Thoracic Surgery: Achalasia

00:01 Thanks for joining me on this discussion of achalasia in the section of cardiothoracic surgery.

00:09 Here, you see a picture of a classic finding – we’ll get to it shortly – of an achalasia patient.

00:15 Do you notice what's wrong with this image? I'll give you a second to think about it.

00:22 Achalasia is described as a progressive degenerative disease of ganglion cells in the myenteric plexus of the esophageal wall.

00:32 Ganglion cells, as you know, in the myenteric plexus induce peristalsis.

00:38 So, if there is a destruction or degeneration of the myenteric plexus, peristalsis is affected.

00:47 The classic association to remember is Chaga’s disease.

00:52 In Chaga’s disease, infection with tropanosoma cruzi results in the loss of these ganglion cells.

00:59 Although, remember, not every patient has Chaga’s disease just because they have achalasia.

01:04 Now, what are some physical findings and historical facts? Patients generally complain of chronic dysphagia.

01:11 When it gets so bad, sometimes they even have a hard time tolerating their own saliva.

01:17 As a result of the decreased PO intake, because of the dysphasia, patients can have weight loss.

01:24 Sometimes, they also complain of atypical chest pain.

01:28 Remember, any time a patient presents with atypical chest pain, don't ignore it.

01:33 Consider it a possibility that the patient may be having a myocardial infarction.

01:38 Now, what about routine labs? Unfortunately, no routine labs is going to be very helpful in your diagnosis of achalasia.

01:46 And how would we diagnose it? Remember, the initial image that we showed on the introductory slide? Did you identify what was wrong? That’s right.

01:56 Take a look at the classic bird’s beak sign.

01:59 What you show here on this image is a dilated proximal esophagus.

02:04 This esophagus is approximately two or three times the normal size.

02:08 And this dilated esophagus tapers down to what's called the bird’s beak, just before entering the stomach.

02:15 This bird’s beak area is the approximate area of the lower esophageal sphincter.

02:21 Hallmark signs are defined by manometry.

02:24 All patients suspected of achalasia or any dysphasia, for that matter, should undergo some sort of a contrast swallowing study, oftentimes followed by manometry.

02:34 On manometry, you're likely to find failure of the lower esophageal sphincter to relax with swallowing.

02:42 That's a natural reflex.

02:44 Whenever you swallow for a meal, your lower esophageal sphincter relaxes.

02:50 And in addition, the other classic finding is aperistalsis of the esophagus.

02:55 Remember, those ganglion cells are damaged or degenerative, so no longer propagate peristalsis.

03:02 Here, you see a classic finding of a manometry.

03:05 Pay close attention to the bottom.

03:07 Notice the pressures on the lower esophageal sphincter.

03:10 It starts off quite high and never relaxes, despite the swallowing.

03:16 And additionally, you notice there's no amplitude changes depending on the length.

03:21 As you go from the top of the graph to the bottom of the graph, this is telling you where along the esophagus you are.

03:28 Generally speaking, the amplitude don't change significantly from the proximal to the distal esophagus, suggesting that there is no significant peristalsis.

03:37 Again, to remind you, classic findings of achalasia are: a failure to – a failure of the lower esophageal sphincter to relax upon swallowing and aperistalsis of the esophagus.

03:49 This sounds very similar to small bowel obstructions, doesn't it? When there's a distal obstruction, the proximal esophagus accommodates over time.

03:58 That's why you see the mega-esophaguses of achalasia.

04:03 Now, are there any medical treatments? Sure, there are.

04:06 And, in fact, if you're presented with a scenario with achalasia, try medical management before offering the patient surgery.

04:13 Patients undergo dilation and it’s actually quite successful.

04:17 Balloon dilation can be repeated multiple times with an EGD.

04:22 There is a downside with dilation, however.

04:24 With multiple attempts, dilation actually disrupts localized muscle.

04:29 As a result, more scarring can form.

04:33 Additionally, with repeated dilations, perforation can happen.

04:38 Be sure you warn your patients.

04:41 Next, botulinum toxin.

04:43 That's right.

04:44 Botox that you know from plastic surgery.

04:46 Botulinum toxin can actually release the hypertonic lower esophageal sphincter when injected.

04:53 This can also be done multiple times.

04:56 Unfortunately, in a subset of patients, medical management fails.

05:00 Therefore, patients need surgery.

05:03 Luckily, we have a very tried-and-true surgery.

05:05 It's called a myotomy.

05:07 Today, myotomies are largely performed laparoscopically.

05:10 But there's nothing wrong with the traditional open approach.

05:13 The goal of the myotomy is to incise or break the circular muscle of the esophagus.

05:19 When the muscle the esophagus is broken, the mucosa actually bulges through.

05:23 This muscle is carried both proximally and distally past the lower esophageal sphincter.

05:28 This allows the obstruction, if you will, to be relieved.

05:33 Remember, we have to incise both layers of the muscle to expose the esophageal mucosa.

05:38 That's how we know that it's gone deep enough.

05:41 Unfortunately, sometimes, despite being very careful, you could actually injure the mucosa.

05:46 That's important to remember and to counsel your patients.

05:49 As a result of incising these muscles, the mucosa is now unprotected.

05:54 Additionally, these patients may have severe reflux disease as a result.

05:59 For this reason, we perform what's called a partial wrap.

06:02 You may remember from our discussion of gastroesophageal reflux disease where a Nissen fundoplication is performed.

06:09 In that situation, it's a full wrap and not appropriate for the surgery.

06:14 Partial wrap the stomach around the esophagus will prevent reflex as well as protect your new myotomy.

06:22 This can be done with what's called a door or anterior fundoplication.

06:28 Here’s a door fundoplication in a laparoscopic view.

06:32 Door fundoplications is essentially using the floppy stomach and covering over your new mucosa that you’ve performed the myotomy.

06:42 Again, the fundoplication prevents reflex as well as protects your esophageal mucosa.

06:48 Now, it's time to review some important clinical information and high-yield facts.

06:54 Remember, trials of non-operative management are first-line therapies.

06:58 Don't be in a hurry to jump to the operating room when presented with this clinical scenario.

07:03 Do you remember what the non-operative management options are? I’ll give you a second to think about it.

07:08 That's right.

07:09 Botulinum toxin (or Botox) and dilations.