Overview
Definition:
Heller myotomy is a surgical procedure involving the division of the circular muscle fibers of the lower esophageal sphincter (LES) and distal esophagus to relieve obstruction caused by spastic esophageal disorders, most commonly achalasia
It is typically performed laparoscopically, often in conjunction with a partial fundoplication to prevent gastroesophageal reflux disease (GERD).
Epidemiology:
Achalasia, the primary indication, affects approximately 1 in 100,000 individuals
Diffuse esophageal spasm (DES) and other spastic disorders are less common
Incidence is relatively uniform across genders, with diagnosis often occurring between the third and sixth decades of life.
Clinical Significance:
Heller myotomy offers a definitive treatment for the debilitating symptoms of spastic esophageal disorders, significantly improving swallowing function and quality of life
It is a cornerstone surgical intervention for achalasia, and understanding its indications, techniques, and potential complications is crucial for surgical residents preparing for DNB and NEET SS examinations.
Clinical Presentation
Symptoms:
Dysphagia (difficulty swallowing), initially to solids, progressing to liquids
Regurgitation of undigested food
Chest pain, often non-cardiac and mimicking angina
Heartburn, which can be a symptom of retained food or reflux post-myotomy
Weight loss due to reduced oral intake
Cough, aspiration, and hoarseness in advanced cases.
Signs:
While physical examination is often unremarkable, advanced cases may show signs of malnutrition or cachexia
Barium swallow may reveal a dilated esophagus with a "bird's beak" or "sigmoid" appearance in achalasia
Esophageal manometry is diagnostic, showing elevated LES pressure, incomplete LES relaxation with swallows, and absent esophageal peristalsis (achalasia) or disorganized contractions (DES).
Diagnostic Criteria:
Diagnosis of achalasia is primarily based on characteristic findings from esophageal manometry and esophagogastroduodenoscopy (EGD)
Manometric criteria for achalasia include: elevated LES basal pressure (>45 mmHg in supine position), incomplete LES relaxation (<2.2 mmHg above baseline) with deglutition, and a 24-hour intraesophageal pressure profile showing elevated end-expiratory pressure
EGD is essential to rule out pseudoachalasia secondary to malignancy.
Diagnostic Approach
History Taking:
Detailed history of dysphagia, including onset, progression, and consistency of food causing difficulty
Characterization of chest pain (quality, duration, aggravating/relieving factors)
Inquiry about regurgitation, weight loss, cough, hoarseness, and aspiration
Previous treatments or investigations for esophageal symptoms.
Physical Examination:
General assessment for nutritional status
Examination of the abdomen for any masses or tenderness
Cardiovascular and respiratory examinations to rule out cardiac or pulmonary causes of chest pain and cough
Oropharyngeal examination for pooling of saliva or food residue.
Investigations:
Esophagogastroduodenoscopy (EGD): To visualize the esophageal mucosa, assess for inflammation, strictures, masses, and to rule out malignancy
may show retained food material
Barium Esophagogram: To assess esophageal diameter, motility, and the characteristic "bird's beak" or "sigmoid" appearance of the LES in achalasia
Esophageal Manometry: Gold standard for diagnosing achalasia and other esophageal motility disorders, quantifying LES pressure, relaxation, and esophageal peristalsis
24-hour pH monitoring: May be used pre- or post-operatively to assess for GERD.
Differential Diagnosis:
Gastroesophageal reflux disease (GERD), peptic stricture, eosinophilic esophagitis, esophageal cancer (primary or metastatic), Schatzki's ring, external compression of the esophagus (e.g., by mediastinal masses or vascular anomalies), Chagas disease (in endemic areas), and other rare esophageal motility disorders.
Management
Initial Management:
For symptomatic relief before definitive treatment: dietary modifications (soft, pureed foods, avoiding triggers), pneumatic dilation of the LES, or long-term medical management with calcium channel blockers or nitrates
However, these are often temporizing measures.
Medical Management:
Pharmacological options are generally less effective and used for patients unsuitable for surgery or dilation
Calcium channel blockers (e.g., nifedipine 10-20 mg orally 30-60 minutes before meals) and nitrates (e.g., isosorbide dinitrate 5-20 mg orally before meals) can provide temporary relief by relaxing the LES
Botulinum toxin injection into the LES is another palliative option, typically lasting for months.
Surgical Management:
Laparoscopic Heller myotomy (LHM) is the gold standard surgical treatment
It involves incising the thickened muscle layers of the distal esophagus and LES
A partial fundoplication (e.g., Dor or Toupet) is almost always performed concurrently to reduce the risk of post-myotomy GERD
Indications include failed medical or dilation therapy, patient preference for surgery, and younger patients seeking durable relief
Contraindications include severe reflux esophagitis, achalasia secondary to malignancy, and significant cardiac disease preventing surgery.
Postoperative Care:
Postoperative care includes pain management, monitoring for complications, and initiation of a gradual diet progression starting with clear liquids
Patients are typically discharged within 1-2 days
Follow-up involves assessment of symptom relief, weight maintenance, and monitoring for GERD
Endoscopic assessment may be considered if symptoms recur or are atypical
Management of potential complications such as GERD and dysphagia post-myotomy is crucial.
Complications
Early Complications:
Bleeding, perforation of the esophagus or stomach, injury to adjacent organs (spleen, diaphragm), intra-abdominal abscess, pneumonia, or atelectasis
Leak from the myotomy or fundoplication site
Dysphagia can sometimes worsen acutely postoperatively due to edema or incomplete relief.
Late Complications:
Gastroesophageal reflux disease (GERD) is the most common late complication, occurring in up to 30-50% of patients, especially without concurrent fundoplication
Recurrent dysphagia due to inadequate myotomy or scarring
Esophagitis
Incisional hernias
In rare cases, achalasia can recur.
Prevention Strategies:
Meticulous surgical technique is paramount
Careful dissection to avoid perforations
Secure closure of any inadvertent defects
Performing a partial fundoplication significantly reduces the risk of GERD
Adequate patient selection and preoperative optimization are also key
Postoperative dietary progression should be managed carefully to avoid stressing the anastomosis.
Prognosis
Factors Affecting Prognosis:
The success of Heller myotomy is generally high, with reported symptom relief rates of 85-95%
Factors influencing prognosis include the specific esophageal disorder (achalasia vs
DES), extent of myotomy, success of fundoplication, and surgeon's experience
Patient adherence to dietary recommendations and follow-up care also plays a role.
Outcomes:
Most patients experience significant and sustained relief from dysphagia and chest pain
Weight gain is common in patients who were previously underweight
Quality of life improves considerably
Long-term studies show durable efficacy of laparoscopic Heller myotomy
Recurrence rates are low but can occur and may require re-intervention.
Follow Up:
Routine follow-up is recommended at 1, 3, 6, and 12 months postoperatively, and annually thereafter, or as needed
This includes clinical assessment of symptoms, weight, and management of any reflux symptoms
Lifestyle modifications and antacid therapy may be necessary for persistent GERD
Patients should be advised to report any recurrent dysphagia or chest pain.
Key Points
Exam Focus:
Laparoscopic Heller myotomy (LHM) is the surgical gold standard for achalasia
Fundoplication is crucial for preventing GERD post-myotomy
Manometry is the definitive diagnostic tool
Distinguish achalasia from pseudoachalasia (due to malignancy)
Recognize complications like GERD and recurrent dysphagia.
Clinical Pearls:
The "bird's beak" sign on barium swallow is classic for achalasia
Always consider malignancy when diagnosing new-onset dysphagia in older patients (pseudoachalasia)
Careful differentiation of spastic disorders is important
Post-operative diet progression is critical for patient recovery and success
Aggressive treatment of GERD is essential.
Common Mistakes:
Forgetting to perform a fundoplication with Heller myotomy, leading to high rates of GERD
Inadequate myotomy resulting in persistent dysphagia
Overly aggressive fundoplication causing a slipped fundoplication or severe dysphagia
Misinterpreting manometry findings or failing to rule out malignancy
Underestimating the importance of long-term GERD management.