Feb 2009 SR1

LECTURE ON

ACHALASIA

S. RAD

With the cases from his own file

Tabriz University of Medical SciencesTabriz-Iran

TECHNIQUE OF EXAMINATION

Apart from clinical manometer no imaging modality compares to fluoroscopic examination with spot-filming for diagnosis of Achalasia.

In fact any functional disorder in gastro-intestinal tract needs to be evaluated studying its state of being in rest and moving modifications. Fluoroscopy, an appropriate means to study movements, can give functional data and is the only one to be implemented for diagnosis in these cases.

Feb 2009 SR2

Feb 2009 SR3

MECHANISM OF PRODUCTION

Achalasia a negative version of chalasia ( wide open gastric cardia with facilitated gastro-esophageal reflux ) means non relaxation of the sphincteric mechanism in the lower esophagus. Absence of the anatomical sphincter in the lower esophagus is compensated with the synergic action of multiple elements to play this role:

The acute angle of the entrance of the lower esophagus into the stomach (namely angle of His), hypertonic circular muscles of the lower esophagus by creating high pressure zone in this area, phrenico-esophageal membrane ( Laimer’s) and freely to and fro moving of the esophageal vestibula in diaphragmatic hiatus surrounded by sling muscles of the diaphragmatic crura, All are collaborating to close the cardia in resting state with the added effect of the gastrin content of the stomach or circulating one to produce tough closure of the cardia to prevent gastro-esophageal reflux.

ETIOLOGY

Cerebral cortex is the dominating commander of the esophageal function. This is done by psychogenic effect or by cranial neural impact on the esophagus, specially vague or pneumo-gastric one (No X) via the ambiguous nucleus of this nerve in the brain as the main factor. This nervous impulse is effectuated by myenteric plexus (Auerbach) of the esophagus itself to autonomic movement of the organ.According to the above-mentioned origins, achalasia may be due to :-Psychogenic disturbance-Vagal transmitting defect, such as seen in vagotomy or Chagas disease.-Lack of myenteric or autonomic nervous plexus of the esophagus itself.

Feb 2009 SR4

The very first sign of the achalasia is the apparition of retention of fluid in its lumen which normally takes no more than 5 or 6 second to empty, subsequently a fluid level in the esopghagus is not seen in standing position, where the gravity should accelerate the stripping function of the organ.To notice this phenomenon it is mandatory to start examination in erect or upright position. This is just the opposite of esophageal involvement in scleroderma ( progressive systemic sclerosis) which demands examination in lying down position to suppress the gravity action of the stagnating fluid to notice the peristalsis only.

Feb 2009 SR5

Air bubble of the stomach is produced by aerophagia or swallowed air. In the case of achalasia stagnation of the fluid inside esophagus prevents air to reach the stomach and so, lack of gastric air bubble or its diminution may be another important sign of the insult.

Feb 20096 SR

Achalasia may be seen at the level of the crico-pharyngeus muscle, called superior achalasia or at the lower end, the ordinary lower achalasia. In any case it is produced by non-relaxation of the sphincters, upper one been a true or anatomical sphinter ( crico-pharyngeus muscle) and a sphincteric mechanism in lower end or sometimes anatomical caused by non-relaxation of the crura sling muscle.A special type of this disorder may also be seen and caused by secondary obtruding factors of the cardia in fact pseudo-achalasia, a justified nomenclature.

Feb 2009 SR7

Feb 2009 SR8Pharyngeal achalasia

Feb 2009 SR9

Cricopharyngeus or UES

There are no stripping waves and inactive peristalsis is

not able to evacuate esophagus . Tubular

esophagus or Ring A is located where the muscular

part transforms to the vestibular region and non-relaxation of the lower end

of the organ affects this point . That is why the

lower end of the esophagus in achalasia appears conical

( Bird’s beak) caused by contractile state of the

circular muscles.Feb 200910 SR

Conical and concentric tapering

of the lower esophagus stands just at the cardia.

Conical and concentric tapering

of the lower esophagus stands just at the cardia.

Feb 200911 SR

Persistent retention because of the inactive

stripping waves despite their force in:

Vigorous achalasia.

Persistent retention because of the inactive

stripping waves despite their force in:

Vigorous achalasia.

Feb 200912 SR

Vigorous achalasia.Vigorous achalasia.

Feb 200913 SR

With previous operation ( Heller type ) there is usually a diverticulum formation at the

cardia.

With previous operation ( Heller type ) there is usually a diverticulum formation at the

cardia.Feb 200914 SR

Deformity due to the previous operation.

Deformity due to the previous operation.

Feb 200915 SR

Before and after operation.

Inefficient operation in advanced cases.

Before and after operation.

Inefficient operation in advanced cases.Feb 200916 SR

Huge epiphrenic diverticulum is the rule

in achalasia.

Huge epiphrenic diverticulum is the rule

in achalasia.Feb 200917 SR

Food retention in epiphrenic diverticulum.Food retention in epiphrenic diverticulum.

Feb 200918 SR

Double epiphrenic diverticulum.

Sorry for the patient’s fore-arm inadvertently overlapping the lower end of the esophagus!

Double epiphrenic diverticulum.

Sorry for the patient’s fore-arm inadvertently overlapping the lower end of the esophagus!

Feb 200919 SR

Huge epiphrenic diverticulum simulating

Heart filled up with food!

Huge epiphrenic diverticulum simulating

Heart filled up with food!

Feb 200920 SR

Diverticulum in achalasia simulating lung tumor.Diverticulum in achalasia simulating lung tumor.

Feb 200921 SR

Achalasia demonstrated in chest CT, only a

morphological evaluation

Achalasia demonstrated in chest CT, only a

morphological evaluation

Feb 200922 SR

Fluid level in the

esophagus.

In CT and barium study. Bird’s beak sign may be shown only

in reformatting

coronal aspect with

MDCT

Fluid level in the

esophagus.

In CT and barium study. Bird’s beak sign may be shown only

in reformatting

coronal aspect with

MDCT

Feb 200923 SR

Achalasia with unusual diverticulum simulating neural tumor.

Achalasia with unusual diverticulum simulating neural tumor.

Feb 200924 SR

CTs of the same patient

Feb 200925 SR

Deviation of dilated esophagus to the right

side:

Men’s socks appearance

Deviation of dilated esophagus to the right

side:

Men’s socks appearance

Feb 200926 SR

Men’s socks appearance.Men’s socks appearance.

Feb 200927 SR

No relevant chest x-ray. There are always exceptions for the rules!

No relevant chest x-ray. There are always exceptions for the rules!

Feb 200928 SR

Paraffinoma due to the oil ingestion

in achalasia to facilitate

swallowing in some way.

Paraffinoma due to the oil ingestion

in achalasia to facilitate

swallowing in some way.

Feb 200929 SR

Esophageal wall seen on the top of

the mediastinal widening is in

favor of achalasia.

Esophageal wall seen on the top of

the mediastinal widening is in

favor of achalasia.

Feb 200930 SR

Operated thoracic transferred stomach usually presents a

thick wall and should not be confused with

achalasia.

Operated thoracic transferred stomach usually presents a

thick wall and should not be confused with

achalasia.

Feb 200931 SR

Odd pattern of the filled up thoracic stomach caused by narrowing of the pylorus or tight hiatus not widened

during operation.

Odd pattern of the filled up thoracic stomach caused by narrowing of the pylorus or tight hiatus not widened

during operation.Feb 200932 SR

Lung abscess simulating cavitating malignancy due to perforate achalasia. Notice fluid level in the esophagus at the plain film, best sign for fluid stagnation.

Lung abscess simulating cavitating malignancy due to perforate achalasia. Notice fluid level in the esophagus at the plain film, best sign for fluid stagnation.

Feb 200933 SR

Lung or mediastinal abscess caused by perforated achalasia.

Lung or mediastinal abscess caused by perforated achalasia.

Feb 200934 SR

Lung abscess caused by repeated aspiration in

achalasia. Notice: esophageal wall in mediastinum and

absence of gastric air bubble.

Lung abscess caused by repeated aspiration in

achalasia. Notice: esophageal wall in mediastinum and

absence of gastric air bubble.

Feb 200935 SR

Pseudo-achalasia due to tumor infiltration of the cardia.Pseudo-achalasia due to tumor infiltration of the cardia.Feb 200936 SR

Different cases of pseudo-

achalasia.

Different cases of pseudo-

achalasia.

Feb 200937 SR

Pseudo-achalasia diagnosed in plain abdominal film.

Pseudo-achalasia diagnosed in plain abdominal film.Feb 200938 SR

Achalasia may occur in children as well:

One-year-old child

Achalasia may occur in children as well:

One-year-old child

One and half-year-oldOne and half-year-old

Feb 200939 SR

Four-year-oldFour-year-old

Seven-year-old Seven-year-old

Feb 200940 SR

Twelve-year-old child having trouble since

infancy.

Twelve-year-old child having trouble since

infancy.

Feb 200941 SR

Onset of malignancy in long standing

achalasia.

Onset of malignancy in long standing

achalasia.

Ninety-year-old patientNinety-year-old patient

Tumor occurrence is almost always above the cardiaTumor occurrence is almost always above the cardia

Feb 200942 SR

Malignancy is located almost always above the cardia.Malignancy is located almost always above the cardia.Feb 200943 SR

ConclusionConclusionAchalasia may be guessed by the absence

of the gastric air bubble on the chest x-rays in clinically suspicious settings. There is no air-fluid level seen on the plain film of the normal esophagus and its apparition is in

favor of achalasia in most of the cases with mediastinal widening. Conical and

concentric tapering of the cardia with reservation of the normal mucosal pattern confirms the diagnosis of the achalasia.

Achalasia may be guessed by the absence of the gastric air bubble on the chest x-rays in clinically suspicious settings. There is no air-fluid level seen on the plain film of the normal esophagus and its apparition is in

favor of achalasia in most of the cases with mediastinal widening. Conical and

concentric tapering of the cardia with reservation of the normal mucosal pattern confirms the diagnosis of the achalasia.

Feb 200944 SR

Feb 200945 SR

THE END