INDICATIONS/CONTRAINDICATIONS
Paraesophageal hiatal hernias (PEH) are a rare surgical problem that afflicts older adults, more commonly women. The relatively occult location of the hernia, a historic impression of minimal or vague symptoms, and an incomplete understanding by most physicians of the differing presentation and significance of PEH and sliding hiatus hernias often leads to a delay in diagnosis. Patients often present with a long history of symptomatic “hiatal hernia” and radiographic findings similar to the chest x-ray shown in Figure 13.1. This patient has >50% of her stomach intrathoracic and thus has a “giant” paraesophageal hernia, most likely type III. This picture of a large air space in the lower mediastinum with an air–fluid level will typically be a PEH, but will be labeled as a “hiatal hernia” by many physicians and radiologists. The symptoms in these patients can be subtle and the symptoms experienced by these patients are quite different from those found in patients with standard GERD or a type I or sliding hernia. Type I hernias are the “sliding” type, often producing symptoms of reflux or regurgitation, with only the gastroesophageal junction (GEJ) herniating into the chest. Type II hernias have the fundus of the stomach, but not the GEJ, herniating into the chest; these are relatively uncommon. The most common PEH is type III, which is where both the GEJ and the fundus or body of the stomach is herniated. The larger type IV hernia encompasses both the stomach and other intra-abdominal viscera such as the colon, small bowel, pancreas or spleen.
The PEH patient will typically describe symptoms that slowly evolved over years. Early satiety, anemia, chest pain, and dyspnea are typical in a patient with a large intrathoracic stomach. When the entire array of symptoms associated with PEH is understood, we believe that patients are rarely asymptomatic at diagnosis. In addition, following repair, the vast majority of patients demonstrate measurable improvements in symptoms and quality of life. Reviews from experienced centers have shown that results of repair have improved, with reports of good or excellent subjective outcomes in 83% to 98% of patients.1
Initial case series suggested that a significant proportion of patients with PEH presented as a surgical emergency with incarceration and/or ischemia of the stomach.2 Older series showed rates of incarceration and strangulation of 30%, with a mortality of 7%. Modern series and population analyses show that urgent or emergent presentations account for 5% to 15% of operative cases and up to 50% of admissions for PEH, although surgical management is required at the time of acute presentation only a third of the time.3 In cases of urgent presentation with incarceration and obstruction or ongoing pain, decompression with endoscopy and subsequent nasogastric (NG) tube placement (which may need to be placed with endoscopic guidance) typically relieves acute symptoms and allows for more thorough preoperative preparation. In a review of 5 years of data from New York state, Polomsky et al. found that as more operations were being done, the number of emergent presentations decreased.3–6 However, a population-based study of a cohort of octogenarians showed that 43% of these patients had urgent surgery, with a mortality of 15% in the urgent group.7
Dyspnea may be related to the hernia as a space-occupying lesion in the chest or an effect on the diaphragmatic function, although effects of the hernia on respiratory function are likely more complex. We have previously demonstrated a measurable improvement in pulmonary function tests (PFTs) for most patients following repair; thus, borderline PFTs should not disqualify a patient from consideration of elective repair.8 We currently advocate that fit, symptomatic patients presenting with giant PEH should meet with a surgeon to discuss elective repair to improve current quality of life and avoid additional symptoms as these hernias continue to grow. The only contraindications to repair would be medically unfit patients.
Paraesophageal Hernia Repair Indications
Increasing symptoms or signs associated with giant PEH, which include heartburn, regurgitation, dysphagia, early satiety, dyspnea, upper gastrointestinal (UGI) blood loss anemia, chest and abdominal pain following meals, as well as eating or lifestyle modification as a result of ongoing early satiety or regurgitation.
Semi-elective repair in patients presenting with acute nonischemic incarceration treated with NG tube or endoscopic decompression.
Urgent repair in the setting of unremitting chest or abdominal pain, UGI obstruction, active gastrointestinal bleeding from Cameron lesions/ulcer disease or evidence of ischemia on upper endoscopy.
PREOPERATIVE PLANNING
Patients presenting with PEH should undergo a barium swallow to confirm the anatomic conformation of the hernia, with an assessment of the esophageal and gastric emptying as well as the degree of esophageal shortening. When possible, being present for the barium swallow gives the surgeon the best information regarding esophagogastric anatomy and motility of the esophagus and whether the hernia is fixed or mobile. If this is not feasible, a video recording of the swallow is a good alternative. Upper endoscopy is utilized to assess the degree of esophageal shortening, document esophagitis or Barrett’s esophagus, and assess the grade of the flap valve, as well as evaluate for Cameron lesions or erosions in the stomach at the diaphragmatic hiatus (see Fig. 13.2). We recommend esophageal manometry routinely to evaluate the lower esophageal sphincter (LES) pressure and assess esophageal motility pattern. Inserting the current high-resolution catheters is often difficult in large PEHs; therefore, we routinely combine manometry with upper endoscopy and insert the catheter over a wire. Nonspecific motility disorders are common in older patients with PEH, although motility pattern will often improve following repair.9 The approach to repair may have to be modified in patients with esophageal dysmotility (poor peristaltic progression) or hypomotility (mean wave amplitude <30 mm Hg). We do not routinely perform gastric emptying or 24-hour pH-impedance studies in this population. We do, however, routinely have patients perform PFTs before and after surgery, as many will have measurable improvements following repair.
Preoperative Assessment
Barium swallow
Upper endoscopy, with or without wire-guided manometry
Esophageal manometry
PFTs, medical or cardiac clearance as necessary
SURGERY
The open Hill repair is performed via a limited upper abdominal midline incision. We currently utilize the laparoscopic approach in virtually all patients with type II hernia and many patients with type III and type IV hernias whose GEJ is seen to be mobile on UGI fluoroscopy study. The open Hill repair is our preferred approach in patients with large type III and type IV hernias involving 75% to 100% of the stomach and significant esophageal shortening, which either does not reduce or only minimally reduces during witnessed preoperative barium studies. The advantages of this approach include the fact that a primary closure of the diaphragm is virtually always possible. The Hill repair is based on anchoring of the GEJ to reliable intra-abdominal structures posteriorly and therefore avoids the need to utilize the Collis gastroplasty, thereby maintaining normal anatomy. Originally, Dr. Hill used the median arcuate ligament to anchor the repair. Most surgeons find the dissection of the celiac axis daunting which is one of the reasons that the Hill repair is not more commonly utilized.10 We advocate utilizing the base of the crura and the pre-aortic fascia for anchoring the repair. The Hill operation has distinct advantages over other methods, as it firmly anchors the GEJ in the abdomen, re-establishing the normal length of intra-abdominal esophagus.
Once the hernia sac is completely reduced and excised, the esophagus can undergo extensive transhiatal mobilization, often up to the carina, to allow the GEJ to be reduced into the abdominal cavity with little or no tension. The esophagus becomes foreshortened and dysfunctional when elevated as in a type III or type IV PEH, and once it is secured to its standard length, its normal contraction pattern is often restored.1 Esophageal shortening is more prevalent when the patient has a history of esophagitis, esophageal stricture, or Barrett’s esophagus. One of the biggest controversies in PEH repair is that of recurrence. The diaphragmatic hiatus must be closed securely, but not to a point that results in postoperative dysphagia. Many have supported the use of various synthetic or biologic meshes to augment the hiatal closure. We have found that with appropriate mobilization of the right and left crura, closure can almost invariably be done primarily. Secure fixation, as performed in the Hill repair, produces a low recurrence rate that is similar to a Collis–Nissen fundoplication.11
Historically, the three options for dealing with clinically significant esophageal shortening were a thoracic approach with extensive esophageal mobilization, an esophageal-lengthening procedure (such as the Collis gastroplasty), or the Hill repair.
Keys to the Operation
Adequate esophageal mobilization and mediastinal dissection, to establish 3 to 4 cm of intra-abdominal esophagus should be the goal.
Excision of the hernia sac
Firmly anchoring repair sutures to reliable intra-abdominal structures to avoid recurrence
Accentuating the angle of His and recreating the gastroesophageal flap valve to produce a viable antireflux mechanism.
Positioning
The patient is placed supine with the right arm tucked and left arm out at 90 degrees. We use the “upper hand” retractor system (V. Mueller, Allegiance, Deerfield, IL) as well as a stationary liver retractor to retract the left lobe of the liver. The “upper hand” retractor blades are placed under the right and left costal margins, retracting each superiorly and laterally. This works to verticalize the diaphragm and allow for good exposure of the esophageal hiatus. A Balfour retractor is used for the lower portion of the incision (see Figure 13.3).
– Martin’s Arm and upper hand retractors in place. Current incision limited to half the distance from xiphoid to umbilicus.
– Retractor setup for exposing the abdominal esophagus. The upper hand retractor verticalizes the diaphragm.
– Following mobilization of left lobe of liver, it is retracted with a Harrington retractor held in place with a Martin’s Arm retractor (See Figure 13.4).