Management of AchalasiaManagement of Achalasia

Dennis KY Ngo

Department of Surgery

Prince of Wales Hospital

Joint Hospital Surgical Grand Round

Background

Greek term : failure to relax

One of esophageal motility abnormalities

Characterized byIncomplete relaxation of the lower esophageal sphincter (LES )

Aperistalsis of the body of esophagus Simultaneous low amplitudes esophageal contraction

No 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/45

Good past health

Presented with acid regurgitation for 5 years

Initially treated as gastroesophageal reflux disease ( GERD )

Refer to us for surgical treatment of GERD

Further questioning : dysphagia symptoms with hold up sensation at lower chest level

F/45

Good past health

Presented with acid regurgitation for 5 years

Initially treated as gastroesophageal reflux disease ( GERD )

Refer to us for surgical treatment of GERD

Further questioning : dysphagia symptoms with hold up sensation at lower chest level

SymptomsDysphagia

Both solid and liquid

Regurgitation and heartburnA common presentation

Often misdiagnosed as GERD, esp. early achalasiaDelayed clearance – generate lactic acid from retained food residue

Howard et al Gut 1992

Chest pain

Weight loss

Investigation

Upper Endoscopy (esophagogastroduodenoscopy)

First choice of investigation of dysphagiaMechanical obstruction

Malignancy, 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 OGJ

Esophageal dilatation

Non-peristaltic esophagus

Signs of aspiration pneumonia

ManometryDiagnostic for achalasia

Diagnostic features : Incomplete relaxation of LES

Normally – to a level < 8 mmHg above the gastric pressure

Aperistalsis of esophagus

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

Pressurization of esophagusresting pressure in the esophagus exceeds the resting pressure in the stomach

Spechler et al Gut 2001

Aim of management

Cannot reverse the underlying the pathogenesis

Focused on reducing the LES pressureFacilitate the emptying of esophageal content by gravity

Symptomatic control and prevention of end organ damage

Treatment OptionsTreatment Options

Treatment OptionsTreatment Options

Pharmacologic therapy

Commonly calcium channel blocker and nitrates

Poor results, effects diminish with time

Significant 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 neurons

Injection to LES Four quadrant manner

Total 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 Dilatation

StudyStudy DesignDesign Pt no.Pt no. FUFUSymptomatic Symptomatic remissionremission

Perf.Perf.

Vaezi et al GUT 1999

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

22 Botox 32% -

Milaeli et al

APT 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. FUFURelief 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 2

Antireflux surgery is needed in myotomy

? Total or partial

Choice of antireflux surgery

Total vs partial Retard the esophageal clearance in a aperistaltic esophagus

Not enough pressure for food propagation

Progressive dilatation of the esophagus, result in dysphagia again

Favour partial fundoplication

Controvery 3

Partial 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 Diltation

One randomized controlled trial recentlyKostic 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