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AchalasiaAchalasiaMark H. Tseng MDMark H. Tseng MD

Kings County HospitalKings County HospitalDecember 17, 2004December 17, 2004

Achalasia: overview and Achalasia: overview and mangagementmangagement

Mark H. Tseng MDMark H. Tseng MDKings County HospitalKings County Hospital

December 17, 2004December 17, 2004

AchalasiaAchalasia

I. I. AnatomyAnatomyII. II. Historical BackgroundHistorical BackgroundIII.III. AchalasiaAchalasiaIV. Clinical presentationIV. Clinical presentationV. EvaluationV. EvaluationVI. TreatmentVI. Treatment

AnatomyAnatomy-- esophagusesophagus-- Muscular tube Muscular tube -- Conduit from the pharynx to Conduit from the pharynx to

the stomachthe stomach-- Length is defined anatomically, from Length is defined anatomically, from cricoidcricoid

cartilage to the gastric orificecartilage to the gastric orifice-- Distance from the incisor 40Distance from the incisor 40--45 cm (actual 45 cm (actual

length: M 22length: M 22--28cm F 2cm shorter)28cm F 2cm shorter)-- Passes behind aortic arch and left main Passes behind aortic arch and left main

bronchus.bronchus.-- Enters abdomen through esophageal hiatus Enters abdomen through esophageal hiatus →→ 22--4 cm below the diaphragm4 cm below the diaphragm

AnatomyAnatomy-- esophagusesophagus

Course of the esophagus:Course of the esophagus:-- Neck and upper esophagus: Neck and upper esophagus: left of midlineleft of midline-- MidMid--esophagus: right of esophagus: right of midlinemidline-- Lower esophagus: left of Lower esophagus: left of midlinemidline

Three area of normal Three area of normal constrictions:constrictions:-- CricopharangealCricopharangeal-- Behind the aortic archBehind the aortic arch-- LES (thickening of the circular LES (thickening of the circular muscles ~ 4cm)muscles ~ 4cm)

AnatomyAnatomy-- esophagusesophagus

-- Fixed in position at two places:Fixed in position at two places:

. Upper: firmly attached to the . Upper: firmly attached to the cricoidcricoid cartilagecartilage

. Lower: . Lower: PhrenoPhreno--esophageal ligament to the esophagus which esophageal ligament to the esophagus which provides an airprovides an air-- tight seal between the thoracic and abdominal tight seal between the thoracic and abdominal cavity.cavity.

(lack of fixation throughout its length allows both transverse a(lack of fixation throughout its length allows both transverse and nd longitudinal mobility)longitudinal mobility)

Vascular supplyVascular supply

ARTERIAL SUPPLYARTERIAL SUPPLYUpper Upper →→ superior and inferior thyroid superior and inferior thyroid artery artery

Middle Middle →→ Bronchial arteries and Bronchial arteries and esophageal branches directly from aortaesophageal branches directly from aorta

Lower Lower →→ L inferior L inferior phrenicphrenic and gastricand gastric

VENOUS SUPPLYVENOUS SUPPLYUpper Upper →→ esophageal venous plexus esophageal venous plexus to to azygosazygos veinvein

Lower Lower →→ esophageal branches of esophageal branches of the coronary vein, a tributary of the the coronary vein, a tributary of the portal veinportal vein

StructureStructure-- Consists of 3 layers: Consists of 3 layers: muscularismuscularis externaexterna, ,

submucosasubmucosa, mucosa , mucosa

AchalasiaAchalasia--historical notehistorical note

First described more than 300yrs agoFirst described more than 300yrs agoReferred to as Referred to as cardiospasmcardiospasmThomas Willis (1621Thomas Willis (1621--1675)1675)

Described a pt starving and unable to swallowDescribed a pt starving and unable to swallowConclusion was due to lower esophageal narrowingConclusion was due to lower esophageal narrowingConstructed the first dilatorConstructed the first dilator--made of whale bone made of whale bone and spongeand spongeFirst successful treatment of First successful treatment of achalasiaachalasia

1914: Ernst Heller 1914: Ernst Heller (1877(1877--1964) 1964) ---- First First successful successful cardiomyotomycardiomyotomy

Anterior and posterior Anterior and posterior myotomiesmyotomiesExtending 8cm or more Extending 8cm or more into esophagus and into esophagus and stomachstomach



1918: De 1918: De BruneBrune GroenveldtGroenveldt and and ZaaijerZaaijer ––performed modified Heller performed modified Heller myotomymyotomy

anterior onlyanterior onlyOriginal technique was to excessiveOriginal technique was to excessive

AchalasiaAchalasia

-- Uncommon (0.5Uncommon (0.5--1 in 100,000)1 in 100,000)

-- No sex predilection M=FNo sex predilection M=F

-- Majority between ages 20Majority between ages 20--50s50s

-- Ineffective relaxation of the LES combined with Ineffective relaxation of the LES combined with loss of esophageal peristalsis loss of esophageal peristalsis →→ impaired impaired esophageal emptying and gradual dilatationesophageal emptying and gradual dilatation

-- Decrease or loss of Decrease or loss of myentericmyenteric ganglion cellsganglion cells

-- Slight increase risk of esophageal carcinoma Slight increase risk of esophageal carcinoma (approx. 10yrs earlier than the general population)(approx. 10yrs earlier than the general population)

Achalasia Achalasia -- PresentationPresentation

-- DysphagiaDysphagia -- delayed and progressive delayed and progressive presentation (mean 2 years)presentation (mean 2 years)

-- ExacerabatedExacerabated by emotional stress or cold by emotional stress or cold fluidfluid

-- 6060--90% report spontaneous or forced 90% report spontaneous or forced regurgitation of undigested food regurgitation of undigested food

-- 10% will have pulmonary complication10% will have pulmonary complication

-- Chest pain (Chest pain (≠≠ heartburn) heartburn) -- 3030--50% resolves 50% resolves with with MyotomyMyotomy

Achalasia Achalasia -- DiagnosisDiagnosis

-- CXRCXR: air fluid levels: air fluid levels

-- Barium swallowBarium swallow: dilated esophagus with Bird's beak : dilated esophagus with Bird's beak deformity. (deformity. (pseudoachalasiapseudoachalasia from extrinsic mass may from extrinsic mass may mimic the classic mimic the classic achalasiaachalasia appearance)appearance)

-- ManometryManometry:: gold standardgold standard

. Elevated LES pressure (greater than 35mmHg). Elevated LES pressure (greater than 35mmHg)

. Incomplete sphincter relaxation. Incomplete sphincter relaxation

. Complete absence of peristalsis. Complete absence of peristalsis

-- EndoscopyEndoscopy: dilated esophagus with tightly closed LES : dilated esophagus with tightly closed LES →→ gentle pressure will admit the scope with a "popgentle pressure will admit the scope with a "pop““..

AchalasiaAchalasia

Achalasia Achalasia -- TreatmentTreatment

Palliation of Palliation of dysphagiadysphagia is the keyis the key→→ relieve functional obstruction of distal relieve functional obstruction of distal esophagus esophagus

-- pharmacotherapypharmacotherapy-- botulinumbotulinum toxintoxin-- esophageal dilationesophageal dilation-- operative operative myotomymyotomy

AchalasiaAchalasia-- algorithmalgorithm

Ferguson MK Ann Ferguson MK Ann ThoracThorac SurgSurg 52:336 199152:336 1991


-- Pharmacotherapy:Pharmacotherapy: (poorly absorbed and (poorly absorbed and

short lived, best reserved as adjunct to other therapies)short lived, best reserved as adjunct to other therapies)

-- NitratesNitrates

-- Ca++ channel blockersCa++ channel blockers

-- AnticholinergicsAnticholinergics

-- OpiodsOpiods

BotulinumBotulinum Toxin TherapyToxin Therapy


BotoxBotox injection:injection:-- Bind to cholinergic nerves and irreversibly Bind to cholinergic nerves and irreversibly

inhibit Acetyl inhibit Acetyl CholineCholine release release -- 6060--85% of patient get relief but 50% get 85% of patient get relief but 50% get

recurrent symptoms within 6 months.recurrent symptoms within 6 months.-- EndoscopicallyEndoscopically injectedinjected-- For pt who are not candidates for other For pt who are not candidates for other

therapiestherapies

AchalasiaAchalasia -- TreatmentTreatment

BotoxBotox injection cont.injection cont.-- Advantages: safety, ease of administration, Advantages: safety, ease of administration,

minimal side effectsminimal side effects-- Disadvantages: expensive, need for multiple Disadvantages: expensive, need for multiple

injections, and efficacy decreased with repeated injections, and efficacy decreased with repeated injectioninjection

-- Cause obliteration of the dissection planes Cause obliteration of the dissection planes between between submucosasubmucosa and muscular layer which will and muscular layer which will make subsequent surgery more difficult and make subsequent surgery more difficult and increase risk of perforation.increase risk of perforation.

EakerEaker, Gordon, Vogel. , Gordon, Vogel. UntowardsUntowards effects of esophageal effects of esophageal botulinumbotulinum toxin injection Dig toxin injection Dig DisDis SciSci 19971997

Pneumatic DilatorPneumatic Dilator


Esophageal dilationEsophageal dilation (under (under fluroscopyfluroscopy))

--Standard Standard nonoperativenonoperative therapytherapy

--Break the muscle fibersBreak the muscle fibers--For pts with limited life expectancyFor pts with limited life expectancy--Can have repeated dilatation Can have repeated dilatation --6060--80% success rate, 5yr recurrence rate 50% 80% success rate, 5yr recurrence rate 50% --Efficacy is decreased after second Efficacy is decreased after second dilatation dilatation --Perforation rate ~ 2%Perforation rate ~ 2%--PPI reduces the need for repeat PPI reduces the need for repeat dilatationdilatation

CsendesCsendes A: Late results of a prospective A: Late results of a prospective randomisiedrandomisied study comparing study comparing forceful dilation Gut 30:299, 1989forceful dilation Gut 30:299, 1989

Esophageal Esophageal myotomymyotomy

Achalasia Achalasia –– Surgical tSurgical treatmentreatment-- Excellent results in 90Excellent results in 90--95%95%-- Gold standard Gold standard -- 1914 1914 -- Ernest HellerErnest Heller-- double double myotomymyotomy-- Modified by Modified by ZaaijerZaaijer-- single single myotomymyotomy-- WorldWorld’’s largest experiences largest experience

--Brazil, Brazil, ChagasChagas’’ diseasedisease--endemic endemic --1 in 8 inhabitants, in which 5% develops 1 in 8 inhabitants, in which 5% develops achalasiaachalasia

-- Traditionally transTraditionally trans--thoracic or transthoracic or trans--abdominalabdominal-- Now minimally invasive Laparoscopic / Now minimally invasive Laparoscopic /

ThoracoscopicThoracoscopic-- Robotic Heller Robotic Heller myotomymyotomy

PinottiPinotti HW: Surgical complications of HW: Surgical complications of ChagasChagas’’ss disease World J disease World J surgsurg 19911991

Achalasia Achalasia –– Surgical tSurgical treatmentreatment

Indications:Indications:Younger than 40yrs old (group which PD is <50%effective) Younger than 40yrs old (group which PD is <50%effective) High risk of perforationHigh risk of perforation

Esophageal Esophageal diverticuladiverticulaPrevious surgery of GE junctionPrevious surgery of GE junctionTortuous or dilated distal esophagusTortuous or dilated distal esophagus

Recurrent symptoms despite Recurrent symptoms despite BotoxBotox or PD therapyor PD therapyPersonal choice of therapyPersonal choice of therapy

Lower risk of perforationLower risk of perforationBetter long term outcomeBetter long term outcomeDecrease chance of reDecrease chance of re--interventionintervention

AchalasiaAchalasia –– Surgical treatmentSurgical treatment

Expose mucosal surfaceExpose mucosal surfaceLength of Length of myotomymyotomy

CephaladCephalad: 1: 1--2 cm beyond 2 cm beyond the dilated esophagusthe dilated esophagusCaudal: 1Caudal: 1--2 cm into the 2 cm into the gastric musculature or gastric musculature or when transverse veins are when transverse veins are encounteredencountered

Check for perforationCheck for perforationMeythleneMeythlene blueblueAir Air

Robotic Robotic hellerheller myotomymyotomy

ComplicationsComplications

IntraIntra--opopMucosa perforationMucosa perforation

PostPost--op: op: DysphagiaDysphagia-- adhesion, inadequate adhesion, inadequate myotomymyotomyGERDGERD-- long long myotomymyotomy, nerve damage, nerve damageDelay perforationDelay perforation-- inadequate inadequate myotomymyotomy

Achalasia Achalasia –– Surgical tSurgical treatmentreatment

Which esophageal technique should be Which esophageal technique should be used?used?Any role for antiAny role for anti--reflux procedure?reflux procedure?

TransTrans--thoracicthoracic

Excellent resultExcellent resultLess GERD* compare to transLess GERD* compare to trans--abdominalabdominal* * PhrenoPhreno--esophageal ligament is not disrupted and shorter esophageal ligament is not disrupted and shorter myotomymyotomyNo No fundoplicationfundoplication is necessaryis necessary

FarshadFarshad AbirAbir: surgical treatments of : surgical treatments of achalasiaachalasia: current status and controversies. Digestive surgery 2004: current status and controversies. Digestive surgery 2004

TransTrans--abdominalabdominal

Excellent result Excellent result –– comparable to transcomparable to trans--thoracicthoracicMore GERD*, less More GERD*, less dysphagiadysphagia*Longer *Longer myotomymyotomy onto stomach (3cm)onto stomach (3cm)


LaparoscopicLaparoscopic

Excellent resultExcellent result*Decrease hospital stay (average 42*Decrease hospital stay (average 42--48hrs post48hrs post--op)op)Improve GERD by Improve GERD by antirefluxantireflux procedureprocedure


AchalasiaAchalasia –– Surgical treatmentSurgical treatment

Currently, no prospective randomized trials Currently, no prospective randomized trials comparing the various approaches to comparing the various approaches to myotomymyotomyExcellent resultsExcellent resultsTechnique used should depend on individual Technique used should depend on individual surgeonsurgeon’’s comfort and experiences comfort and experienceAntiAnti--reflux should be performed with reflux should be performed with abdominal approachabdominal approach

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