,*,单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,*,贲门失弛缓症,ACHALASIA,1,贲门失弛缓症ACHALASIA1,Anatomy-esophagus,-Muscular tube-Conduit from the pharynx to the stomach,-Length is defined anatomically,from cricoid cartilage to the gastric orifice,-Distance from the incisor 40-45 cm(actual length:M 22-28cm F 2cm shorter),-Passes behind aortic arch and left main bronchus.,-Enters abdomen through esophageal hiatus 2-4 cm below the diaphragm,2,Anatomy-esophagus-Muscular,Course of the esophagus,-Neck and upper esophagus:,left of midline,-Mid-esophagus:right of,midline,-Lower esophagus:left of,midline,Three area of normal,constrictions:,-Cricopharangeal,-Behind the aortic arch,-LES(thickening of the,Circular muscles 4cm),3,Course of the esophagus3,-Fixed in position at two places:,.Upper:firmly attached to the cricoid cartilage,.Lower:Phreno-esophageal ligament to the esophagus which,provides an air-tight seal between the thoracic and abdominal cavity.,(lack of fixation throughout its length allows both transverse and longitudinal mobility),4,-Fixed in position at two pla,Vascular supply,ARTERIAL SUPPLY,Upper superior and inferior thyroid,artery,Middle Bronchial arteries and,esophageal branches directly from aorta,Lower L inferior phrenic and gastric,VENOUS SUPPLY,Upper esophageal venous plexus,to azygos vein,Lower esophageal branches of,the coronary vein,a tributary of the,portal vein,5,Vascular supplyARTERIAL SUPPL,Structure,-,Consists of 3 layers:muscularis externa,submucosa,mucosa,6,Structure-Consists of 3 laye,Achalasia-historical note,First described more than 300yrs ago,Referred to as cardiospasm,Thomas Willis(1621-1675),Described a pt starving and unable to swallow,Conclusion was due to lower esophageal narrowing,Constructed the first dilator-made of whale bone,and sponge,First successful treatment of achalasia,7,Achalasia-historical noteFirst,Achalasia-historical note,1914:Ernst Heller,(1877-1964)-First,successful,cardiomyotomy,Anterior and posterior,myotomies,Extending 8cm or more,into esophagus and,stomach,8,Achalasia-historical note1914:,Achalasia-historical note,1918:De Brune Groenveldt and Zaaijer,performed modified Heller myotomy,anterior only,Original technique was to excessive,9,Achalasia-historical note1918:,Achalasia,-Uncommon(0.5-1 in 100,000),-No sex predilection M=F,-Majority between ages 20-50s,-Ineffective relaxation of the LES combined with,loss of esophageal peristalsis impaired esophageal emptying and gradual dilatation,-Decrease or loss of myenteric ganglion cells,-Slight increase risk of esophageal carcinoma,(approx.10yrs earlier than the general population),10,Achalasia10,Achalasia-Presentation,-Dysphagia-delayed and progressive presentation(mean 2 years),-Exacerabated by emotional stress or cold fluid,-60-90%report spontaneous or forced regurgitation of undigested food,-10%will have pulmonary complication,-Chest pain(heartburn)-30-50%resolves with Myotomy,11,Achalasia-Presentation11,Achalasia-Diagnosis,-CXR:air fluid levels,-Barium swallow:dilated esophagus with Birds beak deformity.(pseudoachalasia from extrinsic mass may,mimic the classic achalasia appearance),-Manometry:,gold standard,.Elevated LES pressure(greater than 35mmHg),.Incomplete sphincter relaxation,.Complete absence of peristalsis,-Endoscopy:dilated esophagus with tightly closed LES,gentle pressure will admit the scope with a pop,“,.,12,Achalasia-Diagnosis12,Achalasia,13,Achalasia13,Achalasia,14,Achalasia14,Achalasia-Treatment,Palliation of dysphagia is the key,relieve functional obstruction of distal,esophagus,-pharmacotherapy,-botulinum toxin,-esophageal dilation,-operative myotomy,15,Achalasia-TreatmentPalliatio,Achalasia-algorithm,16,Achalasia-algorithm16,Achalasia-Treatment,Pharmacotherapy:(poorly absorbed and,short lived,best reserved as adjunct to other therapies),-,Nitrates,-,Ca+channel blockers,-,Anticholinergics,-,Opiods,17,Achalasia-TreatmentPharmacot,Botulinum Toxin Therapy,18,Botulinum Toxin Therapy18,Achalasia-Treatment,Botox injection:,-Bind to cholinergic nerves and irreversibly,inhibit Acetyl Choline release,-,60-85%of patient get relief but 50%get,recurrent symptoms within 6 months.,-,Endoscopically injected,-,For pt who are not candidates for other,therapies,19,Achalasia-Treatment19,Achalasia-Treatment,Botox injection cont.,-Advantages:safety,ease of administration,minimal side effects,-Disadvantages:expensive,need for multiple,injections,and efficacy decreased with repeated injection,-Cause obliteration of the dissection planes between submucosa and muscular layer which will make subsequent surgery more difficult and increase risk of perforation.,20,Achalasia-Treatment20,Pneumatic Dilator,21,Pneumatic Dilator21,Achalasia-Treatment,Esophageal dilation(under fluroscopy),-Standard nonoperative therapy,-Break the muscle fibers,-For pts with limited life expectancy,-Can have repeated dilatation,-60-80%success rate,5yr recurrence rate 50%,-Efficacy is decreased after second d