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1、ACHALASIAAnatomy-esophagusn-Muscular tube-Conduit from the pharynx to the stomachn-Length is defined anatomically,from cricoid cartilage to the gastric orificen-Distance from the incisor 40-45 cm(actual length:M 22-28cm F 2cm shorter)n-Passes behind aortic arch and left main bronchus.n-Enters abdome
2、n through esophageal hiatus 2-4 cm below the diaphragm nCourse of the esophagusn-Neck and upper esophagus:left of midlinen-Mid-esophagus:right of midlinen-Lower esophagus:left of midlinenThree area of normal constrictions:n-Cricopharangealn-Behind the aortic archn-LES(thickening of the Circular musc
3、les 4cm)n-Fixed in position at two places:n.Upper:firmly attached to the cricoid cartilagen.Lower:Phreno-esophageal ligament to the esophagus whichnprovides an air-tight seal between the thoracic and abdominal cavity.n(lack of fixation throughout its length allows both transverse and longitudinal mo
4、bility)Vascular supplynARTERIAL SUPPLYnUpper superior and inferior thyroid arterynMiddle Bronchial arteries and esophageal branches directly from aortan Lower L inferior phrenic and gastricnVENOUS SUPPLYnUpper esophageal venous plexus to azygos veinnLower esophageal branches of the coronary vein,a t
5、ributary of the portal veinStructuren-Consists of 3 layers:muscularis externa,submucosa,mucosaAchalasia-historical notenFirst described more than 300yrs agon Referred to as cardiospasmn Thomas Willis(1621-1675)n Described a pt starving and unable to swallown Conclusion was due to lower esophageal na
6、rrowingn Constructed the first dilator-made of whale bone and spongen First successful treatment of achalasiaAchalasia-historical noten1914:Ernst Hellern(1877-1964)-First successful cardiomyotomynAnterior and posterior myotomiesn Extending 8cm or more into esophagus and stomachAchalasia-historical n
7、oten1918:De Brune Groenveldt and Zaaijer performed modified Heller myotomynanterior onlynOriginal technique was to excessiveAchalasian-Uncommon(0.5-1 in 100,000)n-No sex predilection M=Fn-Majority between ages 20-50sn-Ineffective relaxation of the LES combined with loss of esophageal peristalsis imp
8、aired esophageal emptying and gradual dilatationn-Decrease or loss of myenteric ganglion cellsn-Slight increase risk of esophageal carcinoman(approx.10yrs earlier than the general population)Achalasia-Presentationn-Dysphagia-delayed and progressive presentation(mean 2 years)n-Exacerabated by emotion
9、al stress or cold fluidn-60-90%report spontaneous or forced regurgitation of undigested foodn-10%will have pulmonary complicationn-Chest pain(heartburn)-30-50%resolves with MyotomyAchalasia-Diagnosisn-CXR:air fluid levelsn-Barium swallow:dilated esophagus with Birds beak deformity.(pseudoachalasia f
10、rom extrinsic mass may mimic the classic achalasia appearance)n-Manometry:gold standardn.Elevated LES pressure(greater than 35mmHg)n.Incomplete sphincter relaxationn.Complete absence of peristalsisn-Endoscopy:dilated esophagus with tightly closed LESn gentle pressure will admit the scope with a pop“
11、.AchalasiaAchalasiaAchalasia-TreatmentnPalliation of dysphagia is the key relieve functional obstruction of distal esophagusn -pharmacotherapyn -botulinum toxinn -esophageal dilationn -operative myotomyAchalasia-algorithmAchalasia-TreatmentnPharmacotherapy:(poorly absorbed and short lived,best reser
12、ved as adjunct to other therapies)n -Nitratesn -Ca+channel blockersn -Anticholinergicsn -OpiodsBotulinum Toxin TherapyAchalasia-TreatmentnBotox injection:n-Bind to cholinergic nerves and irreversibly inhibit Acetyl Choline releasen-60-85%of patient get relief but 50%get recurrent symptoms within 6 m
13、onths.n-Endoscopically injectedn-For pt who are not candidates for other therapiesAchalasia-TreatmentnBotox injection cont.n-Advantages:safety,ease of administration,minimal side effectsn-Disadvantages:expensive,need for multiple injections,and efficacy decreased with repeated injectionn-Cause oblit
14、eration of the dissection planes between submucosa and muscular layer which will make subsequent surgery more difficult and increase risk of perforation.Pneumatic DilatorAchalasia-TreatmentnEsophageal dilation(under fluroscopy)n -Standard nonoperative therapyn -Break the muscle fibersn -For pts with
15、 limited life expectancyn -Can have repeated dilatationn -60-80%success rate,5yr recurrence rate 50%n -Efficacy is decreased after second dilatationn -Perforation rate 2%n -PPI reduces the need for repeat dilatationEsophageal myotomyAchalasia Surgical treatmentn-Excellent results in 90-95%n-Gold sta
16、ndardn-1914-Ernest Heller-double myotomyn-Modified by Zaaijer-single myotomyn-Worlds largest experiencen-Brazil,Chagas disease-endemicn-1 in 8 inhabitants,in which 5%develops achalasian-Traditionally trans-thoracic or trans-abdominaln-Now minimally invasive Laparoscopic/nThoracoscopicn-Robotic Heller myotomyAchalasia Surgical treatmentnIndications:n Younger than 40yrs old(group which PD is 50%effective)n High risk of perforationn Esophageal diverticulan Previous surgery of GE junctionn Tortuous
