What are the treatment options for achalasia cardia?

Treatment of Achalasia Cardia

For type I and II achalasia, choose between POEM, laparoscopic Heller myotomy with fundoplication, or pneumatic dilation based on shared decision-making and local expertise; for type III achalasia, POEM is the preferred treatment. 1

Diagnostic Workup Required Before Treatment

Before selecting treatment, complete the following evaluation:

• Clinical history with medication review 1
• Upper endoscopy to exclude malignancy and assess mucosal changes 1
• Timed barium esophagram to evaluate esophageal emptying and morphology 1
• High-resolution manometry to classify achalasia subtype (I, II, or III) according to Chicago Classification 1
• Endoscopic functional luminal impedance planimetry as adjunct when diagnosis is equivocal 1

Treatment Selection Algorithm

Type I and II Achalasia

Three equally effective options exist with >90% success rates 1:

POEM (Per-Oral Endoscopic Myotomy):

• Creates submucosal tunnel 10-15 cm proximal to LES, extending 2-4 cm onto gastric cardia 2
• Circular muscle myotomy begins 2-3 cm distal to mucosotomy 2
• Advantages: No abdominal incisions, faster recovery, avoids vagal nerve injury, no intra-abdominal adhesions 2
• Critical caveat: Substantially higher reflux risk with endoscopic/pH-metry evidence of GERD in up to 58% of patients 1, 3, 2
• Erosive esophagitis develops in 23-48% versus 13% after pneumatic dilation 2
• Must be performed by experienced physicians in high-volume centers (20-40 procedures needed for competence) 1

Laparoscopic Heller Myotomy with Fundoplication:

• Requires complete surgical isolation of esophagogastric junction through division of phrenoesophageal ligament and short gastric vessels 3, 2, 4
• Myotomy divides circular and longitudinal muscle layers of LES 3, 4
• Mandatory fundoplication because surgical dissection disrupts anti-reflux mechanisms 3, 2, 4
• Lower reflux rates than POEM due to fundoplication 2
• 94% efficacy with laparoscopic approach versus 84% with open surgery 5

Pneumatic Dilation:

• Graded approach using Rigiflex balloons (3.0,3.5,4.0 cm) achieves symptom improvement in 58-95% 1, 5
• If single session unsuccessful, second and third attempts appropriate before considering surgery 1
• Lower reflux risk (13% erosive esophagitis) compared to POEM 2

Type III Achalasia (Spastic)

POEM is the preferred treatment because longer myotomy is indicated for spastic contractions 1:

• Clinical success rate 98% versus 80.8% for laparoscopic Heller myotomy 2
• Ease of performing extended myotomy length 2
• When expertise available, this should be first-line therapy 1

Patients Not Candidates for Primary Therapies

Botulinum toxin injection into LES:

• Reserved for elderly patients and those at high surgical risk 1
• Modest long-term results with repeated injections often required 1
• Can be performed under endoscopic ultrasound guidance when esophageal varices present 6

Calcium channel blockers and nitrates:

• Only for patients who cannot undergo pneumatic dilation or surgery and those not responding to botulinum toxin 5

Post-Treatment Management

After POEM

Immediate postoperative care:

• Overnight observation with clear liquids if no adverse events 2
• Upper GI contrast study next day to exclude leakage 2
• Same-day discharge possible in select patients meeting discharge criteria 1
• Patients with advanced age, significant comorbidities, poor social support should be admitted 1

Dietary advancement:

• Full-liquid diet for 5-7 days 2
• Then 5-6 small meals daily of low-fiber, low-fat solids 2

Acid suppression (critical):

• Mandatory 8 weeks of proton pump inhibitor therapy to promote mucosal healing 3, 2, 4
• Consider indefinite PPI therapy given reflux affects up to 58% of patients 1, 3, 2, 4
• 31% develop esophagitis at mean 29-month follow-up, including new Barrett's esophagus in some cases 2

Antiemetic therapy:

• Aggressive use of 5-HT3 receptor antagonists plus dexamethasone 3, 4
• Vomiting can disrupt myotomy repair site 3, 4

After Laparoscopic Heller Myotomy

Dietary advancement:

• Similar progression from clear liquids to full liquids for 5-7 days 2
• Then 5-6 small meals of low-fiber, low-fat solids 2

Acid suppression:

• 8 weeks of PPI therapy mandatory 3, 2, 4
• Consider lifelong therapy as reflux occurs in up to 58% despite fundoplication 3, 2, 4

Antiemetic therapy:

• Critical importance as vomiting can disrupt myotomy site and displace fundoplication 3, 4
• Use 5-HT3 receptor antagonists plus dexamethasone 3, 4

After Pneumatic Dilation

Monitoring for perforation:

• Observe closely post-procedure 1
• Suspect perforation if pain, breathlessness, fever, or tachycardia develop 1
• Perform urgent chest x-ray and contrast study if suspected 1
• Can be performed as outpatient in uncomplicated cases 1

Acid suppression:

• Standard-dose PPI more effective than H2 receptor antagonists 1
• Twice-daily PPI dosing may be required if restenosis occurs rapidly 1

Repeat dilation:

• Predictors for repeated dilation include "non-peptic" causes, fibrous strictures, maximum dilator size <14 mm 1
• Weekly dilation until easy passage of >14 mm dilator is common strategy 1

Special Considerations

Esophagogastric outflow obstruction and nonachalasia spastic disorders:

• Undergo comprehensive evaluation with symptom correlation 1
• Evidence for POEM limited; consider only case-by-case after exhausting less invasive approaches 1

Surgical intervention for failed medical therapy:

• Consider for patients requiring frequent dilation despite PPI treatment 1
• Consider for technically difficult dilations 1
• Antireflux surgery may be appropriate 1