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Paraesophageal Hernia: Etiology, Presentation, and Indications for Repair
Paraesophageal hernias are the results of defects in the diaphragmatic hiatus. Widening of the hiatus between the left and right diaphragmatic crura provides the pathway for upward displacement of abdominal contents into the mediastinum. Paraesophageal hernias are an increasingly common type of hiatal hernia. They can be associated with life-threatening complications such as gastric volvulus leading to necrosis or perforation of the stomach. Due to these potential complications, it was thought that all paraesophageal hernias should be repaired upon diagnosis. Recent evidence, however, has suggested that a nonsurgical approach is reasonably safe in asymptomatic patients. Symptoms can be subtle and include chest pain or pressure after meals, dysphagia for solids, dyspnea on exertion out of proportion to general health, early satiety, and the need to eat very small meals to avoid feeling uncomfortable. In addition, anemia is a common condition in patients with a paraesophageal hernia and typically resolves with correction of the hernia. Surgical intervention is recommended for patients who are exhibiting symptoms or signs associated with a paraesophageal hernia. The general etiology, presentation, and indications for repair of paraesophageal hernias are reviewed here.
Etiology
Hiatal hernias occur when portions of the stomach or other abdominal contents herniate superiorly into the mediastinum through a defect in the esophageal hiatus. Hiatal hernias have been associated with gastroesophageal reflux disease (GERD), and the prevalence and size of the hiatal hernia has been described to correlate with the severity of reflux. The presence of a hiatal hernia has also been identified in nearly 40% of obese patients. Some of the causes of hiatal hernias have been attributed to age, stress, and degenerative processes on the diaphragm. Most cases of hiatal hernia are acquired rather than congenital, although familial clustering has been reported.
Classification
Four types of hiatal hernias have traditionally been described. Type I hiatal hernia is a migration of the gastroesophageal (GE) junction into the posterior mediastinum, which is usually the result of deterioration of the phrenoesophageal ligament. The forces exerted during swallowing and the negative intrathoracic pressure combined with the positive intraabdominal pressure contribute to the stretching of the phrenoesophageal ligament. Different types of collagen, particularly types I and III, have been found to be reduced in the phrenoesophageal ligament of patients with GERD and hiatal hernia. Type I hiatal hernia is also known as the “sliding” hiatal hernia ( Fig. 25.1 ). Sliding hiatal hernias can be large, but importantly, the GE junction remains above the herniated stomach.
Types II, III, and IV hiatal hernias are the paraesophageal hernias, where the stomach and esophagus are juxtaposed. A paraesophageal hernia is a true hernia with a hernia sac. The key feature that defines a paraesophageal hernia is that the fundus of the stomach is located above the GE junction, which can either be in a normal intraabdominal location or also herniated into the chest. The location of the fundus relative to the GE junction defines a sliding versus a paraesophageal hernia. Type II, or “rolling” hiatal hernias, occur when the gastric fundus herniates anterior to the esophagus, with a normally positioned intraabdominal GE junction. Type II is also referred to as a “true” paraesophageal hernia. Congenital defects in the esophageal hiatus can lead to paraesophageal hernias. Type III hiatal hernias are a combination of types I and II, in which both the GE junction and a portion of the stomach—usually the gastric fundus—herniate into the mediastinum. Type IV hiatal hernias contain stomach and other abdominal organs such as small bowel, colon, pancreas, or spleen in the mediastinum. The term giant paraesophageal hernia refers to large hiatal hernias where at least 50% of the stomach is in the mediastinum or the hernia measures at least 6 cm on endoscopy.
Prevalence
The actual prevalence of paraesophageal hernias is not known. The most common hiatal hernia is type I, which accounts for up to 95% of all hiatal hernias. Paraesophageal hernias may account for up to 14% of all hiatal hernias, and the majority of paraesophageal hernias are of the type III variety. The incidence of paraesophageal hernias increases with age. Paraesophageal hernias tend to develop on the left anterior aspect of the esophageal hiatus. Women are more likely to develop paraesophageal hernias compared to men, and kyphosis is a risk factor.
Presentation
Symptoms in patients with a paraesophageal hernia may be completely absent, minor and overlooked ( Table 25.1 ), or quite debilitating and interfere with quality of life. Symptomatic patients commonly have typical GERD symptoms including heartburn, regurgitation, and water brash. However, some patients can present with obstructive symptoms, such as dysphagia, anemia secondary to chronic gastric blood loss, and respiratory complaints, such as dyspnea, asthma, chronic obstructive pulmonary disease, and aspiration pneumonia. Other symptoms associated with paraesophageal hernia include lower chest pain or discomfort, bloating, gassiness, and early satiety. Other patients develop these symptoms after undergoing upper gastrointestinal operations. For example, the incidence of symptomatic hiatal hernia after esophagectomy ranges between 1.0% and 4.5%. Many patients have endoscopic evidence of gastritis and Cameron ulcers. It has been postulated these gastric ulcers can result from gastric torsion and poor gastric emptying. Because many of these symptoms are vague or can be attributed to other causes, their relationship to paraesophageal hernia is commonly missed. Therefore, it is not unusual for patients to be suffering from these symptoms for many years.
TABLE 25.1
Paraesophageal Hernias: Preoperative Symptoms and Findings
From Pierre AF, Luketich JD, Fernando HC, et al. Results of laparoscopic repair of giant paraesophageal hernias: 200 consecutive patients. Ann Thorac Surg. 2002;74:1909.
| Typical heartburn | 47% |
| Dysphagia | 35% |
| Epigastric pain | 26% |
| Vomiting | 23% |
| Anemia | 21% |
| Barrett epithelium | 13% |
| Aspiration | 7% |
Caveat: Many paraesophageal hernias are asymptomatic.
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