Management of AchalasiaManagement of Achalasia

Dennis KY NgoDepartment of Surgery

Prince of Wales Hospital

Joint Hospital Surgical Grand Round

BackgroundGreek term : failure to relaxOne of esophageal motility abnormalitiesCharacterized by

Incomplete relaxation of the lower esophageal sphincter (LES )Aperistalsis of the body of esophagus

Simultaneous low amplitudes esophageal contractionNo apparent esophageal contraction

Due to degeneration of inhibitory neurones in the wall of esophagus, preferentially nitric oxide producing.

Cause is unknown ? Viral infection (VZV or HSV-1) ? Immune-mediated

Class II HLA antigen – DQw1

EpidemiologyIncidence : 0.5 per 100 000Prevalence : < 10 per 100 000

No sex predilectionAge ~ 20-50

Kraichely et al Disease of the Esophagus 2006

CaseF/45Good past healthPresented with acid regurgitation for 5 yearsInitially treated as gastroesophageal reflux disease ( GERD )Refer to us for surgical treatment of GERDFurther questioning : dysphagia symptoms with hold up sensation at lower chest level

F/45Good past healthPresented with acid regurgitation for 5 yearsInitially treated as gastroesophageal reflux disease ( GERD )Refer to us for surgical treatment of GERDFurther questioning : dysphagia symptoms with hold up sensation at lower chest level

SymptomsDysphagia

Both solid and liquid

Regurgitation and heartburnA common presentationOften misdiagnosed as GERD, esp. early achalasia

Delayed clearance – generate lactic acid from retained food residue

Howard et al Gut 1992

Chest painWeight loss

Investigation

Upper Endoscopy (esophagogastroduodenoscopy)First choice of investigation of dysphagia

Mechanical obstructionMalignancy, esp around the lower esophageal sphincter ( pseudoachalasia )

Cues for achalasiaEsophageal dilatation Presence of food residue inside the esophagus

Radiology ( Barium swallow )

Features on Fluoroscopic Barium swallow

“Bird beak” like OGJEsophageal dilatationNon-peristaltic esophagusSigns of aspiration pneumonia

ManometryDiagnostic for achalasiaDiagnostic features :

Incomplete relaxation of LESNormally – to a level < 8 mmHg above the gastric pressure

Aperistalsis of esophagus

Other characteristic features: Elevated resting LES ( > 26 mmHg )Pressurization of esophagus

resting pressure in the esophagus exceeds the resting pressure in the stomach

Spechler et al Gut 2001

Aim of managementCannot reverse the underlying the pathogenesisFocused on reducing the LES pressure

Facilitate the emptying of esophageal content by gravity

Symptomatic control and prevention of end organ damage

Treatment OptionsTreatment Options

Treatment OptionsTreatment Options

Pharmacologic therapyCommonly calcium channel blocker and nitratesPoor results, effects diminish with timeSignificant side effects of hypotension, headache and peripheral edema

NOT Applicable in clinical setting now

Lake et al Alimentary Pharmacology & Therapeutics 2006

Botulinum toxin injectionPotent inhibitor of the release of Acetylcholine

Excitatory influence of LES tone

Balance the action between excitation and inhibition neuronsInjection to LES

Four quadrant mannerTotal 100 U

StudyPt. No.

Symptomatic Improvement % LES

pressure %

No. Tx session

immediate 12m

Pasricha et al 31 90 44 - 1-2

Fishman et al 60 70 36 - 1

D’Onofrio et al 37 84 84 30 1-2

Kolbasnik et al 30 77 65 - 1-3

Annese et al 38 84 - 31 1-2

Cuilliere et al 55 72 - 30 1

Endoscopic dilatationDifferent size of balloon

30mm, 35mm and 40mm

Rigiflex balloon dilator

Long term follow-up result2 large scale long term FU results

Retrospective study on 66 patientsSuccess rate : 85.7% ( 12 weeks after procedure )Cumulative success rate : 74% (5 years), 62%(10 years)21% requiring second dilatationPerforation rate : 4.5 % ( all managed conservatively )

Chan et al Endoscopy 2004

Prospective study on 54 patients40% (5 years) and 36% (10 years)One patient with perforation, managed conservatively

Eckardt et al Gut 2004

Predictors of successOlder ageDecrease in LES pressure > 50% after dilatation

Perforation risk : < 5%Risk of gastroesophageal reflux symptoms ~ 4-16%, can be managed by medical therapy

Eckardt et al Gut 2004

Ghoshal et al Am J Gastroenterol 2004

Botulinum toxin vs DilatationStudyStudy DesignDesign Pt no.Pt no. FUFU Symptomatic Symptomatic

remissionremission Perf.Perf.

Vaezi et al GUT 1999

RCT 20 Dilatation 12m 70% (P<0.05) 5%

22 Botox 32% -

Milaeli et alAPT 2001

RCT 20 Dilatation 12m 53% (P<0.05) 0%

20 Botox 15% -

CardiomyotomyHeller’s myotomy

1914Original description

Anterior and posterior myotomy

CurrentlyLess length of myotomyOnly done anteriorly

Open ( transabdominal or transthoracic )Laparoscopic transabdoLaparoscopic transabdominalminal

Result from Laparoscopic cardiomyotomy

StudyStudy No.No. FUFU Relief of Relief of dysphagiadysphagia

LES LES pressurepressure

Patti Ann Surg 1999 133 28m 93% 30 to 9

mmHg

Tsiaoussis Am J Surg 2007 68 8 year 91% 35 to < 8

mmHg

Controversy 1? Antireflux surgery is needed for cardiomyotomy

Variable incidence of reflux symptoms after cardiomyotomy

Richards et al Ann Surg 2004

05

101520253035404550

GERD Acid exp

HellerHeller+Dor

LES pressure was similar : 13.7mmHg vs 13.9 mmHg

Controversy 2Antireflux surgery is needed in myotomy

? Total or partial

Choice of antireflux surgeryTotal vs partial

Retard the esophageal clearance in a aperistaltic esophagusNot enough pressure for food propagationProgressive dilatation of the esophagus, result in dysphagia again

Favour partial fundoplication

Controvery 3Partial fundoplication for myotomy

? Posterior Partial ( Toupet )? Anterior Partial ( Dor )

Studies on individual performance for laparoscopic Heller myotomy + Dor or Toupet fundoplication

Both have good dysphagia relief together with reflux control

However, lack of randomized controlled trial for comparison

The choice is based on the surgeon’s belief and expertise

Treatment options remaining : Laparoscopic cardiomyotomy with partial fundoplication

Endoscopic balloon dilatation

Lap myotomy vs DiltationOne randomized controlled trial recently

Kostic et al World J Surg 2007

51 patients25 Laparoscopic myotomy +

Toupet fundoplication26 Dilatation

FU for 12 monthsResults :

Symptomatic relief96% (Surgery) 77% (Dilatation)

ConclusionAchalasia sometimes mixed up with gastroesophageal reflux disease

High index of suspicion is needed

Manometry is gold standard for Diagnosis of Achalasia

Treatment options available Surgery vs endoscopic balloon dilatation

Trend more towards to Surgery in good operative risk in view of excellent and durable symptomatic risk with low complication rate

Thank you