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Good afternoon, everyone. I'm uh greetings from Abu Dhari. Uh I just want you to thank the uh Baps organization and uh in particular sai for inviting me to speak uh in this very prestigious symposium about the clinical management of Achalasia in Children. And I'm honored and feel grateful uh to be amongst the esteemed speakers that have been included in the symposium for those who don't know me. My name is David Rowett. I'm a consultant, pediatric nephrologist and I currently reside and work in Abu Dhabi in Tli for Medical City in the United Arab Emirates. Uh I have uh worked in London for over 10 years, both from the uh charity minister that were in London and I spent some time in Boston Children's Hospital where I uh refined dispose my interest in the gastrointestinal mortality disorders. So, my remate for today is going to be particularly concentrating on the role of the gastroenterologist in the management of clinical achalasia in Children. Yes. So as we know, Achalasia is a primary esophageal motility disorder, which is not a mechanical but functional obstruction of the gastroesophageal junction for which we now have established pathophysiology. I want to pay. Uh I suppose some respect to Sir Thomas Willis who more than 350 years ago, first described a case presentation of his patient who presented with dysphagia for which he used a, a whale bone and sponge to dilate the lower esophageal sphincter. It wasn't until less than a century ago in 1929 when Achalasia was first named a motility disorder. Greek terminology which stands for failure to relax as we're all aware. Achalasia is a neurodegenerative disorder, which in which you have progressive loss of the inhibitory myenteric neurons of the lower esophageal muscle. It's important to recognize that whilst the initial insult is or the issue is the failure of relaxation of the lower esophageal sphincter. This disorder does affect the esophageal body and ultimately, you get dys peristalsis and aperistalsis. The etiology of uh achalasia remains largely unknown. However, there are uh numerous hypotheses for which the most uh uh likened is that there is initial infection or or toxicity which causes uh uh an insult or inflammation to the Myer plexus. The types of viruses that have been proposed are those like the herpes simplex virus and Chagas, uh which there is uh they're neurotropic and therefore have an affinity to squamous cell mucosa. It affects genetically susceptible individuals and there's a subsequent aberrant autoimmune response which results in progressive uh disruption of the inhibitory neurons of the esophageal myenteric plexus. It's important to also recognize that there are a number of disorders that result in the similar clinical presentation. And indeed, manometric and radiological findings which can resemble primary achalasia. And these secondary achalasia can occur in the form that either uh evolves just the esophagus or that is part of a generalized gi motility disorder affecting uh the esophagus as one part of a generalized motility disorder in adults. This is more recognized that achalasia is also associated with certain cancers and this is by the invasion of esophageal myenteric plexus as part of a paraneoplastic uh syndrome. One of our earlier studies that was undertaken at Chelsea Westminster Hospital including went to had dad uh very, very able and keen. Um in fact, um he was a medical student. Uh Mat Malas did some incredible work. We looked at uh uh we reviewed the incidence of Achalasia in the UK, Northern Ireland. We looked at 25 regional centers using uh the obvious uh ICD and OP CS coding and found that there was an increase in the uh incidence of achalasia uh up to 0.18 per 10,000 cases per year. And this increment has uh this rise rather than the incidence was not just a rise but an incremental rise which was linear and which has been subsequently validated in other international studies, suggesting that there's suddenly an increase in rise or incidence of this uh condition. Achalasia. We're also aware and we went on to look at quality of life using the PS PRL, which is a validated uh quality of life scoring system. And we compared achalasia with other functional disorders such as constipation, gastroesophageal reflux disease, as well as inflammatory bowel disease and healthy individuals. And what we show what we showed was statistical significance that achalasia to Children with achalasia had much lower quality of life scores. So we realize that this is a disorder that has significant symptom burden. So with all this in, in in mind, uh what is the role of the gastroenterologist? So I would suggest to you that the gastroenterologists were there through the diagnosis, were involved in the treatment and most certainly follow up surveillance and then uh enabling transition to adult services uh diagnosis should be suspected by clinical suspicion on obvious symptoms. And this is usually progressive dysphagia with so with liquids and evolving to solids, uh there needs to be a clearly a workup for achalasia incorporating that there may be uh blood blood tests and investigations uh to exclude other possible uh systemic disorders as I've outlined before. And we're also involved in endoscopic assessment and where there's no gi uh physiology unit uh undertaken manometry ourselves. We're also integral to the uh to, to the um treatment. And this is important uh to be addressed as a multidisciplinary team where we involve, of course our pediatric surgeons, our gi physiologists or radiologists, sometimes endocrinologists, sometimes in genetic involvement. And uh it's really involving most crucially the parent and the patient and uh uh families uh to counsel about what the uh the different modalities that are are present that can be offered. And also counseling to uh rationally explain that uh we need to have realistic outcomes because this is uh whatever the intervention is, this is not a cure, purely treating the symptoms. As I said before, diagnosis is on clinical suspicion that the diagnostic modalities that are currently available and are well documented are radiographic studies. Upper geo endoscopies and esophageal manometry. Probably the most uh commonly uh used modality is the upper g contrast study. And typically you see this rat's tail or of the distal esophagus. Uh birds, bird like uh appearance of the esophagus with a dilated esophagus with uh you can see here, distended esophagus with fluid uh fluid uh level and debris, suggesting retention of food. Uh A chest X ray can also be a useful modality in this patient, particularly who presented with uh super eh symptoms of uh uh respiratory distress and uh uh regurgitation. Uh You see this, this is actually the esophagus here being dilated and a patient who presented with pa for which a CT scan can sometimes be useful in in diagnosing a dilated esophagus. Upper endoscopy is often uh the the primary uh diagnostic test in some patients where uh they have atypical symptoms. Uh and this you can often uh be surprised when you go in at endoscopy to find that there's a fluid level that there's retention of food debris in the esophagus. You see a dilated distal esop with food retention, which you sometimes have to use baskets or whatever devices you have to retrieve solid food particles or aspirate uh food residue from the distal esophagus. From what you then see is this uh uh really constricted, lower esophageal sphincter which often uh needs to be dilated at the time of diagnosis. At the same time, it's important to exclude uh Pseudoachalasia syndrome such as eosinophilic esophagitis and Chaga syndrome. Um And you see here uh in this uh pathognomonic endoscopic view of typical findings that you'd expect with eosinophilic esophagitis. And remember the clinical presentation can be uh the same as uh uh achalasia. We always take biopsies because of course, like esophagitis is a histological diagnosis. Esophageal manometry remains of course, the gold standard for the confirmation and diagnosis of achalasia. And there's been huge advances uh over three over the past decade, particularly with introduction of control plot topographic analysis and high resolution manometry, which allows for much better categorization of esophageal motor function and allows for uniform consensus of esophageal uh mortality disorders. I'm not going to go into much detail. However, uh I'm sure uh Jaffa who was a colleague I worked with before and a good friend has explained that there are now three subtypes of achalasia that can be classified according to Chicago classification, which are the classical where you have no pressurization, uh compression or pressurization, which is typical and spastic achalasia. And this is all relevant and was first described in the uh the journal of neurogastroenterology and motility in 2011 by our adult colleagues. Really highlighting that uh this subcategorization is important and crucial because it can predict outcomes. Let me see here that the typical uh or your type two achalasia type pattern is the one that's most amenable to any form of intervention. These are slightly hyperinflated, I suspect outcomes. But this is what was described in the initial uh paper, which is a landmark paper. Uh notably the type three as I'm sure Jeffer has explained before, has the worst outcome. And this should be considered in terms of uh the intervention that's going to be offered to your patient. Uh What about the gastroenterologists role in uh in treating uh achalasia? Well, we are integral to the multidisciplinary team and uh it's important to coordinate. Uh I suppose uh the team that may be required to be involved in the decision making. And ultimately, the goals of treatment are there to improve the patient's or relieve the patient's symptoms uh by improving esophageal emptying and preventing the complications of uh nontreatment. Uh It's, it's important as well to keep, as I said before, uh an open mind because clearly touch up therapies are often required regardless of the intervention that's going to be used. And then ultimately, muscular activity can never be restored. And it's very rarely that you can reverse uh the nonrelaxation of lower esophageal sphincter or esophageal aperistalsis. And clearly, it's important to uh suggest that regardless of the intervention for follow up will always be recommended. The main treatment modalities uh are pharmacological therapeutic endoscopy and surgical of which uh either he myotomy or the more recently home, uh which I'm sure we discussed in more detail by uh surgical colleagues. Um however, important to recognize that the majority of these. And as I said before, there's no cure for this uh pharmacological agents, most of which uh work by relaxing the smooth muscle and mainly involve calcium channel blockers and nitrates. Um have been described in the literature. But really, there's no role for initial or definitive treatment by using pharmacological agents for achalasia. Interesting Terry Botox received some attention uh some time back and there's certainly some data to suggest that it may decrease the low esophageal pressures by up to 33% in some patients and may improve dysphagia. Um However, uh this is uh not an intervention that should be uh used for definitive treatment. It's often used particularly as a hypertensive sphincter in your type one. Achalasia. What about therapeutic endoscopy? I suppose this is where the gastroenterologist uh mostly comes to force in terms of the actual intervention. Uh Pneumatic dilatation is uh well described and uh historically is one of the main uh one of two, I suppose uh interventions to help relieve symptoms. Uh It works by disrupting the lower esophageal sphincter. And uh nowadays, we have uh we protocols which are very simple and easy to follow. This can be undertaken as a day case procedure. And usually, um this involves uh you know, graded uh protocol of using slightly higher balloons and subsequent dilatations. Uh The guidewire is passed through the endoscope over which the balloon is passed. The reverse of the lower esophageal sphincter and pressure of up to 7 to 12 pc uh of air is is applied for 1 to 2 minutes with the ultimate aim uh to relieve or reduce pressures of the lower esophageal sphincter by 10 to 15 millimeters of this is what it looks like. So it's quite a graphically quite a really a, a big balloon uh which is quite robust, it has a very hard rubber um introduction uh I suppose a tip. But if you see on the uh the image on the right hand side, uh when fully dilated, it's the width of a paper clip. So this is quite wide in terms of pediatric data. There's numerous studies looking at uh uh describing the practice of hematic uh dilatation but really small uh small studies. There's only four co studies of which only two were perspective and uh with followups which range from six months to 10 years in these pool studies, there's only 58 Children of which the majority of uh which were males with success rates reported from 60 to 100% at varying levels of follow up with an overall uh need for subsequent uh myotomy reported at 10%. If you look at the adult data, and I'm talking about comparative data here, looking at dramatic uh dilatation versus myotomy. You see that the pooled studies uh suggest a good to excellent outcome for both balloon and laparoscopic myotomy with a slightly higher uh success rate in the group undergoing myotomy and not surprisingly high rates of complications. If you look at though decent studies, there's really not much out there. Even in the adult data, there's only two randomized controlled studies. One single center study where there was no difference shown in success, success rates between uh dilatation and myotomy. These are small numbers 16 patients versus 14 patients, but a higher rate of failure in the group who underwent dilatation. And one multicenter study involving five European centers a long follow up of five years showing a success rate of 92% of uh patients undergoing dilatation versus 87% undergoing surgery. But again, showing higher uh perforation uh rates in the group undergoing myotomy. If you compare this to pediatric data, again, you see a very small number of studies here. Only four studies, you look at full samples of 100 and 97 patients. Again, predominantly male as is uh the case in ecclesia. And uh the reported success rate in Children is much lower uh with hematic dilatation compared to those uh that I've just explained about the adult data and relapse. Uh treatments are required um at a much higher rate, 71 to up to 91% of those who underwent heat dilatation compared to only up to 40% of undergoing myotomy. So a much better outcome in pediatrics as it seems uh with uh myotomy if you look at the data, and this is uh again, the work of Ovaldo Borrelli and his group and they looked at the long term outcomes for helis myotomy and balloon dilatation in uh in Children, small numbers again, 48 Children um over the last follow up of which was three years, but a range of 1 to 5.5 years in the follow up with a meeting age of uh 10 years. And uh the logistic regression analysis that was performed to assess the effect of the various factors on the need. For a second intervention uh showed that the type of procedure uh was the only factor that uh showed a significant difference in the outcome. This is a survival uh analysis with log rank uh test here. Uh graphically demonstrated and you can see that patients undergoing balloon dilatation uh as the first or initial procedure uh had a much, had higher success rates but needed much more intervention for uh uh uh second interventions. Subsequently. In fact, 85% of patients required further procedures uh in the study group regardless. And this is important to recognize only 43.8% were symptom free. Regardless of the intervention. If you look at those patients who were asymptomatic after treatment, there was a larger proportion who were asymptomatic uh of the patients who had undergone primarily uh the uh myotomy as initial treatment compared to uh 30% who were asymptomatic after initial treatment with dilatation. So clearly, there's no robust data in pediatrics and there still remains a debate and controversy about what the optimal initial procedure should be for Children with achalasia. And we still have very conflicting results. If you see specifically if you compare them to your adult uh colleagues, the s began working group for motility, uh decided to look and undertake a survey at worldwide practice of the clinical management of uh pediatric laia. And you can see here what was confirmed was that there is not surprisingly a huge amount of worldwide heterogeneity uh uh with variation uh in not only clinical practices but also the resources that are available depending on where you are. And this needs to be considered in terms of your approach to the treatment of uh eia in Children. So what's the current expert opinion? Well, if you look at here, the subtypes once you've confirmed the diagnosis by whatever measure, either be swallow or high resolution manometry. If that's available, you classify as much as possible. Uh into the you have availability by of high subtype, you subtype your achalasia and regardless of the subtype, uh the first intervention should be uh that's suggested to be offered is thematic balloon dilatation for which you start off with a smaller size and incrementally increase. I usually do monthly increments uh and monitor for a symptom uh response. It is no response. Now, after two new uh dilatations, then uh you can either uh repeat a time barium to look objectively for uh for response or repeat the high reduced tet if available, offer the family a third uh dilatation if they want no more than four dilatations to be offered, if there's no clinical response, and then you need to consider uh myotomy or uh uh it's important to recognize that if you have the availability of high manometry, you should be considering home uh as the initial uh treatment modality. If you have type three, achalasia important to recognize is that in the long term, we all we're doing is improving symptoms. We must must, must understand and it's important that we relate us to uh families and patients that there is no cure for eal. And this is why follow up and surveillance is so necessary. We need to assess symptom uh reduction after any intervention and treat in a timely fashion, relapses uh if they uh occur because we know that repeated relapses are associated with the worse or for outcome. We need to of course, avoid complications. And this is why we need to monitor the current recommendations is that we monitor three monthly post intervention for at least two years. And this is what I do certainly. And after two years, every six months, we're monitoring symptom assessment, the nutritional parameters. And of course, most importantly, patient satisfaction, there are objective for dysphagia scoring systems such as the ed card, which is not validated in pediatrics. But I have to say it's a very useful scoring system to use for objective objective symptom response. What about GERD? It's inevitable that if you are destructing the lower esophageal sphincter, it's inevitable that you're going to have a residual gastroesophageal reflux disease. And uh the pediatric data suggest that up to 8% of patients undergoing dilatation uh by balloon have uh GERD at two years um less. So uh those undergoing a myotomy with frontal location in 3.4% but a much higher rate of GERD, which is noted uh with the co procedure, I think we'll all um recognize and appreciate uh Mauricio Corelli and uh uh marks work uh who, who looked at the hell myotomies. And, and again, showed uh that regardless of fundoplication, uh there was no difference in symptoms of dysphagia and indeed GERD uh following my arty, which is a, a paper, the diligent follow up, of course, is necessary to uh again, to identify GERD and again, to, to, to uh prevent the complications of GERD, which are peptic strictures. Barrett's esophagus and esophageal squamous cell carcinoma, all of which are recognized and have been uh probably certainly even in the pediatric data. Uh the suggestions currently particularly for patients who undergo myotomy or home is to continue on a PPI for at least one year post therapy. What about objective markers of response to treatment? Uh Of course, you can use uh uh manometry if this is available, but of course, technically, this is very difficult and of course, uh not so pleasant. Uh There's a huge now uh move towards using uh timed bury um esophagram protocols. And these are esophageal assessments of uh uh of the ability of the esophagus to empty. And you do timed measurements of uh after uh set eli barium at 13 and five minutes. Uh and you monitor emptying uh which is a very good objective marker of the response lastly. Uh uh and, and, and, and of course, uh there's, there's no way I could uh give a talk uh about the uh uh the value of the gastroenterologist and physician rather without transition. It's something that I II suspect in pediatric surgery is, is uh is, is, is less uh uh common, but certainly as physicians, this is uh important to consider for all young people with chronic conditions. It's important to enable autonomous healthcare decisions and to hand over uh as supposed to enable uh this decision making uh in a handover clinic where we initially do a joint clinic with our adult colleagues at one or two clinics together. And then there's a timely uh handover of care to adults. It should be undertaken in a timely way to prepare them medically psycho, socially, educationally, Vocationally um to use the best use of local um and subspecialist resources that are available uh to make this as easy as possible for the young adult. So in the short time, I hope I managed to convince you that the uh role of gastroenterologist is, is indeed integral to uh the natural history of uh Achalasia in childhood. From the treatment to the diagnosis, to the surveillance and then the handover care uh to adults. Before I end, I just want to acknowledge a few of my triumphant uh patients that I really have a lot of respect for this one. This patient is a patient that uh and the team at Chelsea Westminster Hospital is a young man who was initially uh diagnosed as a eating disorder who went on to win a top National prize for creative Writing. Uh The topic of which was his uh his health and his experience of uh of, of, of dealing with achalasia. Uh The second to the right hand side here is a patient Steve Lamington who's a, who is a fantastic uh young man who was the first patient that I diagnosed with AK A in Char Westminster Hospital. He's gone on to become a really uh uh uh superstar in terms of uh a musician and an actor, I believe. Uh and one of his first albums that he uh recorded and subsequently uh went on to, to, to uh present uh in social media was uh Achalasia. And II urge you all to get a copy and please listen to this ordinarily. I'd love to take some questions. However, of course, this is a uh virtual uh uh uh recording. So I'm very happy to take any of your uh questions or to be involved in any corroborative efforts. Uh My email is de ra at 100 do a ei thank you all for your attention. And once again, thanks the organizers for involving me in this uh symposium. God bless you all and uh keep safe. OK.