0016-5107/95/4102-013053.00+ 0 GASTROINTESTINAL ENDOSCOPY Copyright | 1995 by the American Society for Gastrointestinal Endoscopy Short- and long-term outcome of esophageal perforation Richard Sawyer, MD, Clay Phillips, MD, Nimish Vakil, MD Rochester, New York Esophageal perforation is an important complication of endoscopy and dilation, but few data are available regarding long-term outcome in these patients. Thirty-one consecutive patients with esophageal perforation were studied. Long-term outcome was determined by office follow-up and questionnaires via mail or telephone. The study group included 16 men and 15 women with a mean age of 61 +_ 4 years. Instrumentation was the principal cause of perforation (77%), and pain was the principal symptom in the majority of cases. However, some patients reported no pain, their only symptom being shortness of breath or dysphagia on swallowing saliva. Contrast examinations were used to establish the diagnosis, but results were initially negative in 3 of 27 patients (11%). Seven patients were treated nonsurgically (nasogastric drainage, antibiotics, and intravenous alimentation). Fourteen patients underwent primary closure of the esophageal rent. Six patients underwent surgical drainage, 3 underwent total esophagectomy, and I died during surgery before the esophagus was exposed. Thirty-day mortality was 6.4%. Mean hospital stay was 26 +_ 5 days. Postdischarge follow-up was available in 28 of the 29 patients (97%) surviving initial hospitalization, and the mean follow-up was 47 _+ 9 months. Nineteen patients had persistent dysphagia, and 6 underwent dilation again. One of these patients had a second perforation. Contrary to results of some earlier studies, the mortality from esophageal perforation was low in our study because of early intervention in suspected cases. Results of contrast studies are sometimes negative in the early stages after perforation, and studies may need to be repeated. Long-term dysphagia is a difficult problem in patients with strictures, and some patients need further dilation, with the risk for another perforation. In contrast, long-term dysphagia is rare in patients with barogenic perforation. (Gastrointest Endosc 1995;41:130-4.) Esophageal perforation is a serious complication of instrumentation in the esophagus and also occurs as a result of increased luminal pressure (barogenic perforation), usually as a result of vomiting (Boerhaave's syndrome). 1 With advances in antibiotic therapy and greater awareness of this complication, patients are Received January 11, 1994. For revision February 23, 1994. Accepted May 21, 1994. From the Departments of Medicine and Thoracic Surgery, University of Rochester Medical Center, Rochester, New York. Presented in part at the 58th annual meeting of the American College of Gastroenterology, New York, October 1993. Reprint requests: Nimish Vakil, MD, University of Wisconsin Medical School, SSMC, 945 North 12th Street, A 324 A, Milwaukee, WI 53233. 37/1/58186 130 GASTROINTESTINAL ENDOSCOPY receiving early treatment, and many survive the initial episode of perforation. However, few data are available regarding long-term outcome in these patients. 2 The aim of this study was to determine the short- and long-term outcome of therapy for esophageal perforation. METHODS Strong Memorial Hospital is a regional referral center for the treatment of esophageal perforation. From 1980 to 1993, 31 consecutive patients were treated for esophageal perforation. These patients were identified retrospectively by searching a computerized database of patient diagnoses. The hospital and office records of these patients were reviewed. Long-term outcome was determined by patient interview, review of follow-up data, and telephone or mail questionVOLUME 41, NO. 2, 1995 naires. Dysphagia was assessed and graded as follows: 0, no dysphagia; 1, occasional dysphagia with some solids, minor dietary limitations; 2, dysphagia with most solids, able to eat soft solids only; 3, able to tolerate semisolids only; 4, able to swallow liquids only; 5, severe dysphagia to solids and liquids. Results were expressed as mean + SEM. Techniques used for dilation, postdilation management, and treatment of suspected and proven perforation are described below. Dilation technique and immediate postdilation management Balloon dilation for achalasia. This procedure was performed on an outpatient basis, usually early in the morning to permit observation for several hours. All patients underwent a contrast esophagogram immediately after the procedure. If no evidence of perforation was found, patients were given water to drink after recovery from anesthesia. Pain or dysphagia after water ingestion was considered suggestive of perforation, and these patients then underwent another contrast examination. Both the cases in this study were detected by contrast studies immediately after dilation. M a l o n e y a n d S a v a r y dilation. Savary dilation was always performed under fluoroscopic guidance after endoscopic or combined endoscopic and fluoroscopic placement of a guidewire. Maloney dilation was usually not performed under fluoroscopic guidance. After dilation, patients were observed for at least 30 to 45 minutes if unsedated or until recovery from sedation. If the patient reported chest pain or dysphagia on swallowing saliva, a contrast esophagogram was immediately performed. Patients with minor throat discomfort or no symptoms were given a glass of water to swallow before discharge. Pain, dysphagia, or shortness of breath on ingestion of water were indications for a contrast examination. Criteria for treatment of perforation The choice of therapy for perforation was based on clinical criteria. Patients were treated nonsurgically if they met the following criteria adapted from Cameron et al.3: 1. Recent perforation (within 24 hours); 2. No food intake after the perforation; 3. Perforation not proximal to a high-grade stenosis; 4. Minor symptoms (pain or dysphagia) without clinical signs of sepsis or hemodynamic compromise; 5. Perforation contained within the mediastinum without contamination of adjacent body cavities (e.g., pleural space); 6. Contrast studies showing a small perforation with good drainage of contrast material back into the esophagus. In surgically treated patients, primary closure was usually selected for patients with recent perforations and a normal esophagus or those with recent perforations and minor strictures with a reasonable prospect of good esophageal function. Esophagectomy was performed if the perforation was longstanding within a suppurative environment and if the underlying disease made reasonable function unlikely even with closure of the perforation (e.g., mega-esophagus, neoplasm, and chronic refractory stricture). Decisions were individualized and also based on the general condition of the patient. VOLUME 41, NO. 2, 1995 RESULTS Patient characteristics S t u d y subjects included 16 m e n and 15 women. T h e i r m e a n age was 61 _+ 4 years, with a range of 21 to 89 years. Etiology and presentation I n s t r u m e n t a t i o n was the most c o m m o n cause of esophageal perforation, accounting for 23 patients (77%). Seventeen patients u n d e r w e n t dilation for esophageal strictures proved to be benign by previous endoscopic biopsy and cytology studies. Sixteen of these were chronic strictures caused by reflux esophagitis, and 1 was a radiation stricture. In these 17 patients, perforations developed in 5 with Savary dilators; all of these procedures were p e r f o r m e d u n d e r fluoroscopic guidance. P e r f o r a t i o n developed in 6 patients after dilation with Maloney dilators; these procedures were p e r f o r m e d without fluoroscopy. Perforation developed in 1 p a t i e n t after through-the-endoscope balloon dilation of a benign esophageal stricture. P e r f o r a t i o n occurred in 2 patients after pneumatic dilation for achalasia and in I as a complication of sclerotherapy. A t t e m p t s at i n t u b a t i o n caused perforation in 3 patients: 1 during intubation with a rigid esophagoscope, 1 during endotracheal intubation, and 1 during E R C P with a flexible endoscope, after which a high cervical perforation of a Zenker's diverticulum developed. T h e latter was the only case of perforation after diagnostic flexible endoscopy during this period. P e r f o r a t i o n occurred after vomiting (barogenic perforation) in 5 patients, and no obvious cause (nonbarogenic spontaneous perforation) could be found in 1. P e r f o r a t i o n occurred in 1 patient after foreign body ingestion and in a n o t h e r after blunt t r a u m a to the chest. T h e perforation rate with individual procedures could not be d e t e r m i n e d with accuracy for the entire s t u d y group because 12 of the 31 patients in this s t u d y were t r a n s f e r r e d to our hospital from other institutions and details of annual procedure rates were not available. Accurate d a t a regarding individual procedures from our institution ( J a n u a r y 1992 to J u n e 1993) provide some estimate of the frequency of perforation. During this period, 1844 UGI endoscopic examinations were p e r f o r m e d with no perforations, 6 balloon dilations for achalasia with 1 perforation, 57 pneumatic dilations of stenoses with no perforations, 235 Maloney dilations with no perforations, and 99 Savary dilations with 2 perforations. Symptoms Pain, the most f r e q u e n t presenting symptom, was n o t e d in 28 of the 31 cases (90%). Twelve patients GASTROINTESTINAL ENDOSCOPY 131 Table 2. Long-term dysphagia and type of surgery (n 28*) Table 1. Cause of perforation and type of surgery (n = 30*) Cause Esophageal dilation (n = 19)* Intubation = Nonsurgical Primary Surgical Esophagectomy therapy closure drainage 4 9 3 2 0 3 0 0 0 0 1 0 1 0 0 0 0 0 1 0 1 2 1 1 1 0 0 0 (n = 3) Sclerotherapy (n = 1) Blunt trauma (n = 1) Foreign body (n = 1) Barogenic (n = 5) Spontaneous (n = 1) *One patient died during surgery before the esophagus was exposed; 2 patients undergoing balloon dilation for achalasia had myotomy in addition to closure of the perforation. (36%) had dysphagia and 6 patients (18%) complained of shortness of breath. Diagnosis Chest radiographs were performed in 24 patients and revealed mediastinal air in 2 of them. Contrast studies were initially diagnostic in 24 of 27 subjects in whom they were performed. Results of barium swallow studies were initially normal in 2 cases but when repeated several hours later demonstrated perforation. The third patient with a normal barium swallow study had mediastinal air on the chest radiograph and did not undergo another contrast study. Three patients were diagnosed by gastroscopy, and 1 perforation was recognized at the time of rigid endoscopy. Of the 31 perforations, 6 occurred in the upper esophagus, 4 in the midesophagus, and 21 in the lower esophagus. Treatment Twenty-nine of the 31 patients presented within 24 hours, and all suspected perforations were initially treated with nasogastric aspiration and intravenous antibiotics. Seven patients were treated nonsurgically (nasogastric drainage, antibiotics, and intravenous alimentation) using the criteria outlined above. Fourteen patients underwent primary closure of the esophageal rent. Six patients underwent surgical drainage, and 3 patients underwent total esophagectomy (Table 1). Gross contamination of the pleural space and mediastinitis were noted in i of these cases; in another purulent mediastinitis was present and the exact location of the perforation could not be identified. The third patient had a distal esophageal stricture and a rent that could not be adequately treated with a 132 GASTROINTESTINAL ENDOSCOPY Dysphagia grade Nonsurgical therapy Primary closure Surgical drainage 0 1 2 3 4 5 4 0 0 2 1 0 1 3 1 3 5 0 2 2 0 0 1 1 Es~176 2 0 0 0 0 0 *Two patients died within 30 days of treatment and 1 patient was lost to follow-up. fundal patch. Hyperalimentation or feeding through a jejunostomy tube inserted at surgery was used in all surgically treated patients. Six patients with small perforations who were treated medically could be managed with peripheral alimentation alone. Early outcome Two deaths occurred in the surgically treated patients and none in the medically treated patients. One patient died during surgery of hemorrhage from large collateral vessels near the gastroesophageal junction. Another patient died of sepsis in the postoperative period (2 weeks after esophagectomy). The 30-day mortality was therefore 6.4 %. All the other patients recovered and were discharged from the hospital. Mean duration in the hospital after perforation was 26 + 5 days, with a range of 5 to 115 days. Long-term results Of the 29 patients surviving initial hospitalization, follow-up information was available for 28 (97%). Three patients died during the follow-up period, 2 from unrelated malignancies and 1 from multiple organ failure, also unrelated to the perforation. The mean follow-up was 47 + 9 months, with a range of 3 to 150 months. Nine patients reported no dysphagia after surgery; 1 of these patients had a primary diagnosis of achalasia, and the others had benign strictures of the esophagus. The other patients reported varying grades of dysphagia (Tables 2 and 3). Treatment of the dysphagia was individualized according to underlying disease, overall medical condition of the patient, and patient choice. Understandably, patients were reluctant to undergo any procedures despite symptoms. Dysphagia was sufficiently severe to warrant esophageal dilation in 6 patients. Five of these patients had a benign esophageal stricture secondary to reflux, and 1 had a stricture after sclerotherapy for esophageal varices. All patients underwent esophagoscopy and barium swallow studies, and surgical therapy was considered in all. Three patients were determined to be unfit for surgery, 1 because of advanced liver disease and portal hypertension and 2 because of their poor VOLUME 41, NO. 2, 1995 Table 3. Long-term dysphagia and cause of perforation (n = 28*) Grade Grade Grade Grade Grade Grade Cause 0 1 2 3 4 5 Esophageal dilation 4 2 1 4 5 1 (n = 17) Intubation 2 1 (n = 3) Sclerotherapy 1 (n = 1) Blunt trauma (n = 1) Foreign body (n = 1) Spontaneous 1 1 (n = 5) *Two patients died within 30 days of treatment and 1 patient was lost to follow-up. medical condition. Three patients refused further surgical intervention. Five patients had no complications from subsequent dilation, but another perforation developed in 1 patient as a result of subsequent dilation. A second barogenic perforation, requiring surgery, also occurred in a patient with barogenic perforation. DISCUSSION As in other studies, esophageal dilation was the most frequent cause of esophageal perforation, 4-6 and the most frequent site of perforation was the thoracic esophagus. The only case of perforation caused by fiberoptic endoscopy, a high cervical perforation of a Zenker's diverticulum, occurred during blind intubation for ERCP. Diverticula have been described to predispose to perforation in previous studies; the incidence has ranged from 2 % to 15 % .7, s The absence of patients with malignancy in our study deserves some comment. In part it represents the low incidence of squamous cell carcinoma in our patient population and concurrent trials of radiotherapy and chemotherapy for the palliation of dysphagia. Some studies have suggested that bougienage does not necessarily entail a higher risk for perforation in malignancy.9 Barogenic perforation refers to esophageal disruption caused by an abrupt rise in esophageal pressure. In our study, forceful vomiting was the most common cause of barogenic perforation in otherwise healthy patients; 1 patient had nonbarogenic spontaneous perforation of undetermined cause. Pain, dysphagia, and shortness of breath were the most frequent symptoms, but it is noteworthy that even in the cases of acute perforation after dilation, some patients did not report pain but reported dysphagia when swallowing saliva. Results of initial contrast studies were negative in 3 of 27 patients (11% ), and this figure is similar to data reported in other studies. 1~ All 3 had received V O L U M E 41, NO. 2, 1995 Table 4. Long-term dysphagia and site of perforation Dysphagia Cervical Distal grade esophagus Midesophagus esophagus 0 2 1 6 1 4 0 1 2 0 0 1 3 0 1 4 4 0 1 6 5 0 1 0 barium as the contrast agent. It is important to recognize that this may occur when studies are performed soon after perforation, perhaps because of edema at the site of a small perforation, and that patients should be presumptively treated if a perforation is suspected. The contrast study should then be repeated after several hours. Foley et al. u described 6 patients in whom water-soluble contrast failed to demonstrate a perforation, which could then be demonstrated by administration of barium. Our study demonstrates that the same may also be true with barium. Some experts recommend that the initial contrast examination be performed with a water-soluble contrast agent, which is thought to cause less irritation of the mediastinum; if results are negative, this study should be followed by administration of barium, n Others recommend the use of thinned barium as the agent of choice. 12 In either case, it is important to repeat the study after several hours if the clinical picture is suggestive of perforation but results of the initial examination are negative. Esophagoscopy can be used to identify the site and size of the perforation, t but visualization is quite difficult if a contrast study precedes this examination. Additionally, insuffiation of air may increase contamination of the mediastinum if a perforation is present and convert a small perforation that may be treated medically into an overt perforation requiring surgery. The symptoms of perforation can be subtle, and the low mortality in this study is in part related to early investigation in patients with dysphagia, chest pain, or shortness of breath after dilation. A thoracic surgeon was involved in the care of each patient early in the course, and decisions to treat nonoperatively were made in consultation with the surgeon. This guards against the natural tendency of the endoscopist causing the perforation to favor conservative therapy while precious time is lost. Delay in treatment of more than 24 hours is associated with a greater risk of complications and a higher mortality. In our study, patients who did not meet criteria for nonsurgical management underwent surgery promptly (within 24 hours in the majority of cases), and no patient initially treated with medical therapy required subsequent surgery. The mortality from esophageal perforation is quite variable and has ranged from 7% to 46% .5,13 In a colGASTROINTESTINAL ENDOSCOPY 133 lective review of 450 patients, Jones and Ginsberg 12 reported that iatrogenic and instrumental perforations were associated with a mortality of 19 %, whereas spontaneous perforations had a mortality of 39 %. Two other studies 14,15 have, however, shown no difference between spontaneous and instrumental perforation. In our study, 1 patient with spontaneous and 1 with instrumental perforation died after surgery. One of these patients had previously unrecognized portal hypertension and died of hemorrhage during surgery, and the other died of sepsis in the postoperative period. The higher mortality in the surgical group is to be expected because these patients tend to have large perforations with gross contamination of the mediastinum, a delayed diagnosis, and life-threatening complications that should never be present in the patients treated medically. Few data on the long-term results of surgical therapy for esophageal perforation are found in the literature. Saabye et al. 2 reported 35 patients who survived simple closure and drainage of the esophagus. They found that long-term dysphagia was rare in the patients who had a normal esophagus before perforation. Severe reflux esophagitis and an esophageal stricture that required surgery developed in 1 of the 7 patients with barogenic perforation in that study; long-term dysphagia did not occur in any of the 8 patients with instrumental perforation and a normal esophagus. Of 11 patients who had esophageal strictures, 6 had no postoperative dysphagia, 4 required long-term dilation, and 1 required later esophagectomy. These data are unusual because simple closure of esophageal perforations, particularly in patients with a distal esophageal stricture, has been associated with a poor outcome, and many surgeons recommend more definitive surgery for the stricture in one or two stages. 4 The decision to operate once again on a patient with dysphagia must be determined on a case-by-case basis. If significant dysfunction is present after the initial repair, antireflux surgery or esophagectomy may be indicated depending on the prospect of improved function. An antireflux procedure would be appropriate in a patient with persistent reflux and moderate dysfunction, but dilation might be more effective in the setting of reduced acid reflux. Esophagectomy would be indicated in patients with severe dysfunction and poor prospects of recovery, provided the patient had an acceptable risk for surgery. Both trans-hiatal esophagectomy and Ivor Lewis esophagectomy carry the risk of serious morbidity that must be considered in making this decision. In the follow-up of our patients, we found it noteworthy that some patients refused further therapy with dilation or surgery despite persistent dysphagia and the continued need for supplemental nu- 134 GASTROINTESTINAL ENDOSCOPY trition by jejunostomy feedings. Others with severe dysphagia refused surgery b u t submitted to dilation, and a small number of patients were treated with dilation because they were considered poor risks for surgery. As in Saabye's study, we found that long-term dysphagia was very rare in patients with an intrinsically normal esophagus. In 1 patient with perforation after sclerotherapy for esophageal varices, a postsclerotherapy stricture and significant dysphagia developed, and a reflux stricture developed in a patient with barogenic perforation. Serious dysphagia (grade 3 or more) was confined to midesophageal or distal esophageal lesions. Establishing fixed criteria for the assessment of patients in the postdilation recovery period and recognizing that the symptoms of perforation may be quite subtle may help in the early diagnosis and management of this devastating complication. ACKNOWLEDGMENT We thank Dr. Richard Feins of the Department of Thoracic Surgery for assistance with data acquisition. REFERENCES 1. Curci JJ, Horman MJ. Boarhaave's syndrome: the importance of early diagnosis and treatment. Ann Surg 1976;183:401-8. 2. Saabye J, Nielsen HO, Andersen K. Long-term observation following perforation and rupture of the esophagus. Scand J Thorac Cardiovasc Surg 1988;22:79-80. 3. Cameron JL, Kieffer RF, Hendrix TR, Mehigan DG, Baker RR. Selective nonoperative management of contained intrathoracic esophageal disruptions. Ann Thorac Surg 1978;27:404-8. 4. Moghissi K, Pender D. Instrumental perforations of the esophagus and their management. Thorax 1988;43:642-6. 5. Kim-Deobald J, Kozarek RA. Esophageal perforation: an 8-year review of a multispecialty clinic's experience. Am J Gastroenterol 1992;87:1112-9. 6. Goldstein LA, Thompson WR. Esophageal perforations: a 15 year experience. Am J Surg 1982;143:495-503. 7. Larsen K, Jensen BS, Axelson F. Perforation and rupture of the esophagus. Scand J Thorac Cardiovasc Surg 1983;17:311-8. 8. Schulze S, Pederson VM, Hoier-Madsen K. Iatrogenic perforation of the esophagus. Acta Chir Scand 1982;148:679-82. 9. Heit HA, Johnson LF, Siegel SR, Boyce HW. Palliative dilation for dysphagia in esophageal carcinoma. Ann Intern Med 1978; 89:623-31. 10. Sarr HG, Pemberton JH, Payne WS. Management of instrumental perforations of the esophagus. J Thorae Cardiovase Surg 1982;84:211-8. 11. Foley MJ, Ghahremani G, Rogers L. Reappraisal of contrast media used to detect upper gastrointestinal perforations. Radiology 1982;144:231-7. 12. Jones WG, Ginsberg RJ. Esophageal perforation: a continuing challenge. Ann Thorac Surg 1992;53:534-43. 13. Flynn AE, Verrier ED, Way LW, Thomas AN, Pellegrini CA. Esophageal perforation. Arch Surg 1989;124:1211-4. 14. Graeber GM, Niezgoda JA, Burton NA, Collins GJ, Zajtcbuk R. A comparison of patients with endoscopic esophageal perforations and patients with the Boerhaave's syndrome. Chest 1987; 92:995-8. 15. Triggiani E, Belsey R. Oesophageal trauma: incidence, diagnosis and management. Thorax 1977;32:241-9. VOLUME 41, NO. 2, 1995
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