贲门失弛缓症的处理

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- en

2、ters abdomen 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 (thickeni

3、ng 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 both t

4、ransverse and longitudinal mobility)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 bran

5、ches of the coronary vein, a tributary 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 conclu

6、sion was due to lower esophageal narrowingn 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 esoph

7、agus and stomachachalasia-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 comb

8、ined with loss 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

9、 presentation (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 sw

10、allow: dilated esophagus with birds beak deformity. (pseudoachalasia from 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 esoph

11、agus with tightly closed lesn gentle pressure will admit the scope with a pop“.achalasiaachalasiaachalasia - treatmentnpalliation of dysphagia is the key relieve functional obstruction of distal esophagusn - pharmacotherapyn - botulinum toxinn - esophageal dilationn - operative myotomyachalasia- alg

12、orithmachalasia - treatmentnpharmacotherapy: (poorly absorbed and short 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 irreversibly inh

13、ibit acetyl choline releasen- 60-85% of patient get relief but 50% get recurrent symptoms within 6 months.n- endoscopically injectedn- for pt who are not candidates for other therapiesachalasia - treatmentnbotox injection cont.n- advantages: safety, ease of administration, minimal side effectsn- dis

14、advantages: expensive, need for multiple injections, 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 - treat

15、mentnesophageal 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 reduc

16、es the need for 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% deve

17、lops 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 previou

18、s surgery of ge junctionn tortuous or dilated distal esophagusn recurrent symptoms despite botox or pd therapynpersonal choice of therapyn lower risk of perforationn better long term outcomen decrease chance of re-interventionachalasia surgical treatmentn expose mucosal surfacen length of myotomyn cephalad: 1-2 cm beyond the dilated esophagusn caudal: 1-2 cm into the gastric musculature or when transverse veins are encounteredn check for perforationn me