Overview

Definition:
-Heller myotomy with fundoplication is a surgical procedure performed to treat achalasia cardia, a primary esophageal motility disorder characterized by impaired relaxation of the lower esophageal sphincter (LES) and loss of peristalsis in the esophageal body
-The Heller myotomy involves a myotomy (cutting) of the muscle fibers of the LES, while the fundoplication (wrapping of the gastric fundus around the distal esophagus) is performed to prevent gastroesophageal reflux, a common complication after myotomy.
Epidemiology:
-Achalasia cardia is a rare disorder with an estimated incidence of 1 in 100,000 people
-It can occur at any age but is most commonly diagnosed in individuals between the ages of 30 and 60 years
-There is a slight female preponderance.
Clinical Significance:
-Untreated achalasia leads to progressive dysphagia, regurgitation, chest pain, and weight loss, significantly impacting a patient's quality of life
-Long-standing achalasia is also associated with an increased risk of esophageal cancer
-Surgical intervention, such as Heller myotomy with fundoplication, is the mainstay of treatment for symptomatic achalasia, aiming to restore esophageal transit and improve symptom control.

Indications

Surgical Indications:
-Symptomatic achalasia cardia refractory to medical management or pneumatic dilation
-Significant weight loss or malnutrition due to dysphagia
-Recurrent aspiration pneumonia
-Patients seeking definitive treatment for long-standing symptoms
-Failure of previous pneumatic dilation.
Contraindications:
-Severe comorbidities that make surgery too risky
-Advanced achalasia with sigmoid esophagus and severe esophageal dilation
-Active peptic ulcer disease
-Uncontrolled gastroesophageal reflux disease (GERD) without achalasia
-Previous extensive esophageal surgery in the region.

Preoperative Preparation

Diagnostic Workup:
-Barium swallow to assess esophageal dilation, strictures, and Chicago classification
-Esophageal manometry to confirm diagnosis and assess LES pressure and esophageal peristalsis
-Upper endoscopy to rule out mechanical obstruction and obtain biopsies
-Consider CT scan for advanced disease or suspicion of malignancy.
Patient Optimization:
-Nutritional assessment and optimization
-Consultation with a dietitian if significant weight loss is present
-Review of medications, particularly those affecting esophageal motility or LES pressure
-Smoking cessation advice
-Preoperative antibiotics as per institutional protocol
-Anesthesia assessment and optimization.
Informed Consent:
-Detailed discussion with the patient regarding the procedure, its benefits, risks, and potential complications
-Explanation of the need for both myotomy and fundoplication
-Discussion of alternative treatment options
-The need for lifelong follow-up.

Procedure Steps

Surgical Approach:
-Laparoscopic Heller myotomy with Nissen fundoplication is the preferred approach due to reduced morbidity, shorter hospital stays, and faster recovery
-Open transthoracic or transabdominal approaches are reserved for specific complex cases or when laparoscopy is not feasible.
Laparoscopic Heller Myotomy:
-The surgeon makes several small incisions in the abdomen
-The esophagus is dissected, and a longitudinal myotomy is performed on the anterior or posterior aspect of the distal esophagus and LES, extending approximately 4-6 cm proximally
-Care is taken to avoid transecting the esophageal mucosa
-The muscularis mucosa is also partially divided.
Fundoplication:
-A 360-degree Nissen fundoplication is typically performed, where the gastric fundus is mobilized and wrapped around the distal esophagus
-This helps to buttress the myotomy and prevent gastroesophageal reflux
-Other types of partial fundoplication (e.g., Toupet) may be considered in specific situations.
Intraoperative Checks:
-Confirmation of adequate myotomy length
-Assessment of LES relaxation with intraoperative manometry if available
-Check for any mucosal tears
-Confirmation of secure fundoplication
-Leak test with air insufflation or methylene blue dye instillation if a mucosal tear is suspected.

Postoperative Care

Pain Management:
-Adequate analgesia, typically using patient-controlled analgesia (PCA) or intravenous/oral opioids, followed by NSAIDs
-Epidural analgesia may be used in open procedures.
Dietary Advancement:
-Clear liquids are initiated 24-48 hours postoperatively after a trial of air insufflation or a swallow study to rule out leaks
-Diet is gradually advanced to soft, then regular as tolerated, with emphasis on small, frequent meals and avoiding triggers for reflux or dysphagia.
Monitoring:
-Close monitoring for signs of bleeding, infection, anastomotic leak, or bowel obstruction
-Respiratory monitoring to prevent atelectasis and pneumonia
-Early mobilization to prevent deep vein thrombosis and pulmonary complications.
Discharge Criteria:
-Tolerating an oral diet
-Adequate pain control with oral analgesics
-Ambulating independently
-Absence of significant nausea, vomiting, or abdominal distension
-Satisfactory vital signs.

Complications

Early Complications:
-Esophageal leak (most serious)
-Bleeding
-Pneumonia
-Atelectasis
-Wound infection
-Injury to surrounding organs (spleen, liver, diaphragm)
-Gastric outlet obstruction
-Injury to the vagus nerve.
Late Complications:
-Gastroesophageal reflux disease (GERD) despite fundoplication
-Recurrent dysphagia due to incomplete myotomy or scarring
-Esophageal dysmotility
-Gas bloat syndrome (difficulty belching and bloating)
-Diaphragmatic hernia
-Weight loss.
Prevention Strategies:
-Meticulous surgical technique to avoid mucosal injury during myotomy
-Adequate length of myotomy to ensure LES relaxation
-Secure and appropriate fundoplication to prevent reflux
-Postoperative dietary modifications to aid healing and prevent reflux
-Judicious use of proton pump inhibitors (PPIs) if significant reflux occurs
-Close follow-up to identify and manage late complications.

Prognosis

Factors Affecting Prognosis:
-The success rate of Heller myotomy with fundoplication is generally high, with symptom relief reported in 80-95% of patients
-Factors influencing prognosis include the duration and severity of achalasia, surgical technique, completeness of the myotomy, and the skill of the surgeon
-Early diagnosis and intervention lead to better outcomes.
Outcomes:
-Most patients experience significant improvement in dysphagia and regurgitation, leading to weight gain and improved quality of life
-Long-term recurrence of symptoms can occur, necessitating further intervention
-The risk of GERD needs to be managed
-The risk of esophageal cancer remains elevated in the long term compared to the general population.
Follow Up:
-Regular long-term follow-up is crucial to monitor for symptom recurrence, GERD, and to screen for esophageal cancer
-This typically involves clinical assessment and may include intermittent upper endoscopy or manometry
-Patients should be educated on long-term dietary recommendations and lifestyle modifications.

Key Points

Exam Focus:
-Achalasia is a primary esophageal motility disorder of LES relaxation and esophageal peristalsis
-Heller myotomy severs the LES muscle, and fundoplication prevents reflux
-Laparoscopic approach is standard
-Complications include leak, bleeding, and GERD
-Long-term follow-up is vital.
Clinical Pearls:
-Always perform a complete myotomy extending to the gastroesophageal junction and slightly onto the stomach
-A full 360-degree Nissen fundoplication is often preferred to prevent reflux, but partial fundoplications may be considered in patients with significant pre-existing reflux symptoms or when a very floppy esophagus is encountered
-Consider intraoperative manometry to confirm LES relaxation post-myotomy.
Common Mistakes:
-Incomplete myotomy leading to persistent dysphagia
-Esophageal perforation during myotomy
-Inadequate fundoplication leading to severe reflux
-Overly tight fundoplication causing gastric outlet obstruction or dysphagia
-Failure to consider alternative diagnoses like pseudoachalasia.