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1、贲门失弛缓症 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 bronchu
2、s.n- Enters abdomen through esophageal hiatus 2- 4 cm below the diaphragmnCourse of the esophagusn- Neck and upper esophagus:left of midlinen- Mid-esophagus: right ofmidlinen - Lower esophagus: left of midlinenThree area of normalconstrictions:n- Cricopharangealn- Behind the aortic archn - LES (thic
3、kening of the Circular muscles 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 bo
4、th transverse and longitudinal mobility)Vascular supplynARTERIAL SUPPLYnUpper superior and inferior thyroidarterynMiddle Bronchial arteries andesophageal branches directly from aortan Lower L inferior phrenic and gastricnVENOUS SUPPLYnUpper esophageal venous plexusto azygos veinnLower esophageal bra
5、nches ofthe coronary vein, a tributary of theportal 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 Conclusi
6、on was due to lower esophageal narrowingn Constructed the first dilator-made of whale boneand spongen First successful treatment of achalasiaAchalasia-historical noten1914: Ernst Hellern(1877-1964) - FirstsuccessfulcardiomyotomynAnterior and posteriormyotomiesn Extending 8cm or moreinto esophagus an
7、dstomachAchalasia-historical noten1918: 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 wit
8、hloss of esophageal peristalsis impaired 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 presenta
9、tion (mean 2 years)n- Exacerabated by emotional 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: di
10、lated esophagus with Birds beak deformity. (pseudoachalasia from extrinsic mass maymimic 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
11、tightly closed LESn gentle pressure will admit the scope with a “pop“.AchalasiaAchalasiaAchalasia - TreatmentnPalliation of dysphagia is the key relieve functional obstruction of distalesophagusn - pharmacotherapyn - botulinum toxinn - esophageal dilationn - operative myotomyAchalasia- algorithmAcha
12、lasia - TreatmentnPharmacotherapy: (poorly absorbed andshort lived, best reserved as adjunct to other therapies)n - Nitratesn - Ca+ channel blockersn - Anticholinergicsn - OpiodsBotulinum Toxin TherapyAchalasia - TreatmentnBotox injection:n- Bind to cholinergic nerves and irreversiblyinhibit Acetyl
13、Choline releasen- 60-85% of patient get relief but 50% getrecurrent symptoms within 6 months.n- Endoscopically injectedn- For pt who are not candidates for othertherapiesAchalasia - TreatmentnBotox injection cont.n- Advantages: safety, ease of administration,minimal side effectsn- Disadvantages: exp
14、ensive, need for multipleinjections, and efficacy decreased with repeated injectionn- Cause obliteration of the dissection planes between submucosa and muscular layer which will make subsequent surgery more difficult and increase risk of perforation.Pneumatic DilatorAchalasia - TreatmentnEsophageal
15、dilation (under fluroscopy)n -Standard nonoperative therapyn -Break the muscle fibersn -For pts with 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
16、repeat dilatationEsophageal myotomyAchalasia Surgical treatmentn- Excellent results in 90-95%n- Gold standardn- 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 /
