Ann Thorac Surg 2001;72:592-593
© 2001 The Society of Thoracic Surgeons
Case report
Esophageal perforation: a rare complication of Zollinger-Ellison syndrome
Thomas Ng, MDa,
Donna E. Maziak, MDCMa,
Farid M. Shamji, MDa
a Division of Thoracic Surgery, University of Ottawa, Ottawa, Ontario, Canada
Accepted for publication July 19, 2000.
Address reprint requests to Dr Maziak, Division of Thoracic Surgery, Ottawa Hospital, Civic Campus, 1053 Carling Ave, CPC Room 162, Ottawa, Ontario, Canada, K1Y 4E9
e-mail: dmaziak{at}civich.ottawa.on.ca
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Abstract
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Spontaneous perforation of the esophagus is a rare manifestation of Zollinger-Ellison syndrome (ZES). Failure to recognize its existence can lead to an unsuccessful treatment of the esophageal perforation. We present a rare case of reflux esophagitis–induced esophageal perforation in a patient with ZES. Presence of a gastrinoma should be considered when recurrent or complicated reflux esophagitis is encountered.
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Introduction
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In the largest reported series of Zollinger-Ellison syndrome (ZES), esophageal complications are rarely cited [1]. We report a case of hyperacidic reflux causing spontaneous esophageal perforation in a patient with a gastrinoma. Early recognition of ZES is essential in preventing complications of inappropriate or inadequate therapy for the esophageal perforation.
A 59-year-old woman was referred to the thoracic surgery service for urgent treatment of disabling dysphagia. Esophageal cancer was suspected. Her symptoms began 4 years earlier with diarrhea, duodenitis, and severe esophagitis seen on endoscopy, and she was placed on proton pump inhibitor therapy. On this admission, endoscopic examination of the esophagus revealed a tight stricture beginning at 28 cm. Only the upper end of the stricture could be examined where the mucosa was found to be inflamed and sloughing. No biopsy was taken and no attempt was made to advance past or dilate the stricture. The stomach and duodenum could not be examined therefore a barium contrast study was performed revealing a long tight stricture in the lower 10 cm of the esophagus (Fig 1) and a normal-appearing stomach and duodenum.
Twenty-four hours later, severe right-side chest pain suddenly developed and a chest radiograph revealed a right-side tension hydropneumothorax. There was no history of vomiting. Free perforation of the lower esophagus into the right chest was confirmed by a water soluble contrast study. Within 2 hours of onset of the acute illness, an emergent right thoracotomy with subtotal esophagectomy was performed and in the absence of mediastinal or pleural sepsis, reconstruction with gastric conduit and cervical esophagogastric anastomosis was undertaken. Both the stomach and duodenum appeared healthy at laparotomy. Pathology examination revealed a gastric lesser curvature with normal mucosa and a perforated esophageal ulcer just above the gastroesophageal junction. The esophageal mucosa was completely denuded and replaced by necrotic fibrinopurulent material consistent with severe damage from reflux disease.
In the postoperative period, the only abnormal finding consistently documented was clear nasogastric drainage in excess of 2 L per day. On the seventh postoperative day, bile was noted to drain from the right chest tube. A water soluble contrast study through the nasogastric tube showed extravasation in the mid portion of the gastric conduit and an intact esophagogastric anastomosis. An urgent right thoracotomy was performed with the finding of two gastric perforations. The patient underwent subtotal gastrectomy and cervical esophagostomy. Pathology revealed two perforated ulcers in the gastric body 3.5 cm apart in an otherwise healthy stomach with normal mucosa and no ischemia. At this point ZES was suspected. A fasting serum gastrin level of more than 2,000 pg/mL confirmed the diagnosis (normal < 150 pg/mL). Computerized tomography and arteriography (Fig 2) localized the gastrinoma to the uncinate process of the pancreas.
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Fig 2. Celiac arteriography showing the contrast blush of a hypervascular tumor supplied by the gastroduodenal artery (arrow).
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The patient returned to the operating room 6 weeks later to undergo a successful local resection of the gastrinoma, completion gastrectomy, and esophageal reconstruction with substernal isoperistaltic left colon interposition into a Roux-en-Y loop of jejunum. Pathology revealed an increased prominence of parietal cells within the body-type mucosa of the gastric remnant and a neuroendocrine tumor of the pancreas consistent with gastrinoma. The patient has remained well after 5 years of follow-up and serum gastrin remains low at 40 pg/mL.
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Comment
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In 1964, Ellison and Wilson [1] reported on 260 cases of ZES. Common presentations of this syndrome of acid hypersecretion included recurrent or refractory gastrointestinal ulcer despite maximal medical and surgical therapy, severe complications of ulcer disease, multiple ulcers in atypical locations, and diarrhea. Esophageal involvement was seen in only 2 of 260 cases; however, the details were not elaborated upon. Esophageal complications in ZES were thought to be rare.
Recent studies have noted a high incidence of esophageal involvement in ZES [2, 3]. With close surveillance using endoscopy, biopsy, and pH monitoring, reflux esophagitis was found to be as high as 40% of patients with ZES [2, 3]. However, complicated reflux disease in ZES remains less common. There have been several reports of stricture and Barrett’s epithelium in ZES [2, 3], and one report of adenocarcinoma arising from Barrett’s epithelium [4] but none of perforation until now. Perforation of the esophagus in patients with ZES has been reported in two cases but they were secondary to emesis [5] and paraesophageal hernia [6] and not as a direct result of acid reflux as in our case.
In our patient, free perforation occurred in a damaged esophagus, denuded of its mucosa by unrecognized longstanding acid hypersecretion. After the first operation, persistent hyperacidity, despite truncal vagotomy and resection of the lesser curvature of the stomach, was responsible for the subsequent acute ulceration and perforation of the interposed gastric conduit.
Once the syndrome is recognized, the treatment of choice for ZES is surgery with excision of the gastrinoma. Medical therapy with a proton pump inhibitor and somatostatin is reserved for unresectable disease, metastatic disease, multiple gastrinomas, and medically inoperable patients. As with gastric or duodenal ulcer disease, when refractory or complicated esophageal reflux disease occurs, ZES should be considered.
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References
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Ellison E.H., Wilson D.S. The Zollinger-Ellison syndrome: reappraisal and evaluation of 260 registered cases. Ann Surg 1964;160:512-530.[Medline]
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Miller L.S., Vinayek R., Frucht H., Gardner J.D., Jensen R.T., Maton P.N. Reflux esophagitis in patients with Zollinger-Ellison syndrome. Gastroenterology 1990;98:341-346.[Medline]
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Strader D.B., Benjamin S.B., Orbuch M., et al. Esophageal function and occurrence of Barrett’s esophagus in Zollinger-Ellison syndrome. Digestion 1995;56:347-356.[Medline]
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Symonds D.A., Ramsey H.E. Adenocarcinoma arising in Barrett’s esophagus with Zollinger-Ellison syndrome. Am J Clin Pathol 1980;73:823-826.[Medline]
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Goldstein L.A., Thompson W.R. Esophageal perforations: a 15 year experience. Am J Surg 1982;143:495-502.[Medline]
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Bondeson A.G., Bondeson L., Thompson N.W. Stricture and perforation of the esophagus: overlooked threats in the Zollinger-Ellison syndrome. World J Surg 1990;14:361-363.[Medline]
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Abstract
Introduction
