Esophageal Perforation
Allen Lue, M.D.
May 31, 2001

Esophageal perforation is a true emergency. Prompt diagnosis, in less than 24 hours, is vital to good outcomes. There is a mortality of 10% with early diagnosis and that mortality goes up to 50% with late diagnosis. Surgical treatment is the mainstay of therapy traditionally, but there has been a trend toward more conservative medical management. I will be talking to you today about anatomy and the etiology of esophageal perforation, and in particular, I will be concentrating on rigid endoscopy. I will be talking about how these patients present, diagnosis of perforations, and management.

Going over anatomy; the esophagus is broken up into three parts. The first part is the cervical esophagus, the first five centimeters of the esophagus. It is the only portion of the esophagus that is fixed, between the trachea and vertebra, going from about C6 to the sternal notch. The next section is the thoracic esophagus, about 20 cm long. The aorta lies to the left and causes an indentation that you can often see on an esophagram. The abdominal portion of the esophagus is the last part, starting from the diaphragmatic hiatus and it is about 40 cm from the incisors to the cardia of the stomach. The esophagus is a very thin-walled organ, measuring about 2 mm wide. There is an outer longitudinal muscular layer, and notably there is no serosa surrounding this layer. There is an inner circular muscular layer, as well as a submucosal layer that contains a neuroplexus. There is also the mucosal layer, which is the strongest layer, and great care should be taken with biopsies that would violate this area. You should take into account processes that might erode or weaken this layer, such as gastroesophageal reflux disease. The esophagus has three areas of narrowing: the first area is the cricopharyngeus, which is the narrowest region, about 1.5cm. The next area of narrowing of the esophagus is where the left main stem bronchus and the aorta cross it, and then the third is the gastroesophageal sphincter. This is important because the esophagus is prone for perforation at these areas of narrowing.

There are four main causes of esophageal perforation: the first is instrumentation and that is the most common cause. Spontaneous rupture is another common cause, also known as Boerhaave syndrome, usually seen after vomiting. Foreign bodies can also cause esophageal perforation, as well as trauma. In a series of 52 patients out of LSU reported in 1992, 52% of the perforations that presented to that hospital were iatrogenic in nature and usually have to deal with a dilation. External trauma was also a major cause, usually penetrating injuries. There were 15% spontaneous or barogenic injuries and 10% of them had ingested foreign bodies. We will be concentrating mostly on instrumentation. There were 33 cases of iatrogenic perforation reported by Flynn in 1989. The thing to notice here is that most of these cases had to do with a therapeutic procedure.

In terms of the site of perforation, this is a series of 108 patients and 47 of those patients had perforation in the cervical esophagus. Those are usually due to problems passing the scope. Seventeen of them were in the upper thoracic esophagus and 44% were in the lower thoracic esophagus. Again, they are usually due to underlying pathology. We will hone down even further, just on rigid esophagoscopy. There is a less than 1% instance of perforation reported in most series. Again, most occur in the presence of underlying esophageal lesions or therapeutic maneuvers. The examples are stricture, tumor, and, of course, caustic congestion. We will digress a little in the history. Sword-swallowers were really the first people to perfect the technique of esophagoscopy and it was first mentioned in 300 B.C. in Greece. There are about 50 sword-swallowers left in the world, and there have actually been multiple perforations reported from these performers, with one reported fatality. There was actually a report that came out of the Texas Heart Institute this year of a circus performer who developed a perforation after a performance. He ended up requiring thoracotomy and although he still works for the circus, he is not swallowing swords any more. Kussmaul was the first physician to perform an esophagoscopy. He actually enlisted the help of the some of his contemporary sword-swallowers to try to look into the esophagus and used a straight tube with lighting, which was designed by a Frenchman named Desmormeaux. Wallenburg later used an elastic tube with a laryngeal mirror. McKenzie developed a skeleton esophageal tube, which you see here, and with this tube he was really only able to see into the cervical esophagus. Neitsliter then developed a rigid straight tube, which was lit with a heated platinum wire distally. The esophagoscope, as we know, was really designed by Chevalier Jackson, after Edison invented the light bulb, which permitted him to project light distally instead of the having the light source in the proximal region. He used an auxiliary light tube and developed right-angle instruments, which he made in his own workshop. He generally used this scope for foreign bodies and dilation of lye ingestions, particularly in children.

Rigid esophagoscopy is still widely used as a screening exam in head and neck cancer. There is an approximately 1.8 to 11.8 incidence of secondary primary esophageal lesions in patients with head and neck cancer. The 11% figure actually comes from Japan. Japanese studies have shown that early detection does improve five-year survival. Alternatively, you can use a barium swallow to look for a second primary esophageal lesion, but this is less sensitive. In one series, 245 patients received both esophagoscopy and barium swallow. The barium swallow missed three out of four lesions. Another study from Japan showed that the barium swallow missed 9 out of 13 esophageal lesions. Other reports have shown that using Lugol’s iodine dye during esophagoscopy can improve sensitivity. In fact, the esophageal lesions do not take up the dye. There has been some argument made for the use of the flexible endoscopy as opposed to rigid endoscopy as a screening tool. The reasons cited are that there is a lower perforation rate and better visualization with insufflation of air into the esophagus and the flexible tip. Glaws actually reported that there was a 5% discordance in 195 patients who received both rigid and flexible endoscopy within a six-month period. They showed that rigid endoscopy missed five esophageal cancers and they were usually early distal lesions. The advantage of rigid endoscopy however, is that it better visualizes the cervical esophagus and it is certainly more efficient for foreign body retrieval.

Other than rigid endoscopy, there are other causes of esophageal perforation due to instrumentation. There have been reports of endotracheal and nasogastric intubation causing perforation, especially in neonates. Transesophageal echo is associated with 0.1% to 0.3% perforation rate. Interestingly, there has also been perforation reported after stapling of pharyngeal pouch. A paper published in 2000 describes such a perforation. After they applied the stapler, they could see the prevertebral soft tissue. They theorize that it was because this cricopharyngeal bar was too thick for the staples to hold. In retrospect, they were able to see that there was a thick cricopharyngeal bar on the barium swallow, so they wanted to give everyone a word of caution.

Boerhaave syndrome is a well known cause. There is generally a history of resisting vomiting, but it has also been reported after weight lifting, coughing and childbirth. It often presents silently. It is five times more common in men. There can be a delay in diagnosis because it could be mistaken for pancreatitis; as it presents the same way, with pain, pleural effusion, and an elevated amylase. It has been shown that this delay in diagnosis can lead to poor survival. Boerhaave syndrome usually occurs on the left side in the left pleural cavity right above the GE junction. It has also been found in association with gastroesophageal reflux disease, which compromises the mucosa, which is the strongest layer of the esophagus. As it turns out, vomiting can often exceed 200 mmHg of intragastric pressure, and rupture of the esophagus can occur at 150 mm. Foreign bodies have also been found to cause esophageal perforation. They can puncture the wall directly or they can cause pressure necrosis, which later leads to perforation.

There was an interesting French series of 12 patients who developed perforation after foreign bodies. This series noted that there were psychosocial issues involved in 3 out of those 12 patients. They generally found bones - in 10 out of 12 cases: chicken, pigeon, rabbit, veal, pork and fish. Dentures were found in 2 out of those 12 cases. Other notable foreign bodies would include button batteries, which require urgent retrieval because of their alkaline contents. There have been a couple of cases of bezoars, which were treated with papain, and that enzyme actually degraded the esophageal wall and led to perforation. The other thing to pay attention to when you have possible esophageal foreign body is to look for a sentinel GI bleed, which may lead you to suspect an esophago-aortic fistula, which has been reported in the past.

Trauma is another major cause of esophageal perforation. In terms of penetrating trauma, it is usually a gunshot wound and sometimes a stab wound. There should be a raised index of suspicion from the bullet tract. In these patients however, generally their morbidity is more from associated tracheal and spinal cord injuries. There have been multiple reports of tracheoesophageal fistula from these injuries. Blunt trauma is also a major cause of esophageal perforation. The deceleration causes tears against the vertebral bodies. There have been approximately 100 reported cases, usually motor vehicle accidents, and 82% of these occur in the cervical and upper thoracic esophagus, perhaps because that is just distal to where the esophagus is fixed. Generally there is a delay in diagnosis, about a 66% instance in delay. In terms of diagnosing an esophageal perforation, a high index of suspicion is mandatory since it is vital to improve your results. There are certain symptoms and signs that you should watch out for, in particular, chest pain aggravated by deep breathing or swallowing, upper abdominal rigidity, dyspnea, fever and, of course, subcutaneous emphysema would be a very obvious sign.

The first test that you generally would do would be a chest x-ray. However, the chest radiograph has variable abnormalities depending upon several factors: the time interval between the injury and the chest x-ray, the site of perforation, and the integrity of the mediastinal pleura. Mediastinal emphysema can be seen about 40% of the time and it takes an hour to be demonstrated. You can actually make it out here, from the arrows you can see an air space between the mediastinal pleura and the mediastinal tissue, also seen here. Mediastinal widening is due to soft tissue edema and that usually takes several hours to occur after the injury. Sometimes you can also see air overlying the spinal muscles, not on this picture, but for a cervical perforation, you can often see air in the neck. In this patient you can also see subcutaneous emphysema. You can see a pneumothorax about 77% of the time and that usually occurs when there is violation of the mediastinal pleura; 70% of the pneumothoraces are on the left, 20% on the right and 10% bilaterally. Pleural effusion, which you can see here, is a late finding. Salivary amylase digests the tissue and causes early infection. There is, however, notably a normal chest film in 9% of patients, so any patient in whom you suspect an esophageal perforation should get a contrast esophagogram. The first thing you should use is gastrograffin, which is a water soluble contrast. There is still a 10% false negative rate, however, and that rate is even larger when you look at cervical perforation,s in which it can detect about 60%. It can detect about 90% of distal perforations. A negative scan does not exclude the diagnosis, especially, as I said, in the cervical esophagus because of the rapid transit of the thin contrast.

A negative gastrograffin study and the high suspicion should still lead you to get a thin barium contrast esophagram. Barium has a greater density and it is able to coat the mucosa better and it can detect smaller leaks as well. CT has also been reported as being useful for detection of esophageal perforations. There is a sensitivity of 85% for perforation of the alimentary tract in general. Some of the findings that you see in CT scans of patients with esophageal perforation are mediastinal air, which you can see here; here is the trachea, here is the esophagus and here are air pockets in the mediastinum. You can also detect paraesophageal abscesses and sometimes you can actually see the fistula itself with the CT scan.

So, in terms of management, early recognition is key, as I have already said. You should institute treatment after recognition as early as possible and that should include nothing by mouth, total parenteral nutrition, broad spectrum antibiotics and surgical closure versus medical management. This is a controversial issue: there have not been any trials that compare operative versus non-operative management. The literature includes small, very heterogenous patient population, so there really is not any clear-cut best treatment. In terms of surgical treatment, staged closure has always been the traditional way to go, which includes primary repair with esophageal exclusion and cervical esophagostomy. The advantage of this is that you perform the esophageal anastomosis in a non-infected bed; in a bed where there is not saliva constantly running through it. One study noted that the earlier you close this cervical esophagostomy, the better patients do.

There has also been a lot of literature that looks at a single-stage closure without cervical esophagostomy. They talked about an early, reinforced primary repair with an intercostal muscle graft. There have been other tissues that have been advocated for reinforcement of the primary repair, including pericardium, pleura and gastric fundus. There have been a few small series which have shown success. Another alternative is minimally invasive treatment. Thoracoscopic closures of the esophageal perforations have been performed. The thoracoscope also allows debridement of the pleural cavity and mediastinum. It can be combined with esophageal exclusion to provide an extra layer of safety.

There are patients who are candidates for medical management, that is, non-surgical management. These are patients who are caught early, and patients who have cervical perforations; patients with minimal pain easily controlled by narcotics and absence of shock or sepsis, mild to moderate fever or leukocytosis and a confined cavity draining well into the esophagus. Those are patients who might benefit from watchful waiting. There have been a few series to support this.

Dolgin, in our literature, wrote about medical management of ten pharyngoesophageal perforations and he stressed that prompt diagnosis was vital, as well as close monitoring for abscess formation. He started broad spectrum antibiotics and made patients NPO. He reported no complications or the need for surgery. Westdorp reported on 54 instrumental perforations treated conservatively and he actually reported a mortality of 0% in patients without malignancy. In patients with malignancy, he reported a mortality of only 8.6%. After the preliminary treatment is completed, these patients need to be followed very closely. They are made NPO and given weekly contrast studies until the perforation is shown to be healed. This is a gastrograffin study of our patient in the case study approximately three to four weeks later, which shows healing of the perforation. He was going to start on clear liquids and then eventually ended up with repeat perforation. That is why you should really give patients full liquids for a prolonged period to make sure that the closure is tight. You should perform a repeat contrast study and CT for any suggestion that their condition is deteriorating.

One last word of prevention of an iatrogenic perforation—proper, careful endoscopic technique is essential. One should practice careful head control, direct visualization, and diagnosis of diverticula, vertebral osteophyte, strictures or tumors. You should also recognize any history of gastroesophageal reflux disease in these patients that might weaken the mucosal wall. If you do perform dilation you should use a small caliber scope very carefully.

So, in summary, esophageal perforation is a true emergency with approximately 10% mortality rate. You should always maintain a very high index of suspicion. Prompt recognition of the perforation is essential and thoracic surgery consultation should also be performed promptly. Surgical closure with exclusion is the tradition therapy, but medical management can be instituted in select cases.

Case presentation

The patient is a 66-year-old white male with a history of diabetes mellitus and GERD who presents with hoarseness. He underwent a TVC stripping for dysplasia in 1998.

On physical exam, he is an obese male in no apparent distress. Ears: clear. Nose: clear. OC/OP: edentulous without lesions. Larynx: TVCs mobile bilaterally with a raised, irregular lesion on the L anterior TVC. Neck: no lymphadenopathy.

The patient was taken to the operating room for MSDL with biopsy and rigid esophagoscopy. At esophagoscopy, a polypoid lesion was noted at 30 cm, which was biopsied. The patient complained of epigastric pain post-operatively. A CXR was performed which revealed no evidence of pneumothorax, pleural effusion, or mediastinal air. His symptoms improved after overnight observation, and he remained afebrile. He was advised to return to the hospital if his pain recurred or if he developed a fever.

He presented to the ER on POD #1 with chest pain, and was given oral antibiotics for presumptive pneumonia by the urgent care clinic. On POD #6, he presented to the Otolaryngology clinic with chest pain and shortness of breath. A CXR revealed a left pleural effusion, and a gastrografin swallow showed a distal L esophageal leak. The cardiothoracic service was immediately consulted and an urgent L thoracotomy was performed with pleural debridement and primary esophageal repair using an intercostal muscle flap. Pathologic examination of his L anterior cord lesion revealed SCCA. The esophageal lesion was read as mucosa with adipose tissue.

Three months post-operatively, the patient is G-tube dependent after a trial of oral feedings and recurrence of the leak. He is completing XRT for his laryngeal lesion.

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