Ann Thorac Surg 2004;77:1098-1100
© 2004 The Society of Thoracic Surgeons
Case report
Superimposed spontaneous esophageal perforation in congenital esophageal stenosis
J. Andres Gonzalez, MDa,
Christopher M. Craft, MDa,
T. Theron Knight, MDa,
William H. Messerschmidt, MD*a
a Department of Surgery, Quillen College of Medicine, Johnson City, Tennessee, USA
Accepted for publication May 14, 2003.
* Address reprint requests to Dr Messerschmidt, Department of Surgery, ETSU, Quillen College of Medicine, Johnson City, TN 37614-0575, USA
e-mail: billm1{at}chartertn.net
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Abstract
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Congenital esophageal stenosis (CES) is a very rare embryologic anomaly of tracheoesophageal development that requires lifelong management, usually with frequent esophageal dilations and dietary precautions. We present a patient with spontaneous thoracic esopahgeal perforation in a setting of CES. The patient, a 27-year-old male, recovered uneventfully following open primary esophageal closure. A brief but comprehensive discussion of CES follows the case report.
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Introduction
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Congenital esophageal stenosis (CES) is an often unrecognized cause of childhood and adult dysphagia. Only 500 patients have been reported [1]. Its frequency is thought to be 1 patient in 25,000 to 50,000 live births [2, 3]. Three forms of the anomaly are described: membranous diaphragm, submucosal and muscularis propia fibrosis, and esophageal wall tracheobronchial remnants [4]. Frank T-E fistula may be present in newborns. Symptoms are multiple ringed esophagus and multiple webbed esophagus, accouunting for its description on imaging studies and endoscopy [2]. CES may involve any portion of the thoracic esophagus, but involves the middle third in 80% of the patients. CES exhibits male predominance and typically presents in childhood with progressive dysphagia [2]. The stenotic segment usually contains tracheobronchial elements or maldeveloped muscular tissue, and may be associated with esophageal atresia or tracheoesophageal fistula. Intrauterine stress or anoxia predisposes esophageal lumen canalization defects and stenosis [4]. Most patients can be managed with intermittent dilations and dietary precautions, however surgical intervention is occasionally necessary [1]. Esophageal perforation is a potentially lethal complication of CES with treatment based upon clinical findings. Delayed diagnosis substantially worsens the prognosis [5].
A 27-year-old male, known to have CES, presented with 18 hours of unrelenting, severe substernal pain radiating into the back (Fig 1). The onset of this pain followed an unremarkable meal. He denied other symptoms including retching, emesis, or abdominal pain. Additional history revealed that he had been well since his last esophageal dilatation 6 months previously. Two years earlier he sustained an esophageal perforation during a dilatation procedure. This complication was managed nonoperatively with an uneventful recovery. Physical exam revealed a well nourished man with a low grade fever and no other abnormalities. Laboratory studies were normal except for a white blood cell count of 14,000. Initial electrocardiogram and chest roentgenogram were also unremarkable. A gastrograffin esophagogram revealed no contrast extravasation but did reveal chronic changes of CES without significant stenosis. A computed tomographic (CT) scan of the chest with oral contrast revealed a tear in the mid-esophagus with air and contrast in the adjacent mediastinum (Fig 2).
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Fig 1. Patient esophagogram. Arrows indicate characteristic congenital esophageal stenotic ringed esophagus.
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Fig 2. Computed tomographic scan of the patient. Arrows indicate free air and contrast extravasation in the mediastinum, demonstrating esophageal perforation.
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Because of previous perforation, leukocytosis, and persistent severe chest pain, the patient was taken to the operating room for exploration and repair. A right posterolateral muscle-sparing thoracotomy was performed. There was no pleural contamination, but a 7-mm tear was identified posteriorly in the right midesophagus. The edges were debrided and primary repair was performed using absorbable suture. The parietal pleura was then closed over the repair, and a thoracostomy tube was placed. Ceftazidime and parenteral nutrition were administered for 10 days. The postoperative chest roentgenogram was unremakable. A barium swallow performed on postoperative day 7 demonstrated no esophageal leak or stenosis. An oral diet was started and advanced gradually. The thoracostomy tube was removed 24 hours thereafter. The remainder of the patient's course was unremarkable, and there has been no dysphagia in the follow-up period.
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Comment
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Congenital esophageal stenosis often presents in childhood with chronically progressive dysphagia [2]. Mild forms commonly go unrecognized due to dietary self-modification [3]. Most adults reported were diagnosed younger than 40 years of age, with a mean onset age of 27 years old. Presentation involves worsening dysphagia, chest pain, or food impaction. The pathognomonic radiologic finding is smooth, concentric, ringlike esophageal strictures on barium swallow [4]. Katzka and coworkers [3] reported transmural esophageal hypoechoic thickening as specific findings on ultrasound. The midesophagus is involved in 80% of patients [2].
A high index of suspicion, an accurate history, and an esophagogram are critical in making an early diagnosis [1]. The differential diagnosis includes caustic ingestion, radiation esophagitis, candidiasis, scleroderma, Barrett's esophagus, esophageal tracheobronchial rests, gastroesophageal reflux disease related stricture, achalasia, and vascular ring [1]. Management of CES depends on its type, location, and severity. Esophageal dilation and dietary modification are the treatment of choice for fibromuscular stenosis and membranous diaphragm. Surgical resection is often required with tracheal stenosis. Segmental excision and end-to-end anastomosis is the definitive treatment for CES, with funduplication for lesions at or near the gastroesophageal junction [1].
Spontaneous esophageal perforation with CES has not been previously reported. Perforation of the thoracic and thoracoabdominal esophagus has high morbidity and mortality due to frequent diagnostic delay leading to mediastinitis. Delay of more than 48 hours substantially increases morbidity and mortality [5]. Esophagogram and CT with oral contrast are the best diagnostic options for esophageal perforation.
Treatment of esophageal perforation depends on the clinical presentation and findings. Surgical intervention is mandatory in patients with free perforation or clinical deterioration. Primary repair, with buttressing as indicated, and extensive drainage are recommended. Criteria for nonoperative treatment include contained esophageal perforation with minimal clinical symptoms and without signs of sepsis. Even with appropriate surgical intervention, antibiotic therapy, and nutrition the mortality rate for esophageal perforation remains as high as 20% [5].
Congenital esophageal stenosis is a rare condition typically presenting with dysphagia and requires repetitive esophageal dilations. Herein we describe a patient with spontaneous esophageal perforation. Based on recent literature, CES could be more prevalent than previously thought, and a heightened awareness and clinical suspicion is important to prevent missed diagnoses. One should also bear in mind that spontaneous perforation can occur and, if identified, should be treated promptly.
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References
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- Ramesh J.C., Ramanujan T.M., Jayaam G. Congenital esophageal stenosis: report of three cases, literature review, and proposed classification. Pediatr Surg Int 2001;17:188-192.[Medline]
- Younes Z., Johnson D.A. Congenital esophageal stenosis: clinical and endoscopic features in adults. Dig Dis 1998;17:172-177.
- Katzka D., Levine M.S., Ginsberg G.G., et al. Congenital esophageal stenosis in adults. Am J Gastroenterol 2000;95:32-36.[Medline]
- Oh C.H., Levine M.S., Katzka D.A., et al. Congenital esophageal stenosis in adults: clinical and radiographic findings in seven patients. AJR 2001;176:1179.[Abstract/Free Full Text]
- Okten I., Cangir A.K., Ozedimer N., Kavukcu S., Akai H., Yavuzer S. Management of esophageal perforation. Surg Today 2000;31:36-39.
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Abstract
Introduction
