General considerations The Pharyngo-Esophageal perforation is a diagnostic and therapeutic challenge because of the rarity of the condition and the variability in presentation. The following basic principles are applied to the management of a patient with a pharyngo-esophageal perforation: 1. Rapid diagnosis 2. Appropriate hemodynamic monitoring and support 3. Antibiotic therapy 4. Restoration of luminal integrity when feasible 5. Control of extraluminal contamination Anatomic considerations The esophagus has three anatomical points of narrowing, the cricopharyngeus muscle, the broncho-aortic constriction, and the esophagogastric junction. Perforation may occur anywhere along the esophagus, but there is a predilection for rupture at these key anatomic areas. As an example, iatrogenic injuries to the cervical esophagus can occur during endoscopy at Killian’s triangle, an area lacking a posterior esophageal muscularis and bordered by the horizontal cricopharyngeus muscle inferiorly and the oblique inferior constrictor muscles superiorly. Etiology More than one half of all esophageal perforations are iatrogenic and most of these occur during endoscopy. Other causes of esophageal perforation include: • Spontaneous perforation (Boerhaave’s Syndrome): 15 percent • Foreign body ingestion: 12 percent • Trauma: 9 percent • Intra-operative injury: 2 percent • Malignancy: 1 percent Management 1. Prompt diagnosis and management is critical to minimizing mortality. A delay of greater than 24 hours in diagnosis and treatment of an esophageal perforation is associated with a higher mortality rate compared with an early diagnosis and treatment initiation (27 versus 14 percent). 2. The mortality rate following operative management of an esophageal perforation is dependent on location of the perforation, with cervical perforations having the lowest mortality rate (6 percent) compared with thoracic perforations (27 to 34 percent), and intra-abdominal perforations (21 to 29 percent). 3. A primary repair is the gold standard of care and should be utilized for perforations of the thoracic and abdominal esophagus, as well as for visualized perforations of the cervical esophagus. 4. Drainage alone should only be performed for perforation of the cervical esophagus when the perforation cannot be visualized and when there is no distal obstruction. 5. Diversion is reserved for patients who present with clinical instability and more extensive operative procedure is not possible, or when extensive esophageal damage precludes a primary repair. 6. Patients who show evidence of clinical deterioration (eg, fever, tachycardia) require surgical intervention to control extraluminal contamination and to restore luminal integrity. 7. Non-operative management should be reserved for clinically stable patients with no evidence of systemic inflammation, expediently diagnosed perforations, and no drainage of any collection into the pleura or peritoneum. 8. Essential to non-operative management is careful patient selection; appropriate patient selection can achieve 100 percent survival rates. This requires clinicians experienced in the care of esophageal pathology, careful patient monitoring, and the early involvement of the appropriate surgical team. 9. Cervical perforation is most commonly considered for non-operative management due to the anatomic confinement of the esophagus by surrounding surgical structures. Perforation into the pleural or peritoneal cavity is a relative contraindication to non-operative management due to the difficulties of controlling spillage of contaminated contents in large, free spaces. 10. Patients are maintained on intravenous fluids, nothing per oral (NPO), and broad-spectrum antibiotics for five to seven days. As long as patients remain clinically stable, contrast esophagography is performed at five to seven days and resumption of oral intake under observation is considered depending on the results.