Surgical Aspects of Esophageal Disease
Perforation and Caustic Injury

Joseph B. Zwischenberger, Clare Savage, and Akhil Bidani

Departments of Surgery and Medicine, The University of Texas Medical Branch, Galveston, Texas

	PERFORATION OF THE ESOPHAGUS

TOP PERFORATION OF THE ESOPHAGUS CAUSTIC INJURY REFERENCES

Esophageal perforation presents as an emergency because treatment delay reduces survival. Iatrogenic perforation, spontaneous perforation, and trauma account for a large majority of esophageal perforations (1-4). Endoscopic procedures are the most common cause of iatrogenic esophageal perforation, with the cricopharyngeal area most commonly injured (1). Mid- and distal esophageal perforations usually result from biopsies to document malignancy or from dilatations. Infrequent causes of iatrogenic esophageal perforation include difficult endotracheal intubation, blind insertion of a minitracheostomy, resection of lung cancer, blind dissection of the abdominal esophagus, operations on the cervical spine, thyroidectomy, and palliative intubation, stenting, or laser treatment of esophageal tumors. Boerhaave's syndrome, esophageal rupture induced by straining, is the most common type of spontaneous perforation. Ruptures usually occur in the left posterior aspect of the lower esophagus and are five times more frequent in males (1). Severe reflux and candidal, herpetic, and immunodeficiency infections also cause pathologic perforations. Destruction of the esophageal wall by carcinoma may cause mediastinal or pleural perforations. Esophageal perforations from penetrating or blunt trauma are frequently overshadowed by associated injuries and have a poor prognosis if unrecognized (1, 5, 6).

Diagnosis

Symptoms (pain, vomiting, hematemesis, dysphagia, or tachypnea) and signs (tachycardia, fever, subcutaneous emphysema, cardiac crunch, chest hypersonarity, or dullness) of esophageal perforation vary with cause and location (cervical, thoracic, or abdominal). Pain is the most common symptom, present in 70 to 90% of patients, usually referring directly to the site of perforation (7). Cervical perforation is characterized by neck ache and stiffness due to esophageal attachment to the prevertebral fascia limiting spread of oropharyngeal soilage (1). In the abdomen, subxyphoid pain is present with anterior perforations, and dull epigastric pain radiating to the back may occur if the perforation is posterior and communicates with the lesser sac. Severe retrosternal or chest pain lateralizing to the side of perforation is seen with thoracic perforation. Severe chest pain after straining and hematemesis occur with postemetic ruptures. Tachycardia and tachypnea are documented in most patients with perforation. Hypotension and shock are present when sepsis or significant inflammatory third spacing have occurred. Subcutaneous emphysema is seen frequently with cervical perforations but less often with thoracic or abdominal perforations. Dysphagia appears late and is usually related to a thoracic perforation.

Chest X-ray is suggestive in 90% of patients but may be normal immediately after perforation. Pneumomediastinum, subcutaneous emphysema, mediastinal widening, or a mediastinal air-fluid level prompt investigation to rule out esophageal perforation. Hydropneumothorax on the left is seen in patients with distal third esophageal perforations. Water-soluble contrast esophagogram followed by dilute barium reveals the primary area of leakage and determines whether the perforation is confined to the mediastinum or communicates freely with the pleural or peritoneal cavities. Unfortunately, the rate of false-negative esophagograms may be as high as 10% (8). Chest computed tomography often shows mediastinal fluid and air at the site of perforation. If a perforation is suspected during an endoscopic procedure, careful inspection of the esophagus without air insufflation is warranted. Based on our experience, esophagoscopy can miss a perforation hidden in a mucosal fold or aggravate soilage by air insufflation and is not recommended as a primary diagnostic study.

Treatment

Etiology, location, and delay between rupture and treatment affect prognosis and management of esophageal perforation. Lapse of time before drainage or repair of the perforation, regardless of cause and location, is the most significant influence on outcome (7, 9). Postemetic perforation is the most morbid, with decreased survival from massive contamination and delayed diagnosis. Morbidity and mortality increase as the perforation extends into the thorax (10). Iatrogenic perforation, often noted immediately during endoscopic instrumentation, results in less morbidity and mortality. Patients with perforations of the cervical esophagus have a 94% survival, those with thoracic have a 66% survival, and those with abdominal perforations have a 71% survival (1).

Nonoperative management of esophageal perforations has been associated with a 20 to 38% mortality (11, 12); however, in carefully selected patients, mortality can approach zero (3, 13). The difficulty with nonoperative management is prospective determination of which perforation will remain contained and which will cause ongoing contamination with subsequent uncontrolled infection. Criteria for nonoperative management proposed by Cameron and associates (13) include a well-contained leak in a stable patient without evidence of sepsis or communication with the pleural or peritoneal cavity. The perforation must easily drain back into the esophagus. Signs and symptoms of sepsis during nonoperative management warrant immediate surgical treatment. Pneumothorax, mediastinal emphysema, and respiratory failure are also indications for surgical interventions.

Surgery, including primary repair, exclusion and diversion, or resection (esophagectomy), remains the mainstay of treatment (Figure 1) (1-4, 14, 15). Preoperative preparation includes nasogastric intubation for gastric decompression, broad-spectrum antibiotics for oropharyngeal contamination, and intravenous fluid resuscitation. Cervical perforations are treated by primary closure and drainage of the neck. Thoracic esophageal perforations require a right thoracotomy for exposure of the upper two-thirds and left thoracotomy for the lower third. Lesions at the esophagogastric junction are approached by left thoracotomy or upper midline celiotomy, with repairs often reinforced by fundoplication. Perforations require wide mediastinal drainage by opening the parietal pleura the entire length of the esophagus. Nonviable and grossly contaminated tissue in the mediastinum and the parietal pleura is debrided. The esophagus and often the esophagogastric junction must be dissected completely to identify the site of perforation and mobilize the esophagus for a tension-free repair. Esophagomyotomy (an incision through the longitudinal and circular muscle layers, exposing the submucosa) is often necessary to visualize the mucosal injury. Primary repair is accomplished with closure of the mucosal defect over a bougie and reapproximation of the muscle (15). Thoracic esophageal perforation repairs can be reinforced by an autologous pleural flap or by pedicled muscle flaps from the intercostal muscles, chest wall musculature, diaphragm, or a mobilized pedicle of omentum (16). Reinforcement with vascularized tissue may decrease fistula formation (13%) and mortality (6%), compared with unreinforced primary repair (39% fistula formation, 25% mortality) (7). Esophageal repairs are then drained by a large-bore chest tube.

View larger version (37K): [in this window] [in a new window]   Figure 1.   Algorithm for treatment of esophageal perforation (modified from Zwischenberger and colleagues [37], with permission).

Late perforations can usually still be repaired primarily, reinforced by muscle or pleura (7, 15, 17). If repair is not possible or if severe mediastinitis is present, options include esophageal resection or exclusion and diversion. Exclusion and diversion entails cervical esophagostomy (diversion of the cervical esophagus, creating a salivary fistula), gastric decompression with a gastrostomy, and jejunostomy with delayed (6 months) reconstruction. An alternative to exclusion and diversion is T-tube drainage of the perforation, creating a controlled esophagocutaneous fistula (3, 18). T-tube placement can be used in high-risk patients, but continued leakage can progress to sepsis and is often not recommended as a routine procedure (3, 7, 18).

Esophageal resection, with or without immediate reconstruction, should be considered a first line procedure for perforations in patients with megaesophagus, carcinoma, caustic ingestion or stenosis, or severe undilatable reflux strictures (2, 4, 14, 15). If the underlying pathologic process is esophageal carcinoma, resection and immediate reconstruction are indicated if the lesion is otherwise resectable. If perforation has occurred during palliative treatment of unresectable esophageal carcinoma, a covered metallic stent, which closely follows the contours of the esophagus, usually prevents leakage (19). Treatment of a spontaneous perforation with a flexible covered stent has also been reported (20).

Postoperative care includes nasogastric tube decompression of the stomach until resolution of the postoperative ileus, after which enteral feeding is slowly advanced through a jejunostomy or Dobhoff tube. An esophagogram is obtained 5-10 days postoperatively to document absence of a leak and allow oral intake. Long-term surveillance for stricture formation, reflux, or carcinoma is also recommended.

In summary, treatment of an esophageal perforation entails fluid resuscitation, control of sepsis, operative drainage of the mediastinum and pleural cavity, primary repair of the esophagus, and reinforcement with vascularized tissue. Although delayed diagnosis increases the complexity because of friable tissue at the site of perforation, primary repair may still be possible. In cases with severe inflammation, either resection or exclusion and diversion is recommended. Primary resection is indicated for undilatable strictures, megaesophagus, carcinoma, or caustic ingestion.

	CAUSTIC INJURY

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The severity and site of caustic esophageal injury depend on the substance ingested (alkali versus acid, solid versus liquid, and concentration), quantity ingested, residual food in the stomach, and duration of tissue contact. Alkali causes liquefactive necrosis, resulting in a deep burn, whereas acids cause coagulative necrosis, forming an eschar that limits tissue penetration (21). Solid alkali tends to adhere to and burn the oropharynx, whereas liquid alkali is rapidly swallowed, causing less oropharyngeal but more esophageal and/or gastric injury (22, 23). Sites susceptible to injury because of a relative delay in transit include the upper esophagus in the area of the cricopharyngeus, the mid-esophagus where the aorta and left main stem bronchus impinge, and the distal esophagus proximal to the lower esophageal sphincter (LES).

Caustic injury also results in a hypotensive LES with reflux and prolonged exposure of the distal esophagus (24). Alkali ingestion results in pylorospasm, with regurgitation of caustic agent into the esophagus, followed by cricopharyngeal muscle spasm and propulsion back into the stomach, aggravating both the esophageal and gastric burns. With acid ingestion, the esophagus may escape injury because of relative tolerance by the squamous epithelium. However, in the stomach, acid induces immediate pylorospasm, pooling the acid in the distal antrum, producing severe gastritis that may progress within 24-48 hours to full-thickness necrosis and perforation.

Symptoms of caustic ingestion include oral pain, hematemesis, drooling, and inability or refusal to swallow. Hoarseness, stridor, and dyspnea suggest laryngeal edema or epiglottic injury, prompting a thorough airway evaluation with bronchoscopy and laryngoscopy and possible intubation or tracheostomy to maintain airway patency. Steroids may be administered to relieve airway obstruction due to mucosal edema and bronchospasm. Substernal, back, or abdominal pain may signify mediastinal or peritoneal perforation. Absence of symptoms or evidence of oropharyngeal burns does not exclude esophageal injury (22, 23, 25, 26). A recent report of caustic ingestion in 85 pediatric patients, however, showed absence of symptoms was always associated with no or minimal injury, whereas hematemesis, respiratory distress, or presence of at least three symptoms was consistently associated with severe injury (27).

Management of caustic ingestion involves immediate verification of the etiologic agent, followed by chest and abdominal X-rays and then esophagoscopy. Esophagoscopy is recommended 12-24 hours postinjury to allow gastric emptying and stabilization of the patient (21-23, 25, 26, 28, 29), unless esophageal or gastric perforation is suspected (Figure 2). The severity of esophageal injury is classified as first degree (hyperemia and edema), second degree (ulceration), or third degree (massive edema and eschar formation with or without full-thickness necrosis). Esophagoscopy, which usually requires general anesthesia in children, may not be necessary in asymptomatic pediatric patients. Instead, some advocate close observation (27, 30). A recent prospective study by Millar and coworkers of 22 patients showed a 100% negative predictive value for detection of caustic esophageal injury with radiolabeled sucralfate (30). The authors suggest screening children with suspected caustic ingestion with radiolabeled sucralfate before esophagoscopy. Treatment of first-degree esophageal burns, which do not perforate or form strictures, is usually observation for up to 48 hours (22, 23, 25, 31). Patients with second- or third-degree esophageal burns without evidence of perforation are placed in the intensive care unit (ICU), kept nil per os (NPO), and given intravenous fluids and antibiotics to decrease the risk of aspiration and bacterial contamination of the mediastinum through the injured esophageal wall (22). Because acid reflux may increase stricture formation, prophylactic H₂-blockers, proton-pump inhibitors, or antacids are recommended (27, 32). Steroids have not been shown to be efficacious in preventing stricture formation and may mask signs of peritonitis (31). The endoscope is advanced to the area of the first severe burn to avoid iatrogenic perforation, often precluding full assessment of the esophagus and stomach. In addition, assessment of the depth of burn is difficult during esophagoscopy (22, 26). An alternative approach, advocated by Estrera and associates, is exploratory laparotomy for all patients with second- or third-degree burns seen during esophagoscopy (25). With this approach, all burns with full-thickness necrosis undergo radical esophagogastrectomy, cervical esophagostomy, and jejunostomy for feeding. Those with second- and third-degree burns without full-thickness esophagogastric necrosis have an intraluminal stent placed for at least 21 days to prevent obliteration of the esophageal lumen and provide a template for epithelial ingrowth (25). Estrera and coworkers reported that 4/4 patients with second-degree burns and 3/5 patients with limited third-degree burns treated with a stent did not develop strictures (25).

View larger version (40K): [in this window] [in a new window]   Figure 2.   Algorithm for treatment of caustic ingestion. Abd = abdomen; IVF = intravenous fluids; PPI = proton-pump inhibitor; TPN = total parenteral nutrition.

Patients with second- or third-degree injuries are monitored for evidence of perforation with serial chest and abdominal X-rays plus a barium swallow performed at 24 hours. Treatment alternatives include an elective gastrostomy for feeding with passage of a nasogastric string for retrograde dilatation of strictures, which is commonly used in pediatric patients (26, 28). A second alternative is intraluminal stent placement for prevention of strictures plus gastrostomy, as discussed previously (25). The third option is to continue antibiotics and initiate feeding via a Dobhoff tube or total parenteral nutrition. All patients are kept NPO until they can swallow their saliva without pain, after which the diet is advanced as tolerated. Esophagogastroduodenoscopy is performed at 3 weeks for full evaluation. The esophagus and stomach are also evaluated with a barium swallow performed at 3 weeks, 3 months, and 6 months to rule out stricture formation, gastric outlet obstruction, or the development of either an hourglass or linitis plastica-like appearance (33, 34).

If the physical exam, chest, or abdominal X-rays indicate a perforation has occurred, abdominal exploration is mandatory with resection of all injured organs to prevent extension of the injury (29). Caustic esophageal perforations are best treated by esophagectomy, with the trans-hiatal route preferred (35). Extensive full-thickness esophageal and gastric necrosis is treated with urgent radical total esophagogastrectomy with delayed (6 months) reconstruction. All patients receive a cervical esophagostomy and feeding jejunostomy.

The most frequent complication of second- or third-degree esophageal burns is stricture formation, which usually develops between 3 and 8 weeks after initial injury (31). Some lesions are mild and respond to dilatation without recurrence. Bougienage for early treatment of caustic esophageal burns is performed daily for several weeks, then every other day for 2-3 weeks, and finally once a week for months. Dilatations should not be initiated until esophageal reepithelialization is complete, generally not sooner than 6 weeks after injury. For localized strictures extending beyond 1.5 cm and failing to respond to bougienage alone, local injection of corticosteroids, visualized through an esophagoscope, followed by bougienage may be beneficial (36).

Other complications include tracheoesophageal fistula, hiatal hernia, reflux, and esophageal carcinoma. Diagnosis of a tracheoesophageal fistula is suggested by progressive pneumonia, choking, coughing with feedings, or bile-stained mucus in the airway with confirmation by a contrast study using thin barium or propyliodone (Dionosil). Hiatal hernia and reflux can develop years after injury, causing late esophagitis and peptic stricture. An acquired form of achalasia has also been reported as a consequence of extensive intramural fibrosis. The incidence of esophageal carcinoma in patients with caustic injury is 1,000-fold greater than in the general population; therefore, any change in symptoms warrants immediate radiographic and endoscopic examination.

In summary, management of caustic ingestion requires radiologic and endoscopic evaluation to assess the injury. First-degree burns are observed, whereas second- and third-degree burns without perforation are managed by supportive care, with or without stent placement or gastrostomy, and surveillance for late strictures. Caustic perforation requires immediate esophageal and/or gastric resection.

	Footnotes

Correspondence and requests for reprints should be addressed to Joseph B. Zwischenberger, M.D., Director, General Thoracic Surgery, The University of Texas Medical Branch, Division of Cardiothoracic Surgery, 301 University Boulevard, Galveston, TX 77555-0528. E-mail: jzwische{at}utmb.edu

(Received in original form April 25, 2001 and accepted in revised form January 23, 2002).

Acknowledgments: Supported in part by the Leroy Hillyer, M.D., endowed chair in Surgery.

	References

TOP PERFORATION OF THE ESOPHAGUS CAUSTIC INJURY REFERENCES

1. Jones WG II,, Ginsberg RJ. Esophageal perforation: a continuing challenge. Ann Thorac Surg 1992; 53: 534-543 [Abstract].

2. Altorjay A, Kiss J, Voros A, Sziranyi E. The role of esophagectomy in the management of esophageal perforations. Ann Thorac Surg 1998; 65: 1433-1436 [Abstract/Free Full Text].

3. Bufkin BL, Miller JI Jr,, Mansour KA. Esophageal perforation: emphasis on management. Ann Thorac Surg 1996; 61: 1447-1451 [Abstract/Free Full Text].

4. Iannettoni MD, Vlessis AA, Whyte RI, Orringer MB. Functional outcome after surgical treatment of esophageal perforation. Ann Thorac Surg 1997; 64: 1606-1609 [Abstract/Free Full Text].

5. English GM, Hsu SF, Edgar R, Gibson-Eccles M. Oesophageal trauma in patients with spinal cord injury. Paraplegia 1992; 30: 903-912 [Medline].

6. Pass LJ, LeNarz LA, Schreiber JT, Estrera AS. Management of esophageal gunshot wounds. Ann Thorac Surg 1987; 44: 253-256 [Abstract].

7. Gouge TH, Depan HJ, Spencer FC. Experience with the Grillo pleural wrap procedure in 18 patients with perforation of the thoracic esophagus. Ann Surg 1989; 209: 612-617 [Medline].

8. Sarr MG, Pemberton JH, Payne WS. Management of instrumental perforations of the esophagus. J Thorac Cardiovasc Surg 1982; 84: 211-218 [Abstract].

9. White RK, Morris DM. Diagnosis and management of esophageal perforations. Am Surg 1992; 58: 112-119 [Medline].

10. Dolgin SR, Wykoff TW, Kumar NR, Maniglia AJ. Conservative medical management of traumatic pharyngoesophageal perforations. Ann Otol Rhinol Laryngol 1992; 101: 209-215 [Medline].

11. Jagot P, Sauvanet A, Berthoux L, Belghiti J. Laparoscopic mobilization of the stomach for oesophageal replacement. Br J Surg 1996; 83: 540-542 [Medline].

12. Altorjay A, Kiss J, Voros A, Bohak A. Nonoperative management of esophageal perforations: is it justified? Ann Surg 1997; 225: 415-421 [Medline].

13. Cameron JL, Kieffer RF, Hendrix TR, Mehigan DG, Baker RR. Selective nonoperative management of contained intrathoracic esophageal disruptions. Ann Thorac Surg 1979; 27: 404-408 [Abstract].

14. Orringer MB, Stirling MC. Esophagectomy for esophageal disruption. Ann Thorac Surg 1990; 49: 35-42 [Abstract].

15. Whyte RI, Iannettoni MD, Orringer MB. Intrathoracic esophageal perforation: the merit of primary repair. J Thorac Cardiovasc Surg 1995; 109: 140-144 [Abstract/Free Full Text].

16. Richardson JD, Martin LF, Borzotta AP, Polk HC Jr.. Unifying concepts in treatment of esophageal leaks. Am J Surg 1985; 149: 157-162 [Medline].

17. Wright CD, Mathisen DJ, Wain JC, Moncure AC, Hilgenberg AD, Grillo HC. Reinforced primary repair of thoracic esophageal perforation. Ann Thorac Surg 1995; 60: 245-248 [Abstract/Free Full Text].

18. Shields TW. Esophageal trauma. In: Shields TW, LoCicero J III, Ponn RB, editors. General thoracic surgery, 5th ed. Philadelphia: Lippincott Williams & Wilkins; 2000. p. 1769-1782.

19. Morgan RA, Ellul JP, Denton ER, Glynos M, Mason RC, Adam A. Malignant esophageal fistulas and perforations: management with plastic-covered metallic endoprostheses. Radiology 1997; 204: 527-532 [Abstract].

20. Davies AP, Vaughan R. Expanding mesh stent in the emergency treatment of Boerhaave's syndrome. Ann Thorac Surg 1999; 67: 1482-1483 [Abstract/Free Full Text].

21. DiPalma JA. Esophageal disorders. In: Civetta JM, Taylor RW, Kirby RR, editors. Critical care, 3rd ed. Philadelphia: Lippincott-Raven; 1997. p. 2071-2077.

22. Kirsh MM, Ritter F. Caustic ingestion and subsequent damage to the oropharyngeal and digestive passages. Ann Thorac Surg 1976; 21: 74-82 [Abstract].

23. Greenberg RE, Bank S, Blumstein M, Chow K, Stark B. Common gastrointestinal disorders in the intensive care unit. In: Bone RC, editor. Pulmonary and critical care medicine, 2nd ed. Chicago: Mosby; 1993. p. 1-27.

24. Spitz L, Lakhoo K. Caustic ingestion. Arch Dis Child 1993; 68: 157-158 [Medline].

25. Estrera A, Taylor W, Mills LJ, Platt MR. Corrosive burns of the esophagus and stomach: a recommendation for an aggressive surgical approach. Ann Thorac Surg 1986; 41: 276-283 [Abstract].

26. Symbas PN, Vlasis SE, Hatcher CR Jr.. Esophagitis secondary to ingestion of caustic material. Ann Thorac Surg 1983; 36: 73-77 [Abstract].

27. Lamireau T, Rebouissoux L, Denis D, Lancelin F, Vergnes P, Fayon M. Accidental caustic ingestion in children: is endoscopy always mandatory? J Pediatr Gastroenterol Nutr 2001; 33: 81-84 [Medline].

28. Anderson KD. Corrosive injury. In: Pearson FG, Deslauriers J, Ginsberg RJ, Hiebert CA, McKneally MF, Urschel HC Jr, editors. Esophageal surgery. New York: Churchill Livingstone Inc.; 1995. p. 465-478.

29. Cattan P, Munoz-Bongrand N, Berney T, Halimi B, Sarfati E, Celerier M. Extensive abdominal surgery after caustic ingestion. Ann Surg 2000; 231: 519-523 [Medline].

30. Millar AJ, Numanoglu A, Mann M, Marven S, Rode H. Detection of caustic oesophageal injury with technetium 99m-labelled sucralfate. J Pediatr Surg 2001; 36: 262-265 [Medline].

31. Anderson KD, Rouse TM, Randolph JG. A controlled trial of corticosteroids in children with corrosive injury of the esophagus. N Engl J Med 1990; 323: 637-640 [Abstract].

32. Gunnarsson M. Local corticosteroid treatment of caustic injuries of the esophagus: a preliminary report. Ann Otol Rhinol Laryngol 1999; 108: 1088-1090 [Medline].

33. Goldman LP, Weigert JM. Corrosive substance ingestion: a review. Am J Gastroenterol 1984; 79: 85-90 [Medline].

34. Kikendall JW. Caustic ingestion injuries. Gastroenterol Clin N Am 1991; 20: 847-857 [Medline].

35. Hwang TL, Shen-Chen SM, Chen MF. Nonthoracotomy esophagectomy for corrosive esophagitis with gastric perforation. Surg Gynecol Obstet 1987; 164: 537-540 [Medline].

36. Gandhi RP, Cooper A, Barlow BA. Successful management of esophageal strictures without resection or replacement. J Pediatr Surg 1989; 24: 745-749 [Medline].

37. Zwischenberger JB, Alpard SK, Orringer MB. Esophagus. In: Townsend CM Jr, editor. Sabiston textbook of surgery, 16th ed. Philadelphia: WB Saunders; 2001. p. 709-753.