Paraesophageal Hernia

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1 THE ANNALS OF THORACIC SURGERY Journal of The Society of Thoracic Surgeons and the Southern Thoracic Surgical Association VOLUME 16 * NUMBER 6 DECEMBER 1973 Paraesophageal Hernia A Life-Threatening Disease I. Ayhan Ozdemir, M.D., William A. Burke, M.D., and Phillip M. Ikins, M.D. ABSTRACT Thirty-one patients with paraesophageal hernia were studied, and their treacherous clinical course and complications are emphasized. Ten patients had acute upper gastrointestinal bleeding, 9 had obstruction, and 2 had perforation. Primary repair was done for 28 patients. Two esophagogastric resections were undertaken for irreversible ischemia and perforation. One patient had closure of the perforation and hernia repair. There were 2 operative deaths, both of which were related to complications of this disease (obstruction, perforation, and mediastinitis). Paraesophageal hernia must be repaired promptly to alleviate symptoms and to prevent serious complications. T here are three varieties of diaphragmatic hernia through the esophageal hiatus. These are recognized as the paraesophageal, the sliding, and the combined form of hernia. In our experience the paraesophageal hernia is relatively uncommon, being found in 5.1% of a total of 567 patients with hiatus hernia. In the paraesophageal hernia, the distal esophagus and cardia usually retain their normal position below the diaphragm. The anterior wall and fundus of the stomach roll upward into the chest through the lax esophageal hiatus adjacent to the cardia. The hernia often becomes extremely large, so much so that the entire stomach and other abdominal organs may enter the thorax [I, 81. Serious complications From the Division of Thoracic and Cardiovascular Surgery, State University of New York Upstate Medical Center, Syracuse, N.Y. We wish to acknowledge the help and encouragement of Dr. Watts R. Webb in the preparation of this DaDer. Presente'd it the Ninth Annual Meeting of The Society of Thoracic Surgeons, Houston, Tex., Jan , Address reprint requests to Dr. Ikins, 1200 E. Genesee St., Syracuse, N.Y

2 OZDEMIR, BURKE, AND IKINS such as bleeding, obstruction, strangulation, and perforation can occur in the cardia or fundus of the stomach [l-31. From the years 1960 to 1973, 31 patients with paraesophageal hernia were treated in the Thoracic and Cardiovascular Surgery Service of the Upstate Medical Center in Syracuse, New York. The histories of these 31 patients with paraesophageal hernia were analyzed to evaluate the inherent dangers of such hernias. Clinical Material The 31 paraesophageal hernias were found in 21 women and 10 men. The average age was 60 years, and the age range was from 38 to 84 years. Most of the patients were seriously ill at the time of admission to the hospital. In considering the symptoms that were sufficient to bring the patient to seek medical attention, it is apparent from the Table that the overwhelming majority involved upper gastrointestinal bleeding and obstruction, which are directly related to the size of the hernia. Symptoms from paraesophageal hernia are exclusively mechanical in origin. Following the ingestion of a meal, the distended thoracic stomach can produce severe epigastric and retrosternal pain that is most often described as sharp and crushing in nature; thus the pain is confused with angina pectoris. Pain and respiratory distress were closely related to eating and delayed gastric emptying. Compression of the lungs and heart and impairment of the action of the diaphragm may interfere with cardiorespiratory function, especially in the presence of other respiratory disabilities. Ten of the 31 patients had acute gastrointestinal bleeding due to vascular stasis and active ulceration in the thoracic stomach, which was incarcerated or even partially strangulated in the narrow hernial ring. Nine patients had occult bleeding and anemia. The majority of the patients had had one or two previous hospital admissions for medical therapy. One of them had had repeated transfusions totalling 121 units of blood for her hypo- SYMPTOMS OF PARAESOPHAGEAL HERNIA IN 31 PATIENTS Symptom No. of Patients Pain 18 Respiratory distress 8 Nausea & vomiting 8 Acute upper gastrointestinal bleeding 10 Occult bleeding & anemia 9 Obstruction Acute (incarceration, 3; strangulation, 4; perforation, 2) 2. Intermittent (3) 548 THE ANNALS OF THORACIC SURGERY

3 Paraesophageal Hernia chromic anemia before the diagnosis was made. Nine patients were admitted to the hospital with acute dysphagia and epigastric distress together with nausea but inability to vomit. Three patients had had similar episodes of dysphagia in the past. As the hernia develops, the greater curvature of the stomach rolls upward into the mediastinum to become uppermost. The entire stomach and other abdominal organs can move into the thorax. The pylorus and cardia come together to produce the characteristic roentgenographic entity of the upside-down stomach. The clockwise rotation of the stomach 180 degrees or more along its longitudinal (organoaxial) axis results in incomplete or complete volvulus and obstruction. In complete volvulus, double air-fluid levels behind the heart result in a characteristic roentgenographic picture, the socalled diamond shadow, which represents the dilated proximal and distal portions of the stomach (Fig. 1). Increasing gastric distention and pressure on the stomach from the edges of the hernia may lead to impairment of venous drainage and strangulation (Fig. 2). Diagnosis is usually made by a history of typical symptoms and clinical findings. Complete obstruction and volvulus may lead to epigastric pain and an inability to vomit, and a nasogastric tube cannot be passed into the stomach. This is known as Borchardt s triad [14]. Frontal and lateral chest films and contrast studies of the esophagus and stomach confirm the diagnosis [4]. The entrapped thoracic stomach retains enough gas to show a radiolucent pocket within the normal cardiac silhouette. Double air-fluid levels behind the heart represent complete volvulus with dilated proximal and distal portions of the stomach. FIG. 1. Lateral view of the chest. There is complete volvulus of the stomach with obstruction, and a diamond shadow is seen. VOL. 16, NO. 6, DECEMBER,

4 OZDEMIR, BURKE, AND IKINS FIG. 2. Posteroanterior view of barium swallow study showing upside-down stomach. Esophagoscopy is rarely needed for diagnostic purposes. Only 6 patients had esophagoscopic examination, and none of them showed evidence of reflux or esophagitis. Operative Findings and Results Twelve of the 31 patients underwent emergency operation, and 19 had elective repair of the paraesophageal hernia. All had left thoracotomies through the seventh or eighth intercostal space. Twenty-six patients had Belsey Mark IV operations; 2 had Allison type repairs; 2 had resection for gangrene of the distal esophagus and cardia; and 1 had closure of the perforation, reduction of the hernia, and gastrostomy. Stomach alone was found in the large hernia sac in 25 patients. Other abdominal organs such as the spleen, small bowel, and large bowel were present with the stomach in 6 patients. Four patients had complete volvulus with rotation of the stomach more than 180 degrees clockwise around its long axis. Fo 1 low-up The patients have been followed six months to 11 years with a mean follow-up of 6 years. Five patients were lost to follow-up but were well when last seen (six and nine months and 1, 2, and 4 years postoperatively). Twentyone patients have had excellent results. There were 2 operative deaths in the series. Both of these occurred in patients who had emergency operations for acute obstruction. One patient, a 75-year-old woman who had emergency repair for acute obstruction, died of a myocardial infarction: and the second patient, who had resection of the distal esophagus and stomach for gangrene 550 THE ANNALS OF THORACIC SURGERY

5 Paraesophageal Hernia and perforation, died due to a subsequent small retroperitoneal abscess which eroded into the splenic artery. There were 3 recurrences. One patient had had an Allison repair and 5 years later developed peptic esophagitis which required resection of the distal esophagus. Two of the recurrences were in patients who had had Mark IV repairs initially for their paraesophageal hernia. One developed peptic esophagitis 2 years later and was reoperated upon with the same repair. The other developed an unusual paraesophageal hernia on the right side, and the repair was accomplished with a thoracoabdominal approach. All 3 remain well. Comment In paraesophageal hernia the relationship between the esophagus and stomach remains normal. The esophagogastric junction may or may not be mobile, but it usually stays below the diaphragm. The etiology of paraesophageal hernia is not known, but it appears to be acquired since it is rarely present before middle age. The pathophysiological mechanism of the paraesophageal hernia is related to that of the normal intrathoracic pressure, which is negative relative to intraabdominal pressure, thereby allowing the stomach and abdominal viscera to enter the chest. At the beginning, the fundus of the stomach rolls into the thoracic cavity and posterior mediastinum anterior to the esophagus. Often the entire stomach may herniate into the hernia sac, with the greater curvature of the stomach moving uppermost and the duodenum approaching the esophagogastric junction. The upside-down stomach is partially obstructed and empties inadequately through a narrow hernial ring. Similarly, venous and lymphatic drainage are often impaired. Culver and his associates [6] have proposed that obstruction can occur when the cardiac portion of the herniated stomach redescends through the hiatus, compressing the distal gastric outlet: distention of the proximal stomach ensues. We believe that obstruction in paraesophageal hernia is related to the fixed and anchored esophagogastric junction and clockwise rotation of the stomach along the longitudinal axis. When this rotation takes place, the fundus of the stomach moves into the posterior mediastinum in front of the esophagus and the pylorus comes up anteriorly. If the stomach twists more than 90 degrees with incomplete or complete volvulus, obstruction can occur. Increasing gastric distention and pressure on the stomach from the edges of the hernial ring may lead to impairment of blood supply and strangulation [3, 51. Delay in diagnosis and impaired blood supply result in the catastrophic and lethal complications of the paraesophageal hernia, which are strangulation, perforation, and mediastinitis [I 1, 121. Once these complications occur, operative deaths reach 50% [7, 101. In our experience, upper gastrointestinal bleeding or hypochromic VOL. 16, NO. 6, DECEMBER,

6 OZDEMYR, BURKE, AND IKINS anemia due to vascular stasis and edema, delayed gastric emptying, and ulceration are serious complications of the paraesophageal hernia. The reported incidence of both occult and clinical bleeding is twice as great as in sliding hiatus hernia [13]. Macrocytic anemia also occurs (4 of our patients), possibly from gastric dysfunction; but it is not as common as the hypochromic, microcytic anemia of chronic blood loss. The paraesophageal hernia presents a different clinical picture in that the symptoms are often absent or mild for a long time. Skinner and Belsey [13] reported that of 21 patients treated medically because of minimal symptoms, 6 died from complications. The hernias in the other 15 patients were then repaired. Upon diagnosing obstruction of paraesophageal hernia, operation should be performed immediately or following the least possible delay for the purpose of correcting fluid and electrolyte imbalances. Gastric decompression should be attempted with a soft nasogastric tube in order to prevent aspiration. The left thoracotomy is the optimal approach. The hernia should be freed and reduced. Abnormally large esophageal hiatus should be reconstructed with a Belsey Mark IV repair because dissection of the large hernia sac and simple repair predispose to a sliding hernia and the later problems of gastroesophageal reflux. Wedge or limited esophagogastric resection is indicated for gangrene and perforation. The mediastinum and retroperitoneal area should be widely drained transthoracically. The abdominal approach is said to be quite good and satisfactory [2, 9, 151; but in the presence of obstruction (incarceration, strangulation, volvulus, or perforation), resection and repair are usually not possible. Mildness or absence of symptoms is not an indication against surgical repair. All should be repaired unless there are overwhelming contraindications. References 1. Allison, P. R. Reflux esophagitis, sliding hiatal hernia and the anatomy of repair. Surg. Gynecol. Obstet. 92:419, Babb, R. R., Peck, 0. C., and Jamplis, R. W. Gastric volvulus and obstruction in paraesophageal hiatus hernia. Am. J. Dig. Dis. 17:119, Beardsley, J. M., and Thompson, W. R. Acutely obstructed hiatal hernia. Ann. Surg. 159:49, Blatt, E. S., Schneider, H. J., Wiot, J. F., and Felson, B. Roentgen findings in obstructed diaphragmatic hernia. Radiology 79:648, Bosher, L. H., Fishman, L., Webb, W. R., and Old, L. Strangulated diaphragmatic hernia with gangrene and perforation of the stomach. Dis. Chest 37:504, Culver, G. J., Pirson, H. S., and Bean, B. C. Mechanism of obstruction in para-esophageal diaphragmatic hernias. J.A.M.A. 181 :933, Dialgaard, J. B. Volvulus of the stomach. Actu Chir. Scand. 103:131, Harrington, S. W. Various types of diaphragmatic hernia treated surgically: Report of 430 cases. Surg. Gynecol. Obstet. 86:735, Hill, L. D., and Tobias, J. A. Paraesophageal hernia. Arch. Surg. 96:735, THE ANNALS OF THORACIC SURGERY

7 Paraesophageal Hernia Hoffman, E. Strangulated diaphragmatic hernia. Thorax 23:541, Keshishian, J. M., and Cox, P. A. Diagnosis and management of strangulated diaphragmatic hernias. Surg. Gynecol. Obstet. 115:626, Leininger, B. J., and Puestow, C. B. Esophageal rupture associated with an incarcerated paraesophageal hernia and pyloric obstruction. Am. J. Surg. 119:368, Skinner, D. B., and Belsey, R. H. R. Surgical management of esophageal reflux and hiatus hernia: Long term results with 1030 patients. J. Thorac. Cardiovasc. Surg. 52:33, Soko, A. B., and Morgenstern, L. Gastric volvulus complicating paraesophageal hiatal hernia. Calif. Med. 117:66, Woodward, E. R., Rayl, J. E., and Clarke, J. M. Curr. Probl. Surg., December, Discussion Esophageal hiatus hernia. DR. FRANKLIN HENRY ELLIS, JR. (Boston, Mass.): I enjoyed hearing Dr. Ozdemir s ideas about paraesophageal hiatal hernia, and I agree with most, but not all, of what he said. I would like to emphasize some of his remarks and to point out a few areas of disagreement. It is quite appropriate that he should stress the pathological anatomy of paraesophageal hiatal hernia. For reasons I have never understood, there remains in the minds of some a misconception as to the site of the defect in the diaphragm through which the fundus migrates in a paraesophageal hiatal hernia. The stomach goes through the esophageal hiatus and not through a separate opening in the diaphragm alongside the hiatus. In other words, it is not a parahiatal hernia but a paraesophageal hernia. Parahiatal hernias are practically nonexistent, even though an illustration in a thoracic surgery textbook, which was a classic some years ago, illustrates a parahiatal hernia over the legend paraesophageal hiatal hernia. Dr. Ozdemir emphasized another important point which bears on the physiology of the lower end of the esophagus in paraesophageal hiatal hernia. In contrast to patients with a sliding esophageal hiatal hernia, symptoms of gastroesophageal reflux are absent because the lower esophageal sphincter functions normally and is in its normal position. I am surprised, therefore, at Dr. Ozdemir s selection of the Belsey operation, for if indeed the anatomy and physiology of the lower sphincter are normal, it would seem unwise to disturb this important area, as one must do in a Belsey type of repair. Rather, I would prefer. to concentrate on repairing the defect in the diaphragm anteriorly, as in a Collis repair, and to leave the esophagogastric junction undisturbed. In support of this concept is the fact that 2 of Dr. Ozdemir s patients developed gastroesophageal reflux postoperatively when in fact they did not have reflux preoperatively. Had the esophagogastric junction been left undisturbed, I suspect that reflux would not have developed. DR. ROBERT W. RIEMER (Providence, R.I.): I congratulate the authors for bringing this important subject to our attention, particularly the complications of this type of hernia. I wish to emphasize the prevention of such complications by early repair, before they have a chance to develop. I also want to point out the importance of the abdominal approach when complications do occur. The complications that may develop with this type of hernia are those of incarceration with obstruction; (2) incarceration without obstruction; and 8 strangulation of the stomach. Several types of twisting of the stomach may occur. One may have an organoaxial volvulus in which the stomach rotates on its long axis, or a mesenteroaxial volvulus, in which the pylorus rotates upon the fundus of the stomach. VOL. 16, NO. 6, DECEMBER,

8 OZDEMIR, BURKE, AND IKINS When complications, such as strangulation or obstruction occur, the patient is treated on the medical service as having coronary artery disease. After several days of strangulation and gangrene, an emergency consultation is requested. Many of these patients are over the age of 70 and present a brittle surgical condition. We approach these complicated hernias from the abdomen. Preoperatively we decompress the stomach with a nasogastric tube with care being taken not to shove the tube through the edematous and necrotic gastroesophageal junction. At the time of operation one is hard put, as Dr. Ellis has mentioned, to identify a definite separation between the esophageal hiatus and the paraesophageal portion of the hernial ring. This is usually obliterated by the prolonged position of the stomach in the paraesophageal position. At the time of operation we decompress the stomach by gastrostomy if gangrene or strangulation is present. One only needs to close the defect in the diaphragm to accomplish repair. Again, I congratulate the authors for discussing this important subject. Complications such as strangulation or gangrene of the stomach can be prevented by early repair of these paraesophageal hernias through either a transthoracic or an abdominal approach. DR. IKINS: I wish to express my thanks to Dr. Ellis for his erudition and his very fine, instructive comments concerning his differing approach to our plan. I am aware of his preference for an abdominal approach in this most particular operation, and aside from several points, I would think that the abdominal approach and the thoracic approach probably accomplish the same thing. Unfortunately, in a large number of these patients we suspect we have incarceration when in fact we have strangulation. In a large number we expect that we have strangulation only, and we actually have perforation and mediastinitis. And in that small, very important and mortal group who have perforation and gangrene, it is infinitely more important to be in the chest where one can perform anastomosis. The use of the Belsey repair does indeed disturb the cardioesophageal angle and the integrity of the gastroesophageal mechanism, and I know Dr. Ellis disagrees with this. I think it can be repaired in the manner that God and Mr. Belsey intended and that it works very well. The ultimate point, I think, of Dr. Ozdemir s paper and subsequent papers is to emphasize that although we assume that the hiatus hernia is sort of a normal variant of the gastrointestinal series in this country, in fact it should be considered a potentially dangerous and lethal one, and there are many forms of gastroesophageal reflux that in themselves are dangerous. Other papers will touch on the hiatus hernia that presents as an acute respiratory disease. 554 THE ANNALS OF THORACIC SURGERY

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