CASE REPORT Boerhaave's syndrome
A Case of Boerhaave's Syndrome Presenting as Diffuse Left Pulmonary Infiltrate A case of spontaneous esophageal rupture (Boerhaave's syndrome) is presented. The patient was referred from an outside hospital emergency department to Los Angeles County/University of Southern California Medical Center with a history of acute left-sided chest pain immediately after an episode of forceful vomiting. An upright chest radiograph revealed a left hydropneumothorax. The diagnosis of Boerhaave's syndrome was confirmed with the placement of a chest tube and extraction of serosanguinous fluid and partially digested food particles from the left hemithorax. The patient underwent surgical repair and was discharged from the hospital in good condition. Boerhaave's syndrome is extremely rare. The predominant symptoms of chest pain and dyspnea also are found in many common disease entities, making early diagnosis difficult. Delay in diagnosis and treatment results in substantial morbidity and mortality. This case exemplifies the importance of obtaining an upright chest radiograph to make a prompt diagnosis. [Kyriacou DN: A case of Boerhaave's syndrome presenting as diffuse left pulmonary infiltrate. Ann Emerg Med November 1991;20:1239-1242.]
Demetrios N Kyriacou, MD Los Angeles, California From the Department of EmergencY Medicine, University of Southern California School of Medicine, Los Angeles, California. Received for publication November 12, 1990. Revision received May 13, 1991. Accepted for publication May 28, 1991. Address for reprints: Demetrios N Kyriacou, MD, LAC/USC Medical Center, Department of Emergency Medicine, 1200 North State Street, Los Angeles, California 90033.
INTRODUCTION Spontaneous rupture of the esophagus received its eponym from the preeminent 18th-century Dutch physician Hermann Boerhaave. One evening in 1724, Boerhaave was hastily called to see Baron John van Wassenaer, Grand Admiral of The Netherlands, who had suddenly become extremely ill. After feasting, the baron attempted to relieve his gastric discomfort by vomiting with the help of an emetic. On retching, the baron experienced severe sudden chest pain. He complained that something near the upper part of his stomach had ruptured and that the pain was so severe that death was coming and inevitable. The baron's prediction was accurate. During the autopsy, Boerhaave found a tear in the distal esophagus extending through the pleura with food particles and fluid in the pleural cavities. 1 Boerhaave's syndrome is extremely rare. Because of the unusual occurrence and variable presentation, it is not often considered in the initial differential diagnoses of chest or abdominal pain. Delay in treatment results in a high level of morbidity and mortality. The case of a man with Boerhaave's syndrome referred to Los Angeles County/University of Southern California Medical Center is presented.
CASE REPORT A 33-year-old man was transferred to the ED of the LAC/USC Medical Center from an outside hospital with a diagnosis of diffuse left pulmonary infiltrate of unknown etiology. He was transferred for further evaluation and definitive treatment at a tertiary-care facility. The patient was in good health until approximately four hours before presentation when after overindulging at a Chinese restaurant, he felt midepigastric discomfort. In an attempt to relieve his discomfort, the patient drank water with an over-the-counter antacid preparation. Shortly thereafter, he vomited forcefully, retching four or five times. He immediately felt a severe, sharp left anterior chest pain associated with dyspnea. There was no hematemesis or melena. The patient stated that he may have aspirated while vomiting, causing the referring physician to entertain the possible
20:11 November 1991
Annals of Emergency Medicine
1239/99
BOERHAAVE'S SYNDROME Kyriacou
FIGURE 1. Initial chest radiograph in
supine position showing opacification of left lung field. Close examinatio~ reveals partially collapsed left upper and lower lobes. FIGURE 2. Second chest radiograph in upright position (portable anteroposterior) reveals a large left hydropneumothorax. diagnosis of aspiration pneumonitis. In addition, while retching in his bathroom, the patient fell, striking his left posterior chest against the bathtub. The patient had no history of a similar episode of v o m i t i n g w i t h chest pain. There was no history of peptic ulcer disease, esophagitis, abdominal pain, s p o n t a n e o u s pneumothorax, or respiratory difficulty. The patient stated that he drank alcohol only occasionally. Blood pressure was 136/98 m m Hg; pulse, 64; respirations, 18 with moderate distress; and oral temperature, 36.4 C. On physical examination, the patient was writhing in pain but alert and responsive to questioning. The skin was pale and dry. The head was normocephalic, and the neck was supple and w i t h o u t palpable subc u t a n e o u s e m p h y s e m a or jugular venous distension. Examination of the lungs r e v e a l e d a b s e n t b r e a t h sounds at the left base with dullness to percussion and tenderness over the inferolateral left chest wall. The patient was splinting with respiration. The heart tones were normal, and there was no murmur or Ham100/1240
m a n ' s sign. The abdomen was flat and s o f t w i t h d e c r e a s e d b o w e l sounds. There was 1 + tenderness in the left upper quadrant and diffuse guarding but no rigidity, h e p a t o splenomegaly, or masses. There was n o r m a l rectal t o n e and rio gross blood per rectum. Laboratory examination from the referring hospital showed sodium, 141 mEq/L; potassium, 4.6 mEq/L; chloride, 100 mEq/L; urea nitrogen, 17 mg%; creatinine, 1.3 mg%; and glucose, 175 mg%. The WBC count was 12,600/mm3; hemoglobin was 15.4 g/dL; and h e m a t o c r i t w a s 46.8%, w i t h 81% s e g m e n t s , 5% bands, 12% l y m p h o c y t e s , and 2% monocytes. The platelet count was 304,000/mm a. The ECG showed sinus bradycardia at a rate of 47 without Q waves, ST-T wave changes, or ectopy. The chest radiograph from the referring hospital was taken with the patient in the supine position and interpreted as left lung opacification suggesting diffuse p u l m o n a r y infiltrate. On presentation to LAC/USC Medical Center, re-examination of the chest radiograph revealed partial collapse of the left upper and lower lobes with a possible hydrothorax (Figure 1). A repeat chest radiograph taken with the patient in an upright position (portable anteroposterior) revealed a large left hydropneumothorax with partial collapse of the lower and upper lobes. No pneum o m e d i a s t i n u m or fractured ribs were identified (Figure 2). The p a t i e n t was given 8 mg of morphine sulfate IV for pain. A tube Annals of Emergency Medicine
t h o r a c o s t o m y was placed into the left chest with immediate extraction of 1,150 mL of serosanguinous fluid and partially digested food particles. A nasogastric tube was placed with aspiration of 50 mL of similar contents. The patient was admitted to the surgical admitting ward and placed on p e n i c i l l i n , g e n t a m i c i n , clindamycin, and ranitidine IV. He underwent an upper gastrointestinal radiographic series with contrast dye, which revealed extravasation from the distal esophagus into the left pleural space. A p p r o x i m a t e l y ten hours after his initial episode of vomiting and chest pain, the patient underwent exploratory celiotomy with primary repair of the distal esophagus using a Thai patch and Hill gastropexy. A S t a m m gastrostomy (for gastric drainage) and feeding jejunostomy were also done. The postoperative hospital course was unremarkable except for a lowgrade fever (38~0 to 38.1 C) on days 3 and 4. The patient was continued on IV penicillin, gentamicin, and clindamycin for ten days. Oral feedings were begun on day 8 and were well tolerated. The patient was discharged in good condition 14 days after admission.
DISCUSSION Boerhaave's syndrome is the panm u r a l a t r a u m a t i c r u p t u r e of the esophagus. This definition excludes perforations resulting from iatrogenic instrumentation, surgical complications, or foreign bodies. Rupture is a result of an acute, dramatic increase 20:11 November 1991
BOERHAAVE'S SYNDROME Kyriacou
in the intraluminal pressure of the esophagus, most often from vomiting. There is often a history Of overeating. Numerous other causes of increased i n t r a - a b d o m i n a l pressure have been implicated in rare cases, including defecation, parturition, coughing, and seizure. 2-4 A p p r o x i m a t e l y 90% to 95% of spontaneous ruptures of the esophagus occur at the posterolateral aspect of the esophagus just proximal to the gastroesophageal junction. The rupture involves all layers of the esophageal wall. The rent is usually 1 to 6 cm in length and almost always longitudinal. The reason for the common occurrence of rupture at this specific a n a t o m i c location is not clear, but it may be related to lack of supporting anatomic structure.2,4, 5 It has also been postulated that there is an inherent weakness in the distal esophagus from embryologic development. 6 Pre-existing factors that have been implicated in Boerhaave's syndrome include peptic ulcer disease, esophagitis, esophageal stricture, MalloryWeiss syndrome, and excessive alcohol use, although none of these predispositions is required for rupture to occur.2-4, 7 In addition, in a review of 47 cases of Boerhaave's syndrome, Abbott et al found neurological or neurosurgical disease in 14 patients and severe hypertension in 11, suggesting that imbalance of autonomic nerve activity might be responsible for esophageal rupture. 8 Establishing the correct diagnosis can be extremely difficult. This is due to the variable presentation and rarity of Boerhaave's syndrome. Pain is the most common symptom, often preceded by a violent episode of vomiting. It is described as severe and incapacitating, with a sharp q u a l i t y . The pain is substernal, left chest, or midepigastric and may radiate to the neck, shoulder, back, or left upper quadrant.2-4, 8 It is not relieved by position, and large doses of parenteral pain medication are usually required to provide relief to the patient. Vomiting is also very common but may not be present in as many as 25% of cases. In the absence of any history of vomiting, the diagnosis is especially difficult.3,4, a Hematemesis is often seen with the vomiting, although massive hematemesis may suggest bleeding ulcer, erosive gastritis, or Mallory-Weiss syndrome as 20:11 November 1991
the cause of emesis.4, 7-9 Dyspnea is a result of the p l e u r i t i c i r r i t a t i o n caused by gastric contents in the pleural space and mediastinum and from respiratory embarrassment secondary to the hydropneumothorax. The vital signs m a y be normal early in the presentation of Boerhaave's syndrome, with the patient becoming tachycardic and hypotensive if diagnosis and treatment are delayed. Pallor, cyanosis, and diaphoresis are indicative of impending hemodynamic collapse. Decreased or absent breath sounds, if elicited, are more often found in the left lung field because rupture is usually on the left posterolateral aspect of the distal esophagus, releasing gastric contents into the left pleural cavity. Hamman's sign is almost pathognomonic for mediastinal emphysema when found, but this sound may be confused with a pericardial friction rub or abnormal heart sound, obscuring the true diagnosis. Tenderness may be elicited over the chest wall. Midepigastric and left upper quadrant abdominal tenderness and rigidity may be found, often associated w i t h d e c r e a s e d or a b s e n t b o w e l sounds. Subcutaneous emphysema of the anterior neck is usually a late (12 to 24 hours) finding. 8 Laboratory examination may reveal an elevated WBC count with a left shift, especially in cases with a delayed presentation. Hemoconcentration in the range of a 46% to 50% hematocrit is a common finding. This is secondary to loss of fluids through excessive gastric secretions into the mediastinal tissues and pleural spaces. In the review by Abbott et al, half of the patients had a hematocrit of more than 50%.8 The most valuable diagnostic examination is the upright chest radiograph. Pneumomediastinum and hydropneumothorax are the most comm o n findings, but s u b c u t a n e o u s emphysema, pneumothorax, pleural effusion, pulmonary infiltrate, and pneumoperitoneum also can be seen. Pneumomediastinum is most likely to be found along the left cardiac border, but it m a y be p r e s e n t as a V-shaped linear density formed by air dissecting through the fascial planes of the mediastinal and diaphragmatic pleura near the lower esophagus. T M Radiographic findings are predominantly in the left hemithorax, but in delayed cases may be present bilatAnnals of Emergency Medicine
erally; they are rarely found exclusively in the right hemithorax# o If an upright chest radiograph is not feasible, a lateral decubitus radiograph may elucidate pleural air-fluid levels. Occasionally, the chest radiograph is normal, and a repeat radiograph taken two to three hours later may show significant changes to assist in establishing the diagnosis. The upright chest radiograph is rapid and efficient and, because mortality and m o r b i d i t y increase s u b s t a n t i a l l y with even modest delays in definitive treatment, its value is obvious. Water-soluble contrast media studies are used to verify the diagnosis and determine the exact anatomic location of the perforation. In cases without a clear clinical presentation and normal or minimal radiographic findings, computed tomography has been shown to be effective in elucidating mediastinal emphysema and may be useful in differentiating Boerhaave's syndrome from other causes of acute, severe chest pain (ie, aortic dissection). 12 If contrast media studies and computed tomography are not available, m e t h y l e n e blue swallows can be performed, confirming rupture if the pleural fluid becomes stained. 13 Many disease entities encompass a clinical presentation of chest or abdominal pain with vomiting, dyspnea, and pallor. The differential diagnoses include myocardial ischemia, acute pericarditis, pulmonary embolism, spontaneous pneumothorax, Mallory-Weiss syndrome, incarcerated hiatal hernia, perforated peptic ulcer, acute pancreatitis, biliary colic, and mesenteric occlusion. Misdiagnosis on initial presentation is extremely common, with the resulting delay in treatment leading to an alarmingly high mortality rate.2, 3 The most frequent misdiagnoses are perforated peptic ulcer and myocardial infarction.g,4,7,14 In our case, the diagnosis of aspiration pneumonitis was considered by the referring physician, possibly due to the questionable history of aspiration with vomiting. The history of trauma to the left posterior chest and the size of the effusion on the upright chest radiograph suggested a large hemopneumothorax. But the patient's vital signs were stable, and the hemoglobin and hematocrit were elevated, not lowered. Insertion of the left chest tube thoracostomy and ex1241/101
BOERHAAVE'S SYNDROME Kyriacou
traction of serosanguinous fluid and food particles confirmed the diagnosis of Boerhaave's syndrome. Definitive treatment is the surgical repair of the esophageal perforation with irrigation and debridement of the mediastinal and pleural tissues, is Preoperative treatment involves fluid resuscitation, evacuation of gastric contents from the pleural cavity and stomach, and initiation of broads p e c t r u m antibiotics. R a n i t i d i n e s h o u l d be s t a r t e d to r e d u c e the acidity of the gastric secretions. The high rate of p o s t o p e r a t i v e complications is related to the breakd o w n of the e s o p h a g e a l w o u n d . B r e a k d o w n can r e s u l t in m e d i astinitis, empyema, fistula formation, and sepsis. Delay of treatment results in decreased efficacy of the surgical repair and a marked increase in m o r b i d i t y and m o r t a l i t y . The cause of death in untreated patients is hemodynamic failure secondary to hypovolemia, sepsis, and compression on the mediastinum by gastric contents or a pneumothorax.
SUMMARY Boerhaave's syndrome is the panm u r a l a t r a u m a t i c r u p t u r e of the esophagus resulting in spillage of gastric contents into the mediastinal tissues and pleural cavity. It occurs
102/1242
most often in men between the ages of 40 and 60 years. The most common symptom is severe, acute left anterior or substernal chest pain or abdominal pain, often preceded by forceful vomiting after ingestion of a large meal. Respiratory difficulty and signs of impending h e m o d y n a m i c collapse are also common. The upright chest radiograph is the single most important laboratory examination. Because of the common symptoms of chest pain and dyspnea and the extremely low incidence of spontaneous esophageal rupture, misdiagnosis often occurs. A significant delay in proper diagnosis and treatment can result in substantial morbidity and mortality. Because survival is directly related to the early onset of treatment, the importance of correct i n i t i a l d i a g n o s i s in the case of Boerhaave's s y n d r o m e c a n n o t be overstated. The author thanks Dr Michael Kirk for his astute and rapid interpretation of the initial chest radiograph in this case.
REFERENCES 1. Boerhaave H: Atrocis nec descripti prius, morbi historia. The first translation of the classic case report of the rupture of the esophagus, with annotations. Bull Med Libr Assoe 1955;43:217-240. 2. Derbes VJ, Mitchell RE: Rupture of the esophagus.
Annals of Emergency Medicine
Surgery 1956;39:688-709,865-888. o 3. Wilson RF, Sarver EJ, Arbuln A, et al: Spontaneous perforation of the esophagus. A n n Thorac Surg 1971; 12:291-296. 4. Callaghan J: The Boerhaave syndrome (spontaneous rupture of the oesphagus). Br J Surg 1972;59:41-44. 5. Sawyers JL, Lane CE, Foster JH, et al: Esophageal perforation: An increasing challenge. A n n Thorac Surg 1975;19:233-238. 6. Mosher HP: The lower end of the esophagus at birth and in the adult. 7 Laryngol OtoI 1930;45:161-180. 7. Curci JJ, Horman MJ: Boerhaave's syndrome: The importance of early diagnosis and treatment. Ann Surg 1976;183:401-408. 8. Abbott, OA, Mansour KA, Logan WD, et al: Atram matic so-called "spontaneous rupture of the esophagus": A review of 47 personal cases with comments on a new method of surgical therapy. 7 Thorac Cardiovasc Surg 1970;59:67-83. 9. Uehara DT, Dymowski JJ, Schwartz J, et al: Chest pain, shock, and pneumomediastiuenm in a previously healthy 56-year-old man. A n n Emerg M e d 1987;16: 359-364. 10. Rogers L£, Puig AW, Dooley BN, et al: Diagnostic considerations in pathophysiologic-roentgenologic approach to Boerhaave's syndrome and spontaneous pneumomediastinum. A m J Roentgenol 1972~115:495 511. 11. Naclerio EA: The "V-sign" in the diagnosis of spontaneous rupture of the esophagus. Arn J Surg 1957;93: 291-298. 12. Jaworski A, Fischer R, Lippmann M: Boerhaave's syndrome: Computed tomographic findings and diagnostic considerations. Arch Intern M e d 1988;148: 223-224. 13. Walker WS, Cameron EWJ, Walbaum PR: Diagnosis and management of spontaneous transmural rupture of the oeseophagus (Boerhaave's syndrome). Br J Surg 1985;72:204-207. 14. Bennett DJ, Deveridge RJ, Wright JS: Spontaneous rupture of the esophagus: A review with reports of six cases. Surgery 1970;68:766-770. 15. Thai AP, Hatafuku T: Improved operation for esophageal rupture, lAMA 1964;188:126-128.
20:11 November 1991
A Case of Boerhaave's Syndrome Presenting as Diffuse Left Pulmonary Infiltrate A case of spontaneous esophageal rupture (Boerhaave's syndrome) is presented. The patient was referred from an outside hospital emergency department to Los Angeles County/University of Southern California Medical Center with a history of acute left-sided chest pain immediately after an episode of forceful vomiting. An upright chest radiograph revealed a left hydropneumothorax. The diagnosis of Boerhaave's syndrome was confirmed with the placement of a chest tube and extraction of serosanguinous fluid and partially digested food particles from the left hemithorax. The patient underwent surgical repair and was discharged from the hospital in good condition. Boerhaave's syndrome is extremely rare. The predominant symptoms of chest pain and dyspnea also are found in many common disease entities, making early diagnosis difficult. Delay in diagnosis and treatment results in substantial morbidity and mortality. This case exemplifies the importance of obtaining an upright chest radiograph to make a prompt diagnosis. [Kyriacou DN: A case of Boerhaave's syndrome presenting as diffuse left pulmonary infiltrate. Ann Emerg Med November 1991;20:1239-1242.]
Demetrios N Kyriacou, MD Los Angeles, California From the Department of EmergencY Medicine, University of Southern California School of Medicine, Los Angeles, California. Received for publication November 12, 1990. Revision received May 13, 1991. Accepted for publication May 28, 1991. Address for reprints: Demetrios N Kyriacou, MD, LAC/USC Medical Center, Department of Emergency Medicine, 1200 North State Street, Los Angeles, California 90033.
INTRODUCTION Spontaneous rupture of the esophagus received its eponym from the preeminent 18th-century Dutch physician Hermann Boerhaave. One evening in 1724, Boerhaave was hastily called to see Baron John van Wassenaer, Grand Admiral of The Netherlands, who had suddenly become extremely ill. After feasting, the baron attempted to relieve his gastric discomfort by vomiting with the help of an emetic. On retching, the baron experienced severe sudden chest pain. He complained that something near the upper part of his stomach had ruptured and that the pain was so severe that death was coming and inevitable. The baron's prediction was accurate. During the autopsy, Boerhaave found a tear in the distal esophagus extending through the pleura with food particles and fluid in the pleural cavities. 1 Boerhaave's syndrome is extremely rare. Because of the unusual occurrence and variable presentation, it is not often considered in the initial differential diagnoses of chest or abdominal pain. Delay in treatment results in a high level of morbidity and mortality. The case of a man with Boerhaave's syndrome referred to Los Angeles County/University of Southern California Medical Center is presented.
CASE REPORT A 33-year-old man was transferred to the ED of the LAC/USC Medical Center from an outside hospital with a diagnosis of diffuse left pulmonary infiltrate of unknown etiology. He was transferred for further evaluation and definitive treatment at a tertiary-care facility. The patient was in good health until approximately four hours before presentation when after overindulging at a Chinese restaurant, he felt midepigastric discomfort. In an attempt to relieve his discomfort, the patient drank water with an over-the-counter antacid preparation. Shortly thereafter, he vomited forcefully, retching four or five times. He immediately felt a severe, sharp left anterior chest pain associated with dyspnea. There was no hematemesis or melena. The patient stated that he may have aspirated while vomiting, causing the referring physician to entertain the possible
20:11 November 1991
Annals of Emergency Medicine
1239/99
BOERHAAVE'S SYNDROME Kyriacou
FIGURE 1. Initial chest radiograph in
supine position showing opacification of left lung field. Close examinatio~ reveals partially collapsed left upper and lower lobes. FIGURE 2. Second chest radiograph in upright position (portable anteroposterior) reveals a large left hydropneumothorax. diagnosis of aspiration pneumonitis. In addition, while retching in his bathroom, the patient fell, striking his left posterior chest against the bathtub. The patient had no history of a similar episode of v o m i t i n g w i t h chest pain. There was no history of peptic ulcer disease, esophagitis, abdominal pain, s p o n t a n e o u s pneumothorax, or respiratory difficulty. The patient stated that he drank alcohol only occasionally. Blood pressure was 136/98 m m Hg; pulse, 64; respirations, 18 with moderate distress; and oral temperature, 36.4 C. On physical examination, the patient was writhing in pain but alert and responsive to questioning. The skin was pale and dry. The head was normocephalic, and the neck was supple and w i t h o u t palpable subc u t a n e o u s e m p h y s e m a or jugular venous distension. Examination of the lungs r e v e a l e d a b s e n t b r e a t h sounds at the left base with dullness to percussion and tenderness over the inferolateral left chest wall. The patient was splinting with respiration. The heart tones were normal, and there was no murmur or Ham100/1240
m a n ' s sign. The abdomen was flat and s o f t w i t h d e c r e a s e d b o w e l sounds. There was 1 + tenderness in the left upper quadrant and diffuse guarding but no rigidity, h e p a t o splenomegaly, or masses. There was n o r m a l rectal t o n e and rio gross blood per rectum. Laboratory examination from the referring hospital showed sodium, 141 mEq/L; potassium, 4.6 mEq/L; chloride, 100 mEq/L; urea nitrogen, 17 mg%; creatinine, 1.3 mg%; and glucose, 175 mg%. The WBC count was 12,600/mm3; hemoglobin was 15.4 g/dL; and h e m a t o c r i t w a s 46.8%, w i t h 81% s e g m e n t s , 5% bands, 12% l y m p h o c y t e s , and 2% monocytes. The platelet count was 304,000/mm a. The ECG showed sinus bradycardia at a rate of 47 without Q waves, ST-T wave changes, or ectopy. The chest radiograph from the referring hospital was taken with the patient in the supine position and interpreted as left lung opacification suggesting diffuse p u l m o n a r y infiltrate. On presentation to LAC/USC Medical Center, re-examination of the chest radiograph revealed partial collapse of the left upper and lower lobes with a possible hydrothorax (Figure 1). A repeat chest radiograph taken with the patient in an upright position (portable anteroposterior) revealed a large left hydropneumothorax with partial collapse of the lower and upper lobes. No pneum o m e d i a s t i n u m or fractured ribs were identified (Figure 2). The p a t i e n t was given 8 mg of morphine sulfate IV for pain. A tube Annals of Emergency Medicine
t h o r a c o s t o m y was placed into the left chest with immediate extraction of 1,150 mL of serosanguinous fluid and partially digested food particles. A nasogastric tube was placed with aspiration of 50 mL of similar contents. The patient was admitted to the surgical admitting ward and placed on p e n i c i l l i n , g e n t a m i c i n , clindamycin, and ranitidine IV. He underwent an upper gastrointestinal radiographic series with contrast dye, which revealed extravasation from the distal esophagus into the left pleural space. A p p r o x i m a t e l y ten hours after his initial episode of vomiting and chest pain, the patient underwent exploratory celiotomy with primary repair of the distal esophagus using a Thai patch and Hill gastropexy. A S t a m m gastrostomy (for gastric drainage) and feeding jejunostomy were also done. The postoperative hospital course was unremarkable except for a lowgrade fever (38~0 to 38.1 C) on days 3 and 4. The patient was continued on IV penicillin, gentamicin, and clindamycin for ten days. Oral feedings were begun on day 8 and were well tolerated. The patient was discharged in good condition 14 days after admission.
DISCUSSION Boerhaave's syndrome is the panm u r a l a t r a u m a t i c r u p t u r e of the esophagus. This definition excludes perforations resulting from iatrogenic instrumentation, surgical complications, or foreign bodies. Rupture is a result of an acute, dramatic increase 20:11 November 1991
BOERHAAVE'S SYNDROME Kyriacou
in the intraluminal pressure of the esophagus, most often from vomiting. There is often a history Of overeating. Numerous other causes of increased i n t r a - a b d o m i n a l pressure have been implicated in rare cases, including defecation, parturition, coughing, and seizure. 2-4 A p p r o x i m a t e l y 90% to 95% of spontaneous ruptures of the esophagus occur at the posterolateral aspect of the esophagus just proximal to the gastroesophageal junction. The rupture involves all layers of the esophageal wall. The rent is usually 1 to 6 cm in length and almost always longitudinal. The reason for the common occurrence of rupture at this specific a n a t o m i c location is not clear, but it may be related to lack of supporting anatomic structure.2,4, 5 It has also been postulated that there is an inherent weakness in the distal esophagus from embryologic development. 6 Pre-existing factors that have been implicated in Boerhaave's syndrome include peptic ulcer disease, esophagitis, esophageal stricture, MalloryWeiss syndrome, and excessive alcohol use, although none of these predispositions is required for rupture to occur.2-4, 7 In addition, in a review of 47 cases of Boerhaave's syndrome, Abbott et al found neurological or neurosurgical disease in 14 patients and severe hypertension in 11, suggesting that imbalance of autonomic nerve activity might be responsible for esophageal rupture. 8 Establishing the correct diagnosis can be extremely difficult. This is due to the variable presentation and rarity of Boerhaave's syndrome. Pain is the most common symptom, often preceded by a violent episode of vomiting. It is described as severe and incapacitating, with a sharp q u a l i t y . The pain is substernal, left chest, or midepigastric and may radiate to the neck, shoulder, back, or left upper quadrant.2-4, 8 It is not relieved by position, and large doses of parenteral pain medication are usually required to provide relief to the patient. Vomiting is also very common but may not be present in as many as 25% of cases. In the absence of any history of vomiting, the diagnosis is especially difficult.3,4, a Hematemesis is often seen with the vomiting, although massive hematemesis may suggest bleeding ulcer, erosive gastritis, or Mallory-Weiss syndrome as 20:11 November 1991
the cause of emesis.4, 7-9 Dyspnea is a result of the p l e u r i t i c i r r i t a t i o n caused by gastric contents in the pleural space and mediastinum and from respiratory embarrassment secondary to the hydropneumothorax. The vital signs m a y be normal early in the presentation of Boerhaave's syndrome, with the patient becoming tachycardic and hypotensive if diagnosis and treatment are delayed. Pallor, cyanosis, and diaphoresis are indicative of impending hemodynamic collapse. Decreased or absent breath sounds, if elicited, are more often found in the left lung field because rupture is usually on the left posterolateral aspect of the distal esophagus, releasing gastric contents into the left pleural cavity. Hamman's sign is almost pathognomonic for mediastinal emphysema when found, but this sound may be confused with a pericardial friction rub or abnormal heart sound, obscuring the true diagnosis. Tenderness may be elicited over the chest wall. Midepigastric and left upper quadrant abdominal tenderness and rigidity may be found, often associated w i t h d e c r e a s e d or a b s e n t b o w e l sounds. Subcutaneous emphysema of the anterior neck is usually a late (12 to 24 hours) finding. 8 Laboratory examination may reveal an elevated WBC count with a left shift, especially in cases with a delayed presentation. Hemoconcentration in the range of a 46% to 50% hematocrit is a common finding. This is secondary to loss of fluids through excessive gastric secretions into the mediastinal tissues and pleural spaces. In the review by Abbott et al, half of the patients had a hematocrit of more than 50%.8 The most valuable diagnostic examination is the upright chest radiograph. Pneumomediastinum and hydropneumothorax are the most comm o n findings, but s u b c u t a n e o u s emphysema, pneumothorax, pleural effusion, pulmonary infiltrate, and pneumoperitoneum also can be seen. Pneumomediastinum is most likely to be found along the left cardiac border, but it m a y be p r e s e n t as a V-shaped linear density formed by air dissecting through the fascial planes of the mediastinal and diaphragmatic pleura near the lower esophagus. T M Radiographic findings are predominantly in the left hemithorax, but in delayed cases may be present bilatAnnals of Emergency Medicine
erally; they are rarely found exclusively in the right hemithorax# o If an upright chest radiograph is not feasible, a lateral decubitus radiograph may elucidate pleural air-fluid levels. Occasionally, the chest radiograph is normal, and a repeat radiograph taken two to three hours later may show significant changes to assist in establishing the diagnosis. The upright chest radiograph is rapid and efficient and, because mortality and m o r b i d i t y increase s u b s t a n t i a l l y with even modest delays in definitive treatment, its value is obvious. Water-soluble contrast media studies are used to verify the diagnosis and determine the exact anatomic location of the perforation. In cases without a clear clinical presentation and normal or minimal radiographic findings, computed tomography has been shown to be effective in elucidating mediastinal emphysema and may be useful in differentiating Boerhaave's syndrome from other causes of acute, severe chest pain (ie, aortic dissection). 12 If contrast media studies and computed tomography are not available, m e t h y l e n e blue swallows can be performed, confirming rupture if the pleural fluid becomes stained. 13 Many disease entities encompass a clinical presentation of chest or abdominal pain with vomiting, dyspnea, and pallor. The differential diagnoses include myocardial ischemia, acute pericarditis, pulmonary embolism, spontaneous pneumothorax, Mallory-Weiss syndrome, incarcerated hiatal hernia, perforated peptic ulcer, acute pancreatitis, biliary colic, and mesenteric occlusion. Misdiagnosis on initial presentation is extremely common, with the resulting delay in treatment leading to an alarmingly high mortality rate.2, 3 The most frequent misdiagnoses are perforated peptic ulcer and myocardial infarction.g,4,7,14 In our case, the diagnosis of aspiration pneumonitis was considered by the referring physician, possibly due to the questionable history of aspiration with vomiting. The history of trauma to the left posterior chest and the size of the effusion on the upright chest radiograph suggested a large hemopneumothorax. But the patient's vital signs were stable, and the hemoglobin and hematocrit were elevated, not lowered. Insertion of the left chest tube thoracostomy and ex1241/101
BOERHAAVE'S SYNDROME Kyriacou
traction of serosanguinous fluid and food particles confirmed the diagnosis of Boerhaave's syndrome. Definitive treatment is the surgical repair of the esophageal perforation with irrigation and debridement of the mediastinal and pleural tissues, is Preoperative treatment involves fluid resuscitation, evacuation of gastric contents from the pleural cavity and stomach, and initiation of broads p e c t r u m antibiotics. R a n i t i d i n e s h o u l d be s t a r t e d to r e d u c e the acidity of the gastric secretions. The high rate of p o s t o p e r a t i v e complications is related to the breakd o w n of the e s o p h a g e a l w o u n d . B r e a k d o w n can r e s u l t in m e d i astinitis, empyema, fistula formation, and sepsis. Delay of treatment results in decreased efficacy of the surgical repair and a marked increase in m o r b i d i t y and m o r t a l i t y . The cause of death in untreated patients is hemodynamic failure secondary to hypovolemia, sepsis, and compression on the mediastinum by gastric contents or a pneumothorax.
SUMMARY Boerhaave's syndrome is the panm u r a l a t r a u m a t i c r u p t u r e of the esophagus resulting in spillage of gastric contents into the mediastinal tissues and pleural cavity. It occurs
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most often in men between the ages of 40 and 60 years. The most common symptom is severe, acute left anterior or substernal chest pain or abdominal pain, often preceded by forceful vomiting after ingestion of a large meal. Respiratory difficulty and signs of impending h e m o d y n a m i c collapse are also common. The upright chest radiograph is the single most important laboratory examination. Because of the common symptoms of chest pain and dyspnea and the extremely low incidence of spontaneous esophageal rupture, misdiagnosis often occurs. A significant delay in proper diagnosis and treatment can result in substantial morbidity and mortality. Because survival is directly related to the early onset of treatment, the importance of correct i n i t i a l d i a g n o s i s in the case of Boerhaave's s y n d r o m e c a n n o t be overstated. The author thanks Dr Michael Kirk for his astute and rapid interpretation of the initial chest radiograph in this case.
REFERENCES 1. Boerhaave H: Atrocis nec descripti prius, morbi historia. The first translation of the classic case report of the rupture of the esophagus, with annotations. Bull Med Libr Assoe 1955;43:217-240. 2. Derbes VJ, Mitchell RE: Rupture of the esophagus.
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Surgery 1956;39:688-709,865-888. o 3. Wilson RF, Sarver EJ, Arbuln A, et al: Spontaneous perforation of the esophagus. A n n Thorac Surg 1971; 12:291-296. 4. Callaghan J: The Boerhaave syndrome (spontaneous rupture of the oesphagus). Br J Surg 1972;59:41-44. 5. Sawyers JL, Lane CE, Foster JH, et al: Esophageal perforation: An increasing challenge. A n n Thorac Surg 1975;19:233-238. 6. Mosher HP: The lower end of the esophagus at birth and in the adult. 7 Laryngol OtoI 1930;45:161-180. 7. Curci JJ, Horman MJ: Boerhaave's syndrome: The importance of early diagnosis and treatment. Ann Surg 1976;183:401-408. 8. Abbott, OA, Mansour KA, Logan WD, et al: Atram matic so-called "spontaneous rupture of the esophagus": A review of 47 personal cases with comments on a new method of surgical therapy. 7 Thorac Cardiovasc Surg 1970;59:67-83. 9. Uehara DT, Dymowski JJ, Schwartz J, et al: Chest pain, shock, and pneumomediastiuenm in a previously healthy 56-year-old man. A n n Emerg M e d 1987;16: 359-364. 10. Rogers L£, Puig AW, Dooley BN, et al: Diagnostic considerations in pathophysiologic-roentgenologic approach to Boerhaave's syndrome and spontaneous pneumomediastinum. A m J Roentgenol 1972~115:495 511. 11. Naclerio EA: The "V-sign" in the diagnosis of spontaneous rupture of the esophagus. Arn J Surg 1957;93: 291-298. 12. Jaworski A, Fischer R, Lippmann M: Boerhaave's syndrome: Computed tomographic findings and diagnostic considerations. Arch Intern M e d 1988;148: 223-224. 13. Walker WS, Cameron EWJ, Walbaum PR: Diagnosis and management of spontaneous transmural rupture of the oeseophagus (Boerhaave's syndrome). Br J Surg 1985;72:204-207. 14. Bennett DJ, Deveridge RJ, Wright JS: Spontaneous rupture of the esophagus: A review with reports of six cases. Surgery 1970;68:766-770. 15. Thai AP, Hatafuku T: Improved operation for esophageal rupture, lAMA 1964;188:126-128.
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