Scott Gabbard, MD
Scott Gabbard, MD

Siva Raja, MD, PhD
Siva Raja, MD, PhD

Wednesday, October 24, 2018 | Noon


Description

Achalasia is a condition where the esophagus is unable to move food into the stomach. The lower esophageal sphincter (LES), a valve located at the end of the esophagus, stays closed during swallowing, resulting in the back up of food. Slowly, over a number of years, people with achalasia experience an increasing difficulty in eating solid food and in drinking liquids. As their condition advances, achalasia can cause considerable weight loss and malnutrition. Cleveland Clinic gastroenterologist Scott Gabbard, MD, and Sydell and Arnold Miller Family Heart & Vascular Institute thoracic surgeon Siva Raja, MD, PhD answer your questions.

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Diagnostic Testing

earl359: I've been to the doctor with my mother many times, and have told him that I was concerned about when she eats she has a choking sensation while swallowing food. His response many times was to just look at me, then look at her. I guess the pill cam is a higher class of medical treatment then what her insurance will pay, either that or they just want to save them a few bucks and don't care.

Scott Gabbard, MD: The pill-cam is mainly used to evaluate for bleeding in the small intestine. Tests used to evaluate swallowing: – Modified barium swallow is a barium study done with a speech pathologist to evaluate the throat phase of the swallow – Barium esophagram is a barium study of the esophagus – rules out mechanical blockage – Upper endoscopy (EGD) can rule out mechanical obstruction, mass, inflammation from reflux – Esophageal manometry can evaluate the muscle function of the esophagus.

Siva Raja, MD, PhD: I agree. The pill-cam is a great test for a different problem. It is to identify mechanical problems in the bowel that we have difficulty getting to. The barium swallow is a better test for functional problems such as problems with the swallowing mechanism and upper endoscopy is a better test for mechanical problems in the esophagus.


Symptoms – Problem Swallowing

Brent1992: Hi! I'm a 26-year-old male. About three months ago I started experiencing difficulty swallowing and it has progressed to the point that I can't eat anything solid, have difficulty with soft foods, and liquids come back right back up. My PCP thought maybe it was anxiety at first. Really? I am going to see a GI doc next week. What can I expect in testing or any thoughts on diagnosis?

Scott Gabbard, MD: Yes. In general, we recommend having an upper endoscopy (EGD) in anyone with dysphagia (difficulty swallowing), to ensure no mechanical blockage, and no inflammatory disorder like acid reflux or eosinophilic esophagitis (allergic esophagus). Eosinophilic esophagitis is an allergic condition of the esophagus, most commonly seen in young individuals. It is important that your gastroenterologist take biopsies from the top and bottom of the esophagus to evaluate for this condition. It is very treatable with medications and diet. But yes, would definitely recommend that you be evaluated by a gastroenterologist.

bonita: Is it achalasia if you have problems swallowing - but it is only certain types of foods. Sometimes I feel like I am choking on my food but it is not liquids it is more like solid foods, and even worse with crunchy foods. Or smaller items like rice? Should I be checked by someone?

Scott Gabbard, MD: Yes. In general, we recommend having an upper endoscopy (EGD) in anyone with dysphagia (difficulty swallowing), to ensure no mechanical blockage, and no inflammatory disorder like acid reflux or eosinophilic esophagitis (allergic esophagus). If EGD is normal, we then recommend esophageal manometry to rule out muscle problem in the esophagus like achalasia or spasm. But yes, would definitely recommend that you be evaluated by a gastroenterologist.

Siva Raja, MD, PhD: I agree with Dr. Gabbard. Many reasons can cause swallowing difficulty - only one is achalasia. Arguably it is probably one of the least common causes of swallowing difficulty. Endoscopy, manometry and other tests mentioned in previous answers will help your physician accurately diagnose this and treat appropriately.

JTRS24: I get food stuck in my throat. I can't eat or drink - it just comes back up. I had a manometry test and it was negative. Does that mean it is not achalasia? I am scared to eat, afraid i will choke.

Scott Gabbard, MD: Difficult to say. I would make sure that your manometry was performed at an expert center. There are adjudicative tests like barium swallow and endoscopy that can ensure nothing like achalasia was missed.

Siva Raja, MD, PhD: As mentioned before, achalasia is one of the less common swallowing problems, therefore we should insure all other diagnoses have been ruled out. If you are still having problems, despite all tests being normal, it may be time for a second opinion in a high volume experienced center.


Symptoms – Heartburn

MarkH: Having been diagnosed with achalasia and then talking to other sufferers I am confused as to why heartburn is prevalent in my symptoms. This does NOT always occur at night and can occur whilst upright. Why can heartburn occur in an achalasia sufferer during a normal day whilst upright? Connected to this, if food is 'stuck' how come water can still be swallowed?

Scott Gabbard, MD: Your docs should consider using manometry, timed barium esophagram, PH testing, and endoscopy to help determine the cause of pain. If it is not the first two, then it is nerve pain. Nerve pain is indistinguishable from acid reflux pain or esophageal spasm – it is important to differentiate and then direct treatment at the cause.

Siva Raja, MD, PhD: If you have had treatment for achalasia then the esophageal sphincter should be open. This can result in acid reflux day or night. The reasons why it happens at different times is different for different people - diet choices, size of the meal, body size can all have influence on this. If you have not been treated, then you are suffering more from regurgitation of food within the esophagus rather than true acid reflux from the stomach.


Symptoms – Chest Pain

MissieG: I’m type III. I had open HM with wrap in 2004. Onset was fast, first symptom of extreme chest pains to not being able to get solid or liquids down was about 5-6 months. I now take morphine only for the extreme pains I get. What can be done about chest pain? They are horrendously debilitating, yet there seems to be no real answer. My registrar said to possible have more surgery??

Scott Gabbard, MD: The cause of chest pain in achalasia can be confusing. In general, there are three major causes: – Spasm in the esophagus – Acid from reflux (after myotomy) or acid from fermentation of food sitting in esophagus (if not treated) – Nerve pain – patients with achalasia often get nerve pain in the esophagus, which requires medications aimed at the nerves.

Siva Raja, MD, PhD: Attributing chest pain due to achalasia is common but we should first rule out mechanical problems such as acid reflux, spasms and persistent tight sphincter. Acid reflux should be considered and treated sometimes even when the patient does not appreciate symptoms of reflux. The sphincter should be evaluated to make sure it is open as it should be. In patients where there is a delay in esophageal drainage - can be considered for repeat myotomy such as a POEM procedure. If the drainage is optimal, then surgery should not be entertained.


Medications

Yole: Good afternoon, it is again Yole from Italy. I am 53 with diagnosed Achalasia type 2, operated Heller’s myotomy with fundoplication eight years ago. I recently discovered to suffer of advanced Osteoporosis and my GP prescribed me Alendronic Acid by mouth, but I read it is not suitable for people with Achalasia. In which way i can take this acid, or can I substitute it with something less harmful for my condition and effective as well? Many Thanks. Kind Regards.

Scott Gabbard, MD: I would not recommend oral bisphosphonate or certain antibiotics like doxycycline in someone with achalasia (even if well treated), as these can cause severe ulcers. In the US, we do have some IV preparations of bisphosphonate, which may be preferred in someone with achalasia.


Achalasia Treatment

flowergal: I've been diagnosed with achalasia, and have scheduled to have a Heller Myotomy with fundoplication. This will be done laparoscopic. What is recovery like? How long until I can eat again? What about exercise?

Siva Raja, MD, PhD: Generally the recovery is quick. Our pathway keeps people on a full liquid diet for one week. Soft diet for three weeks after. By one month, they can eat regular food. We do have them meet with a nutritionist to make sure they are educated on the anti-reflux diet which we recommend they follow life-long. In terms of exercise, we usually ask that they do not perform strenuous exercise for six weeks to avoid hernia in the incision site. But exercise like walking and slow jog would be fine at our institute.


Gastroesophageal reflux disease (GERD)

carolinaS: I have GERD. Five years ago had a Nissen fundoplication. Having problems again. Doctor said that maybe I have weakening of esophageal muscles. Doctor was discussing redoing surgery - full Nissen or full Nissen - I don't understand what my options are.

Scott Gabbard, MD: This is a complicated answer. At CCF, we use manometry, PH testing, esophagram, and EGD to determine the cause of your recurrent symptom. It depends on what is causing the problem. As noted previously, sometimes we find it is a nerve issue that needs to be treated with nerve medication (if testing is negative).

Siva Raja, MD, PhD: If the problem is that you are having recurrent reflux, then a redo surgery can be considered. The main thing for redo surgery is that the side effects may be greater therefore the threshold to entertain surgery is often higher. We try medical therapy as much as possible before doing redo surgery. Once the decision for a redo fundoplication is made, based on the mechanism of the failure of the first procedure, we often consider performing an esophageal lengthening procedure. It is very important to redo the manometry and a gastric emptying study before embarking on redo esophageal surgery, especially if the weakening of esophageal muscles have been brought up. If your problem is that food is getting stuck, then a redo surgery should possibly involve a partial wrap if the manometry confirms that the esophageal muscles are actually weak.


Laryngopharyngeal reflux (LPR) vs. Gastroesophageal reflux disease (GERD)

DHill: What is the difference between LPR and GERD? What tests are used to diagnose one vs. the other? Is there a difference in treatment?

Scott Gabbard, MD: This is tricky. LPR is thought to be when acid and refluxed contents come up to the throat and cause symptoms such as cough. Unfortunately, studies have found that patients with LPR who do not respond to PPI medications will not respond to antireflux surgery such as fundoplication – this was a study done at CCF ~10 years ago: GERD is when stomach contents reflux and cause symptoms. Typical symptoms are heartburn and regurgitation, which are much more responsive to medications.

Siva Raja, MD, PhD: Reflux has two components - the acid and the regurgitation. Medical management to decrease acid can be very effective. As such, when patients do not respond to medical management for acid, we often try to look for alternate diagnoses. Regurgitation on the other hand, is a mechanical problem where medical therapy is not very successful. For this problem, the easiest option is to modify ones diet and modify one's weight if the person is overweight. The patients who do best with surgery are the ones who have relief of heartburn symptoms with medication but have persistent regurgitation. Specifically to your question of LPR, anyone with significant regurgitation can get regurgitation in the proximal esophagus, insuring the label of LPR. We treat GERD and LPR similarly when considering surgical treatment.


Recurrent symptoms after treatment

Yole: Hi, i had a Heller’s myotomy eight years ago and after some painful troubles for the first two years after surgery, everything went well till few months ago. Five months ago I started to suffer of painful flare up of arthritis in my wrists hands and knees and at the same time the number quick spasms and acid reflux episodes are increased. Also at the same time I started again to choke on food very often and sometimes also saliva instead of go down chokes and come back up. Question 1) Are the two problems somehow connected? Question 2) is it normal that after some years the peristalsis failure get worse? Question 3) Can this worsening be at cause of menopause? (Whereas I am very careful to follow the achalasia nutritional protocol and to keep all the appropriate precautions, such as: eating small bites, chewing for a long time, eating wet food, etc. I do daily supplementation of Magnesium, Vitamin D and E, and 1 HCL betaine + Pepsin tablet at each meal due to low acidity in the stomach. Thanks

Scott Gabbard, MD: Your docs should consider using manometry, timed barium esophagram, and endoscopy to help determine the cause of difficulty swallowing. If you have recurrent achalasia, options would be POEM or pneumatic dilation – we have great experience with each option after Heller I worry about using Pepsin in someone with achalasia – this may cause damage to the lining of the esophagus. I would make sure no erosive disease on endoscopy.

Siva Raja, MD, PhD: Any time we see patients with arthritis, we see patients on high doses of NSAIDs, this can increase acid production and worsen symptoms. Make sure that maximal acid suppression is considered and endoscopy is used to insure there is no damage to the esophagus due to acid reflux. This test will also tell you if there is a problem with your lower sphincter which may be the source of your symptoms.

looloo: Fifteen years ago I had a laparoscopic heller myotomy with wrap. In the past month - everything seems to be falling apart. I am back to where I was before surgery not able to eat or drink. Surgeon wants to under the myotomy I had before. What is next step in this situation?

Scott Gabbard, MD: Your docs should consider using manometry, timed barium esophagram, and endoscopy to help determine the cause of difficulty swallowing. If you have recurrent obstruction after Heller/Dor, options would be POEM or pneumatic dilation (30-40mm balloon, not regular 20mm balloon). It is important to ensure you don’t have sigmoid esophagus, which can be seen on esophagram. If you have severe sigmoid esophagus, treatment may need to be esophagectomy – hopefully this is not the case.

Siva Raja, MD, PhD: If you are having symptoms it is important to establish that the problem is related to the sphincter before embarking on interventional therapies. If the problem is clear, then surgical treatment is reasonable. However, I personally recommend trying one pneumatic dilatation in folks who responded to surgery in the past before performing a repeat myotomy, POEM or otherwise.

marmitetoast: Achalasia diagnosed 2015. Operation 2016. Laparoscopic (Da Vinci Xi) Heller myotomy and Dor semifundoplication. Rapid onset of symptoms with rapid weight loss. 3 unsuccessful balloon dilatations. I am doing really well after the operation. I can eat most foods although I stay away from beef so not really eat meat. On occasional I have Acid reflux my question is can the esophageal muscle try to grow back? As I experience pain in this area. The onset of pain does not have a pattern. So I don't know when it’s going to occur. Should I have another Endoscopy? Last one eight weeks after the operation.

Scott Gabbard, MD: Your docs should consider using manometry, timed barium esophagram, and endoscopy to help determine the cause of difficulty swallowing. If you have recurrent obstruction after Heller/Dor, options would be POEM or pneumatic dilation (30-40mm balloon, not regular 20mm balloon). It is important to ensure you don’t have sigmoid esophagus, which can be seen on esophagram. If you have severe sigmoid esophagus, treatment may need to be esophagectomy – hopefully this is not the case.

Siva Raja, MD, PhD: If you are having worsening symptoms, an endoscopy is reasonable. The esophageal muscle does not grow back but it is a circular muscle whose cut ends could technically be reattached with scar, causing the symptoms. If this is the case, a pneumatic dilation would be a reasonable solution.

marmitetoast: It is again marmitetoast from Switzerland. I was reading once that the cold sore virus could be a factor in Achalasia. Is there any new research to support this?

Scott Gabbard, MD: The exact pathogenesis of achalasia is still not understood. Something triggers loss of the nerves that release nitrous oxide in the lower esophageal sphincter. To my knowledge the exact reason has not been discovered.

Ann: My name is Annie and I suffer from severe Achalasia. I’m from the UK by the way. Sadly for me I was born with a hiatus hernia which seemed to dominate everything resulting in no one medically or surgically picking up the Achalasia. Then by 2012, after countless ops on my hernia to try and stop my symptoms, including three majors, one Angle Chic prosthesis (don’t know if you will have heard of that one) and two Nissan fundoplication’s (both causing further problem) until they finally realized the Achalasia was the main problem. By then I required a gastrostomy tube, in fact begged for it as I’d lost 1/3 of my body weight, and it is still in situ, in fact the problem is worsening. Sadly for me also I haven’t seen a specialist for six years as there is such sketchy care here and it depends purely on the individual specialist’s interest. There are only 6,000 of us over here who obviously suffer from it in various degrees. I therefore ‘depend’ very much on Professor Google and Dr Wikipedia and have found research is going on that 1) recognizes Achalasia also as an autoimmune disease and 2) it can also travel to other parts of the gut. Which I have suspected for some time. I would love to know if any of your specialist know anything about this research. I have also seen that an oesophagus/esophagus has recently been transplanted which will use the patient’s stem cells, again I’d love to ask if any more details have surfaced on this. I hope you don’t mind me writing to you as I’m struggling to really find any answers. I do hope to hear back from you and thank you in anticipation.

Siva Raja, MD, PhD: The research topics mentioned in the email are extensive and beyond the scope of this chat, however, in this case, it would be hard to determine the difference between achalasia and pseudo achalasia due to the history of multiple hiatal hernia operations. At some point no further repair or additional surgery is possible on the esophagus and esophagectomy may be the only option. There are esophageal specialists in the UK who may be able to help if such a thing is necessary. If you want a second opinion, you can come here through our global patient services. Eventually we are in the process of opening Cleveland Clinic London but it is several years away.

neil39: Hi, I am a 39-year-old male and had a heller without fundoplication operation back in 2013-my eating is now ok albeit I do have to drink water. My biggest issue was chest spasms and this is also now more controlled thanks to having Gaviscon tablets every night - I would like to know if there are any long term side effects of having regular Gaviscon? I do notice that when I sleep on my right I get more chance of chest spasms - why is this? Furthermore, my achalasia I've been told has resulted in my esophagus width being enlarged to about 2.8cm when the norm is about 1.5cm - what impact will this have later in life & are there any precautionary measures I can take-i.e. to stop cancers, etc.? In addition, I used to have annual endoscopies for the first three years but have now stopped - is it worth doing any annual checks to ensure the achalasia is not returning (I feel fine). Finally, in what likelihood would the achalasia return? I had my operation when I was 34 and I'm now 39. Many thanks.

Scott Gabbard, MD: Gaviscon is safe to take long term. In my patients, I have used a sleep positioning pillow system that locks patients on the left side and this has been very successful.

Siva Raja, MD, PhD: The heller myotomy treats the symptoms of achalasia but you still have achalasia. If you are having sig reflux you may qualify or benefit from a 180 degree fundoplication to decrease reflux. In a recently published study we noted that in patients with esophageal width greater than 3 cm were at a higher risk for re-interventions. The only thing you should consider being done is to insure there is no obstruction at the level of the sphincter. If you do, then the dilation is likely to be progressive. We recommend endoscopies every five years - yearly is probably not necessary. However timed barium esophagrams every 1 - 2 years is recommended to follow the esophageal dilation.

Reviewed: 11/18

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