Okay. Well, welcome back again to our section two of the virtual esophagology course. And it's my pleasure to moderate this along with Dr. Reginald Bell, who's a surgeon in Denver. And Reg, you're around. Let's get started with this section. And our first speaker is Dr. Philip Katz. And Philip is professor of medicine at Cornell Medical School in New York. And again, we'll follow the same format as we did for section one, is two lectures, followed by a case, followed by, again, a great panel discussion, hopefully, just like we had for the first section. So making a diagnosis of achalasia and EG junction outflow obstruction in 2021. Welcome, Phil. Looking forward to your lecture. I'm Philip Katz. I'd like to thank the course organizers, Drs. Konda and Sharma, as well as the ASGE, for giving me the opportunity to give this talk, Making a Diagnosis of Achalasia and EGJ outflow obstruction in 2021. These are my disclosures. So achalasia is a disease with functional abnormalities of the esophagogastric junction, characterized by incomplete relaxation and opening, esophageal contractions that exhibit a loss of normal peristalsis, and in most cases, contractile vigor, and abnormal bolus emptying, either by barium or impedance, showing delayed transit through the esophageal body and the EGJ. An EGJ outflow obstruction starts with a manometric finding of an elevated IRP associated with the symptom of dysphagia. Evidence of other criteria for an EGJ outflow obstruction on high-resolution manometry, either via rapid drink challenge, increased pressurization pattern, or a solid bolus, or an abnormal time barium swallow, or appropriate findings on endoflip, all of which I will briefly review. So the diagnosis begins with the clinical history, dysphagia, solids greater than liquids, usually both, heartburn, not typical of GERD, regurgitation during the meal, or immediately after, which of course must be distinguished from rumination, but rarely occurs as an isolated symptom, and can be distinguished from GERD with a careful history. Chest pain suggestive of spasm during a meal, and anyone failing a PPI trial should be considered to have achalasia. Now, achalasia rarely presents with a food impaction, nor does an ESGJ outlet obstruction, as these are not mechanical obstructions. So food impactions should not necessarily make one think about achalasia. Now, the diagnostic tools include endoscopy, a time barium swallow, appropriately performed high-resolution manometry, and with the addition of endoflip, another diagnostic intervention that can help us. You don't necessarily need them all for every case, but together they should allow you to make the diagnosis. Now, I always perform an endoscopy with the intent to find an esophageal abnormality, so I do not rush to the stomach. I look for excess saliva in the proximal esophagus as a soft sign of poor emptying. I look for retained food and liquids, which in the esophagus after fasting reflects an emptying delay, mechanical or functional. I look for esophageal dilatation, the appropriate appearance of the GE junction with a pop, and I consider performing endoflip at the time of the initial EGD. This slide illustrates several of the endoscopic findings seen in achalasia, dilated empty esophagus, retained food that should give you the suggestion that there is an outlet obstruction, perhaps some sigmoidization here, dilated esophagus with old food, the tight rosette with tight LES region that will exhibit a pop, the subtle features of spasm, and a subtle area of retained food here, and this violaceous reticular pattern with food retention in the esophagus, all suggestive of achalasia. Now, barium is still helpful. I believe necessary in many patients to evaluate for achalasia. A good study will not miss, or at least always give you a suspicion, and a well-performed normal study will eliminate achalasia in most cases. It offers information on anatomy which will affect therapy, and while it is a great test for hiatal hernias and a parasophageal hernia, these findings do not exclude achalasia. This is achalasia, typical bird beak with retained barium column and a dilated esophagus. This is achalasia. The black arrow is the thin bird beak, not classic, and the white arrow, probably a mass lesion, so this is secondary achalasia. This is spastic achalasia, and this is achalasia in the setting of a hiatal hernia. So what I suggest is that you order a timed barium swallow. You be ready to tell the radiologist how to do it until they get used to you ordering them, and what it does is to measure esophageal emptying in the upright position at 1 in 5 minutes after drinking 100 to 250 ml of low-density barium sulfate, followed by a 13 mm tablet, repeating radiographs at 5 minutes. An abnormal study is defined at more than a centimeter of retained residual liquid barium in the esophagus at 1 in 5 minutes, and pill retention after 5 minutes. This slide illustrates a timed swallow demonstrating in panels A, B, and C, highlighted by the orange arrow, the barium tablet and its slow movement, and actually here, stuck at the GEJ, and the thin trail of barium in the classic bird beak. That is an EGJ-alpha obstruction that's likely achalasia. So a timed barium swallow is an excellent standardized way to assess functional outlet obstruction. You must do it with a tablet. It's highly accurate for the diagnosis or at least a high clinical suspicion of achalasia, and is good in differentiating an isolated EGJ-alpha obstruction from achalasia. The height at 5 minutes is the most reliable number to assess both emptying and delay in the barium tablet. Ultimately, achalasia is confirmed by high-resolution manometry. The latest iteration, Chicago 4, continues to use the hierarchical approach, starting with measurement of the EGJ, that is the IRP. If the IRP is elevated, you move to the left side of the graph into making a diagnosis of the disorders of EGJ-alpha obstruction with the tricheria as indicated here. Now if we drill down to achalasia and EGJ-alpha obstruction, we still divide achalasia into three types. Type 1, an elevated IRP with 100% failed peristalsis. Type 2, median elevated IRP, 100% failed peristalsis and panesophageal pressurization. Or type 3, an elevated median IRP, supine and or upright with premature contractions in greater than 20% and no evidence of normal peristalsis. An EGJ-alpha obstruction is an elevated mean IRP, both supine and upright, with greater than 20% elevated intrabolus pressure supine, not meeting the criteria for achalasia. This slide illustrates the three manometric phenotypes. Type 1, absent contractility and elevated IRP. Type 2, panesophageal pressurization, elevated IRP. And type 3, elevated IRP with premature contractions, a short distal latency reflective of spastic achalasia. Again, to make the diagnosis of an EGJ-alpha obstruction, you need an elevated IRP, predominantly supine but confirmed with an upright one. And in clear situations or unclear situations, if you perform additional testing with a rapid drink challenge or a solid test meal, you can aid in the diagnosis. The manometric designations are varied. Here, an esophagogastric junction outlet obstruction with hypercontractile features demonstrating an elevated IRP and this jackhammer-like contraction with elevated intrabolus pressure. This would indicate a clinically important EGJ outlet obstruction. Here, esophagogastric junction outlet obstruction with no evidence of disordered peristalsis but a compartmentalized pressurization and the elevated IRP. This also would be potentially clinically important. This is a potential artifact, an elevated IRP with no abnormalities in pressurization or peristalsis. This you might confirm with an upright IRP. For certain, do a rapid drink challenge and maybe proceed on to other testing with a time swallow and or endoflip. These pressurization patterns are important to recognize, compartmentalized pressurization here and the pan-esophageal pressurization typical of type 2a kalasia. Proximal compartmentalized pressurization is also abnormal and can give you a suggestion of bolus delay and perhaps an EGJ outlet obstruction. Now, the newest entry into our testing armamentarium is the functional luminal imaging probe or endoflip, endoflip with panometry. This is a very elegant technology that uses impedance with a balloon inflated with saline that is passed via endoscopy, positioned across the esophagogastric junction, allowing you to measure EGJ opening, measured by a distensibility index, allows you to measure diameter at the EGJ, in fact in the esophagus, as well as to do planimetry, which is a way to measure secondary peristalsis in response to esophageal distention. So the functional luminal imaging probe or FLIP panometry makes two assessments. It gives you distensibility of the EGJ during volumetric distention using a 60 ml balloon, can actually go to 70, and allows you to measure the distensibility index at the EGJ, a reflection of sphincter opening, and to assess contractility in response to balloon distention. A normal panometry, if you would, gives you these very clean repetitive antigrade contractions, and a distensibility index that, based on limited normal data, is below a value of 2.8. This is an abnormal FLIP study. What it shows is this increased pressure here, with a calculation showing an EGJ distensibility index of 0.3, which is quite low, and these repetitive retrograde contractions, somewhat disordered, and some sense here of perhaps a decrease in contractility. This would be suggestive of spasm in the setting of an EGJ outflow obstruction, which, of course, would have to be distinguished from a callasia. At this point, FLIP is quite useful as an adjunct to manometry. It allows us to measure esophagogastric junction distensibility index, with values here reflecting an esophagogastric junction outlet obstruction, diameter, which in these values is reflective of an EGJOO, and perhaps give you clinically important esophagogastric junction outlet obstructions, those that may be clinically important, differentiated from normal and gastroesophageal reflux disease. So, ultimately, an EGJ outflow obstruction, a manometric finding that must be confirmed with the clinical picture and other objective findings, dysphagia should be present, a time swallow should show objective evidence of delayed emptying, a column or delayed pill passage, and endoFLIP should show a low distensibility index less than 3. Demonstrating again, delayed emptying of the barium tablet, hold up at the GE junction, and the thin escape. So, ultimately, in conclusion, a high-resolution manometry can look for an EGJ outflow obstruction, an elevated IRP pressurization, a rapid drink challenge can aid by showing panesophageal pressurization and elevated IRP, multiple repetitive swallows can show compartmentalized pressurization, a time barium swallow can show an elevated barium height at 5 centimeters, and a 12-millimeter tablet that does not pass. FLIP decreased EGJ distensibility index less than 2.8, and panometry showing abnormal repetitive contractions. With that, I want to thank the ASGE and the course organizers. I will look forward to discussion.

achalasia

esophagogastric junction

EGJ outflow obstruction

diagnosis

virtual esophagology course