REVIEWS Evolving Options in the Management of Esophageal Perforation Clayton J. Brinster, BA, Sunil Singhal, MD, Lawrence Lee, BS, M. Blair Marshall, MD, Larry R. Kaiser, MD, and John C. Kucharczuk, MD Department of Surgery, Division of Cardiothoracic Surgery, University of Pennsylvania Medical Center, Philadelphia, Pennsylvania Esophageal perforation remains a devastating event that The overall mortality associated with esophageal perfo- is difficult to diagnose and manage. The majority of ration can approach 20%, and delay in treatment of more injuries are iatrogenic and the increasing use of endo- than 24 hours after perforation can result in a doubling of scopic procedures can be expected to lead to an even mortality. Surgical primary repair, with or without rein- higher incidence of esophageal perforation in coming forcement, is the most successful treatment option in the years. Accurate diagnosis and effective treatment depend management of esophageal perforation and reduces mor- on early recognition of clinical features and accurate tality by 50% to 70% compared with other interventional interpretation of diagnostic imaging. Outcome is deter- therapies. mined by the cause and location of the injury, the presence of concomitant esophageal disease, and the (Ann Thorac Surg 2004;77:1475–83) interval between perforation and initiation of therapy. © 2004 by The Society of Thoracic Surgeons sophageal perforation continues to present a diag- Historical Background E nostic and therapeutic challenge despite decades of The signs and symptoms of esophageal perforation have clinical experience and innovation in surgical technique. been described in the literature for centuries. In 1946, Accurate diagnosis and early treatment are essential to Barrett [1] reviewed the first report of spontaneous the successful management of patients with this increas- esophageal perforation: Hermann Boerhaave’s 1723 de- ingly frequent condition, but presentation is often am- tailed account of the barogenic esophageal rupture suf- biguous and resembles that of other disorders. The fered by the High Admiral of the Dutch Navy, Baron van diagnostic errors and delayed treatment that result sig- Wassenaer, was due to intense and prolonged vomiting REVIEWS nificantly increase morbidity and mortality. Optimal following excessive ingestion of food and alcohol. The therapy, especially after delayed diagnosis, continues to first successful surgical repairs following esophageal per- evolve. Since the original description of esophageal per- foration were accomplished by Barrett [2] and by Olson foration more than 250 years ago, diagnosis remains and Clagett [3] in 1947. Satinsky and Kron [4] performed challenging, management remains controversial, and the first successful esophagectomy following perforation mortality remains high. in 1952. As early recognition of signs and symptoms improved and antibiotics became widely available, the Material and Methods mortality associated with esophageal perforation de- clined through the 1960s and 1970s. The incidence of A review of the literature by search of the MEDLINE database limited to human studies published in English esophageal perforation has increased with the advance- was performed with the keywords “esophageal perfora- ment of invasive diagnostic technology and the etiology tion,” and “Boerhaave’s syndrome,” which were then has changed from mostly spontaneous or traumatic to matched to “surgery,” “nonoperative,” and “primary mostly iatrogenic. repair.” Articles cited in the references retrieved by Etiology MEDLINE search were reviewed. The etiology, patho- genesis, and clinical, diagnostic, surgical, and nonopera- In 559 patients from recent series, iatrogenic injury to the tive features of esophageal perforation, as well as the esophagus was the most frequent cause of esophageal current mortality rate associated with each respective perforation, with instrumentation accounting for 59% of treatment modality in case series between 1990 and 2003, all patients [5–17]. Spontaneous perforations accounted were examined. for 15% of all patients. Other injuries included foreign body ingestion (12%), trauma (9%), operative injury (2%), tumor (1%), and other causes (2%). The relationship of Address reprint requests to Dr Kucharczuk, Department of Surgery, cause to location is illustrated in Figure 1. Types of Thoracic Surgery Section, Hospital of the University of Pennsylvania, 3400 Spruce St, Philadelphia, PA 19104-4227; e-mail: john.kucharczuk@ instrumentation that commonly cause esophageal perfo- uphs.upenn.edu. ration include esophagoscopy, sclerotherapy, variceal © 2004 by The Society of Thoracic Surgeons 0003-4975/04/$30.00 Published by Elsevier Inc doi:10.1016/j.athoracsur.2003.08.037 1476 REVIEW BRINSTER ET AL Ann Thorac Surg MANAGEMENT OF ESOPHAGEAL PERFORATION 2004;77:1475–83 [29], and atrial surgery [30] have been associated with esophageal perforation. Penetrating trauma causes perforations mainly in the cervical esophagus, and morbidity and mortality are usually due to associated injuries [31]. Perforation sec- ondary to blunt trauma is exceedingly rare, but may present following motor vehicle accident [32] or at- tempted Heimlich maneuver [33]. Ingestion of foreign bodies or caustic materials can produce perforation in areas of anatomic narrowing such as the cricopharyn- geus, the impingement of the aortic arch and left main Fig 1. Association of cause to anatomic location of esophageal perfo- stem bronchus, and in the distal esophagus just proximal ϭ ration in 250 patients from recent series [5, 6, 8, 14, 90]. ab- to the lower esophageal sphincter. Spontaneous, or dominal; ᮀ ϭ thoracic; ■ ϭ cervical. barogenic, esophageal perforation results from a sudden increase in intraesophageal pressure like that associated with hyperemesis, or, much less frequently, childbirth, ligation, pneumatic dilation, bougienage, and laser ther- seizure, prolonged coughing or laughing, or weightlifting apy. Over the past 30 years, the actual risk of esophageal [34]. The rupture usually involves the left wall of the perforation during flexible esophagoscopy has remained supradiaphragmatic esophagus, dissects all esophageal low and is estimated to occur at a frequency of 0.03% layers in a longitudinal manner, and frequently drains during flexible upper endoscopy compared with 0.11% into the left pleural or peritoneal cavity [35]. Erosion by during rigid endoscopy [18, 19]. Simultaneous dilation primary or metastatic esophageal carcinoma [36], Bar- during endoscopy increases the risk of perforation to rett’s ulcers [37], surrounding infection [38], or immuno- between 0.09% for Maloney-Hurst-type dilators and 2.2% deficiency [39] can also cause esophageal perforation. for the Celestin-type dilator [18, 19]. Endoscopic sclero- therapy for esophageal varices leads to perforation in 1% Clinical Presentation to 6% of patients in which the sclerosing agent induces a The cause and location of the injury, as well as the transmural necroinflammatory injury in the esophagus interval between perforation and diagnosis, determine [20]. Decreasing the volume and concentration of sclero- the clinical features of esophageal perforation. Diagnosis sant may reduce this risk [20]. Esophageal perforation is often difficult because presentation is inconclusive and REVIEWS can also occur during endoscopic variceal ligation, when often mimics that of other disorders such as myocardial the esophageal mucosa is caught and torn between the infarction, peptic ulcer perforation, pancreatitis, aortic overtube and the endoscope [20]. The risk of perforation aneurysm dissection, spontaneous pneumothorax, or during pneumatic dilation for achalasia is 1% to 5%, with pneumonia. Common clinical manifestations of esopha- higher inflation pressure and previous pneumatic dila- geal perforation include chest pain, dysphagia, dyspnea, tion increasing this risk [21]. The placement of nasogas- subcutaneous emphysema, epigastric pain, fever, tachy- tric tubes [22], endotracheal tubes [23], Sengstaken- cardia, and tachypnea. Any combination of these signs Blakemore or Minnesota tubes for tamponading variceal and symptoms following instrumentation of the esopha- bleeding [20], or endoesophageal prostheses [24] can also gus or respiratory tract implies perforation until proven cause esophageal perforation. otherwise. In a normal esophagus, the location at greatest risk of Cervical perforation of the esophagus is generally less instrumental injury is Killian’s triangle, which is formed by severe and more easily treated than intrathoracic or the inferior constrictor pharyngeus and the cricopharyn- intraabdominal perforation. Spread of contamination to geus muscles. In this region, the posterior esophageal the mediastinum through the retroesophageal space is mucosa is unprotected by muscularis, and is separated slow after cervical perforation, and attachments of the from the retroesophageal space by buccopharyngeal fascia esophagus to the prevertebral fascia in this region limit only. Cervical osteophytic spurs, kyphosis of the spine, or lateral dissemination of esophageal flora. Patients with hyperextension of the neck can further increase the risk cervical perforations can present with neck pain, cervical of perforation in this area [25]. Instrumental perforation dysphagia, dysphonia, or bloody regurgitation. Subcuta- also occurs at anatomic areas of narrowing such as the neous emphysema is commonly found on physical ex- distal esophagus just proximal to the gastroesophageal amination