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“OCCULT” Boerhaave's syndrome
The Journa(of
Emergency
Medcrne,
Vol. 6, pp. 13-16,
Printed In the USA
1988
l
Copyright
0 1988 Pergamoil
Journals
Ltd
“OCCULT” BOERHAAVE’S SYNDROME Gobind S. Singh, MD,* and Corey M. Slovis, Reprint
‘Diviaon address:
of Emergency Medicine and the tDepartment Corey M. Slovis, MD, Director, Emergency
FACP,
FACEP*
,t
of Medicine, Emory Unwersrty School of Mediane, Medicine, Grady Memorial Hospital, 69 Butler Street.
Atlanta, Georgia SE, Atlanta, GA 30303
spontaneous rupture of the esophagus may, however, be quite subtle and easily missed. We report a case of “occult” Boerhaave’s syndrome in a stable patient who presented with signs and symptoms of upper gastrointestinal (GI) bleeding.
Cl Abstract -Spontaneous rupture of the esophagus (Boerhaave’s syndrome) usually presents in a dramatic fashion. Classically, following repeated episodes of vomiting, patients present with chest pain, dyspnea, cyanosis, shock, and cardiovascular collapse. We present a case of occult Boerhaave’s syndrome diagnosed by an upper gastrointestinal series in a 33-year-old man who arrived at the emergency department with a chief complaint of hematemesis. This case report reviews the usual presenting signs and symptoms of Boerhaave’s syndrome and concludes with a caution to physicians not to ignore the possibility of this diseaseentity in relatively stable patients.
CASE REPORT
A 33-year-old man presented to the Medical Emergency Clinic of Grady Memorial Hospital with the chief complaint of vomiting blood. The patient was in his usual state of health until two days prior to admission when he had begun to feel weak and tired and had lost his appetite. He noted that this coincided with the development of nausea, vomiting, and abdominal pain. The patient stated that his stools had become black and that his vomitus contained blood-streaked material. On the morning of admission he had vomited bright red blood, prompting him to seek medical attention. He denied previous such episodes, chest pain, shortness of breath, or syncope. The patient consumed 2 to 3 pints of vodka per week. He had a history of peptic ulcer disease, alcohol withdrawal seizures, and probable alcoholic hepatitis. The patient had previously been prescribed cimetidine (Tagamet) 400 mg every night and phenytoin (Dilantin) 300 mg every night. There was no known history of esophageal varices, hematemesis, hematochezia, or cardiac disease. The patient had no known allergies. On examination, the patient appeared somewhat weak and complained of abdominal pain. Supine blood pressure was 1501100 mm Hg, with a pulse rate of 82 beats per minute. No orthostatic changes were
q Keywords- Boerhaave’s syndrome: spontaneous rupture: esophagus
INTRODUCTION
In 1724 Herrnann Boerhaave, a Dutch physician, described the autopsy findings of an overindulgent Grand Admiral who, after gorging himself with duck and Moselle wine, tried to induce vomiting to relieve his gastric upset.] In the process, the Admiral gave out a terrifying cry, collapsed, and later died. This was the first reported case of spontaneous rupture of the esophagus. The mortality of Boerhaave’s syndrome remained close to 100% until 1947, when Barrett performed the first successful operation for repair of distal esophageal rupture.* Because the mortality of this disease approaches 100% if left untreated, physicians are taught to be suspicious of Boerhaave’s syndrome in any patient who presents with emesis associated with severe chest pain, respiratory distress, or shock.3 The diagnosis of =zzxzzx
MD,
Clinical Communications, focusing primarily on adult emergencies, is coordinated by Michael Tornlanovich, MD,
~
of Henry Ford Hospital in Detroit. RECEIVED: 23 March 1986; ACCEPTED: 20 April 1987
0736-4679/88 $3.00 + .OO 13
14
noted. The skin was warm and dry. The head and neck examination findings were within normal limits; no subcutaneous emphysema was noted in the neck or supraclavicular region. The lungs were clear to auscultation; and the heart was normal. A Hamman’s crunch was not present. Abdominal examination revealed a moderate amount of tenderness in the epigastric region without rebound or guarding. The abdomen was soft with normal bowel sounds. The liver and spleen were not felt. The genitalia were normal. A rectal exam revealed a normal size prostate. A stool specimen was tarry and gave a 4+ test for occult blood. The remainder of the physical exam findings, including that of the neurological exam, were normal. Laboratory studies revealed the following values: WBC, 10,2OO/pL, with 70 segs, 6 bands, 15 lymphocytes, 7 monocytes, and 2 basophils; hemoglobin was 12.9 g/dL; hematocrit, 37.7%; platelet count, 193,0OO/~L. Prothrombin and partial thromboplastin times were normal. The BUN was 18 mg/dL; creatinine, 0.7 mg/dL; blood glucose, 80 mg/dL, sodium, 134 mEq/L; potassium, 5.1 mEq/L; CO,,21 mEq/L; chloride, 93 mEq/L. The amylase was 150 U. Total bilirubin was 1.1 mg/dL with a direct bilirubin of 0.2 mg/dL. The SGOT was 53 U/L and the alkaline phosphotase was 201 U/L. A 12-lead ECG revealed a sinus rhythm at a rate of 64, without evidence of acute ischemia or injury. The admission chest film was read by an attending radiologist as normal. There was no evidence of pneumomediastinum, pneumothorax, or pleural effusion (Figure 1). Flat and upright x-ray studies of the abdomen showed a 2-mm pancreatic calcification but were otherwise normal. The patient was admitted to the medical intensive care unit for close monitoring and hydration. The patient was started on ranitidine (Zantac) 50 mg intravenously (IV) q6h, phenytoin 100 mg IV qSh, and metoclopramide (Reglan) 10 mg IV qSh and placed on a nothing-by-mouth order (NPO). Within 12 hours of admission an elective esophagi-am and upper GI series were performed to determine the site of his GI bleeding. They revealed a contained perforation of the posterior distal esophagus about 4 cm proximal to the cardia. (Figure 2) There was no evidence of peptic ulcer disease. A follow up chest x-ray study showed excellent free flow of barium back in to the esophagus. An immediate thoracic surgery consultation was obtained. In view of the patient’s stable condition, without evidence of mediastinitis or sepsis, and due to the contained nature of the perforation with excellent backflow into the esophagus, a conservative, nonoperative course was pursued. Penicillin 2 million units IV every four hours and gentami-
Gobind S. Singh and Corey M. Slovis
Figure 1. Admission mality.
chest x-ray without
evidence
of abnor-
tin 30 mg every eight hours were added to the above regimen. The patient was kept NPO and peripheral hyperalimentation was begun. Over the next 2 weeks, the patient underwent weekly upper GI series, all showing essentially no change in the contained rupture. Clinically, the patient improved to the point of feeding himself against medical advice, Daily peripheral WBC counts and hematocrit remained stable. On the 16th day of his hospitalization the patient left the hospital against medical advice because “he was tired of being hungry all the time”.
DISCUSSION Spontaneous rupture of the esophagus, commonly called Boerhaave’s syndrome, is a disease that can have a dramatic and rapidly fatal outcome.“7 Patients commonly complain of substernal or epigastric pain that may radiate to the back or left shoulder. The pain typically starts after one or more episodes of vomiting and may be associated with dyspnea, cyanosis, or shock, or cardiovascular collapse. The sign symptom complex of: (1) pain in the lower chest with vomiting and hematemesis, (2) subcutaneous emphysema of the neck, (3) respiratory distress, and (4) prostration is referred to as the tetrad of Gott.8 However, this combination is seen in less than about half
“Occult”
Figure posterior
Boerhaave’s
2. Esophagram distal esphosus.
15
Syndrome
showing
__ ----
contained
perforation
of
the patients.‘” It is more common for the pain to occur after repeated episodes of vomiting rather than after the first episode.4 Hematemesis is seen in about half the patients, though massive gastrointestinal hemorrhage is uncommon.4 Although uncommon in the first 12 hours, subcutaneous emphysema is seen in up to 60% of the patients.5 A “mediastinal crunch,” the so-caIIed Hamman’s sign, is audible in 20% of the patients5 Fever is seen in approximately half of patients, and leukocytosis or leftward shift is present in up to 85% of patients with esophageal rupture.4vS Although most patients are middle-aged men with a history of vomiting post food ingestion, Boerhaave’s syndrome has been associated with childbirth, seizures, severe coughing, swallowing, weight lifting, blunt trauma, and hyperemesis gravidarum.g-‘2 Most patients have no preexisting esophageal disease, although esophagitis, hiatal hernia, and strictures are conditions that predispose to spontaneous rupture.5,7 Although reported, this disease is uncommon in children.‘3,14
The diagnosis of this syndrome is rarely made on initial presentation.j-5 In the first 12 hours when the salvage rate is quite high, the presenting signs and symptoms may be deceptively benign and the diagnosis is likely to be missed.T-5 In one series, Abbot et al noted that the correct diagnosis was made within the first 12 hours in only 21% of the patients.j They suggested that multiple factors were involved in causing a delay in correctly diagnosing this disease including: physicians’ lack of familiarity with the disease; failure to appreciate the fact that this disease may readily mimic other entities, including peptic ulcer disease, pericarditis with audible rub, gastritis, pancreatitis, aortic dissection, cholecystitis, acute myocardial infarction, and spontaneous mediastinum; that alcoholic patients are more likely to present later than 12 hours; and the coexistence of coma at time of presentation. Pathophysiologicaliy, the rapid increase in the esophageal intraluminal pressure that accompanies vomiting leads to expansion and rupture.’ Following esophageal rupture, gastric contents pour into the mediastinum and pleural cavity, resulting in an intense inflammatory response and fluid collection. This leads to respiratory compromise, mediastinitis, sepsis, shock, and ultimately, death. Anatomically, the tears in the esophageal wall are longitudinal and confined to the left posterolateral wall of the distal esophagus 3 to 6 cm above the diaphragm in 9OOi’oof the cases.5-7 This specific location is due to multiple factors, including the weakness in the distal esophagus created by its thin musculature and the segmental defects, which are common in the esophagus’ lower lateral aspects.7z9 An erect chest x-ray study is probably the single most valuable diagnostic tool available to the emergency physician. Up to 90% of the patients have a pleural effusion and 80% have a pneumothorax.s,J Since most ruptures are on the left side, the pulmonary findings are usually confined to that side.11 Mediastinal emphysema is seen in up to two thirds of the patients, and widening of the mediastinum may also be seen.5 The “V” sign described by Emil Niclerio is an early roentgenographic sign of esophageal rupture.15 It is formed by the radiolucent streaks of air within the fascial planes along the aorta and the diaphragm. A barium esophagram confirms the diagnosis in the majority of cases. If left untreated, the mortality for spontaneous rupture of the esophagus in unstable patients is 100% within seven days4v5 With surgical intervention the overall survival is about 70% .+l6-17The high mortality of this disease is documented in a series of 157 cases reported by Derbes and Mitchell who noted that 25% of the patients died within 12 hours.~ Of the 71 un-
16
Gobind S. Singh and Corey M. Slovis
treated cases, only 11% survived for 48 hours. The survival rate with surgical intervention was 64%. Most authorities recommend immediate surgical repair with pleural and mediastinal drainage in patients who present with pneumothorax, mediastinal emphysema, pneumoperitoneum, systemic sepsis, shock, or respiratory failure.2OJl However, in recent years several authors have reported success with conservative nonoperative management in a subset of patients with contained perforations.22s23 In stable patients with well-draining, contained perforations and no evidence of sepsis, shock, or respiratory compromise, Cameron et al recommended nonoperative management. 22 Patients who meet the above criteria should have all oral feedings withdrawn and peripheral hyperalimentation begun to provide a positive nitrogen balance. High-dose antibiotic therapy is started early in the course to cover for anaerobic bacteria and oral flora. The patient should be monitored closely for clinical or laboratory evidence of decompensation.
Owing to the high mortality of unrecognized Boerhaave’s syndrome, emergency physicians must consider this diagnosis in any patient with an upper GI hemorrhage. In patients who have been diagnosed as having acute bleeding from peptic ulcer disease or a Mallory-Weiss tear of the esophagus, Boerhaave’s syndrome should be suspected in the following clinical settings: increasing or unexplained leukocytosis; unexplained “third spacing” of volume; mediastinal air seen on x-ray film; or signs of mediastinitis or sepsis. This case represents an instance of an occult stable presentation of a usually dramatic and catastrophic disease entity. Although clinicians often think Boerhaave’s syndrome occurs in a classic fashion, multiple reviews have consistently demonstrated the contrary. This case report is not meant to suggest that all patients with upper GI bleeding should be worked up to rule out Boerhaave’s syndrome, but to urge emergency physicians to be suspicious for the possibility of this diagnosis.
REFERENCES 1. Boerhaave H: Atrocis, net descripti prius, morbi historia. Secundum Medicae Artis Leges Conscripta Lugduni. Batavorum Boutesteniana, 1724. Translated in BUN Med Libr Assoc 1955; 43:217-240. 2. Barrett NR: Report of case of spontaneous perforation of the esophagus successfully treated by operation. Br J Surg 1947; 35:216. 3. Patton AS, Lawson DW, Shannon JM: Re-evaluation of Boerhaave’s Syndrome, a review of 14 cases. Am J Surg 1979; 137:560-565. 4. Abbott OA, Mansour KA, Logan WD Jr, et al: A traumatic socalled “spontaneous” rupture of the esophagus; a review of 47 personal cases with comments on a new method of surgical therapy. J Thorac Cardiovas Surg 1970; 59:67-83. 5. Cruci JJ, Horman MJ: Boerhaave’s Syndrome, the importance of early diagnosis and treatment. Ann Surg 1976; 183:401-408. 6. Callaghan J: The Boerhaave’s Syndrome. Br J Surg 1972; 59:41-44. 7. Derbes VJ, Mitchell RG: Rupture of the esophagus. Surgery 1956; 39:688-709,865-888. 8. Van Heerden JA, Toxopeus M, Troxel G: Boerhaave’s Syndrome, a case report. Va Med Mon 1974; 101:452. 9. Mosher HP: The lower end of the esophagus at birth and in the adult. JLaryngoI Otoll930; 45:161-180. 10. Fernandez D: Another esophageal rupture after alcohol and disulfiram (let). N Engl JMed 1972; 286:610. 11. Maver JH. Herlocher JE. Parisian J: Esoohageal ruuture after mu~hrod&alcohol ingestion (let). N En& J ied 1971; 285:1323. 12. Chirino 0: Barogenic rupture of the esophagus associated with
hyperemesis gravidarum. Obstet Gynecol 1978; 52(suppl):5 15535. 13. Wiseman HJ, Celano ER, Hester FC III: Spontaneous rupture of the esophagus in a newborn infant. JPediatr 1959; 55:207. 14. Mennen FR, Moore CV: Spontaneous rupture of the esophagus in an infant. Arch Pediatr 1921; 38:672-676. 15. Naclerio ES: The “V-sign” in the diagnosis of spontaneous rupture of the esophagus (an early roentgen clue). Am J Surg 1957; 93:291-298. 16. Berne CJ, Shader AE, Dotty DB: Treatment of effort rupture of the esophagus by epigastric celiotomy. Surg Gynecol Obstet 1969; 129:277. 17. Wilson FR, Sarver EJ, Arbulu A: Spontaneous rupture of the esophagus. Ann Thorac Surg 1971; 12:291-296. 18. Bennett DJ, Deverege RJ, Wright JS: Spontaneous rupture of the esophagus: a review with reports of six cases. Surgery 1970; 68~766-770. 19. Sealy WR: Rupture of the esophagus. Am J Surg 1963; 105:-505-510. 20. Michel L, Grill0 HC, Malt RA: Esophageal perforation. Ann Thorac Surf 1982; 33:203-210. 21. Phillips LG; Coghill CL, Makkar JC, et al: Barogenic rupture of the esophagus (Boerhaave’s Syndrome). Am Surg 1982; 48:430-434. 22. Cameron JL, Kieffer FR, Hendrix TR, et al: Selective nonoperative management of contained intrathoracic esophageal disruption. Ann Thorac Surg 1979; 27:404-409. 23. Anderson OS, Guistra PE: Non operative management of contained esophageal perforation. Arch Surg 1981; 116:12141217.
Emergency
Medcrne,
Vol. 6, pp. 13-16,
Printed In the USA
1988
l
Copyright
0 1988 Pergamoil
Journals
Ltd
“OCCULT” BOERHAAVE’S SYNDROME Gobind S. Singh, MD,* and Corey M. Slovis, Reprint
‘Diviaon address:
of Emergency Medicine and the tDepartment Corey M. Slovis, MD, Director, Emergency
FACP,
FACEP*
,t
of Medicine, Emory Unwersrty School of Mediane, Medicine, Grady Memorial Hospital, 69 Butler Street.
Atlanta, Georgia SE, Atlanta, GA 30303
spontaneous rupture of the esophagus may, however, be quite subtle and easily missed. We report a case of “occult” Boerhaave’s syndrome in a stable patient who presented with signs and symptoms of upper gastrointestinal (GI) bleeding.
Cl Abstract -Spontaneous rupture of the esophagus (Boerhaave’s syndrome) usually presents in a dramatic fashion. Classically, following repeated episodes of vomiting, patients present with chest pain, dyspnea, cyanosis, shock, and cardiovascular collapse. We present a case of occult Boerhaave’s syndrome diagnosed by an upper gastrointestinal series in a 33-year-old man who arrived at the emergency department with a chief complaint of hematemesis. This case report reviews the usual presenting signs and symptoms of Boerhaave’s syndrome and concludes with a caution to physicians not to ignore the possibility of this diseaseentity in relatively stable patients.
CASE REPORT
A 33-year-old man presented to the Medical Emergency Clinic of Grady Memorial Hospital with the chief complaint of vomiting blood. The patient was in his usual state of health until two days prior to admission when he had begun to feel weak and tired and had lost his appetite. He noted that this coincided with the development of nausea, vomiting, and abdominal pain. The patient stated that his stools had become black and that his vomitus contained blood-streaked material. On the morning of admission he had vomited bright red blood, prompting him to seek medical attention. He denied previous such episodes, chest pain, shortness of breath, or syncope. The patient consumed 2 to 3 pints of vodka per week. He had a history of peptic ulcer disease, alcohol withdrawal seizures, and probable alcoholic hepatitis. The patient had previously been prescribed cimetidine (Tagamet) 400 mg every night and phenytoin (Dilantin) 300 mg every night. There was no known history of esophageal varices, hematemesis, hematochezia, or cardiac disease. The patient had no known allergies. On examination, the patient appeared somewhat weak and complained of abdominal pain. Supine blood pressure was 1501100 mm Hg, with a pulse rate of 82 beats per minute. No orthostatic changes were
q Keywords- Boerhaave’s syndrome: spontaneous rupture: esophagus
INTRODUCTION
In 1724 Herrnann Boerhaave, a Dutch physician, described the autopsy findings of an overindulgent Grand Admiral who, after gorging himself with duck and Moselle wine, tried to induce vomiting to relieve his gastric upset.] In the process, the Admiral gave out a terrifying cry, collapsed, and later died. This was the first reported case of spontaneous rupture of the esophagus. The mortality of Boerhaave’s syndrome remained close to 100% until 1947, when Barrett performed the first successful operation for repair of distal esophageal rupture.* Because the mortality of this disease approaches 100% if left untreated, physicians are taught to be suspicious of Boerhaave’s syndrome in any patient who presents with emesis associated with severe chest pain, respiratory distress, or shock.3 The diagnosis of =zzxzzx
MD,
Clinical Communications, focusing primarily on adult emergencies, is coordinated by Michael Tornlanovich, MD,
~
of Henry Ford Hospital in Detroit. RECEIVED: 23 March 1986; ACCEPTED: 20 April 1987
0736-4679/88 $3.00 + .OO 13
14
noted. The skin was warm and dry. The head and neck examination findings were within normal limits; no subcutaneous emphysema was noted in the neck or supraclavicular region. The lungs were clear to auscultation; and the heart was normal. A Hamman’s crunch was not present. Abdominal examination revealed a moderate amount of tenderness in the epigastric region without rebound or guarding. The abdomen was soft with normal bowel sounds. The liver and spleen were not felt. The genitalia were normal. A rectal exam revealed a normal size prostate. A stool specimen was tarry and gave a 4+ test for occult blood. The remainder of the physical exam findings, including that of the neurological exam, were normal. Laboratory studies revealed the following values: WBC, 10,2OO/pL, with 70 segs, 6 bands, 15 lymphocytes, 7 monocytes, and 2 basophils; hemoglobin was 12.9 g/dL; hematocrit, 37.7%; platelet count, 193,0OO/~L. Prothrombin and partial thromboplastin times were normal. The BUN was 18 mg/dL; creatinine, 0.7 mg/dL; blood glucose, 80 mg/dL, sodium, 134 mEq/L; potassium, 5.1 mEq/L; CO,,21 mEq/L; chloride, 93 mEq/L. The amylase was 150 U. Total bilirubin was 1.1 mg/dL with a direct bilirubin of 0.2 mg/dL. The SGOT was 53 U/L and the alkaline phosphotase was 201 U/L. A 12-lead ECG revealed a sinus rhythm at a rate of 64, without evidence of acute ischemia or injury. The admission chest film was read by an attending radiologist as normal. There was no evidence of pneumomediastinum, pneumothorax, or pleural effusion (Figure 1). Flat and upright x-ray studies of the abdomen showed a 2-mm pancreatic calcification but were otherwise normal. The patient was admitted to the medical intensive care unit for close monitoring and hydration. The patient was started on ranitidine (Zantac) 50 mg intravenously (IV) q6h, phenytoin 100 mg IV qSh, and metoclopramide (Reglan) 10 mg IV qSh and placed on a nothing-by-mouth order (NPO). Within 12 hours of admission an elective esophagi-am and upper GI series were performed to determine the site of his GI bleeding. They revealed a contained perforation of the posterior distal esophagus about 4 cm proximal to the cardia. (Figure 2) There was no evidence of peptic ulcer disease. A follow up chest x-ray study showed excellent free flow of barium back in to the esophagus. An immediate thoracic surgery consultation was obtained. In view of the patient’s stable condition, without evidence of mediastinitis or sepsis, and due to the contained nature of the perforation with excellent backflow into the esophagus, a conservative, nonoperative course was pursued. Penicillin 2 million units IV every four hours and gentami-
Gobind S. Singh and Corey M. Slovis
Figure 1. Admission mality.
chest x-ray without
evidence
of abnor-
tin 30 mg every eight hours were added to the above regimen. The patient was kept NPO and peripheral hyperalimentation was begun. Over the next 2 weeks, the patient underwent weekly upper GI series, all showing essentially no change in the contained rupture. Clinically, the patient improved to the point of feeding himself against medical advice, Daily peripheral WBC counts and hematocrit remained stable. On the 16th day of his hospitalization the patient left the hospital against medical advice because “he was tired of being hungry all the time”.
DISCUSSION Spontaneous rupture of the esophagus, commonly called Boerhaave’s syndrome, is a disease that can have a dramatic and rapidly fatal outcome.“7 Patients commonly complain of substernal or epigastric pain that may radiate to the back or left shoulder. The pain typically starts after one or more episodes of vomiting and may be associated with dyspnea, cyanosis, or shock, or cardiovascular collapse. The sign symptom complex of: (1) pain in the lower chest with vomiting and hematemesis, (2) subcutaneous emphysema of the neck, (3) respiratory distress, and (4) prostration is referred to as the tetrad of Gott.8 However, this combination is seen in less than about half
“Occult”
Figure posterior
Boerhaave’s
2. Esophagram distal esphosus.
15
Syndrome
showing
__ ----
contained
perforation
of
the patients.‘” It is more common for the pain to occur after repeated episodes of vomiting rather than after the first episode.4 Hematemesis is seen in about half the patients, though massive gastrointestinal hemorrhage is uncommon.4 Although uncommon in the first 12 hours, subcutaneous emphysema is seen in up to 60% of the patients.5 A “mediastinal crunch,” the so-caIIed Hamman’s sign, is audible in 20% of the patients5 Fever is seen in approximately half of patients, and leukocytosis or leftward shift is present in up to 85% of patients with esophageal rupture.4vS Although most patients are middle-aged men with a history of vomiting post food ingestion, Boerhaave’s syndrome has been associated with childbirth, seizures, severe coughing, swallowing, weight lifting, blunt trauma, and hyperemesis gravidarum.g-‘2 Most patients have no preexisting esophageal disease, although esophagitis, hiatal hernia, and strictures are conditions that predispose to spontaneous rupture.5,7 Although reported, this disease is uncommon in children.‘3,14
The diagnosis of this syndrome is rarely made on initial presentation.j-5 In the first 12 hours when the salvage rate is quite high, the presenting signs and symptoms may be deceptively benign and the diagnosis is likely to be missed.T-5 In one series, Abbot et al noted that the correct diagnosis was made within the first 12 hours in only 21% of the patients.j They suggested that multiple factors were involved in causing a delay in correctly diagnosing this disease including: physicians’ lack of familiarity with the disease; failure to appreciate the fact that this disease may readily mimic other entities, including peptic ulcer disease, pericarditis with audible rub, gastritis, pancreatitis, aortic dissection, cholecystitis, acute myocardial infarction, and spontaneous mediastinum; that alcoholic patients are more likely to present later than 12 hours; and the coexistence of coma at time of presentation. Pathophysiologicaliy, the rapid increase in the esophageal intraluminal pressure that accompanies vomiting leads to expansion and rupture.’ Following esophageal rupture, gastric contents pour into the mediastinum and pleural cavity, resulting in an intense inflammatory response and fluid collection. This leads to respiratory compromise, mediastinitis, sepsis, shock, and ultimately, death. Anatomically, the tears in the esophageal wall are longitudinal and confined to the left posterolateral wall of the distal esophagus 3 to 6 cm above the diaphragm in 9OOi’oof the cases.5-7 This specific location is due to multiple factors, including the weakness in the distal esophagus created by its thin musculature and the segmental defects, which are common in the esophagus’ lower lateral aspects.7z9 An erect chest x-ray study is probably the single most valuable diagnostic tool available to the emergency physician. Up to 90% of the patients have a pleural effusion and 80% have a pneumothorax.s,J Since most ruptures are on the left side, the pulmonary findings are usually confined to that side.11 Mediastinal emphysema is seen in up to two thirds of the patients, and widening of the mediastinum may also be seen.5 The “V” sign described by Emil Niclerio is an early roentgenographic sign of esophageal rupture.15 It is formed by the radiolucent streaks of air within the fascial planes along the aorta and the diaphragm. A barium esophagram confirms the diagnosis in the majority of cases. If left untreated, the mortality for spontaneous rupture of the esophagus in unstable patients is 100% within seven days4v5 With surgical intervention the overall survival is about 70% .+l6-17The high mortality of this disease is documented in a series of 157 cases reported by Derbes and Mitchell who noted that 25% of the patients died within 12 hours.~ Of the 71 un-
16
Gobind S. Singh and Corey M. Slovis
treated cases, only 11% survived for 48 hours. The survival rate with surgical intervention was 64%. Most authorities recommend immediate surgical repair with pleural and mediastinal drainage in patients who present with pneumothorax, mediastinal emphysema, pneumoperitoneum, systemic sepsis, shock, or respiratory failure.2OJl However, in recent years several authors have reported success with conservative nonoperative management in a subset of patients with contained perforations.22s23 In stable patients with well-draining, contained perforations and no evidence of sepsis, shock, or respiratory compromise, Cameron et al recommended nonoperative management. 22 Patients who meet the above criteria should have all oral feedings withdrawn and peripheral hyperalimentation begun to provide a positive nitrogen balance. High-dose antibiotic therapy is started early in the course to cover for anaerobic bacteria and oral flora. The patient should be monitored closely for clinical or laboratory evidence of decompensation.
Owing to the high mortality of unrecognized Boerhaave’s syndrome, emergency physicians must consider this diagnosis in any patient with an upper GI hemorrhage. In patients who have been diagnosed as having acute bleeding from peptic ulcer disease or a Mallory-Weiss tear of the esophagus, Boerhaave’s syndrome should be suspected in the following clinical settings: increasing or unexplained leukocytosis; unexplained “third spacing” of volume; mediastinal air seen on x-ray film; or signs of mediastinitis or sepsis. This case represents an instance of an occult stable presentation of a usually dramatic and catastrophic disease entity. Although clinicians often think Boerhaave’s syndrome occurs in a classic fashion, multiple reviews have consistently demonstrated the contrary. This case report is not meant to suggest that all patients with upper GI bleeding should be worked up to rule out Boerhaave’s syndrome, but to urge emergency physicians to be suspicious for the possibility of this diagnosis.
REFERENCES 1. Boerhaave H: Atrocis, net descripti prius, morbi historia. Secundum Medicae Artis Leges Conscripta Lugduni. Batavorum Boutesteniana, 1724. Translated in BUN Med Libr Assoc 1955; 43:217-240. 2. Barrett NR: Report of case of spontaneous perforation of the esophagus successfully treated by operation. Br J Surg 1947; 35:216. 3. Patton AS, Lawson DW, Shannon JM: Re-evaluation of Boerhaave’s Syndrome, a review of 14 cases. Am J Surg 1979; 137:560-565. 4. Abbott OA, Mansour KA, Logan WD Jr, et al: A traumatic socalled “spontaneous” rupture of the esophagus; a review of 47 personal cases with comments on a new method of surgical therapy. J Thorac Cardiovas Surg 1970; 59:67-83. 5. Cruci JJ, Horman MJ: Boerhaave’s Syndrome, the importance of early diagnosis and treatment. Ann Surg 1976; 183:401-408. 6. Callaghan J: The Boerhaave’s Syndrome. Br J Surg 1972; 59:41-44. 7. Derbes VJ, Mitchell RG: Rupture of the esophagus. Surgery 1956; 39:688-709,865-888. 8. Van Heerden JA, Toxopeus M, Troxel G: Boerhaave’s Syndrome, a case report. Va Med Mon 1974; 101:452. 9. Mosher HP: The lower end of the esophagus at birth and in the adult. JLaryngoI Otoll930; 45:161-180. 10. Fernandez D: Another esophageal rupture after alcohol and disulfiram (let). N Engl JMed 1972; 286:610. 11. Maver JH. Herlocher JE. Parisian J: Esoohageal ruuture after mu~hrod&alcohol ingestion (let). N En& J ied 1971; 285:1323. 12. Chirino 0: Barogenic rupture of the esophagus associated with
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