Thoracic Surgery: Achalasia

by Kevin Pei, MD

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    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.

    About the Lecture

    The lecture Thoracic Surgery: Achalasia by Kevin Pei, MD is from the course Special Surgery. It contains the following chapters:

    • Cardiothoracic Surgery – Achalasia
    • Management of Achalasia

    Included Quiz Questions

    1. Trypanosomacruzi
    2. Enterobiusvermicularis
    3. Cestoda
    4. Pseudoterranova
    5. Clonorchissinensis
    1. Proton pump inhibitors
    2. Surgical myotomy
    3. Pneumatic dilation
    4. Botulinum toxin
    1. Bird beak sign
    2. String of beads sign
    3. Snowman sign
    4. Sandwich sign
    5. Shaggy esophagus

    Author of lecture Thoracic Surgery: Achalasia

     Kevin Pei, MD

    Kevin Pei, MD

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