Overview
Definition:
Epiphrenic diverticulum is a pulsion outpouching of the esophageal wall located in the distal esophagus, typically within 10 cm of the gastroesophageal junction, often associated with a motility disorder
Epiphrenic diverticulectomy with myotomy is a surgical procedure to remove the diverticulum and relieve the underlying esophageal dysmotility by incising the thickened muscle layer.
Epidemiology:
Epiphrenic diverticula are rare, representing about 5-20% of all esophageal diverticula
They predominantly affect middle-aged to elderly individuals, with a slight male predominance
The prevalence is not well-established due to asymptomatic cases, but they are more frequently identified incidentally during investigations for dysphagia or other symptoms.
Clinical Significance:
This condition is significant as it can lead to debilitating symptoms such as dysphagia, regurgitation, chest pain, and aspiration pneumonia
Unmanaged diverticula carry risks of perforation, bleeding, and malignancy (rarely)
Surgical intervention is crucial for symptomatic patients to improve quality of life and prevent complications, making it a key topic for surgical residents preparing for DNB and NEET SS examinations.
Clinical Presentation
Symptoms:
Difficulty swallowing (dysphagia) for solids and liquids
Regurgitation of undigested food, particularly when lying down
Chest pain, often postprandial or exertional, mimicking angina
Persistent cough, especially at night, due to aspiration
Hoarseness
Weight loss
Halitosis.
Signs:
Generally, physical examination may be unremarkable in asymptomatic patients
In symptomatic individuals, cachexia might be present
Auscultation may reveal crackles in the lungs if aspiration pneumonia is present
Palpation of the abdomen is usually normal
Vital signs are typically stable unless acute complications like aspiration pneumonia occur.
Diagnostic Criteria:
Diagnosis is primarily based on imaging
Symptoms suggestive of esophageal dysmotility and/or a distal esophageal outpouching
While no formal diagnostic criteria exist for epiphrenic diverticula, a diagnosis is confirmed by barium esophagography and/or upper endoscopy with manometry demonstrating a distal outpouching and an associated motility disorder like achalasia or diffuse esophageal spasm.
Diagnostic Approach
History Taking:
Detailed history of dysphagia (solids vs
liquids, progression), regurgitation (timing, content), chest pain (character, triggers, relief), aspiration symptoms (cough, recurrent pneumonia), weight loss, and past medical history including gastrointestinal surgeries or motility disorders
Red flags include severe dysphagia, significant weight loss, and signs of aspiration.
Physical Examination:
A complete physical examination focusing on the head and neck for signs of aspiration (e.g., vocal cord paralysis)
Examination of the chest for respiratory findings (crackles)
Abdominal examination to rule out other causes of dysphagia or referred pain
General assessment for nutritional status and signs of chronic illness.
Investigations:
Barium esophagography: The gold standard for diagnosis, showing the diverticulum's size, location, and relation to the GE junction, often revealing abnormal esophageal motility
Upper gastrointestinal endoscopy: To rule out malignancy, assess the extent of mucosal involvement, and evaluate for reflux esophagitis
Esophageal manometry: Essential to identify and characterize the underlying esophageal motility disorder (e.g., achalasia, hypercontractile esophagus), guiding the extent of myotomy
Chest CT scan: To assess for complications like perforation, mediastinitis, or fistulae, and to evaluate mediastinal lymph nodes
24-hour pH monitoring: If reflux symptoms are prominent.
Differential Diagnosis:
Achalasia (especially type III), diffuse esophageal spasm, nutcracker esophagus, peptic stricture, esophageal cancer, Zenker's diverticulum (though typically higher), hiatal hernia, mediastinal masses, cardiac causes of chest pain.
Management
Initial Management:
For asymptomatic or mildly symptomatic patients, conservative management with dietary modifications (soft diet, avoiding trigger foods) and upright posture after meals may be considered
However, due to the risk of complications and the presence of underlying dysmotility, most symptomatic patients require surgical intervention.
Medical Management:
Medical management is generally not curative for epiphrenic diverticula but may be used for symptom control in select cases or in patients unfit for surgery
This can include pharmacologic agents to relax smooth muscle (e.g., calcium channel blockers, nitrates) for underlying motility disorders, but efficacy is limited and short-lived.
Surgical Management:
Surgical indications include symptomatic disease (dysphagia, regurgitation, chest pain), recurrent aspiration, or suspicion of malignancy
The procedure involves: 1
**Diverticulectomy:** Excision of the diverticular sac, usually via a left thoracotomy approach, though laparoscopic or robotic approaches are increasingly utilized
2
**Myotomy:** Incision of the thickened circular and longitudinal muscles of the esophagus distal to the diverticulum and extending into the LES to relieve the underlying motility disorder
The length of myotomy is determined by manometric findings
3
**Fundoplication:** Often performed to prevent gastroesophageal reflux post-myotomy.
Supportive Care:
Preoperative: Nutritional optimization, management of aspiration pneumonia
Postoperative: Intensive monitoring for respiratory complications, anastomotic leak, or bleeding
Pain management
Gradual oral intake advancement
Mobilization
Nutritional support (e.g., nasogastric tube feeding if oral intake is insufficient)
Management of potential GERD.
Complications
Early Complications:
Bleeding, mediastinitis, esophageal perforation, vocal cord paralysis (recurrent laryngeal nerve injury), pneumothorax, atelectasis, aspiration pneumonia, wound infection, anastomotic leak (rare with diverticulectomy itself but a concern with concurrent esophagectomy if performed).
Late Complications:
Gastroesophageal reflux disease (GERD), dysphagia recurrence, stricture formation at the myotomy site or diverticulum closure site, recurrent aspiration, weight loss, retained food in the diverticulum.
Prevention Strategies:
Meticulous surgical technique with careful identification of vital structures
Adequate length of myotomy based on manometry to address the full extent of dysmotility
Careful closure of the diverticulum neck
Performing fundoplication to mitigate reflux
Aggressive postoperative pulmonary toilet and early mobilization.
Prognosis
Factors Affecting Prognosis:
Severity of underlying esophageal dysmotility, presence of aspiration, success of complete diverticulectomy and adequate myotomy, patient's overall health status, and promptness of diagnosis and treatment
Patients with severe achalasia or significant aspiration risk may have a poorer prognosis.
Outcomes:
Symptomatic relief is generally good following successful surgery, with significant improvement in dysphagia and regurgitation in most patients
However, some degree of dysphagia or reflux may persist
Long-term outcomes depend on the resolution of the underlying motility disorder and prevention of complications.
Follow Up:
Regular follow-up appointments are crucial, especially in the early postoperative period, to monitor for complications, assess nutritional status, and evaluate symptom resolution
Long-term follow-up may involve periodic clinical assessments and possibly endoscopy or manometry if symptoms recur or worsen
Patients should be advised on dietary modifications and lifestyle changes to manage potential GERD.
Key Points
Exam Focus:
Epiphrenic diverticula are pulsion diverticula in the distal esophagus associated with motility disorders
The surgical management involves diverticulectomy and myotomy
Left thoracotomy is the traditional approach, but minimally invasive techniques are evolving
Manometry is key to guide myotomy length.
Clinical Pearls:
Always suspect an underlying motility disorder when an epiphrenic diverticulum is found
Consider the length of myotomy based on manometric findings rather than just the diverticulum size
Fundoplication is often necessary to prevent post-operative reflux
Be vigilant for aspiration pneumonia in the postoperative period.
Common Mistakes:
Failure to identify and treat the underlying esophageal motility disorder, leading to persistent or recurrent dysphagia
Performing an inadequate myotomy
Ignoring the risk of GERD post-myotomy
Inadequate pulmonary toilet leading to aspiration complications.