ACHALASIA

INTRODUCTION:

SYONONYMOUS:

.CARDIOSPASM.

.MEGA-ESOPHAGUS.

.OESOPHAGEAL DYSTONIA.

DEFINITION;

ACHALASIA IS A MOTILITY DISORDER OF THE OESOPHAGUS CHARACTERIZED BY ABSENCE OF PERISTALSIS AND FAILURE OF RELAXATION OF LOWER ESOPHAGEAL SPHINCTER.

THIS CAUSES OBSTRUCTION AT LEVEL OF OESOPHAGO-GASTRIC JUNCTION.

INCIDENCE

COMMON IN ADULTS (AGE 25-40 YRS).

ONLY 5% IN CHILDREN.

ALSO OCCURS IN ELDERLY IN ASSOCIATION WITH MALIGNANCY.

STRUCTURE OF ESOPHAGUS OESOPHAGUS IS A MUSCULAR TUBE,

ABOUT 25cm LONG,BEGINS FROM THE PHARYNX (C6) AND ENDS AT THE CARDIAC ORIFICE OF STOMACH (T11).

ESOPHAGUS CONSISTS OF FOUR LAYERS.THESES ARE FROM INSIDE OUTWARDS;

1. MUCOSA.2. SUBMUCOSA.3. MUSCULARIS EXTERNA.4. SEROSA.

a.SEROSA b.LONGITUDINAL c.CIRCULAR MSL LAYERSd.SUBMUCOSA e.MUSCULARIS MUCOSA f.MUCOUS MEMBRANEg.STRATIFIED SQUAMOUS EPITHELIUM.

LAYERS OF OESOPHAGUS.

LAYERS OF OESOPHAGUS

PERISTALSIS

DEFINITION:

IT IS A WAVE LIKE CONTRACTIONS OF SMOOTH MUSCLES OF THE ESOPHAGUS THAT PROPEL THE FOOD CONTENTS ONWARD BY ALTERNATIVE CONTRACTION AND RELAXATION.

PATHOPHYSIOLOGY ACHALASIA IS CHARECTERIZED BY

INCREASED LOWER ESOPHAGEAL SPHINCTER PRESSURE,

DECREASED or ABSENT PERISTALSIS IN THE DISTAL PORTION OF THE ESOPHAGUS,

AND LACK OF LES RELAXATION IN RESPONSE TO SWALLOWING.

NEUROTRANSMITTERS IN MYENTERIC PLEXUS MYENTERIC PLEXUS LIES IN B/W

LONGITUDINAL AND CIRCULAR LAYERS.

ACETYLCHOLINE AND SUBSTANCE ‘P’ ARE EXCITATORY NEUROTRANSMITTERS CAUSING CONTRACTIONS OF THE ESOPHAGUS.

NITRIC OXIDE (NO) AND VASOACTIVE

INTESTINAL PEPTIDE (VIP) ARE INHIBITORY NEUROTRANSMITTERS AND RESPONSIBLE FOR RELAXATION OF THE L.E.S AND COORDINATED ESOPHAGEAL PERISTALSIS.

MYENTERIC PLEXUS

MYENTERIC PLEXUS PROVIDES MOTOR INNERVATION TO BOTH MUSCULAR LAYERS,

SECRETOMOTOR INNERVATION TO THE MUCOSA AND

CONTROLS ESOPHAGEAL MOTILITY.

MYENTERIC PLEXUS

AETIOLOGY EXACT CAUSE IS NOT KNOWN. DEGENERATION OF MYENTERIC PLEXUS. THE LOSS OF INHIBITORY NEURONS

ALLOWS UNOPPOSED STIMULATION BY CHOLINERGIC NEURONS,

WHICH LEADS TO FAILURE IN L.E.S RELAXATION AND APERISTALSIS OF DISTAL OESOPHAGUS.

SIGN AND SYMPTOMS DYSPHAGIA. .MORE FOR SOLIDS. .PROGRESSIVE AND WORSENING. .MAY LEAD TO WAIT LOSS.

CHEST PAIN. .INDUCED BY EATING. .RETEROSTERNAL.

INFECTED TEETH AND GUMS DUE TO ACID REFLUX, FERMENTED FOOD AND POOR ORAL HYGIENE.

SIGN AND SYMPTOMS. REGURGITATION OF UNDIGESTED FOOD. .SOME PATIENTS LEARN TO INDUCE IT TO

RELIEVE PAIN.

HEARTBURN.

SENSATION OF FOOD STICKING IN LOWER ESOPHAGUS.

NOCTURNAL COUGH.

SIGN AND SYMPTOMS

LATE FEATURES

INHALATION OF REFLUXED CONTENTS LEADING TO PNEUMONIA.

WEIGHT LOSS.

INVESTIGATIONS

CHEST X-RAY. IT SHOWS DILATED ESOPHAGUS

WITH AIR-FLUID LEVEL IN DISTAL ESOPHAGUS.

BARIUM SWALLOW IN ACHALASIA THE CHARACTERISTIC FEATURES

ARE; DILATED OESOPHAGUS, INCOORDINATED ESOPHAGEAL

CONTRACTIONS, OBSTRUCTION AT THE

OESOPHAGOGASTRIC JUNCTION AND

RATE-TAIL APPEARANCE.

ENDOSCOPY

IT SHOWS NARROWING OR OBSTRUCTION OF DISTAL PART OF THE ESOPHAGUS.

ALSO TO EXCLUDE ORGANIC CAUSE OF OBSTRUCTION.

ESOPHAGEAL MANOMETRY ESOPHAGEAL MANOMETRY TEST

MEASURES THE MOTILITY AND FUNCTION OF THE ESOPHAGUS AND ESOPHAGEAL SPHICTER.

A TUBE IS PASSED THROUGH NOSE INTO THE ESOPHAGUS.

THE PRESSURE OF THE SPHINCTER AND CONTRACTIONS WAVES ARE RECORDED.

THIS PROCEDURE HAS RISKS OF ASPIRATION DUE TO INCREASED SALIVATION.

TREATMENT THERE ARE FOUR TREATMENT

OPTIONS;

1. PHARMACOLOGICAL.

2. FORCEFUL DILATATION.

3. BOTULINUM TOXIN.

4. OESOPHAGEAL MYOTOMY.

PHARMACOLOGICAL TX CALCIUM CHANNEL BLOCKERS LIKE NIFEDIPINE

AND NITRATES LIKE ISOSORBIDE DINITRATE;

AS CALCIUM IONS ARE RESPONSIBLE FOR ACTIVITY OF MYOFIBRILS AND TENTION GENERATION.

C.C.B ACT BY DECREASING CALCIUM ENTRY AND REDUCING THE PRESSURE IN LOWER ESOPHAGEAL SPHINCTER.

THESE BRING ONLY SHORT TERM RELIEF.

SIDE EFFECTS ARE LOW BLOOD PRESSRE AND HEADACHE.

FORCEFUL DILATATION IT IS THE TREATMENT OF CHOICE IN

ADULTS.

PROCEDURE A BALLOON CATHETERA BALLOON CATHETER IS PLACED INTO THE IS PLACED INTO THE

ESOPHAGUS AND FORCEFULLY INFLATING IT ESOPHAGUS AND FORCEFULLY INFLATING IT WITHIN THE LOWER ESOPHAGEAL WITHIN THE LOWER ESOPHAGEAL SPHINCTER.SPHINCTER.

TEAR IS PRODUCCED IN LES WHICH BECOME TEAR IS PRODUCCED IN LES WHICH BECOME RELAX AND ALLOW THE FOOD TO PASS RELAX AND ALLOW THE FOOD TO PASS INTO STOMACH.INTO STOMACH.

IT HAS SUCCESS RATE OF 60-90 %. THE ADVANTAGE OF DILATATION

IS THAT IT CAN BE REPEATED IF SYMPTOMS RETUN.

INCIDENCE OF PERFORATION VARIES FROM 1 TO 5 PERCENT.

BOTULINUM TOXIN BOTULINUM TOXIN IS INJECTED INTO THE

LOWER ESOPHAGEAL SPHINCTER USING ENDOSCOPE AND CAUSES LOCAL RELAXATION.

IT IS EFFECTIVE IN 90% BUT EFFECT WEARS OFF AFTER SEVERAL MONTHS.

50% OF PATIENTS WILL REQUIRE ANOTHER TX WITHIN A YEAR.

SIDE EFFECT IS CHEST PAIN AFTER INJECTION.

SURGICAL PROCEDURE

MYOTOMY THIS INVOLVES A CONTROLLED THIS INVOLVES A CONTROLLED

INCISION INTO THE MUSCLE OF INCISION INTO THE MUSCLE OF LES TO CAUSE IT TO RELAX.LES TO CAUSE IT TO RELAX.

IT HAS A SUCCESS RATE OF UPTO IT HAS A SUCCESS RATE OF UPTO 90%.90%.

HELLER MYOTOMY IT WAS FIRST PERFORMED BY

ERNEST HELLER IN 1913.

IT CAN BE PERFORMED EITHER BY OPEN PROCEDURE, THROUGH THE CHEST (THORACOTOMY) OR THROGH THE ABDOMEN (LAPROTOMY),OR BY LAPROSCOPIC TECHNIQUES.

PROCEDURE

PATIENT POSITION AND PREPARATION

PATIENT IS PLACED IN MODIFIED LITHOTOMY PROCEDURE.

THE SURGEON STANDS BETWEEN THE PATIENT’S LEGS, AND THE ASSISTANT AT THE PATIENT’S LEFT SIDE.

MONITOR SHOULD BE LOCATED ABOVE THE PATIENT’S HEAD.

PORT PLACEMENT

ABDOMINAL ACCESS IS OBTAINED AT THE COSTAL MARGIN OF LEFE UPPER QUADRANT.

PNEUMOPERITONEUM IS ESTABLISHED AND TROCAR IS INSERTED.

FOUR WORKING PORTS ARE THEN PLACED UNDER DIRECT VISUALIZATION.

PROCEDURE

Excellent visualization and exposure is essential to performing a safe and adequate myotomy.

The myotomy is begun approximately 3 cm below the GEJ and an L-shaped hook electrocautery is used to divide the muscle fibers.

Individual muscle fibers are divided by hooking them and applying gentle upward traction.

Bleeding from the muscle or submucosa is controlled with pressure.

Progressive division of the longitudinal and then circular muscle layer is performed as the myotomy is carried superiorly, 6 to 8 cm above the GEJ.

PROCEDURE Thus, the entire myotomy spans

approximately 9 to 11 cm (3cm below to 6cm above the GEJ).

The most difficult dissection involves the 3-cm myotomy on the stomach where the plane of dissection becomes blurred with intervening muscular fibers and the underlying gastric mucosa is thinner which increases the risk of perforation.

Mucosal perforations are repaired with a fine (4-0 or 5-0) absorbable suture.

COMPLICATIONS OF ACHALASIA

WEIGHT LOSS. MALNUTRITION. BREATHLESNESS. PNEUMONIA. CARCINOMA.

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