Esophageal achalasia
Esophageal achalasia | |
---|---|
Other names | Achalasia cardiae, cardiospasm, esophageal aperistalsis, achalasia |
laparoscopic surgery | |
Symptoms | Anorexia (but willing and trying to eat), inability to swallow food, chest pain comparable to heart attack, lightheadedness, dehydration, excessive vomiting after eating (often without nausea). |
Usual onset | Normally in mid-to-late life, rarely during youth |
Duration | Lifelong |
Types | 1st stage – 2–3 cm dilated,
2nd stage – 4–5 cm dilated, bird beak looking, 3rd stage – 5–7 cm, dilated 4th / Late-stage – 8+ cm dilated, sigmoid |
Causes | Unknown |
Risk factors | Inconclusive, but possibly: history of autoimmune disorders, air-hunger that accompanies anxiety, faulty eating habits, improper diet |
Diagnostic method | Esophageal manometry, biopsy, X-ray, barium swallow study, endoscopy |
Prevention | No method of prevention |
Treatment | Heller myotomy and fundoplomy, POEM, pneumatic dilation, botulinum toxin |
Prognosis | ~76% chance of survival after 20 years (in a western country such as Germany)[2] |
Frequency | ~1 in 100,000 people[2] |
Deaths | 829 in a period of 1–8 years study out of a 28 demographic, 754 million record pool.[3] |
Esophageal achalasia, often referred to simply as achalasia, is a failure of
Esophageal achalasia is an
Achalasia is characterized by
The most common form is primary achalasia, which has no known underlying cause. It is due to the failure of
Achalasia can also manifest alongside other diseases as a rare syndrome such as achalasia microcephaly.[12]
Signs and symptoms
The main symptoms of achalasia are dysphagia (difficulty in swallowing), regurgitation of undigested food, chest pain behind the sternum, and weight loss.[13] Dysphagia tends to become progressively worse over time and to involve both fluids and solids. Some people may also experience coughing when lying in a horizontal position. The chest pain experienced, also known as cardiospasm and non-cardiac chest pain can often be mistaken for a
Late-stage achalasia
End-stage achalasia, typified by a massively dilated and tortuous oesophagus, may occur in patients previously treated but where further dilatation or myotomy fails to relieve dysphagia or prevent nutritional deterioration, and esophagectomy may be the only option.[14]
End stage disease, characterised by a markedly dilated and tortuous "burned-out" esophagus and recurrent obstructive symptoms, may require oesophageal resection in order to restore gastro-intestinal function, reverse nutritional deficits and reduce the risk of aspiration pneumonia.[15][16][17]
![Image of a surgically removed esophagus due to late-stage Achalasia](http://upload.wikimedia.org/wikipedia/commons/thumb/2/29/Transhiatal_oesophagectomy_specimen.jpg/260px-Transhiatal_oesophagectomy_specimen.jpg)
A review of the literature shows similar results with good symptom control reported in 75–100% of patients undergoing such a procedure. However, oesophagectomy is not without risk, and every patient must be fully informed of all associated risks. Reported mortality rates of 5–10% are described, while morbidity rates of up to 50% have been reported, and
Mechanism
The cause of most cases of achalasia is unknown.
Autopsy and myotomy specimens have, on
Diagnosis
![](http://upload.wikimedia.org/wikipedia/commons/thumb/0/06/AchalasiaCT.jpg/220px-AchalasiaCT.jpg)
Due to the similarity of symptoms, achalasia can be mistaken for more common disorders such as
Barium swallow
Esophageal manometry
![](http://upload.wikimedia.org/wikipedia/commons/thumb/5/51/Achalasia_EMS.jpg/220px-Achalasia_EMS.jpg)
Because of its sensitivity,
Characteristic manometric findings are:[citation needed]
- Lower esophageal sphincter (LES) fails to relax upon wet swallow (<75% relaxation)
- Pressure of LES <26 mm Hg is normal, >100 is considered achalasia, > 200 is nutcracker achalasia.
- Aperistalsis in esophageal body
- Relative increase in intra-esophageal pressure as compared with intra-gastric pressure
Biopsy
Treatment
Lifestyle changes
Both before and after treatment, achalasia patients may need to eat slowly, chew very well, drink plenty of water with meals, and avoid eating near bedtime. Raising the head off the bed or sleeping with a wedge pillow promotes emptying of the esophagus by gravity. After surgery or pneumatic dilatation,
Medication
Drugs that reduce LES pressure are useful. These include
Pneumatic dilatation
In
Surgery
Heller myotomy helps 90% of achalasia patients. It can usually be performed by a
![Image of a stomach which has undergone Fundoplomy, where the Fundus, the upper part of the stomach (similar to the top part of a kidneybean) is wrapped around the attached esophagus and sewn back to the itself](http://upload.wikimedia.org/wikipedia/commons/thumb/a/ad/Fundoplicatio_nach_Nissen_Zeichnung.jpg/220px-Fundoplicatio_nach_Nissen_Zeichnung.jpg)
A partial fundoplication or "wrap", where the fundus (Part of the stomach which hangs above the connection to the oesophagus) is wrapped around said lower oesophagus and sewn to itself, secured to the diaphragm to create pressure on the sphincter post-myotomy, is generally added in order to prevent excessive reflux, which can cause serious damage to the esophagus over time. After surgery, patients should keep to a soft diet for several weeks to a month, avoiding foods that can aggravate reflux.[33] The most recommended fundoplication to complement Heller myotomy is Dor fundoplication, which consists of a 180- to 200-degree
The shortcoming of laparoscopic esophageal myotomy is the need for a fundoplication. On the one hand, the myotomy opens the esophagus, while on the other hand, the fundoplication causes an obstruction. Recent understanding of the gastroesophageal antireflux barrier/valve has shed light on the reason for the occurrence of reflux following myotomy. The gastroesophageal valve is the result of infolding of the esophagus into the stomach at the esophageal hiatus. This infolding creates a valve that extends from 7 o'clock to 4 o'clock (270 degrees) around the circumference of the esophagus. Laparoscopic myotomy cuts the muscle at the 12 o'clock position, resulting in incompetence of the valve and reflux. Recent
Endoscopic myotomy
A new endoscopic therapy for achalasia management was developed in 2008 in Japan.[35] Per-oral endoscopic myotomy or POEM is a minimally invasive type of natural orifice transluminal endoscopic surgery that follows the same principle as the Heller myotomy. A tiny incision is made on the esophageal mucosa through which an endoscope is inserted. The innermost circular muscle layer of the esophagus is divided and extended through the LES until about 2 cm into the gastric muscle. Since this procedure is performed entirely through the patient's mouth, there are no visible scars on the patient's body.[citation needed]
Patients usually spend about 1–4 days in the hospital and are discharged after satisfactory examinations. Patients are discharged on full diet and generally able to return to work and full activity immediately upon discharge.
POEM has been performed on over 1,200 patients in Japan and is becoming increasingly popular internationally as a first-line therapy in patients with achalasia.[38]
Monitoring
Even after successful treatment of achalasia, swallowing may still deteriorate over time. The esophagus should be checked every year or two with a timed barium swallow because some may need pneumatic dilatations, a repeat myotomy, or even esophagectomy after many years. In addition, some physicians recommend pH testing and endoscopy to check for reflux damage, which may lead to a premalignant condition known as Barrett's esophagus or a stricture if untreated.[citation needed]
History of the understanding and treatment of achalasia
- In 1672, the English physician whale bone.
- In 1881, the German Polish-Austrian physician Johann Freiherr von Mikulicz-Radecki described the disease as cardiospasm, and felt it was a functional problem rather than a mechanical one.
- In 1913, Ernest Heller became the first person to successfully perform an esophagomyotomy, now known in his namesake as the Heller myotomy.[39]
- In 1929, two physicians – Hurt and Rake – figured out that the problem was due to the LES not relaxing. They named the disease achalasia, meaning inability to relax.
- In 1937, F.C. Lendram affirmed the conclusions of Hurt and Rake, forwarding the term achalasia over cardiospasm. (Hard to say who really changed the name between the last two entries) In 1937, the physician F.C. Lendram affirmed the conclusions of Hurt and Rake in 1929, forwarding the usage of the term achalasia over cardiospasm.
- In 1955, the German physician Rudolph Nissen, a student of Ferdinand Sauerbruch, performs the first fundoplomy, now known in his namesake as the Nissen fundoplication, eventually publishing the results of two cases in a 1956 copy of Swiss Medical Weekly.[40][41]
- In 1962, the physician Dor reports the first anterior partial fundoplication,[42] acting as a solution to the intense post-surgery GERD, and risk of stomach acid inhalation accompanying Heller myotomy.
- In 1963, the physician Toupet reports first posterior partial fundoplication.[43]
- In 1991, the physician Shimi and his colleagues perform the first laproscopic Heller's in England.
- In 1994, Paricha et al. introduces Botox as a method for reducing LES pressure.[44]
- In 2008, the newest method of surgically treating achalasia, the per-oral endoscopic myotomy, was devised by H. Inoue in Tokyo, Japan.[45] This method is currently considered experimental in many countries such as the United States.
Epidemiology
Incidence of achalasia has risen to approximately 1.6 per 100,000 in some populations. Disease affects mostly adults between ages 30s and 50s.[46]
Notable patients
Planetary scientist Carl Sagan had achalasia from the age of 18.[47] The Zambian government announced that the President of Zambia Edgar Lungu has achalasia, bearing symptoms that sometimes occur during official engagements, in particular lightheadedness.[48]
References
- ^ ACHALASIA | Meaning & Definition for UK English | Lexico.com
- ^ S2CID 235200001.
- PMID 3259530.
- ^ "Achalasia".
- S2CID 33583131.
- PMID 11413123.
- PMID 15633138.
- PMID 25965233.
- ^ PMID 9718057.
- ^ S2CID 243821.
- PMID 20600038.
- S2CID 583462.
- PMID 18614976.
- PMID 21111851.
- PMID 10343255.
- S2CID 8248607.
- PMID 19207550.
- PMID 11565670.
- PMID 2649031.
- PMID 12192319.
- S2CID 30462116.
- ^ Achalasia at eMedicine
- PMID 22529685.
- ISBN 978-0-7817-4733-2.[page needed]
- S2CID 54522267.
- ^ S2CID 25927258.
- S2CID 37740591.
- ^ "Barrett's Esophagus and GERD". 10 October 2017.
- ^ "Nifedipine". NHS UK. 29 August 2018. Retrieved 2021-06-25.
- PMID 25485740.
- PMID 16181839.
- PMID 11981208.
- ^ "Achalasia". The Lecturio Medical Concept Library. 14 October 2020. Retrieved 2021-06-25.
- S2CID 32101221.
- S2CID 25573758.
- PMID 26206634.
- PMID 27020899.
- S2CID 21665171.
- .
- NAID 10008497300.
- S2CID 29470586.
- ISBN 978-3-319-25092-2.
- S2CID 22728462.
- ^ "All About Achalasia". achalasia.us. Archived from the original on 2021-12-05. Retrieved 2021-12-05.
- PMID 20845759.
- PMID 24124325.
- ^ Porco, Carolyn (20 November 1999). "First reach for the stars". The Guardian. Retrieved 14 September 2022.
- ^ "Zambia: President collapses from dizziness during televised ceremony". MSN. 14 June 2021.
External links
- Esophageal achalasia at Curlie
- U.S. Society for Surgery of the Alimentary Tract – Achalasia treatment guidelines