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Paraesophageal Hernias

Definition & Pathophysiology

  • Paraesophageal hernias result from defects in the diaphragmatic hiatus, allowing abdominal contents to be displaced into the mediastinum.
  • Widening of the hiatus between the left and right diaphragmatic crura provides the pathway for the upward displacement.
  • Complications include life-threatening conditions like gastric volvulus, which can lead to necrosis or perforation of the stomach.

Clinical Considerations

  • Previously, it was recommended to repair all paraesophageal hernias immediately due to potential complications.
  • Recent evidence suggests that a nonsurgical approach is safe for asymptomatic patients.

Symptoms

  • Symptoms can be subtle and include:
    • Chest pain or pressure after meals.
    • Dysphagia for solids.
    • Dyspnea on exertion.
    • Early satiety.
    • Need to eat small meals to avoid discomfort.
    • Anemia (common, resolves with hernia correction).

Surgical Indications

  • Surgical intervention is recommended for patients with symptoms or signs associated with paraesophageal hernia.

Etiology of Hiatal Hernias

  • Hiatal hernias occur when portions of the stomach or abdominal contents herniate into the mediastinum via a defect in the esophageal hiatus.
  • Associated conditions:
    • GERD (Gastroesophageal Reflux Disease): Prevalence and size of the hernia correlate with reflux severity.
    • Nearly 40% of obese patients have hiatal hernias.
  • Causes:
    • Age, stress, degenerative processes on the diaphragm.
    • Most hiatal hernias are acquired, though familial clustering has been reported.

Classification of Hiatal Hernias

Hiatal hernias are classified into four types:

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Type I: Sliding Hiatal Hernia

  • Migration of the gastroesophageal (GE) junction into the posterior mediastinum.
  • Caused by the deterioration of the phrenoesophageal ligament.
  • Forces during swallowing, negative intrathoracic pressure, and positive intraabdominal pressure stretch the ligament.
  • Found in patients with GERD and hiatal hernia due to reduced collagen types I and III.
  • Also known as sliding hiatal hernia.
  • Key feature: GE junction remains above the herniated stomach.

Types II, III, IV: Paraesophageal Hernias

  • Paraesophageal hernias involve the juxtaposition of the stomach and esophagus, where a portion of the stomach herniates into the chest, sometimes accompanied by other abdominal organs.

Type II: Rolling Hiatal Hernia

  • Also called true paraesophageal hernia.
  • Gastric fundus herniates anterior to the esophagus, with a normally positioned GE junction.

Type III: Combined Sliding and Rolling Hernia

  • A combination of Types I and II where both the GE junction and gastric fundus herniate into the mediastinum.

Type IV: Complex Hernia

  • Contains the stomach along with other abdominal organs, such as:
    • Small bowel, colon, pancreas, or spleen.

Giant Paraesophageal Hernia

  • Refers to large hiatal hernias where at least 50% of the stomach is displaced into the mediastinum or the hernia measures at least 6 cm on endoscopy.

Prevalence

  • Actual prevalence is not well known.
  • Type I hiatal hernias account for up to 95% of all hiatal hernias.
  • Paraesophageal hernias may account for up to 14% of all hiatal hernias.
  • Type III paraesophageal hernias are the most common variety.
  • The incidence increases with age and is more common in women.
  • Kyphosis is a recognized risk factor.

Presentation

  • Symptoms can be absent, minor, or debilitating.
  • Common symptoms:
    • GERD symptoms: Heartburn, regurgitation, water brash.
    • Obstructive symptoms: Dysphagia, anemia, respiratory complaints (e.g., dyspnea, COPD, aspiration pneumonia).
    • Other symptoms: Chest pain, bloating, early satiety.
  • Gastritis and Cameron ulcers are common findings on endoscopy.
  • Symptomatic hiatal hernias occur in 1.0% to 4.5% of patients post-esophagectomy.

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1. Incarceration and Strangulation

  • Rare occurrences in paraesophageal hernia patients.
  • Symptoms: Epigastric pain, anterior chest pain.
  • Can lead to obstruction, ischemia, anemia, perforation, or death.
  • Gastric volvulus can occur:
    • Organoaxial (stomach turns on its long axis).
    • Mesenteroaxial (stomach turns on its short axis).
  • Borchardt triad: Chest pain, retching without vomiting, inability to pass a nasogastric tube.
  • Acute gastric volvulus is a surgical emergency.

2. Compression of the Esophagus or Stomach

  • Mechanical compression from a displaced stomach can cause:
    • Chest pain, epigastric pain, retching without emesis.
    • Dysphagia due to esophageal compression.
  • Some patients report relief from acid reflux when they develop symptoms of mechanical obstruction.

3. Anemia

  • About one-third of patients develop chronic anemia.
  • Bleeding can occur from ischemia or ulcers in the herniated stomach.
  • Cameron lesions (single or multiple erosions/ulcerations at the diaphragmatic hiatus):
    • Seen in up to 4.7% of patients with hiatal hernias.
    • Common location: Lesser curve of the stomach.
  • Patients with anemia from paraesophageal hernia have a 7x higher likelihood of linear ulcerations or erosions.
  • Anemia resolves in the majority of patients post-surgical repair.

4. Respiratory Symptoms

  • Dyspnea and cough are common.
  • Symptoms typically worsen throughout the day.
  • GE reflux can present as respiratory complaints (more common in Type II hiatal hernia).
  • Respiratory symptoms may improve post-surgery.
  • Hiatal hernia size inversely correlates with lung capacity (total lung capacity and vital capacity improve after repair).

Diagnostic Approach for Paraesophageal Hernias

1. Initial Evaluation

  • Begins with history and physical examination.
  • Many patients with paraesophageal hernias are asymptomatic.
  • Physical exam findings may include:
    • Decreased breath sounds on the affected side.
    • Presence of bowel sounds in the chest.
  • Chest pain evaluation often leads to further gastrointestinal investigation and diagnosis.
  • Incidental findings of paraesophageal hernias may occur during radiographic or endoscopic evaluations for other conditions.

2. Radiographic Studies

  • Chest Radiographs:
    • Upright chest X-ray may reveal the pathognomonic retrocardiac air-fluid level.
    • Lateral radiographs often demonstrate retrosternal opacities or air-fluid levels.
    • Coiling of a nasogastric tube in the thorax suggests an intrathoracic stomach.

  • Computed Tomography (CT) Scan:

    • Useful for anatomic detail, though not typically used in routine work-up.
    • Helpful in differentiating between paraesophageal hernias and other types of diaphragmatic hernias (e.g., Morgagni hernia, traumatic hernias).

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3. Contrast Esophagography

  • Barium Esophagram:

    • Provides accurate information regarding the hernia’s anatomy and location.
    • Differentiates between Type II and Type III hiatal hernias.
    • Helps assess esophageal peristalsis and reflux.
    • The right anterior oblique technique can improve diagnostic accuracy in patients undergoing bariatric surgery.

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4. Upper Endoscopy (EGD)

  • Used to assess esophageal and gastric pathology such as:
    • Ulceration and mucosal ischemia.
  • Allows for evaluation of the GE junction and the size of the hernia.
  • Retroflexion technique helps better visualize the GE junction and adjacent areas:
    • Helps diagnose Type II hiatal hernia by identifying a second orifice next to the GE junction.
  • Can screen for Barrett esophagus and malignancy, which may influence management.

5. Esophageal Manometry and pH Monitoring

  • Manometry:
    • Not routinely used in paraesophageal hernias due to distorted anatomy from the large hernia.
    • Useful in patients with dysphagia to identify weak esophageal peristalsis.
    • Assists in planning the appropriate type of fundoplication.
  • pH Monitoring:
    • Not necessary in symptomatic paraesophageal hernias.
    • The indication for repair is based on symptoms, not on reflux status.
    • Fundoplication is included in the repair for these patients.

Management: Indications for Paraesophageal Hernia Repair

Historical Context

  • Initially, operative repair was recommended for all paraesophageal hernias, whether symptomatic or asymptomatic, due to the fear of incarceration and strangulation.
    • In the 1960s, Skinner and Belsey reported that 29% of patients managed conservatively died due to hernia-related causes.
    • By the 1970s, Hill showed an incidence of incarceration in 30% of patients with paraesophageal hernias.

Current Indications

  • Good-risk surgical patients should still undergo repair due to the risk of complications.
    • Laparoscopic repair of large hiatal hernias has shown to be effective and durable.
  • Pooled risk estimates:
    • Annual risk of developing symptoms requiring emergency surgery: 0.69% to 1.93%.
    • The lifetime risk of acute symptoms decreases with age.
  • Watchful waiting may be appropriate for asymptomatic or minimally symptomatic patients:
    • Over 80% of these patients may benefit more from observation than elective surgery.

Surgical vs. Nonsurgical Outcomes

  • Elective repair:
    • Lower mortality rate: 1%.
  • Nonelective repair:
    • Associated with 6-7 fold increased mortality, especially in octogenarians.
    • 50% longer hospital stay and higher inpatient mortality in patients aged over 80.

When to Operate

  • Patients with acute incarceration or strangulation require immediate surgery.
  • Symptoms warranting surgical repair include:
    • Obstructive symptoms, bleeding, or respiratory symptoms attributed to the hernia.

Risk Factors for Surgical Morbidity

  • Increased mortality in patients over the age of 75.
  • Higher morbidity in patients with:
    • ASA Class 3 or 4.
    • Type IV hiatal hernia.

Need for Antireflux Procedure

  • GERD symptoms are common after paraesophageal hernia repair.
  • Fundoplication is often added to the repair to prevent GERD unless contraindicated.

Key Considerations

  • Surgical management should be individualized, especially for elderly patients with high surgical risk.