Esophageal Achalasia
Esophageal Achalasia.

Video Endoscopic Sequence 1 of 10.

Esophageal Achalasia.

This 32 year old man has been suffering from esophageal achalasia for about 10 years, which he has managed by meticulously cutting and chewing all his food.

At that time they offered him a surgery, in a public hospital, but he declined it.At endoscopy, copious amounts of foo and liquid were. Seen in the obviously dilated esophagus.

The incidence of achalasia is approximately 1 per 100,000 people per year. Chagas disease may cause a similar disorder due to Trypanosoma Cruzi.           Medline.

The exact cause of achalasia is unknown                                                     Medline

The diagnosis of achalasia should be suspected in anyone complaining of dysphagia for solids and liquids with regurgitation of food and saliva.

The clinical suspicion should be confirmed by a barium esophagram showing smooth tapering of the lower esophagus leading to the closed lower esophageal sphincter (LES), resembling a "bird's beak." Esophageal manometry establishes the diagnosis showing esophageal aperistalsis and insufficient LES relaxation.
All patients should undergo upper endoscopy to exclude pseudoachalasia arising from a tumor at the gastroesophageal junction.

For more endoscopic details, download the video clip by clicking on the endoscopic image. Wait to be downloaded complete then Press Alt and Enter for full screen.
All endoscopic images shown in this Atlas contain video clips.We recommend seeing the video clips in full screen mode.


 

Esophageal Achalasia.

Video Endoscopic Sequence  2 of 10.

Esophageal Achalasia.

Achalasia. Detail of a barium swallow study demonstrating the classic bird's beak deformity of the distal esophagus showing a dilated esophagus.

Achalasia is an esophageal motor disorder characterized by incomplete relaxation of the lower esophageal sphincter and by the absence of esophageal peristalsis.

Progressive dysphagia and regurgitation can compromise oral intake and lead to malnutrition and weight loss. Treatment of moderate to severe cases of achalasia involves either balloon dilation or myotomy.

  Medline.

Barium Swallow, Esophageal Achalasia.

Video Endoscopic Sequence 3 of 10.

 Barium Swallow, Esophageal Achalasia.

Barium sulfate is a metallic compound that shows up on x-ray and is used to help see abnormalities in the esophagus and stomach.

When taking the test, you drink a preparation containing this solution. The x-rays track its path through your digestive system.

A simple chest x-ray may reveal distortion of the esophagus and absence of air in the stomach, two abnormalities that suggest achalasia.

 Download the video clip by clicking on the Rx image.

Upper GI series, Esophageal Achalasia

Video Endoscopic Sequence  4 of 10.

Upper GI series, Esophageal Achalasia.

These problems can be detected with a barium swallow:

1.Narrowing or irritation of the esophagus (the muscular tube between the back of the throat and the stomach).
2. Disorders of swallowing.
3. Hiatal hernia (an internal defect that causes the stomach to slide partially into the chest).
4. Abnormally enlarged veins in the esophagus that cause bleeding.

5. Ulcers.
6. Tumors.
7. Polyps (growths that are usually not cancerous, but could be precancerous).



Upper GI series, Esophageal Achalasia.

Video Endoscopic Sequence 5 of 10.

Upper GI series, Esophageal Achalasia.

Barium swallow showing the entire length of the esophagus with extreme narrowing of the esophago-gastric junction;There is dilatation, tortuosity, S-shaped bend in the lower oesophagus (sigmoid esophagus). There is a smooth narrowing at the lower end of the esophagus referred to as "bird-beak" appearance. The above appearance is very typical of achalasia of the cardia or cardiospasm.






The bird-beak appearance.

"The bird-beak" appearance

Barium Swallow, Esophageal Achalasia.

Video Endoscopic Sequence 6 of 10.

Barium Swallow, Esophageal Achalasia.

The esophagus appears dilated, and contrast material passes slowly into the stomach as the LES opens intermittently.
The distal esophagus is narrowed and has been described as resembling a bird's beak.

The test shows esophageal dilatation





Achalasia: Pneumatic dilatation under endoscopic guidance.

Video Endoscopic Sequence  7 of 10.

Achalasia: Pneumatic dilatation under endoscopic guidance.

Our patient underwent a dilatation with special ballon used for achalasia. We do not used fluoroscopic control.

Mechanical therapy for achalasia consists of esophageal dilation, the object of which is to disrupt muscle fibers of the LES, effecting a decrease in

LES pressure. Dilation is mostcommonly performed by using pneumatic balloons. The therapy is successful in decreasing LES pressure in 60-80% of patients; however, this change does not always translate into the relief or improvement of symptoms.

Approximately one half of patients experience recurrent symptoms within 5 years. In most of these patients, the disease responds well to repeated dilation therapy.

Esophageal Achalasia: Pneumatic dilatation under endoscopic guidance.

Video Endoscopic Sequence 8 de 10.

Esophageal Achalasia: Pneumatic dilatation under endoscopic guidance.

Pneumatic balloon dilation is considered by many to be the treatment of choice for achalasia of the esophagus.

Dilation procedures are done as an outpatient procedure using only mild sedation with fentanyl and midazolam. Sedation is kept to true conscious sedation to assist the operator in evaluating severity of dilation induced chest pain.


Medline: Long-term follow-up after pneumatic dilation for
 achalasia cardia: factors associated with treatment failure
 and recurrence.


Esophagusl Achalasia: Pneumatic dilatation under endoscopic guidance.

Video Endoscopic Sequence  9 of 10.

Esophagusl Achalasia: Pneumatic dilatation under endoscopic guidance.

After the esophagus being dilated, we performed a retroflexed maneuver in the esophagus seen the endoscope and the catheter of the balloon.

Pneumatic dilatation is considered to be the first line achalasia, but long-term outcome studies are scarce and by their retrospective design.

Medline: Pneumatic balloon dilatation in achalasia: a prospective comparison of safety and efficacy with different balloon diameters.


 

Esophageal Achalasia: Final status of the dilatation.

Video Endoscopic Sequence  10 of 10.

Esophageal Achalasia: Final status of the dilatation.

We have performed 78 pneumatic dilations for achalasia in the past 8 years and have had no complication using this protocol.

One of the patient of 21 year-old female has DownSyndrome Most prospective studies have shown theeffectiveness of pneumatic dilation to be between 60% and80% and comparable to myotomy.


Esophageal Achalasia and Bronchoaspiration.

Esophageal Achalasia and Bronchoaspiration.

 Tracheobronchitis.

In this image and the video clip show a bronchoscopy, performed with a regular GI video endoscope in which is observed, material that came from the esophagus that has a achalasia.

This 78 year-old female 20 years previously underwent a surgery due to Esophageal Achalasia, (Esophagomyotomy of the lower esophageal sphincter (LES) ). Heller esophagomyotomy, since one month she has vomiting and weight.

Esophageal dysfunction progresses through accumulation of ingested material in the superior portion of the esophagus, facilitating the aspiration of this material. This condition is more common in individuals in whom the cough reflex has been lost or suppressed due to neuromuscular disturbances, or due to the use of sedatives or other drugs. In such cases, aspiration pneumonia becomes established.

Pub Med: Surgery for achalasia: long-term results in
 operated achalasic patients.

 

Esophageal Achalasia.

Video Endoscopic Sequence 1 of 16.

Esophageal Achalasia.

A 75 year-old female, who 3 months ago, had started with weight loss and persiting vomiting. A forceful maneuvering of the endoscope had to be done, in order to overcome the sphincter.

The incidence of achalasia is approximately 1 per 100,000 people per year.
Chagas disease may cause a similar disorder due to Trypanosoma Cruzi.

The exact cause of achalasia is unknown.





Esophageal Achalasia.

Video Endoscopic Sequence  2 of 16.

The esophagus is found to be dilated with rest of food. Achalasia is a rare disease of the muscle of the esophagus which is usually diagnosed in young adults.

The term achalasia means "failure to relax" and refers to one of the abnormalities of the esophagus seen in the disease, specifically. the inability of the muscle at the lower end of the esophagus (the lower esophageal sphincter) to open and let food pass into the stomach.

In addition, the muscle of the lower half of the esophagus does not contract normally to propel food down the esophagus and into the stomach.

Both of these abnormalities result in food sticking in the esophagus after it is swallowed.

 

Esophageal Achalasia.

Video Endoscopic Sequence  3 of 16.

Retroflexed view of the fundus shows a cardias so tight, the retroflex maneuver can not be performed into the esophagus.

Esophageal Achalasia.

Video Endoscopic Sequence 4 of 16.


Esofagogram.

 X-ray finding in a patient with achalasia.

Esophageal Achalasia.

Video Endoscopic Sequence  5 of 16.

Pneumatic dilation in achalasia under direct visualization.

The image and the video display a guide wire placed first To introduce the balloon.

A nonsurgical treatment of esophageal achalasia, where the lower esophageal and cardial sphincter is disrupted by overdistension of the circular muscular fibres.

The balloon is positioned over a guide wire and positioned through an endoscope under direct vision.

Esophageal Achalasia.

Video Endoscopic Sequence  6 of 16.

The image and the video display the cardias with the guide wire and the distal tip of the balloon catether.

Esophageal Achalasia.

Video Endoscopic Sequence 7 of 16.

Achalasia Dilator.

Starting with the maneuvers to perform dilatation of the cardias.

Pneumatic dilation is the most common first-line therapy for the treatment of achalasia.

The aim of dilation is a controlled disruption of circular muscle fibres of the lower esophageal sphincter to reduce the functional obstruction.




Esophageal Achalasia.

Video Endoscopic Sequence  8 of 16.

Balloon Dilatation.

The image and the video display the balloon with water insuflation.

Considering the pros and cons of other effective forms of treatment of achalasia (esophagomyotomy and intrasphincteric injection of botulinum toxin), pneumatic dilation is still the treatment of choice in the majority of patients with achalasia.

Balloon Dilatation of the achalasia

Video Endoscopic Sequence 9 of 16.

Balloon Dilatation of the achalasia

The image and the video clip display the balloon in retroflexed image.

 Medline: Short-term and long-term results of endoscopic
 balloon dilation for achalasia: 12 years' experience
.

 

Balloon Dilatation of the achalasia

Video Endoscopic Sequence 10 of 16.

The expansion causes certain perístalsis.

Balloon Dilatation of the achalasia

Video Endoscopic Sequence  11 of 16.

The video clip exhibit more maneuvers.

Achalasia is an esophageal motor disorder characterized by increased lower esophageal sphincter (LES) pressure, diminished-to-absent peristalsis in the distal portion of the esophagus composed of smooth muscle, and lack of a coordinated LES relaxation in response to swallowing.

Primary achalasia is the most common subtype and is associated with loss of ganglion cells in the esophageal myenteric plexus These important inhibitory neurons induce LES relaxation and coordinate proximal-to-distal peristaltic contraction of the esophagus.

Secondary achalasia is relatively uncommon. This condition exists when a process other than intrinsic disease of the esophageal myenteric plexus is the etiology. Examples of maladies causing secondary achalasia include certain malignancies, diabetes mellitus, and Chagas disease.

Balloon Dilatation of the achalasia

Video Endoscopic Sequence  12 of 16.

Achalasia Dilator

The downside is that this balloon is much larger than the balloon dilators normally used to dilate an esophageal stricture or tight hiatal hernia. It has to be big enough to actually rip the tight LES valve and weaken it.

Achalasia

Video Endoscopic Sequence  13 of 16.

Achalasia

 Neuromuscular disorder of the esophagus characterized by a lack of reflex relaxation of the distal esophageal sphincter, normally induced by swallowing.

Achalasia produces a functional obstruction of the esophagus with proximal dilatation. However, the disease involves the entire organ in which the normal neuromuscular disorder peristaltic waves are replaced by simultaneous contractions.

Achalasia occurs mainly in adults and rarely in children.


.

Balloon Dilatation of the achalasia

Video Endoscopic Sequence 14 of 16.

More forceful maneuver to perform dilation Forceful balloon dilation was the mainstay of treatment for achalasia for many years.
In this procedure, a pneumatic balloon is passed down the esophagus, half above and half below the LES.

The balloon is then rapidly inflated for about a minute. As the balloon expands, it forcefully stretches and weakens the LES.
Forceful pneumatic dilatation can be very successful and may last for a decade or longer.


Balloon Dilatation of the achalasia

Video Endoscopic Sequence 15 of 16.

Final status of the dilatation of the cardias.

Best long term results are obtained if the lower esophageal sphincter pressure can be reduced below 10 mm/Hg.



Balloon Dilatation of the achalasia

Video Endoscopic Sequence 16 of 16.

A endoscopic follow up five days after the dilatation.

We underline the simplicity, safety and effectiveness of pneumatic dilatation under direct visualization it should be used as first-line treatment of achalasia, surgery being performed only when dilatation fails.

In conclusion, pneumatic dilatation performed using a consistent technique is effective long-term therapy for achalasia patients of all ages.
Most patients require only one dilatation.


Video Endoscopic Sequence  1 of 7.

Esophageal Achalasia

A 49 year-old female who have been complained of solid food dysphagia and liquids intermittently, weight loss, retroesternal fullness.

The image and video display liquid and food retained as a consequence of aperistalsis.


Video Endoscopic Sequence   2 of 7.

Esophageal Achalasia.

Image shows cardias, the cardias proved to be so tight its appears more like a narrows pylorus.

The patient with achalasia classically present with progressive dysphagia for both solids and liquids, bland regurgitation of food and saliva with chest pain.




Video Endoscopic Sequence 3 of 7.

Achalasia.

It took forceful maneuvering of the endoscope to overcome the sphincter. Pathophysiologic studies of achalasia have primarily identified neural lesion involving loss of ganglion cell within the myenteric plexus, degeneration of the vagus nerve, and changes in the dorsal motor nucleus of the vagus.

These changes result in aperistalsis in the esophageal body impairment of lower esophageal sphincter relaxation.


 

 

Video Endoscopic Sequence  4 of 7.

Achalasia.  

Monilias are observed

Pathophysiology: The exact etiology of achalasia is not known. The most widely accepted current theories implicate autoimmune disorders, infectious diseases, or both.
The last decade has witnessed much progress in the understanding of the cellular and molecular derangements in achalasia.

Degeneration of the esophageal myenteric plexus of Auerbach is the primary histologic finding. However, with early achalasia, a mixed inflammatory infiltrate of T cells, mast cells, and eosinophils is found in association with myenteric neural fibrosis and with a selective loss of inhibitory postganglionic neurons from the Auerbach plexus.

In these patients with early achalasia, neurons of the myenteric plexus are relatively well preserved.



Video Endoscopic Sequence 5 of 7.


Esophageal Achalasia.

Maneuvering with the biopsy forceps troughs the cardias
and taken the biopsy to rule out malignancy.

 

Video Endoscopic Sequence 6 of 7.

Esophageal Achalasia.

Sir Thomas Willis first described achalasia in 1674. Willis successfully treated a patient by dilating the LES with a cork-tipped whalebone.

Not until 1929 did Hurt and Rake first realize that the primary pathophysiology resulting in achalasia was a failure in LES relaxation.

Video Endoscopic Sequence 7 of 7.

Achalasia.

Retroflexed view of the fundus shows a cardias so tight,
the retroflex maneuver can not be performed into the
esophagus

 

 

Video Endoscopic Sequence 1 of 5.

Impaction of foreign bodies (piece of meat) due to a Esophageal Achalasia.

This 65 year-old female presented dysphagia, endoscopy finding: piece of meet impacted in the middle third of the esophagus and spasm of the lower third consistently of esophageal achalasia.

this piece of meat was founded 3 days previously and it persisted in such position

Video Endoscopic Sequence 2 of 5.

Meat Bolus.

With diathermy loop this piece of meat was extracted

Video Endoscopic Sequence 3 of 5.

The GE junction was tight suggestive of motor dysphagia.

Video Endoscopic Sequence 4 of 5.

Balloon dilatation (pneumatic dilatation.

This case the balloon was filled with water instead of air.

Pneumatic dilatation is considered to be the first line therapy for achalasia, but long-term outcome studies are scarce and limited by their retrospective design. There is also no consensus on the optimal method for performing pneumatic dilation as regard to balloon diameter, amount and the rate inflation pressure.

Graded pneumatic balloon dilatation with 30 mm diameter and slower rate of balloon inflation is an effective and safe initial method of therapy for achalasia.

The duration of remission can be extended by repeated dilatation with larger size balloon.

 



Video Endoscopic Sequence  5 of 5

Status after dilatation with balloon .

Estatus posterior a la dilatación con balón

Achalasia is the best understood example of an esophageal motility disorder and characterized by esophageal aperistalsis and impaired relaxation of the lower esophageal sphincter.

The histopathology of achalasia involves inflammation of the myenteric plexus of the esophagus with diminution of ganglion cells. Significant reduction in nitric oxide synthase containing neurons has been demonstrated using immunohistochemical staining.

Autoimmune, neurodegenerative, and viral etiologies have been implicated in the pathogenesis of achalasia. However, the exact cause has yet to be elucidated.

Pharmacologic studies in achalasia patients support the selective loss of inhibitory, itrergic neurons with preservation of cholinergic innervation.

Video Endoscopic Sequence  1 of 11.

This 50 year-old lady presented with progressive dysphagia to solids and liquids.



Video Endoscopic Sequence  2 of 11.

 A "tight" gastroesophageal junction retroflexed image.

Sir Thomas Willis is credited with the first report of a patient with achalasia in 1674.
Von Mikulicz in 1882 and Einhorn in 1888 hypothesized that the disease was due to the absence of opening of the cardia or "cardiospasm." Over the past three centuries, achalasia has emerged as an important model by which to understand the pathophysiology and therapy of motility disorders emanating from a defect in the enteric nervous system. It is the most extensively studied and readily treatable gastrointestinal motor disorder.

This review discusses current concepts in achalasia with an emphasis on the pathophysiology and etiology of the disease. Specific secondary etiologies of achalasia are discussed that provide insight into mechanisms responsible for the neurodegeneration that characterizes the disorder. Diffuse esophageal spasm is also discussed, although there is a paucity of data regarding this condition.

Video Endoscopic Sequence  3 of 11.

Achalasia occurs with an incidence of approximately 1:100,000 with an equal gender distribution.
It occurs at all ages with an increase in incidence observed after the seventh decade.

Dysphagia is the predominant symptom and it is typically accompanied by regurgitation.
Upper endoscopy is often the first test used to evaluate patients with suspected achalasia and may detect esophageal dilatation with retained saliva or food.

A barium esophagram can be highly suggestive of the diagnosis of achalasia, particularly when there is the combination of esophageal dilatation with retained food and barium and a smooth, tapered constriction of the gastroesophageal junction.

Quantitative assessment of the degree of esophageal emptying of barium over time may increase the diagnostic sensitivity of the esophagram for achalasia and serves as a valuable means by which to follow patients response to therapy.



Video Endoscopic Sequence 4 of 11.

The symptoms have a slow onset and progress gradually; many people delay seeking medical attention until symptoms are advanced.

The major symptom is difficulty swallowing (liquids or solids). Some people compensate by eating more slowly and using specific maneuvers, such as lifting the neck or throwing the shoulders back, to improve emptying of the esophagus Other symptoms can include chest pain, regurgitation of swallowed food and liquid, heartburn, difficulty burping, a sensation of fullness or a lump in the throat, hiccups, and weight loss.

 

 


Video Endoscopic Sequence 5 of 11.

Balloon dilatation mechanically stretches the contracted LES.

A Rigiflex balloon with a 30 mm diameter was advanced
over the guide wire with the middle of the balloon
traversing the GE junction


Video Endoscopic Sequence  6 of 11.

Performing balloon dilatation under endoscopic observation as an outpatient procedure is simple, safe and efficacious treating patients with achalasia and referral of surgical myotomy should be considered for patients who do not respond to medical therapy or individuals that do not desire pneumatic dilatations.

The middle of the balloon is generally held about 1 cm above the GE junction as traction of the balloon upon inflation moves the balloon distally into the stomach. Once the balloon is in place, the catheter is held firmly against the bite block to prevent migration of the balloon.

Here, the waist of the balloon is noted, which is at the tight lower esophageal sphincter.

The balloon is inflated slowly until the waist is obliterated, which is usually 7-10 psi. There is no consensus on the duration of inflation. Some centers repeat the dilation to document that the subsequent pressure is lower.

Video Endoscopic Sequence  7 of 11.

The image and the video clip show the GI junction that has been dilated.

Pneumatic dilatation is an effective procedure in the treatment of primary achalasia during the short- and long-term period.


Video Endoscopic Sequence  8 de 11.

The rationale for dilation is to produce a controlled tear of the lower esophageal sphincter, which will result in relief of distal esophageal obstruction.

This is the most effective non-surgical treatment of achalasia

Video Endoscopic Sequence  9 of 11.

The image and the video clip show in retroflexed the balloon in the gastric fundus.

Video Endoscopic Sequence  10 of 11

Balloon dilatation reduces the basal LES pressure by tearing muscle fibers.

Video Endoscopic Sequence  11 of 11.

Status after dilatation with balloon.

 

 

Pseudo Achalasia

Video Endoscopic Sequence 1 of 4.

 Pseudo Achalasia

A 42 year-old man, who presented weight loss of more than 30 pounds and vomiting, during the previous 6 months. At that time, the upper endoscopy performed in a public.

Hospital only explored the esophagus, since they could not get into the stomach due to an obstruction. 3 months after that he was refered to our endoscopic unit and a balloon dilation of esophageal stricture, under direct endoscopic visualization, had to be perform. However, the biopsies were negative to carcinoma.




Pseudo Achalasia

Video Endoscopic Sequence 2 of 4.

The patient did not returned until the date of a new
endoscopy; he had gained 30 pounds but the cardias
needed a new dilatation.


Pseudo Achalasia

Video Endoscopic Sequence 3 of 4.

The image and video display some typical parameters of criteria of a malign ulcer, gastric cardias in retrofled image.



Pseudo Achalasia

Video Endoscopic Sequence 4 of 4.

This image, which did not appear during the previous three months endoscopy. It is a typical neoplastic infiltration.

There are, depressed mucosal surface, abrupt termination of folds, fold tapering and ulceration.

The biopsies were negatives to malignancy.


Achalasia and Esophageal Cancer

Video Endoscopic Sequence 1 of 8.

Achalasia and Esophageal Cancer

This is the case of a 80 year-old male, who has suffered from esophageal achalasia of 20 years of evolution.

Fifteen years before in order to relieve his dysphagia some colleague offered a surgery but he do not accepted.

Reported incidence rates of carcinoma in patients with achalasia and the prevalence of achalasia in patients with esophageal cancer vary widely in the literature. The prognosis of an "achalasia-carcinoma" is generally considered poor, although systematic studies assessing the
incidence, prevalence, and prognosis of patients with "achalasia-carcinoma" are scant.

 

Achalasia and Esophageal Cancer


Achalasia and Esophageal Cancer

Video Endoscopic Sequence 2 of 8.

Achalasia and Esophageal Cancer

The neoplasia is displayed with the endoscope in retroflexed maneuver.

Although the prevalence of patients with achalasia developing an esophageal carcinoma is low the risk is nearly 140-fold; there is no difference in prognosis between patients with achalasia-carcinoma and those with esophageal cancer without achalasia.

Although the gastro-esophageal cancer risk in patients with longstanding achalasia is much higher than in the general population, the absolute risk is rather low. Despite structured endoscopical surveillance, most neoplastic lesions remain undetected until an advanced stage. Efforts
should be made to identify high-risk groups and develop adequate surveillance strategies.

Endoscopy of Achalasia and Esophageal Cancer

Video Endoscopic Sequence 3 of 8.

Endoscopy of Achalasia and Esophageal Cancer

Image shows cardias, the cardias proved to be so tight its appears more like a narrows pylorus.

 

Endoscopy of Achalasia and Esophageal Cancer

Video Endoscopic Sequence 4 of 8.

Endoscopy of Achalasia and Esophageal Cancer

Pneumatic balloon dilation in achalasia and esophageal cancer



Endoscopy of Achalasia and Esophageal Cancer

Video Endoscopic Sequence 5 of 8.

Endoscopy of Achalasia and Esophageal Cancer

Pneumatic balloon dilation in achalasia and esophageal cancer

Image and Video clip of Achalasia and Esophageal Cancer

Video Endoscopic Sequence 6 of 8.

Image and Video clip of Achalasia and Esophageal Cancer

Pneumatic Balloon Dilation in Achalasia

Achalasia and esophageal cancer

Video Endoscopic Sequence 7 of 8.

Achalasia and esophageal cancer

Final status of the dilatation

Achalasia and esophageal cancer

Video Endoscopic Sequence 8 of 8.

Achalasia and esophageal cancer

Some biopsies are taken proven to be a squamous cell carcinoma of the esophagus.

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