Boerhaave syndrome: Effort rupture of the esophagus
- George Triadafilopoulos, MD
George Triadafilopoulos, MD
- Clinical Professor of Medicine
- Stanford University School of Medicine
Effort rupture of the esophagus or Boerhaave syndrome is associated with high morbidity and mortality and is fatal in the absence of therapy. The occasionally nonspecific nature of the symptoms may contribute to a delay in diagnosis and a poor outcome .
This topic will review the epidemiology, pathogenesis, clinical manifestations, diagnosis, and management of Boerhaave syndrome. The clinical manifestations, diagnosis, and management of traumatic and iatrogenic causes of esophageal perforation are discussed in detail, separately. (See "Overview of esophageal perforation due to blunt or penetrating trauma" and "Surgical management of esophageal perforation" and "Overview of gastrointestinal tract perforation".)
Effort rupture of the esophagus, or Boerhaave syndrome, is a spontaneous perforation of the esophagus that results from a sudden increase in intraesophageal pressure combined with negative intrathoracic pressure (eg, severe straining or vomiting).
ETIOLOGY AND PATHOGENESIS
Boerhaave syndrome usually occurs in patients with a normal underlying esophagus. However, a subset of patients with Boerhaave syndrome has underlying eosinophilic esophagitis, medication-induced esophagitis, Barrett's or infectious ulcers. Sudden increase in intraesophageal pressure combined with negative intrathoracic pressure such as that associated with severe straining or vomiting, and less frequently with childbirth, seizure, prolonged coughing or laughing, or weightlifting, results in a longitudinal esophageal perforation [4,5].
- Brauer RB, Liebermann-Meffert D, Stein HJ, et al. Boerhaave's syndrome: analysis of the literature and report of 18 new cases. Dis Esophagus 1997; 10:64.
- Vidarsdottir H, Blondal S, Alfredsson H, et al. Oesophageal perforations in Iceland: a whole population study on incidence, aetiology and surgical outcome. Thorac Cardiovasc Surg 2010; 58:476.
- Brinster CJ, Singhal S, Lee L, et al. Evolving options in the management of esophageal perforation. Ann Thorac Surg 2004; 77:1475.
- Pate JW, Walker WA, Cole FH Jr, et al. Spontaneous rupture of the esophagus: a 30-year experience. Ann Thorac Surg 1989; 47:689.
- Herbella FA, Matone J, Del Grande JC. Eponyms in esophageal surgery, part 2. Dis Esophagus 2005; 18:4.
- Saha A, Jarvis M, Thorpe JA, O'Regan DJ. Atypical presentation of Boerhaave's syndrome as Enterococcal bacterial pericardial effusion. Interact Cardiovasc Thorac Surg 2007; 6:130.
- Michel L, Grillo HC, Malt RA. Operative and nonoperative management of esophageal perforations. Ann Surg 1981; 194:57.
- McGovern M, Egerton MJ. Spontaneous perforation of the cervical oesophagus. Med J Aust 1991; 154:277.
- Wilson RF, Sarver EJ, Arbulu A, Sukhnandan R. Spontaneous perforation of the esophagus. Ann Thorac Surg 1971; 12:291.
- Attar S, Hankins JR, Suter CM, et al. Esophageal perforation: a therapeutic challenge. Ann Thorac Surg 1990; 50:45.
- Maher MM, Lucey BC, Boland G, et al. The role of interventional radiology in the treatment of mediastinal collections caused by esophageal anastomotic leaks. AJR Am J Roentgenol 2002; 178:649.
- Larsen K, Skov Jensen B, Axelsen F. Perforation and rupture of the esophagus. Scand J Thorac Cardiovasc Surg 1983; 17:311.
- Han SY, McElvein RB, Aldrete JS, Tishler JM. Perforation of the esophagus: correlation of site and cause with plain film findings. AJR Am J Roentgenol 1985; 145:537.
- Bladergroen MR, Lowe JE, Postlethwait RW. Diagnosis and recommended management of esophageal perforation and rupture. Ann Thorac Surg 1986; 42:235.
- Dodds WJ, Stewart ET, Vlymen WJ. Appropriate contrast media for evaluation of esophageal disruption. Radiology 1982; 144:439.
- Backer CL, LoCicero J 3rd, Hartz RS, et al. Computed tomography in patients with esophageal perforation. Chest 1990; 98:1078.
- de Lutio di Castelguidone E, Merola S, Pinto A, et al. Esophageal injuries: spectrum of multidetector row CT findings. Eur J Radiol 2006; 59:344.
- Tonolini M, Bianco R. Spontaneous esophageal perforation (Boerhaave syndrome): Diagnosis with CT-esophagography. J Emerg Trauma Shock 2013; 6:58.
- Gubbins GP, Nensey YM, Schubert TT, Batra SK. Barogenic perforation of the esophagus distal to a stricture after endoscopy. J Clin Gastroenterol 1990; 12:310.
- Ivey TD, Simonowitz DA, Dillard DH, Miller DW Jr. Boerhaave syndrome. Successful conservative management in three patients with late presentation. Am J Surg 1981; 141:531.
- Carrott PW Jr, Low DE. Advances in the management of esophageal perforation. Thorac Surg Clin 2011; 21:541.
- Schweigert M, Beattie R, Solymosi N, et al. Endoscopic stent insertion versus primary operative management for spontaneous rupture of the esophagus (Boerhaave syndrome): an international study comparing the outcome. Am Surg 2013; 79:634.
- Dasari BV, Neely D, Kennedy A, et al. The role of esophageal stents in the management of esophageal anastomotic leaks and benign esophageal perforations. Ann Surg 2014; 259:852.
- Siersema PD, Homs MY, Haringsma J, et al. Use of large-diameter metallic stents to seal traumatic nonmalignant perforations of the esophagus. Gastrointest Endosc 2003; 58:356.
- Petruzziello L, Tringali A, Riccioni ME, et al. Successful early treatment of Boerhaave's syndrome by endoscopic placement of a temporary self-expandable plastic stent without fluoroscopy. Gastrointest Endosc 2003; 58:608.
- Chung MG, Kang DH, Park DK, et al. Successful treatment of Boerhaave's syndrome with endoscopic insertion of a self-expandable metallic stent: report of three cases and a review of the literature. Endoscopy 2001; 33:894.
- Dumonceau JM, Devière J, Cappello M, et al. Endoscopic treatment of Boerhaave's syndrome. Gastrointest Endosc 1996; 44:477.
- Gelbmann CM, Ratiu NL, Rath HC, et al. Use of self-expandable plastic stents for the treatment of esophageal perforations and symptomatic anastomotic leaks. Endoscopy 2004; 36:695.
- Fischer A, Thomusch O, Benz S, et al. Nonoperative treatment of 15 benign esophageal perforations with self-expandable covered metal stents. Ann Thorac Surg 2006; 81:467.
- Eisendrath P, Devière J. Plastic stents in the treatment of benign esophageal conditions. Gastrointest Endosc 2008; 68:402.
- van Boeckel PG, Dua KS, Weusten BL, et al. Fully covered self-expandable metal stents (SEMS), partially covered SEMS and self-expandable plastic stents for the treatment of benign esophageal ruptures and anastomotic leaks. BMC Gastroenterol 2012; 12:19.
- Koivukangas V, Biancari F, Meriläinen S, et al. Esophageal stenting for spontaneous esophageal perforation. J Trauma Acute Care Surg 2012; 73:1011.
- Freeman RK, Ascioti AJ, Wozniak TC. Postoperative esophageal leak management with the Polyflex esophageal stent. J Thorac Cardiovasc Surg 2007; 133:333.
- Madanick RD. Medical management of iatrogenic esophageal perforations. Curr Treat Options Gastroenterol 2008; 11:54.
- van Heel NC, Haringsma J, Spaander MC, et al. Short-term esophageal stenting in the management of benign perforations. Am J Gastroenterol 2010; 105:1515.
- Swinnen J, Eisendrath P, Rigaux J, et al. Self-expandable metal stents for the treatment of benign upper GI leaks and perforations. Gastrointest Endosc 2011; 73:890.
- Van Weyenberg SJ, Stam FJ, Marsman W. Successful endoscopic closure of spontaneous esophageal rupture (Boerhaave syndrome). Gastrointest Endosc 2014; 80:162.
- Braun A, Richter-Schrag HJ, Fischer A, Hoeppner J. Minimally invasive therapy of perforations at the esophagogastric junction by over-the-scope clipping. Endoscopy 2013; 45 Suppl 2 UCTN:E133.
- Wehrmann T, Stergiou N, Vogel B, et al. Endoscopic debridement of paraesophageal, mediastinal abscesses: a prospective case series. Gastrointest Endosc 2005; 62:344.
- Kobara H, Mori H, Rafiq K, et al. Successful endoscopic treatment of Boerhaave syndrome using an over-the-scope clip. Endoscopy 2014; 46 Suppl 1 UCTN:E82.
- Harries K, Masoud A, Brown TH, Richards DG. Endoscopic placement of fibrin sealant as a treatment for a long-standing Boerhaave's fistula. Dis Esophagus 2004; 17:348.
- ETIOLOGY AND PATHOGENESIS
- CLINICAL FEATURES
- Clinical manifestations
- Laboratory findings
- Thoracic and cervical radiography
- Contrast esophagram
- Computed tomography
- Upper endoscopy
- DIFFERENTIAL DIAGNOSIS
- Initial management
- Subsequent management
- - Medical management
- - Surgery
- - Endoscopic therapy
- EXCLUSION OF UNDERLYING ESOPHAGEAL PATHOLOGY
- SUMMARY AND RECOMMENDATIONS