贲门失弛缓症的处理

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1、ACHALASIA Anatomy- 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.

2、 n - Enters abdomen through esophageal hiatus 2-4 cm below the diaphragm n Course of the esophagusn - Neck and upper esophagus: left of midlinen - Mid-esophagus: right of midlinen - Lower esophagus: left of midlinen Three area of normal constrictions:n - Cricopharangeal n - Behind the aortic archn -

3、 LES (thickening 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 whichn provides an air- tight seal between the thoracic and abdominal cavity.n (lack of fixation throughout its

4、length allows both transverse and longitudinal mobility) Vascular supplyn ARTERIAL SUPPLYn Upper superior and inferior thyroid arteryn Middle Bronchial arteries and esophageal branches directly from aortan Lower L inferior phrenic and gastricn VENOUS SUPPLYn Upper esophageal venous plexus to azygos

5、vein n Lower esophageal branches of the coronary vein, a tributary of the portal vein Structuren - Consists of 3 layers: muscularis externa, submucosa, mucosa Achalasia-historical noten First described more than 300yrs agon Referred to as cardiospasmn Thomas Willis (1621-1675)n Described a pt starvi

6、ng and unable to swallown Conclusion was due to lower esophageal narrowingn Constructed the first dilator-made of whale bone and sponge n First successful treatment of achalasia Achalasia-historical noten 1914: Ernst Hellern (1877-1964) - First successful cardiomyotomyn Anterior and posterior myotom

7、iesn Extending 8cm or more into esophagus and stomach Achalasia-historical noten 1918: De Brune Groenveldt and Zaaijer performed modified Heller myotomyn anterior onlyn Original technique was to excessive Achalasian - Uncommon (0.5-1 in 100,000)n - No sex predilection M=Fn - Majority between ages 20

8、-50sn - Ineffective relaxation of the LES combined with loss of esophageal peristalsis impaired esophageal emptying and gradual dilatationn - Decrease or loss of myenteric ganglion cells n - Slight increase risk of esophageal carcinoman (approx. 10yrs earlier than the general population) Achalasia -

9、 Presentationn - Dysphagia - delayed and progressive 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 Myotomy

10、 Achalasia - Diagnosisn -CXR: air fluid levelsn - Barium swallow: 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 relaxatio

11、nn . Complete absence of peristalsis n - Endoscopy: dilated esophagus with tightly closed LESn gentle pressure will admit the scope with a pop“. Achalasia Achalasia Achalasia - Treatmentn Palliation of dysphagia is the key relieve functional obstruction of distal esophagusn - pharmacotherapyn - botu

12、linum toxinn - esophageal dilationn - operative myotomy Achalasia- algorithm Achalasia - Treatmentn Pharmacotherapy: (poorly absorbed and short lived, best reserved as adjunct to other therapies)n - Nitratesn - Ca+ channel blockersn - Anticholinergicsn - Opiods Botulinum Toxin Therapy Achalasia - Tr

13、eatmentnBotox 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 months.n - Endoscopically injectedn - For pt who are not candidates for other therapies Achalasia - TreatmentnBotox injection

14、 cont.n - Advantages: safety, ease of administration, minimal side effectsn - Disadvantages: 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

15、more difficult and increase risk of perforation. Pneumatic Dilator Achalasia - Treatmentn Esophageal 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

16、50%n -Efficacy is decreased after second dilatationn -Perforation rate 2% n -PPI reduces the need for repeat dilatation Esophageal myotomy Achalasia Surgical treatmentn - Excellent results in 90-95%n - Gold standardn - 1914 - Ernest Heller- double myotomyn - Modified by Zaaijer- single myotomyn - Wo

17、rlds largest experiencen -Brazil, Chagas disease-endemicn -1 in 8 inhabitants, in which 5% develops achalasian - Traditionally trans-thoracic or trans-abdominal n - Now minimally invasive Laparoscopic /n Thoracoscopicn - Robotic Heller myotomy Achalasia Surgical treatmentn Indications:n Younger than

18、 40yrs old (group which PD is 50%effective)n High risk of perforationn Esophageal diverticulan Previous surgery of GE junctionn Tortuous or dilated distal esophagusn Recurrent symptoms despite Botox or PD therapyn Personal choice of therapy n Lower risk of perforationn Better long term outcomen Decr

19、ease chance of re-intervention Achalasia 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 Meythlene blue n Air Complicationsn Intra-opn Mucosa perforationn Post-op:n Dysphagia- adhesion, inadequate myotomyn GERD- long myotomy, nerve damagen Delay perforation- inadequate myotomy Achalasia Surgical treatmentn Which esophageal technique should be used?n Any role for anti-reflux procedure?