Feb 2009 SR1

LECTURE LECTURE ONON

ACHALASIAACHALASIA

S. RADS. RAD

With the cases from his own fileWith the cases from his own file

Tabriz University of Medical SciencesTabriz University of Medical SciencesTabriz-IranTabriz-Iran

TECHNIQUE OF EXAMINATIONTECHNIQUE OF EXAMINATION

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

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

Feb 2009 SR2

Feb 2009 SR3

MECHANISM OF PRODUCTIONMECHANISM OF PRODUCTION

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

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

ETIOLOGYETIOLOGY

Cerebral cortex is the dominating commander of the esophageal function. Cerebral cortex is the dominating commander of the esophageal function. This is done by psychogenic effect or by cranial neural impact on the This is done by psychogenic effect or by cranial neural impact on the esophagus, specially vague or pneumo-gastric one (No X) via the esophagus, specially vague or pneumo-gastric one (No X) via the ambiguous nucleus of this nerve in the brain as the main factor. ambiguous nucleus of this nerve in the brain as the main factor. This nervous impulse is effectuated by myenteric plexus (This nervous impulse is effectuated by myenteric plexus (AuerbachAuerbach) of ) of the esophagus itself to autonomic movement of the organ.the esophagus itself to autonomic movement of the organ.According to the above-mentioned origins, achalasia may be due to :According to the above-mentioned origins, achalasia may be due to :-Psychogenic disturbance-Psychogenic disturbance-Vagal transmitting defect, such as seen in vagotomy or-Vagal transmitting defect, such as seen in vagotomy or Chagas Chagas disease.disease.-Lack of myenteric or autonomic nervous plexus of the esophagus itself.-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 bubble of the stomach is produced by aerophagia or swallowed air. In the case of achalasia stagnation of the fluid inside esophagus 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 prevents air to reach the stomach and so, lack of gastric air bubble or its diminution may be another important sign of the insult.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 achalasiaPharyngeal achalasia

Feb 2009 SR9

Cricopharyngeus or Cricopharyngeus or UESUES

There are no stripping There are no stripping waveswaves and inactive peristalsis is and inactive peristalsis is

not able to evacuate not able to evacuate esophagus . 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 Conical and concentric tapering concentric tapering

of the lower of the lower esophagus stands esophagus stands just at the cardia.just at the cardia.

Conical and Conical and concentric tapering concentric tapering

of the lower of the lower esophagus stands esophagus stands just at the cardia.just at the cardia.

Feb 200911 SR

Persistent retention Persistent retention because of the inactive because of the inactive

stripping waves stripping waves despite their force in:despite their force in:

Vigorous achalasia.Vigorous achalasia.

Persistent retention Persistent retention because of the inactive because of the inactive

stripping waves stripping waves despite their force in:despite their force in:

Vigorous achalasia.Vigorous achalasia.

Feb 200912 SR

Vigorous achalasia.Vigorous achalasia.Vigorous achalasia.Vigorous achalasia.

Feb 200913 SR

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

cardia.cardia.

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

cardia.cardia.Feb 200914 SR

Deformity due to the Deformity due to the previous operation.previous operation.

Deformity due to the Deformity due to the previous operation.previous operation.

Feb 200915 SR

Before and after operation.Before and after operation.

Inefficient operation in advanced cases.Inefficient operation in advanced cases.

Before and after operation.Before and after operation.

Inefficient operation in advanced cases.Inefficient operation in advanced cases.Feb 200916 SR

Huge epiphrenic Huge epiphrenic diverticulum is the rule diverticulum is the rule

in achalasia.in achalasia.

Huge epiphrenic Huge epiphrenic diverticulum is the rule diverticulum is the rule

in achalasia.in achalasia.Feb 200917 SR

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

Feb 200918 SR

Double epiphrenic Double epiphrenic diverticulum.diverticulum.

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

Double epiphrenic Double epiphrenic diverticulum.diverticulum.

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

Feb 200919 SR

Huge epiphrenic diverticulum Huge epiphrenic diverticulum simulatingsimulating

Heart filled up with food!Heart filled up with food!

Huge epiphrenic diverticulum Huge epiphrenic diverticulum simulatingsimulating

Heart filled up with food!Heart filled up with food!

Feb 200920 SR

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

Feb 200921 SR

Achalasia demonstrated in Achalasia demonstrated in chest CT, only a chest CT, only a

morphological evaluationmorphological evaluation

Achalasia demonstrated in Achalasia demonstrated in chest CT, only a chest CT, only a

morphological evaluationmorphological evaluation

Feb 200922 SR

Fluid level in Fluid level in the the

esophagus.esophagus.

In CT and In CT and barium barium

study. Bird’s study. Bird’s beak sign beak sign may be may be

shown only shown only in in

reformatting reformatting coronal coronal

aspect with aspect with MDCTMDCT

Fluid level in Fluid level in the the

esophagus.esophagus.

In CT and In CT and barium barium

study. Bird’s study. Bird’s beak sign beak sign may be may be

shown only shown only in in

reformatting reformatting coronal coronal

aspect with aspect with MDCTMDCTFeb 200923 SR

Achalasia with unusual diverticulum simulating neural Achalasia with unusual diverticulum simulating neural tumor.tumor.

Achalasia with unusual diverticulum simulating neural Achalasia with unusual diverticulum simulating neural tumor.tumor.

Feb 200924 SR

CTs of the same patientCTs of the same patient

Feb 200925 SR

Deviation of dilated Deviation of dilated esophagus to the right esophagus to the right

side:side:

Men’s socks Men’s socks appearanceappearance

Deviation of dilated Deviation of dilated esophagus to the right esophagus to the right

side:side:

Men’s socks Men’s socks appearanceappearance

Feb 200926 SR

Men’s socks appearance.Men’s socks appearance.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 Paraffinoma due to the oil ingestion to the oil ingestion

in achalasia to in achalasia to facilitate facilitate

swallowing in swallowing in some way. some way.

Paraffinoma due Paraffinoma due to the oil ingestion to the oil ingestion

in achalasia to in achalasia to facilitate facilitate

swallowing in swallowing in some way. some way.

Feb 200929 SR

Esophageal wall Esophageal wall seen on the top of seen on the top of

the mediastinal the mediastinal widening is in widening is in

favor of achalasia.favor of achalasia.

Esophageal wall Esophageal wall seen on the top of seen on the top of

the mediastinal the mediastinal widening is in widening is in

favor of achalasia.favor of achalasia.

Feb 200930 SR

Operated thoracic Operated thoracic transferred stomach transferred stomach usually presents a usually presents a

thick wall and thick wall and should not be should not be confused with confused with

achalasia.achalasia.

Operated thoracic Operated thoracic transferred stomach transferred stomach usually presents a usually presents a

thick wall and thick wall and should not be should not be confused with confused with

achalasia.achalasia.

Feb 200931 SR

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

during operation.during operation.

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

during operation.during operation.Feb 200932 SR

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

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

Feb 200933 SR

Lung or mediastinal abscess caused by perforated Lung or mediastinal abscess caused by perforated achalasia.achalasia.

Lung or mediastinal abscess caused by perforated Lung or mediastinal abscess caused by perforated achalasia.achalasia.

Feb 200934 SR

Lung abscess caused by Lung abscess caused by repeated aspiration in repeated aspiration in

achalasia.achalasia. Notice: Notice: esophageal wall in esophageal wall in mediastinum and mediastinum and

absence of gastric air absence of gastric air bubble.bubble.

Lung abscess caused by Lung abscess caused by repeated aspiration in repeated aspiration in

achalasia.achalasia. Notice: Notice: esophageal wall in esophageal wall in mediastinum and mediastinum and

absence of gastric air absence of gastric air bubble.bubble.

Feb 200935 SR

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

Different Different cases of cases of pseudo-pseudo-

achalasia.achalasia.

Different Different cases of cases of pseudo-pseudo-

achalasia.achalasia.

Feb 200937 SR

Pseudo-achalasia diagnosed in plain abdominal Pseudo-achalasia diagnosed in plain abdominal film.film.

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

Achalasia may occur in Achalasia may occur in children as well:children as well:

One-year-old childOne-year-old child

Achalasia may occur in Achalasia may occur in children as well:children as well:

One-year-old childOne-year-old child

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

Feb 200939 SR

Four-year-oldFour-year-oldFour-year-oldFour-year-old

Seven-year-old Seven-year-old Seven-year-old Seven-year-old

Feb 200940 SR

Twelve-year-old Twelve-year-old child having child having trouble since trouble since

infancy.infancy.

Twelve-year-old Twelve-year-old child having child having trouble since trouble since

infancy.infancy.

Feb 200941 SR

Onset of malignancy Onset of malignancy in long standing in long standing

achalasia.achalasia.

Onset of malignancy Onset of malignancy in long standing in long standing

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

ConclusionConclusionConclusionConclusionAchalasia may be guessed by the absence Achalasia may be guessed by the absence

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

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

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

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

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

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

Feb 200944 SR

Feb 200945 SR

THE END