , great but more importantly, you just need to make sure that they get all these vaccines before or after. So moving on to the next slide, quickly, I'll touch on open splenectomy. This is an interesting article that I found for patients undergoing open splenectomy. Apparently, there are four different incisions that these authors described.
ELIZABETH HECHENBLEIKNER: I am most familiar with the midline life laparatomy incision. Again, this is really going to be your trauma patients. People are in the supine position, but apparently, you can also use [INAUDIBLE] incisions. This is more for cancer cases. And then there are some upper abdominal transverse incisions, as well as this long oblique incision for giant [INAUDIBLE] here, that can also be used.
ELIZABETH HECHENBLEIKNER: I am most familiar with midline laparatomies. Moving on, to get into more the MIS splenectomy, for this position is obviously very different. It's a right or semi-right lateral decubitus position. The MIS splenectomy is now really the preferred method for taking the spleen out. Mostly, it's been described laparoscopically, but you can also do it robotically, in a hybrid hand-assist approach.
ELIZABETH HECHENBLEIKNER: This technique was first described in 1991, and it certainly has evolved a lot since then. Some of the advantages are decreased pain, more rapid recovery. People get out of the hospital faster and disadvantages are, of course, longer operating times, and it's sometimes really difficult to remove these large organs.
ELIZABETH HECHENBLEIKNER: In terms of just basic setup for this laparoscopic case here, in this picture featured from the Sabiston textbook, as you can see, this person is on an OR bed that's flexed, such that there is appropriate distance between the iliac crest, as well as the costal margin. It's important when people are in this decubitus position that they are usually on a beanbag or some type of a kidney rest to help maximize exposure and stability.
ELIZABETH HECHENBLEIKNER: It's important that you're taping people very safely, and making sure that the legs and the arms and all the pressure points are padded well. So lots of taping, and be careful, don't take shortcuts here. As you can see here, there's a four-fork technique that's featured in this picture. You usually have your liver retractor in ports 1 or 4, and you can have the camera either in 2 or 3, and then you can sort of hop around the ports.
ELIZABETH HECHENBLEIKNER: And the surgeon can work through 1 and 3, or through 2 and 4, depending on what side of the spleen they're working on and what type of exposure you need. So typically, you can do this with the four-port technique. And then when you think about your incisions, most of these can be done with 5 millimeters, but ultimately, you are going to have to up-size a port at the end to get your specimen out. So you just have to think about using one of the sites that you've already created, or making another incision to get the specimen out through the back.
ELIZABETH HECHENBLEIKNER: I'm moving on to the next slide. Again, this is just a sort of reviewing what we discussed earlier-- sites for accessory spleen. And again, this is very important for your ITP and HS cases. The most common sites that we typically see are the gastrosplenic ligament for accessory tissue, splenic hilum, the tail of your pancreas, your splenocolic ligament, your transverse mesocolon, and your greater omentum.
ELIZABETH HECHENBLEIKNER: So we can, I think, go ahead and skip to the next slide, just because I want to get to some of the-- An overview of the important operative steps for the MIS splenectomy. So this is just sort of a review of nine key steps for this procedure. The first step is usually mobilizing your splenic flexure. You're going to get it down off its lateral attachments and sort of medialize it as much as possible.
ELIZABETH HECHENBLEIKNER: You're going to divide the splenocolic ligament. Then you're going to divide some of the lateral peritoneal attachments and splenic attachments on the spleen, leaving a rim, so that you can move the spleen around. You're going to enter the lesser sac, divide your short gastric vessels. So remember, you'll be mobilizing the gastric splenic ligament when you're doing that. In terms of dissecting up at the splenic pedicle, which is your splenorenal area, you obviously need to take caution as you're working around the pancreas.
ELIZABETH HECHENBLEIKNER: You don't want to injure it. Basically, in terms of some of the basic techniques that I have reviewed for both the magistral and the distributive types, or in the magistral technique, since the main splenic artery and vein are dividing really close the spleen and hilum, you can typically take those vessels and block together with a vascular stapler. However, in a distributive technique since things are branching further away from the capsule, you typically need to completely divide out, and ligate and divide your vessels separately.
ELIZABETH HECHENBLEIKNER: And you also just have to be mindful when you're doing this that any other little isolated branches will need to be ligated and divided separately for this particular blood supply. Ultimately, then once you've mobilized all of your spleen and divided the hilum and the vascular structures, your spleen should be attached to the superior-- Superior pole should be attached to the diaphragm. And then when it's sort of hanging down like that, it's really ideal to get your bag in and then place your specimen in the bag, and then divide the spinal splenophrenic attachments so it's ready for you to remove it right away.
ELIZABETH HECHENBLEIKNER: And then the next step is to figure out where your extraction side is going to be, and then to remove the spleen. And a piecemeal fashion, of course, being, careful on those hematologic cases where you don't want to spill any splenic tissue. So moving onto the next slide, complications. So we'll go over this pretty quickly, so I can just talk about OPSI for a little bit.
ELIZABETH HECHENBLEIKNER: One thing that you can see in the acute-- [INAUDIBLE] is post-splenectomy thrombocytosis. This is particularly in patients who have milely proliferative disorders. This can result in thrombosis of mesenteric portal and renal veins. It can be severe and life-threatening because it can not only lead to bleeding but also can lead to bad complications from thromboembolism.
ELIZABETH HECHENBLEIKNER: More importantly, and what's commonly tested on, and what you need to practically speaking as well, and also for educating your patients is OPSI or overwhelming post-splenectomy sepsis. This is the most common fatal complication that happens after splenectomy in terms of late-timing. It's especially high-risk for this in the first two years after splenectomy. However, you can really get it at any time.
ELIZABETH HECHENBLEIKNER: The risk is higher for anyone undergoing splenectomy for malignant processes, hematologic conditions, especially children who are under the age of four. It's very important that you educate all your patients as well as their providers on taking the appropriate precautions, not only for recognizing symptoms early, but also for making sure that the kids and adults are getting appropriate vaccines, and just staying on top of what the symptoms are.
ELIZABETH HECHENBLEIKNER: Moving on to the next slide, and discussing OPSI in a little bit more detail, this overwhelming post-splenectomy sepsis is honestly something that I have never seen in splenical practice, but it's very scary from its description. It typically has a prodromal phase with fevers, rigors, and other non-specific symptoms. Patients can actually present with focal sites of infection, like pneumonia and meningitis, but they can also sometimes only present with bacteremia.
ELIZABETH HECHENBLEIKNER: But the hallmark of this disease process is a rapid splenical progression in hours to hypotension, DIC, respiratory failure, and coma. And the mortality here, as you can see, and some serious, it's very scary. It's up to 50% to 70%. So early recognition is important. Going to the next slide here, this is just-- I'm sorry.
ELIZABETH HECHENBLEIKNER: Here, I see that we have a little pause. We're almost finished with the topic, and we're going to jump to the questions.
ALBERT SHAHEEN: Actually, at this point, let me just ask-- Dr. Joshi, ask about morcellization as an extraction tool, would it be appropriate for non-oncological cases like ITP, for example?
ELIZABETH HECHENBLEIKNER: My understanding, [? morcellazation ?] is, it is OK as long as you're not-- if you want to, like, for example, minimize your [INAUDIBLE] site. But it's fine as long as you're not spilling any tissue.
ALBERT SHAHEEN: And not using the morcellator? [INAUDIBLE] ELIZABETH
HECHENBLEIKNER: Oh, no.
HECHENBLEIKNER: No, not an actual morcellator. It's really, I don't know anybody that does that in splenical practice, but more like either finger-fracture technique or moving it with reinforcers, but not an actual morcellator.
ALBERT SHAHEEN: OK. Thank you.
ELIZABETH HECHENBLEIKNER: So for the next two slides, you can skip over the next slide on vaccines, and then the next slide too. Let's just skip over that. It's just talking-- Has a brief primer on antibiotic prophylaxis. So why don't we just jump in to our questions as we are nearing completion of the hour? So for this first question, I'll go ahead and read it and then open it up for the chat for responses.
ELIZABETH HECHENBLEIKNER: So the first patient is a 20-year-old healthy female who presented with recurrent nosebleeds. A dark red rash and new onset bleeding of her guns. She takes her medications. Has normal vital signs and has labs that are remarkable for platelets of 46. The rest of her work-up is completely negative, as you can see here, on the question. And her physical exam just demonstrates no hepatosplenomegaly.
ELIZABETH HECHENBLEIKNER: So what is the best initial treatment strategy for this patient? Yes, I see a lot of responses. Everyone is saying A, and that is correct. So this is a patient who is symptomatic, and who has platelet levels that are less than 50.
ELIZABETH HECHENBLEIKNER: And based on what we discussed for ITP treatment, this man needs some type of medical intervention. And you guys are right. The first-line of therapy is going to be glucocorticoids, prednisone. And as you can see, the correct doses are listed here. So moving on to the next question, so we got a 10-year-old boy with hereditary spherocytosis, who is presenting with worsening fatigue and upper abdominal pain with eating.
ELIZABETH HECHENBLEIKNER: He had a viral upper respiratory illness three weeks ago, blood transfusions, three years ago for a similar episode of fatigue. Takes the medications and has all appropriate vaccines up to date. He's afebrile. His heart rate is a 110. His blood pressure is 90 over 60. He's a little bit low for this age range.
ELIZABETH HECHENBLEIKNER: So you know, he's pale with mild scleral icterus, and on exam, has a firm mildly tender mass in his left upper quadrant. His lab show hematocrit at 24%, platelets at 105. A total bili of 6.4 and a direct bili of 1.2. The abdominal ultrasound shows a very enlarged spleen, he's got multiple mobile gallstones with no evidence of cholecystitis. The extrahepatic billiary tree is otherwise normal. And after stabilization, what would you guys recommend as the next best step in management of this patient?
ELIZABETH HECHENBLEIKNER: So we go to the next slide, it'll show everyone the answers. All right, I see some answers for C, which are splenectomy with cholecystectomy. I see an answer for B, splenectomy alone. Seems like right now, two people are favoring splenectomy with cholecystectomy.
ELIZABETH HECHENBLEIKNER: So the answer C, is the correct one for this particular question. First and foremost, this child has significant splenomegally, and life-limiting symptoms, and the setting of hereditary spherocytosis. So that alone mandates splenectomy. However, his history of abdominal pain with eating is a bit concerning for biliary colic. And he has an ultrasound that's showing gallstones that are presumably pigmented gallstones.
ELIZABETH HECHENBLEIKNER: And so this patient should have a concurrent cholecystectomy. So for our final question, we've got a 56-year-old female, who presents to the ED. Status-- post a high speed motor vehicle collision. Her primary survey is intact upon presentation. Her secondary survey revealed left flank ecchymosis, as well as right lower extremity open fractures.
ELIZABETH HECHENBLEIKNER: She has a CAT scan of her abdomen, which is a grade-III splenic injury. She's hemodynamically stable and is going to the ER with ortho for her lower extremity fractures. What is the most appropriate management of splenic injury in this patient? So far, we've got a couple of E's.
ELIZABETH HECHENBLEIKNER: So you guys are right. And this is a grave splenic injury and a person who is presenting with hemodynamic stability, and no reports of any active extravasation. So non-operative management would be very appropriate in this patient. What this will typically entail after going to the OR for fracture management is going to the ICU bed-rest, serial hemoglobin levels, and staying NPO, to make sure that the patient stays stable.
ELIZABETH HECHENBLEIKNER:
ALBERT SHAHEEN: All right. Any final questions for Dr. Hechenbleikner?
ELIZABETH HECHENBLEIKNER: Thank you, guys, very much.
ALBERT SHAHEEN: Thank you so much. We really appreciate your time. This was lovely and very helpful. Thank you so much, and good night, everybody. We'll see you next week.
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