Atypical presentation of Boerhaave's syndrome as Enterococcal bacterial pericardial effusion
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Department of Cardiothoracic Surgery, The General Infirmary at Leeds, Great George Street, Leeds, LS1 3EX, West Yorkshire, UK
Abstract
Boerhaave's perforation is a serious condition describing spontaneous transmural perforation of the oesophagus. The classical presentation of this condition is vomiting, lower thoracic pain and subcutaneous emphysema. However, the condition often presents atypically and it is important to reach the correct diagnosis quickly. We present the case of a 54-year-old woman with a Boerhaave's perforation that presented as Enterococcal bacterial pericardial effusion.
Key Words: Boerhaave's; Perforation; Enterococcus; Pericardial; Effusion
1. Introduction
Boerhaave's perforation is spontaneous transmural perforation of the oesophagus, first described by Herman Boerhaave in 1724 [1]. It is usually secondary to violent vomiting or retching and is thought to occur in response to the sudden rise in intraluminal oesophageal pressure produced during vomiting. The Mackler triad describes the classical presentation and consists of vomiting, lower thoracic pain and subcutaneous emphysema. Other frequent findings include pleural effusions, tachypnoea and abdominal rigidity. However, approximately one third of cases are clinically atypical and present with a variety of unusual symptoms including peripheral cyanosis, cervical vein distension and hoarseness of voice due to recurrent laryngeal nerve involvement [2]. The mortality rate of oesophageal perforation is high and survival is usually dependent on early diagnosis and appropriate surgical intervention [3].
2. Case history
A 54-year-old woman initially attended her local casualty department with diarrhoea and violent vomiting associated with severe, sharp chest pain. She was discharged with a diagnosis with food poisoning. Her clinical condition subsequently deteriorated at home and after a four-week history of night sweats and nausea, with regular treatment from her general practitioner, she was readmitted. Other than a background of meningitis fifteen years earlier, from which she made a complete recovery, she had no significant past medical history.
On admission, she was hypertensive and pyrexial and had a neutrophilia (27.29x109/l) and a raised CRP (372 mg/l). She was treated for infective gastroenteritis and was started on intravenous cefuroxime, metronidazole and fluid resuscitation. However, she continued to deteriorate and subsequent plain radiographs of the chest revealed bilateral pleural effusions (Fig. 1) which were treated with bilateral pleural drains. An echocardiogram showed a moderately large pericardial effusion and she underwent a subxiphoid percutaneous pericardial drain insertion which drained 750 ml of purulent fluid. Microbiological investigation identified Enterococcus on enrichment broth culture with no evidence of malignant cells or tuberculosis. Immunoglobulin levels were normal and auto-antibody screens were negative. Her working diagnosis was an oesophageal perforation secondary to the violent vomiting and retching at the beginning of her history.
A computerised tomography (CT) scan showed a loculated pericardial effusion compressing the right atrium, persistent bilateral pleural effusions and a mediastinal effusion with para-tracheal lymphadenopathy (Fig. 2). At this time, she was referred to the cardiothoracic unit for further investigation and management.
Antibiotics were changed to intravenous Vancomycin and she remained apyrexial throughout her admission. Cardiac signs improved and a repeat CT scan three weeks after her initial scan showed that the pericardial effusion had reduced in size. Drainage volumes from the pericardial drain reduced to zero two weeks after commencing Vancomycin. A contrast barium swallow did not demonstrate any persisting oesophageal leak and non-operative management was continued and on discharge, her inflammatory markers had returned to normal.
3. Discussion
Boerhaave's perforation of the oesophagus is a rare but life-threatening condition. The principles of treatment comprise fluid resuscitation, antibiotics, enteral or parenteral feeding, pleural and mediastinal drainage and usually repair of the oesophageal defect.
In this case, our patient had an extremely uncommon presentation of pericardial effusion but on review of her history and investigations, she had the classical signs and symptoms of a Boerhaave's perforation, namely the violent vomiting, the severe chest pain and the presence of bilateral pleural effusions. The presence of Enterococci in the pericardial fluid first raised the possibility of a non-cardiac underlying aetiology, as it is particularly unusual as the sole causative microbial agent in pericarditis. Although it has been identified as the cause of pericarditis in other animals, it has not to our knowledge been the sole agent involved in human pericarditis before. Enterococci have previously been part of a microbial spectrum that has caused an epidemic of equine pericarditis [4] and Enterococcus durans has also been found with Pseudomonas aeruginosa in a SCID mouse colony [5].
We surmise that our patient developed a Boerhaave's oesophageal perforation whilst suffering from violent vomiting. This allowed oesophageal microbes to communicate with the pericardium and lead to the isolation of Enterococcus from the pericardial sac.
It is important to note that this diagnosis, although likely, is based on indirect evidence with no direct confirmation of an oesophageal rupture. This is often the case with delayed diagnosis of Boerhaave's perforation. There are a number of other possible differential diagnoses which could lead to the presence of gastro-intestinal microbes to be present within the pericardial sac. These include colonic diverticular disease (leading to perforation and fistulation into the pericardial sac), intestinal trauma and metastatic intestinal neoplastic disease. We also suspect that antibiotics given by the general practitioner may have modified the microbial spectrum present in the pericardial fluid.
This case highlights a number of important issues. Firstly, Boerhaave's perforation can be difficult to diagnose and does not always present in the classical way. It can also present late, providing even more of a diagnostic challenge. Careful elucidation of the history and accurate examination allied with appropriate investigations are necessary to make the diagnosis. CT scanning and contrast studies have a role to play in investigating the presence of a perforation and identifying the site.
Secondly, when an unusual microbe is found to be the cause of pericarditis, careful steps need to be taken to exclude any other pathology such as HIV infection, occult neoplasia or, as in this case, Boerhaave's perforation.
Finally, it is important to ensure that treatment follows a two-pronged approach of appropriate antibiotic therapy and drainage of any effusion. In the majority of patients with oesophageal perforation, repair of the defect is indicated. However, in this case, the patient improved with non-operative treatment and as such was able to avoid the mortality and morbidity associated with a general anaesthetic and surgical approach to the oesophagus although we recognise that in the future, she may develop signs of pericardial constriction and require pericardectomy [6].
References
Derbes VJ, Mitchell RE Jr. Hermann Boerhaave's (1) atrocis, nec Descripti priu, morbi Historia; (2) the first translation of the classic case report of rupture of the esophagus, with annotations. Bull Med Libr Assoc 1955; 43:217.
Henderson JA, Peloquin AJ. Boerhaave revisited: spontaneous esophageal perforation as a diagnostic masquerader. Am J Med May 1989; 86:559–567.
Curci JJ, Horman MJ. Boerhaave's syndrome: the importance of early diagnosis and treatment. Ann Surg Apr 1976; 183:401–408.
Bolin DC, Donahue JM, Vickers ML, Harrison L, Sells S, Giles RC, Hong CB, Poonacha KB, Roberts J, Sebastian MM, Swerczek TW, Tramontin R, Williams NM. Microbiologic and pathologic findings in an epidemic of equine pericarditis. J Vet Diagn Invest Jan 2005; 17:38–44.
Dietrich HM, Khaschabi D, Albini B. Isolation of Enterococcus durans and Pseudomonas aeruginosa in a scid mouse colony. Lab Anim Apr 1996; 30:102–107.
Tomkowski WZ, Kuca P, Gralec R, Burakowski J, Caban P, Orlowski T, Kurzyna M. Management of purulent pericarditis. Monaldi Arch Chest Dis 2003;Oct-Dec 59:308–309.(Arin Saha, Martin Jarvis, James A.C. Tho)
Abstract
Boerhaave's perforation is a serious condition describing spontaneous transmural perforation of the oesophagus. The classical presentation of this condition is vomiting, lower thoracic pain and subcutaneous emphysema. However, the condition often presents atypically and it is important to reach the correct diagnosis quickly. We present the case of a 54-year-old woman with a Boerhaave's perforation that presented as Enterococcal bacterial pericardial effusion.
Key Words: Boerhaave's; Perforation; Enterococcus; Pericardial; Effusion
1. Introduction
Boerhaave's perforation is spontaneous transmural perforation of the oesophagus, first described by Herman Boerhaave in 1724 [1]. It is usually secondary to violent vomiting or retching and is thought to occur in response to the sudden rise in intraluminal oesophageal pressure produced during vomiting. The Mackler triad describes the classical presentation and consists of vomiting, lower thoracic pain and subcutaneous emphysema. Other frequent findings include pleural effusions, tachypnoea and abdominal rigidity. However, approximately one third of cases are clinically atypical and present with a variety of unusual symptoms including peripheral cyanosis, cervical vein distension and hoarseness of voice due to recurrent laryngeal nerve involvement [2]. The mortality rate of oesophageal perforation is high and survival is usually dependent on early diagnosis and appropriate surgical intervention [3].
2. Case history
A 54-year-old woman initially attended her local casualty department with diarrhoea and violent vomiting associated with severe, sharp chest pain. She was discharged with a diagnosis with food poisoning. Her clinical condition subsequently deteriorated at home and after a four-week history of night sweats and nausea, with regular treatment from her general practitioner, she was readmitted. Other than a background of meningitis fifteen years earlier, from which she made a complete recovery, she had no significant past medical history.
On admission, she was hypertensive and pyrexial and had a neutrophilia (27.29x109/l) and a raised CRP (372 mg/l). She was treated for infective gastroenteritis and was started on intravenous cefuroxime, metronidazole and fluid resuscitation. However, she continued to deteriorate and subsequent plain radiographs of the chest revealed bilateral pleural effusions (Fig. 1) which were treated with bilateral pleural drains. An echocardiogram showed a moderately large pericardial effusion and she underwent a subxiphoid percutaneous pericardial drain insertion which drained 750 ml of purulent fluid. Microbiological investigation identified Enterococcus on enrichment broth culture with no evidence of malignant cells or tuberculosis. Immunoglobulin levels were normal and auto-antibody screens were negative. Her working diagnosis was an oesophageal perforation secondary to the violent vomiting and retching at the beginning of her history.
A computerised tomography (CT) scan showed a loculated pericardial effusion compressing the right atrium, persistent bilateral pleural effusions and a mediastinal effusion with para-tracheal lymphadenopathy (Fig. 2). At this time, she was referred to the cardiothoracic unit for further investigation and management.
Antibiotics were changed to intravenous Vancomycin and she remained apyrexial throughout her admission. Cardiac signs improved and a repeat CT scan three weeks after her initial scan showed that the pericardial effusion had reduced in size. Drainage volumes from the pericardial drain reduced to zero two weeks after commencing Vancomycin. A contrast barium swallow did not demonstrate any persisting oesophageal leak and non-operative management was continued and on discharge, her inflammatory markers had returned to normal.
3. Discussion
Boerhaave's perforation of the oesophagus is a rare but life-threatening condition. The principles of treatment comprise fluid resuscitation, antibiotics, enteral or parenteral feeding, pleural and mediastinal drainage and usually repair of the oesophageal defect.
In this case, our patient had an extremely uncommon presentation of pericardial effusion but on review of her history and investigations, she had the classical signs and symptoms of a Boerhaave's perforation, namely the violent vomiting, the severe chest pain and the presence of bilateral pleural effusions. The presence of Enterococci in the pericardial fluid first raised the possibility of a non-cardiac underlying aetiology, as it is particularly unusual as the sole causative microbial agent in pericarditis. Although it has been identified as the cause of pericarditis in other animals, it has not to our knowledge been the sole agent involved in human pericarditis before. Enterococci have previously been part of a microbial spectrum that has caused an epidemic of equine pericarditis [4] and Enterococcus durans has also been found with Pseudomonas aeruginosa in a SCID mouse colony [5].
We surmise that our patient developed a Boerhaave's oesophageal perforation whilst suffering from violent vomiting. This allowed oesophageal microbes to communicate with the pericardium and lead to the isolation of Enterococcus from the pericardial sac.
It is important to note that this diagnosis, although likely, is based on indirect evidence with no direct confirmation of an oesophageal rupture. This is often the case with delayed diagnosis of Boerhaave's perforation. There are a number of other possible differential diagnoses which could lead to the presence of gastro-intestinal microbes to be present within the pericardial sac. These include colonic diverticular disease (leading to perforation and fistulation into the pericardial sac), intestinal trauma and metastatic intestinal neoplastic disease. We also suspect that antibiotics given by the general practitioner may have modified the microbial spectrum present in the pericardial fluid.
This case highlights a number of important issues. Firstly, Boerhaave's perforation can be difficult to diagnose and does not always present in the classical way. It can also present late, providing even more of a diagnostic challenge. Careful elucidation of the history and accurate examination allied with appropriate investigations are necessary to make the diagnosis. CT scanning and contrast studies have a role to play in investigating the presence of a perforation and identifying the site.
Secondly, when an unusual microbe is found to be the cause of pericarditis, careful steps need to be taken to exclude any other pathology such as HIV infection, occult neoplasia or, as in this case, Boerhaave's perforation.
Finally, it is important to ensure that treatment follows a two-pronged approach of appropriate antibiotic therapy and drainage of any effusion. In the majority of patients with oesophageal perforation, repair of the defect is indicated. However, in this case, the patient improved with non-operative treatment and as such was able to avoid the mortality and morbidity associated with a general anaesthetic and surgical approach to the oesophagus although we recognise that in the future, she may develop signs of pericardial constriction and require pericardectomy [6].
References
Derbes VJ, Mitchell RE Jr. Hermann Boerhaave's (1) atrocis, nec Descripti priu, morbi Historia; (2) the first translation of the classic case report of rupture of the esophagus, with annotations. Bull Med Libr Assoc 1955; 43:217.
Henderson JA, Peloquin AJ. Boerhaave revisited: spontaneous esophageal perforation as a diagnostic masquerader. Am J Med May 1989; 86:559–567.
Curci JJ, Horman MJ. Boerhaave's syndrome: the importance of early diagnosis and treatment. Ann Surg Apr 1976; 183:401–408.
Bolin DC, Donahue JM, Vickers ML, Harrison L, Sells S, Giles RC, Hong CB, Poonacha KB, Roberts J, Sebastian MM, Swerczek TW, Tramontin R, Williams NM. Microbiologic and pathologic findings in an epidemic of equine pericarditis. J Vet Diagn Invest Jan 2005; 17:38–44.
Dietrich HM, Khaschabi D, Albini B. Isolation of Enterococcus durans and Pseudomonas aeruginosa in a scid mouse colony. Lab Anim Apr 1996; 30:102–107.
Tomkowski WZ, Kuca P, Gralec R, Burakowski J, Caban P, Orlowski T, Kurzyna M. Management of purulent pericarditis. Monaldi Arch Chest Dis 2003;Oct-Dec 59:308–309.(Arin Saha, Martin Jarvis, James A.C. Tho)