Overview
Definition:
A giant paraesophageal hernia (GPEH) is defined as a large hiatal hernia where a significant portion of the stomach, and often other abdominal organs like the colon, omentum, or spleen, herniates into the thoracic cavity through the esophageal hiatus
It is typically characterized by the stomach essentially becoming an intrathoracic organ, often with significant displacement of the gastroesophageal junction (GEJ) into the chest.
Epidemiology:
Paraesophageal hernias account for approximately 5-10% of all hiatal hernias
Giant paraesophageal hernias are less common but represent the more severe end of the spectrum
They are more prevalent in older individuals, with a higher incidence reported in women
Factors contributing to their development include increased intra-abdominal pressure, obesity, and weakened diaphragmatic hiatus.
Clinical Significance:
GPEHs pose significant risks, including gastric volvulus, incarceration, strangulation, perforation, hemorrhage, and aspiration pneumonia
They can lead to chronic symptoms such as dysphagia, regurgitation, chest pain, and profound weight loss
Early diagnosis and surgical repair are crucial to prevent life-threatening complications and improve patient quality of life
Mastery of surgical techniques is paramount for residents preparing for DNB and NEET SS examinations.
Clinical Presentation
Symptoms:
Vague epigastric or chest pain, often postprandial
Dysphagia or odynophagia, particularly with solid foods
Postprandial fullness or early satiety
Nausea and vomiting, sometimes intractable
Weight loss due to poor oral intake
Regurgitation of undigested food
Shortness of breath due to compression of lung parenchyma
Occasional symptoms of gastric outlet obstruction
Hematemesis or melena from mucosal ischemia or Mallory-Weiss tears.
Signs:
Often subtle or absent on physical examination
Palpable epigastric mass in some cases
Signs of malnutrition or dehydration if severe
Tachycardia or hypotension if complications like strangulation occur
Chest auscultation may reveal decreased breath sounds on the affected side
Abdominal distension can be present.
Diagnostic Criteria:
No formal diagnostic criteria exist beyond radiographic and endoscopic evidence
Diagnosis is based on characteristic findings on barium swallow, CT scan, and upper GI endoscopy, demonstrating significant herniation of abdominal contents into the chest, often with significant displacement of the GEJ superiorly.
Diagnostic Approach
History Taking:
Detailed inquiry about the onset, character, and exacerbating/relieving factors of symptoms
Focus on dietary habits, weight changes, and any history of prior abdominal or thoracic surgery
Assess for symptoms suggestive of gastric volvulus, obstruction, or ischemia
Inquire about associated conditions like GERD, obesity, or connective tissue disorders
Red flags include acute severe chest pain, persistent vomiting, and signs of hemodynamic instability.
Physical Examination:
Thorough abdominal examination to assess for tenderness, masses, or distension
Assess for signs of dehydration or malnutrition
Careful cardiopulmonary examination to rule out respiratory compromise
Evaluate for any visible or palpable masses in the chest or upper abdomen.
Investigations:
Barium swallow: The gold standard for initial diagnosis, demonstrating the size and contents of the hernia, gastric anatomy, and potential for volvulus
CT scan of the chest and abdomen: Provides excellent anatomical detail of the hernia, its contents, and surrounding structures, useful for pre-operative planning and assessing complications
Upper GI endoscopy: Evaluates for mucosal pathology within the herniated stomach and esophagus, assesses GEJ competence, and rules out other upper GI pathologies
Esophageal manometry: May be considered to assess esophageal motility and GEJ function, especially if achalasia is suspected
Chest X-ray: Can show a retrocardiac opacity, but less sensitive for diagnosis compared to barium swallow or CT.
Differential Diagnosis:
Other causes of mediastinal masses, such as esophageal duplication cysts, bronchogenic cysts, mediastinal tumors, or enlarged lymph nodes
Congenital diaphragmatic hernias (in infants)
Large hiatal hernias that are not yet giant
Boerhaave syndrome (esophageal perforation), presenting with similar chest pain and distress
Gastric cancer or peptic ulcer disease presenting with epigastric pain and vomiting.
Surgical Management
Indications:
Symptomatic patients with dysphagia, pain, vomiting, or weight loss
Asymptomatic patients with evidence of gastric volvulus, incarceration, or a very large hernia posing a significant risk of complications
Patients with recurrent aspiration pneumonia
Elective repair is generally recommended for most GPEHs due to the risk of acute complications.
Preoperative Preparation:
Nutritional assessment and optimization
Respiratory assessment and optimization, especially for patients with significant intrathoracic component
Preoperative imaging review to understand hernia anatomy and contents
Broad-spectrum antibiotics
DVT prophylaxis
Informed consent covering potential risks and benefits, including recurrence, need for gastrostomy, and extent of repair.
Procedure Steps:
Approach: Either abdominal (laparoscopic or open) or thoracic (video-assisted thoracic surgery - VATS, or open thoracotomy)
Combined approaches (laparotbomy/thoracotomy) may be necessary for very large or complex hernias
Reduction of hernia contents: Careful mobilization and gentle reduction of herniated abdominal organs back into the abdomen
Hernia sac excision/plication: Excision of the sac or plication to reduce the size of the hiatus
Hiatal repair: Crural repair to narrow the diaphragmatic hiatus, often reinforced with mesh (e.g., synthetic or biologic mesh) to prevent recurrence
Fundoplication: A 360-degree Nissen fundoplication or a partial fundoplication (e.g., Toupet) is crucial to prevent recurrent GERD and provide GEJ stability
Gastropexy: May be performed in select cases to further anchor the stomach
Placement of a feeding tube (e.g., gastrostomy or jejunostomy) may be considered for patients with preoperative dysphagia or poor nutritional status.
Postoperative Care:
Pain management
Early ambulation
Diet progression: Initially clear liquids, advancing as tolerated
Monitoring for complications such as bleeding, infection, ileus, or respiratory compromise
Chest tube management if a thoracic approach was used
Gastrostomy tube care if placed
Pulmonary toilet to prevent pneumonia
Close monitoring of vital signs and fluid balance
Swallow evaluation before advancing diet if significant dysphagia was present preoperatively
Follow-up appointments to monitor for recurrence and GERD symptoms.
Complications
Early Complications:
Bleeding from surgical site or staple lines
Infection of surgical wound or chest cavity
Pneumonia or atelectasis
Injury to adjacent organs (esophagus, spleen, colon, lung)
Gastric perforation
Anemia from occult bleeding
DVT/PE
Re-herniation or paraesophageal leak.
Late Complications:
Recurrent hiatal hernia or GERD
Dysphagia or odynophagia post-fundoplication
Gastroparesis or gastric outlet obstruction
Mesh-related complications (infection, erosion, pain) if mesh was used
Nutritional deficiencies
Chronic chest pain.
Prevention Strategies:
Meticulous surgical technique with careful handling of tissues
Appropriate choice and placement of mesh if used
Secure and well-performed hiatal closure and fundoplication
Aggressive pulmonary toilet postoperatively
DVT prophylaxis
Careful diet advancement
Close patient follow-up for early detection of recurrence or complications.
Prognosis
Factors Affecting Prognosis:
The extent of the hernia, presence of complications at presentation (e.g., incarceration, strangulation), patient comorbidities, and the skill of the surgeon
Successful surgical repair generally leads to resolution of symptoms and prevention of life-threatening complications.
Outcomes:
With appropriate surgical management, the majority of patients experience significant symptom relief and improved quality of life
Recurrence rates for large hiatal hernias, even with repair and mesh, can range from 5-20%
Long-term management of GERD may still be required in some patients.
Follow Up:
Regular follow-up appointments are essential to monitor for recurrence of the hernia, persistent or new-onset GERD symptoms, and overall well-being
Endoscopic evaluation may be considered if there is suspicion of recurrence or new upper GI pathology
Patients should be educated on warning signs of complications or recurrence.
Key Points
Exam Focus:
Understand the definition and epidemiological factors of GPEH
Know the classic symptoms and risks of complications like gastric volvulus and strangulation
Recognize the role of barium swallow and CT in diagnosis
Master the surgical approaches (abdominal vs
thoracic) and essential components of repair: reduction, hiatal closure, and fundoplication
Be aware of mesh use and its indications/risks
Identify key complications and recurrence factors.
Clinical Pearls:
For GPEHs, always consider the possibility of gastric volvulus
Intraoperative assessment of the GEJ is critical
it should be brought down into the abdomen and secured
Mesh reinforcement of the crural repair is often necessary for large defects but requires careful consideration of mesh type and placement to minimize morbidity
Fundoplication is almost always indicated to prevent recurrent GERD
Be prepared for difficult dissections due to adhesions and inflammation within the chest cavity.
Common Mistakes:
Underestimating the size and complexity of the hernia
Inadequate reduction of stomach contents
Incomplete excision or plication of the hernia sac
Insufficient narrowing of the diaphragmatic hiatus
Omitting fundoplication leading to recurrent GERD
Over-reliance on mesh without adequate crural closure
Failure to recognize intraoperative injury to adjacent organs
Inadequate postoperative care leading to respiratory or gastrointestinal complications.