A multiplicity of terms has been used over the years to describe paraesophageal hernia. These reflect the considerable confusion that persists to this day concerning its pathophysiology and treatment. Terms like up-side-down stomach, rolling hernia, intrathoracic stomach, parahiatal, and paraesophageal hernia have all been used to describe this clinical condition. Any herniation of the fundus and/or body of the stomach into the chest anterior or lateral to the esophagus is considered to be a paraesophageal hernia. Once considered an immediate indication for surgical repair, the role of surgery in this operation is changing and patients receive treatment appropriate to their complaints. Persistent symptoms, in particular, those related to the mechanical effects of paraesophageal hernia, eventually will result in an indication for surgery.
Pathophysiology and Classification
The common classification for paraesophageal hernia distinguishes four types (Table 46-1). The sliding type hiatal hernia (Type I) is the most common. It occurs when the gastroesophageal junction (GEJ), along with a leading portion of the gastric cardia, slides through the esophageal hiatus into the mediastinum. The sliding hernia may progress to a rolling hernia. As the muscles of the hiatus weaken, the hiatus becomes wider, causing an ever increasing portion of the stomach along with the GEJ to slip through the opening, until the entire greater curvature rolls into the posterior mediastinum (hence the name rolling hernia). A rolling hernia is therefore seen as a progression from a small sliding hernia to complete herniation of the greater curvature of the stomach. It is now generally accepted that most paraesophageal hernias begin as sliding hiatal hernias.
In rare cases, the GEJ may remain in its normal intra-abdominal position. This is called the “true” paraesophageal hernia (Type II). Here a portion of the stomach and sometimes other parts of the abdominal viscera migrate into the mediastinum either through the esophageal hiatus or through an adjacent defect in the diaphragm.
Occasionally, the stomach may further migrate into the chest causing organoaxial volvulus, in which usually more than half the stomach lies in the mediastinum, a condition known as “giant” paraesophageal hernia (Type III). The lesser curvature remains in the abdomen, fixed by the gastrohepatic omentum and the left gastric vessels, which explains the asymmetric displacement of the stomach generally observed. With organoaxial volvulus, the fundus folds over anteriorly to the esophagus and toward the right side of the mediastinum.
Less frequently, if the defect becomes very large, colon together with omentum, spleen, and eventually small bowel may migrate into the chest, together with the stomach (Type IV).
Types III and IV are most commonly seen in elderly patients. Aging is associated with diminished muscle tone and loss of strength and elasticity of the connective tissues in the phrenoesophageal membrane, which predispose to thinning and widening of the esophageal hiatus. In addition, elderly patients often develop kyphosis. The resultant change in posture may further stretch and weaken the diaphragmatic structures. Finally, obesity can be a predisposing factor because it causes an increase of intra-abdominal pressure on the stomach.
If the herniated stomach becomes trapped between the margin of the esophageal hiatus and the esophagus, this may result in an unreducible incarcerated hernia. This condition may eventually lead to life-threatening complications, such as dilatation of the intrathoracic stomach with partial or complete obstruction, ischemia of a strangulated intrathoracic stomach with subsequent necrosis and perforation, and soiling of the mediastinum or pleural space with gastric contents resulting in infection. Localized pressure ulceration at the margin of the hiatus may result in bleeding (often occult) and anemia.
The anatomical derangements associated with the full spectrum of paraesophageal hernias are summarized in Fig. 46-1.
General Therapeutic Principles
Paraesophageal hernias are usually discovered on diagnostic radiographic images in patients with symptoms of heartburn and chest pain. They also may be discovered incidentally in asymptomatic individuals. After careful questioning to rule out potential life-threatening pathology, treatment appropriate to the patient’s symptoms is recommended. Patients without symptoms do not need any treatment. Patients with symptoms of reflux are treated lege artis. Moderate dysphagia can be remediated by small meals of semisolid consistency. A prokinetic, for example, metoclopramide, is helpful for treating symptoms of early satiety and fullness. The presence of moderate anemia requires iron replacement. If there is a high index of suspicion for incarceration, the introduction of a nasogastric tube will provide relief. However, persistence of symptoms, in particular, those related to the mechanical effects of paraesophageal hernia, eventually will result in an indication for surgery.
In 1967, Skinner and Belsey published a classic paper1 that drew attention to the potentially life-threatening complications of symptomatic paraesophageal hernias. In their series, over 25% of patients with a paraesophageal hernia treated with medical therapy died of catastrophic complications, chiefly, aspiration-related pneumonia. This led to the long-standing practice of treating hernias surgically, even those with minimal symptoms. That practice has been challenged over the past several decades with the advent of improved diagnostic procedures, minimally invasive approaches, and long-term outcome data from clinical studies. Even so, giant paraesophageal hernias are more complex than the garden variety sliding hernia, and this influences both surgical approach and outcome. Besides the more complex technical aspects of the surgery, itself, these interventions carry a higher rate of postoperative mortality and morbidity, even in expert hands, causing some surgeons to challenge the necessity of surgical intervention for many large or giant paraesophageal hernias.
A study by Allen et al.2 from the Mayo Clinic in 1993 reported on 147 patients, 23 of which were not operated but instead followed for a median of 6.5 years. Not a single patient of this series developed life-threatening complications. In four patients, symptoms progressed resulting in elective surgery in two.
In 2002, using a Markov Monte Carlo decision analytic model, Stylopoulos et al.3 calculated that for the many patients who have only minor symptoms, such as heartburn, bloating, and so on, a policy of watchful waiting entails a lifetime risk of only 1.1% per year for the development of acute symptoms requiring surgery, with mortality related to emergency surgery of only 5.4%.3 This means that the overall lifetime risk of death is approximately 1% which is less than the reported 30-day mortality in series from high volume centers. Moreover, the older the patient, the higher the postoperative mortality. In the series published by Luketich et al.,4 the 30-day mortality in patients older than 80 years was 8%.
Given the fact that progression toward life-threatening symptoms/complications is low and occurs mostly in elderly populations, elderly patients with mild symptoms should not undergo surgery. Therefore, for every patient, the advantages of repair should always be carefully weighed against the risks of surgery.
An attempt should always be made to decompress the herniated stomach in patients presenting with symptoms of incarceration. This avoids the necessity of performing an emergency procedure, which is associated with high mortality, and gives adequate time, subsequently, for a thorough preoperative investigation and preparation for elective surgery under optimal conditions, for example, correction of anemia, treatment of aspiration-related pneumonia.
In cases involving clear incarceration that cannot be decompressed, gastric ischemia due to strangulation, massive bleeding, and perforation, emergency surgery is unavoidable. Depending on the experience of the surgeon, an open transthoracic or transabdominal or laparoscopic approach can be used.
Symptoms that accompany paraesophageal hernias are either related to reflux or to the mechanical aspects of the herniation. It is important to acquire a complete history and physical examination to elicit the typical symptoms of gastroesophageal reflux disease (heartburn and regurgitation) and dysphagia. A detailed discussion of the symptoms related to reflux is provided in Chapter 36.
Many patients with paraesophageal hernias remain asymptomatic for years or have only vague, intermittent symptoms. Although patients may initially deny symptoms, careful questioning can reveal complaints that are related to the esophagus, for example, dysphagia, epigastric or substernal pain, postprandial substernal fullness, nausea, and hiccupping.
Symptoms related to intermittent incarceration are postprandial distress or early satiety and fullness, epigastric pain, intermittent vomiting, or regurgitation. A very large volvulus may cause dyspnea, and on occasion, in this respect, atelectasis may be seen on chest CT.
Anemia and iron deficiency are usually the result of chronic occult bleeding from a pressure ulcer at the margin of the hiatus. In such cases, the patient may present with complaints of exercise-related dyspnea, fatigue, weakness, palpations, and in the case of concomitant coronary disease, angina pectoris. Painful ulceration of the herniated fundus with perforation and massive bleeding may occasionally occur. The perforation may extend into the pleura, mediastinum, or pericardium. When volvulus is present, complete obstruction of food passage may occur.
A posteroanterior and lateral chest x-ray often will show air or an air–fluid level in the mediastinum posterior to the heart indicating the presence of a herniated stomach.
The barium swallow is the gold standard diagnostic study for paraesophageal hernias (Fig. 46-2). It reveals the anatomy and relationship of the esophagus and stomach. It also will reveal whether there is free passage of contrast material (this may include a more solid contrast bolus) into the stomach. Less often, it may be helpful in locating the GEJ in relation to the diaphragm. This study reveals the anatomy and relationship of the esophagus and stomach. It also will reveal whether there is free passage of the contrast material (this may include a more solid contrast bolus) into the stomach. Often, it may be helpful in locating the GEJ in relation to the diaphragm. CT scan may be used to assess the extent of volvulus and the size of the hiatus or in identifying other abdominal organs that have migrated into the chest. Contrast studies may also reveal esophageal motility disorders, strictures, endoluminal masses, or diverticula.