Ann Thorac Surg 2002;73:416-419
© 2002 The Society of Thoracic Surgeons
Original article: general thoracic
"True" parahiatal hernia: a rare entity radiologic presentation and clinical management
Michael G. Scheidler, MDb,
Robert J. Keenan, MDa,b,
Richard H. Maley, MD, Jra,b,
Robert J. Wiechmann, MDa,
Dennis Fowler, MDb,
Rodney J. Landreneau, MD*a,b
a Division of General Thoracic Surgery, Allegheny General Hospital, Pittsburgh, Pennsylvania, USA
b Division of Minimally Invasive Surgery, Allegheny General Hospital, Pittsburgh, Pennsylvania, USA
Accepted for publication September 25, 2001.
* Address reprint requests to Dr Landreneau, Division of General Thoracic Surgery, Allegheny General Hospital, 320 East North Ave, Pittsburgh, PA 15213, USA
e-mail: rlandren{at}wpahs.org
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Abstract
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Background. True parahiatal diaphragmatic hernias are rare entities that are sparsely accounted for in the literature. The current report is intended to depict the clinical profile and assess the feasibility of laparoscopic repair of parahiatal hernias.
Methods. We conducted a retrospective review of all patients diagnosed and treated for parahiatal hernias. Clinical presentation and radiological assessment, as well as operative findings and repair, are discussed.
Results. Of the 917 laparoscopic hiatal hernia repairs, 2 (0.2%) patients were identified with a parahiatal hernia. The presenting symptoms and preoperative testing were similar to those with more common paraesophageal hernias. Laparoscopic repair was successful in repairing the diaphragmatic defect and alleviating symptoms up to 4 years postoperatively.
Conclusions. Parahiatal hernias of the diaphragm appear to be rare primary diaphragmatic defects. The clinical presentation of parahiatal hernias is often indistinguishable from the more common paraesophageal pathology. Laparoscopic repair of this rare entity can be safely and successfully accomplished in conjunction with antireflux surgical interventions when indicated.
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Introduction
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Diaphragmatic hernias include subcostal anterior hernias of Morgagni, posterolateral hernias of Bochdalek, and, most commonly, esophageal hiatal herniation. The latter is frequently identified by barium esophagography and endoscopic examination. There are two common forms of hiatal herniation [14]. The overwhelming majority are described as "sliding" in nature. These "sliding" hiatal hernias are characterized by a laxity and stretching of the phreno-esophageal attachments at the esophageal hiatus resulting in cephalad migration of the gastroesophageal junction through the diaphragmatic hiatus into the mediastinum. The primary significance of sliding hernias is their common association with gastroesophageal reflux disease. The second, less common form of hiatal herniation, representing approximately 5% of all hernias, is described as "paraesophageal" in nature. These hiatal hernias are characterized by gastric fundic herniation through a large opening in the esophageal hiatus anterior to the gastroesophageal junction, which is usually in its normal anatomic location.
A third form of hiatal herniation, designated as "parahiatal," is even more rare. In fact, controversy exists among esophageal surgeons as to existence of a true de novo parahiatal hernia in the absence of previous diaphragmatic trauma or surgical manipulation [3]. As defined, parahiatal hernias arise lateral to the crural musculature adjacent to but separate from the esophageal diaphragmatic hiatus [38]. To our knowledge there have been only three documented reports of true parahiatal herniation surgically repaired in the literature [57]. Only one of these [6] was repaired laparoscopically. The current report is intended to depict the clinical profile and assess the feasibility of laparoscopic repair of parahiatal hernias. We describe and illustrate our clinical experience managing 2 patients with true parahiatal hernias among the 917 patients undergoing laparoscopic surgical correction of hiatal hernia and gastroesophageal reflux disease over the last 5 years. We further describe the radiologic characteristics of these hernias and the unique surgical management problems that may be encountered.
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Patients and methods
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Between 1996 and 2001, 917 patients underwent laparoscopic fundoplication by one of four surgeons at Allegheny General Hospital. Preoperative evaluation for each patient was similar for all patients and is detailed below. Within this group of patients with a hiatal hernia, a subset (n= 2; 0.2%) had a true parahiatal hernia. Their presenting symptoms, preoperative evaluation including radiological assessment, and the conduit of the operation are described in detail below.
Clinical summaries
Patient 1
A 68-year-old woman presented with a 2-year history of progressive intermittent postprandial nausea and emesis associated with epigastric pain. The patient also described regurgitation of small amounts of undigested, non-foul-smelling food particles hours after large meals. Except for an open hysterectomy 20 years ago, the patient had no concomitant medical condition or history of trauma. She had received treatment with proton pump inhibitors for 1 year before this presentation to manage gastroesophageal reflux disease symptoms. Inadequate control of her symptoms with this medical therapy led to the performance of esophagogastroduodenoscopy, which demonstrated grade I-II distal esophagitis and the perception of a large hiatal hernia on retroflexed imaging of the gastroesophageal junction. Chest roentgenography and upper gastrointestinal barium contrast studies were subsequently performed. A large air-filled structure to the far left of the midline was identified on the posterior anterior roentgenogram (Fig 1).
The corresponding lateral chest roentgenogram confirmed a posterior mediastinal location of this abnormality (Fig 2).
The barium contrast study confirmed an intrathoracic location of a portion of the gastric fundus with preservation of the normal location of the gastroesophageal junction. The patient was considered for surgical correction of what was perceived to be a paraesophageal hernia. Preoperative esophageal manometric studies demonstrated normal esophageal body peristalsis and a low normal resting pressure and length of the lower esophageal sphincter. Twenty-four-hour pH monitoring demonstrated abnormal acid exposure with a composite DeMeester score of 48. Upon analysis of the preoperative data, a decision was made to attempt laparoscopic repair of the hiatal herniation and Nissen fundoplication.

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Fig 1. Posterior-anterior chest roentgenogram demonstrating intrathoracic air fluid level to the left of the midline.
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Fig 2. Lateral chest roentgenogram demonstrating the posterior mediastinal air fluid level within the posterior mediastinum consistent with an incarcerated intrathoracic stomach.
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After general anesthesia and carbon dioxide insufflation of the celiomic cavity, laparoscopic exploration was performed. Gastric and omental herniation was appreciated within a peritoneal-lined defect immediately lateral to the esophageal hiatus. This parahiatal defect was separated from the esophageal hiatus by the muscular substance of the left crus of the diaphragm. The gastric fundus and omentum were reduced and the peritoneal lining of the hernia defect was excised. Because of the patients documented pathologic gastroesophageal reflux, hiatal dissection and Nissen fundoplication were also performed. The dissected esophageal hiatus and lateral parahiatal defect are seen in Figure 3.
In retrospect, an unusual area of tissue separation between the distal esophagus and the herniated stomach could be appreciated on the oblique views of the gastroesophageal junction (Fig 4).
The parahiatal defect was closed by approximating the lateral aspect of the hernia defect and the left crus of the diaphragm with nonabsorbable heavy suture. A standard Nissen fundoplication was created about a 54F intraesophageal bougie after careful approximation of the right and left crus of the diaphragm posterior to the esophagus. Although we achieved adequate approximation of the crura, some degree of tension upon the crural repair was appreciated as a result of the lateral repair of the parahiatal defect. Additional pexing sutures were placed between the upper lateral aspects of the fundoplication and each crura. Pexing sutures were also placed between the lower aspect of the fundoplication and the arcuate ligament. In total, these sutures anchored the gastroesophageal junction and the fundoplication so that the risk of postoperative paraesophageal herniation and slippage of the fundoplication was minimized. The patients postoperative course was uneventful and she was discharged to home 2 days after surgery. A barium esophagram obtained at 10-month follow-up demonstrated normal esophageal hiatal anatomy and an intact fundoplication without gastroesophageal reflux. Clinically, the patient has no symptoms of heartburn or dysphagia without medication at one year.

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Fig 3. Laparoscopic view of the esophageal hiatus and the lateral parahiatal defect in the diaphragm after hernia sac excision and hiatal dissection.
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Fig 4. Oblique view of barium esophagram demonstrating band of crural musculature between the distal esophagus and the herniated stomach.
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Patient 2
A 57-year-old man presented with a complaint of postprandial, substernal chest pain of several months duration [9]. The patient had no previous surgery or thoracoabdominal trauma history. Cardiac evaluation was negative; however, chest roentgenography demonstrated a posterolateral air fluid level within the posterior mediastinum similar to that described for the first patient. Again, a presumed diagnosis of paraesophageal hernia was made. A workup similar to the first patient was performed, which also confirmed the intrathoracic herniation of the gastric fundus. Manometric evaluation confirmed intact esophageal motility, and 24-hour pH monitoring resulted in a high normal DeMeester score of 21.
Esophagogastroduodenoscopy demonstrated an uninflammed esophagus and the impression of a true paraesophageal hernia on examination of the stomach. As was the case with patient 1, we identified a separate diaphragmatic "parahiatal" defect through which the gastric fundus was herniated into the chest. The nature of this defect was appreciated on further review of the lateral images of the barium contrast studies (Fig 5).
The operative management and postoperative course of this patient paralleled that of the first patient. The patient continues to be symptom-free 4 years postoperatively, and a barium esophagram revealed normal anatomy without reflux.

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Fig 5. Lateral image from barium contrast study of the stomach from the second patient demonstrating small-necked hernia defect in the diaphragm independent of the esophageal hiatus.
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Results
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Until recently, objective evidence for the existence of primary or true parahiatal hernias has been limited. The increasing popularity and efficacy of video-assisted surgery has allowed for the documentation of this rare clinical entity. In our practice, the prevalence of this diaphragmatic defect is quite uncommon, representing 0.2% (2 of 917) of our entire laparoscopic antireflux surgical experience and 1.6% (2 of 127) of our patients with a presumptive clinical diagnosis of paraesophageal hernia.
As previously indicated, most parahiatal hernias are presumably the result of traumatic injury to the diaphragm or the result of iatrogenic injury after previous surgery in the left upper quadrant of the abdomen [3, 8]. The current report is only the third documenting parahiatal herniation that appears to be congenital in nature or progressively acquired through evolution of an inherent weakness within the perihiatal musculature of the diaphragm.
The studies of our patients illustrate that there are some subtle but specific roentgenographic findings that may aid in discriminating parahiatal herniation from paraesophageal hernias. As illustrated in Figure 1, the position of the intrathoracic herniated abdominal viscus was noticeably removed toward the left of the midline. Most posterior mediastinal air-fluid levels representing the herniated viscus associated with paraesophageal hernias are midline in nature or favoring a right-sided predominance. The second roentgenographic feature associated with these parahiatal hernias is the presence of a defined band of tissue separating the contrast within the distal esophagus from the contrast within the herniated viscus (Fig 3). This tissue band may also be seen on lateral barium esophagram and upper gastrointestinal studies, as seen in Figure 5.
The primary symptoms described by our 2 patients in the current report are similar to those of patients with the more commonly occurring paraesophageal hiatal hernias. The unique parahiatal characteristics of the diaphragmatic herniations were not identified until laparoscopic examination, but in retrospect, important clues were present on preoperative roentgenographic studies.
The same potentially catastrophic complications of visceral incarceration and strangulation inherent to paraesophageal herniation appear also to be shared by patients with parahiatal hernias. Elective surgery is advocated when this problem is identified to avoid such complications. Laparoscopic repair of paraesophageal hernias is increasingly utilized [4], with a success rate equivalent to previously reported open repairs [7]. This same approach should be favored for repair of these rare parahiatal hernias.
After excision of the parahiatal hernia sac, careful inspection and mobilization of the lateral extent of the diaphragmatic opening must be done. Correction of the parahiatal defect must be performed without undue tension and to allow secondary approximation of the right and left limbs of the crura during the course of any associated antireflux surgery. In most instances, this can be performed without the use of prosthetic material [10]. The use of pexing sutures between the fundoplication, crural margins, and the arcuate ligament can reduce the possibility of postoperative paraesophageal herniation.
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Comment
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In conclusion, true parahiatal hernias of the diaphragm appear to be rare primary diaphragmatic defects occurring in the absence of previous diaphragmatic trauma or surgical misadventure. The clinical presentation of parahiatal hernias are often indistinguishable from the more common paraesophageal pathology, and the risks associated with these two diaphragmatic abnormalities are similar. Laparoscopic repair of this rare entity can be safely and successfully accomplished in conjunction with antireflux surgical interventions when indicated.
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