Ann Thorac Surg 2005;80:1120-1122
© 2005 The Society of Thoracic Surgeons
Case report
Fatal Esophageal Perforation Caused by Invasive Candidiasis
Michael Gock, MD,
Markus Schäfer, MD,
Aurel Perren, MD,
Nicolas Demartines, MD,
Pierre-Alain Clavien, MD, PhD
*
Department of Visceral and Transplantation Surgery, and Pathology, University of Zürich, Zürich, Switzerland
Accepted for publication February 20, 2004.
* Address reprint requests to Dr Clavien, Department of Visceral and Transplantation Surgery, University of Zürich, Rämistr 100, Zürich 8091, Switzerland; (Email: pierre-alain.clavien{at}usz.ch).
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Abstract
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Instrumental lesions, spontaneous rupture, and trauma cause most esophageal perforations. Transmural fungal infection is extremely rare, although Candida may be detected in as many as 25% of normal esophagus. In this report we present a case of fatal esophageal perforation due to transmural Candida infection in a 76-year-old woman. The patient died from septic shock and multiorgan failure, despite esophageal resection and systemic antifungal therapy. Pathogenetic aspects and treatment strategies are discussed.
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Introduction
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Esophageal perforation is an uncommon clinical entity, which is associated with increased morbidity and mortality rates [1]. Among different reasons that may lead to esophageal perforation, transmural Candida infection of the esophageus is extremely rare. We could only identify four cases in current English literature.
Particularly characterized by its ability to survive in distinct anatomical sites, Candida albicans mucositis of the gastrointestinal and urogenital tract is further facilitated by changes of a host immunity (eg, depletion of CD4+ leukocytes in patients with AIDS) [2]. Immunosuppression regimen after organ transplantation and prolonged stay in intensive care units due to major surgery particularly promote Candida infection of the esophagointestinal mucosa [3].
We report a case of fatal esophageal perforation as a sequel of transmural esophageal Candida infection in a woman with a symptomatic paraesophageal hernia.
A 76-year-old woman had been treated as in-hospital patient for actinic skin lesions that occurred after radiation therapy for vaginal carcinoma, when she had acute left-sided chest pain and shortness of breath develop. Coronary heart disease, mild impairment of renal function, and ileocecal resection for small bowel carcinoma were preexisting comorbidities.
Chest roentgenogram revealed left pleural effusion and pulmonary infiltration, as well as an enlarged mediastinum. Cardiac ischemia was suspected because of electrocardiographic changes, whereas laboratory findings were all within normal ranges, particularly troponin and D-dimer. Empirical antibiotic treatment was started without further investigations, and the patient slightly improved during the following 2 days. Then the patient became rapidly septic with cardiopulmonary disturbances, increasing pain, and C-reactive protein (CRP) levels. Computed tomographic scan of the thorax demonstrated a seropneumothorax and extensive left-sided pulmonary atelectasis formation. In addition, multiple mediastinal air bubbles and a large paraesophageal hernia were detected (Fig 1).
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Fig 1. Computed tomographic scan of the thorax shows a seropneumothorax with extensive pulmonary atelectasis formation on the left side, multiple mediastinal air bubbles, a large paraesophageal hernia, and a right-sided descending aorta.
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The patient underwent emergency surgical exploration by a median upper laparotomy. Intraoperatively, a 4-cm longitudinal opening of the distal esophagus was detected that had induced extensive necrosis formation in the posterior mediastinum. Due to the advanced inflammatory changes, esophageal resection and mediastinal drainage were performed through an additional right anterior thoracotomy. A right-sided descending aorta further complicated the operation. Restoration of gastrointestinal continuity was achieved by creating a gastric tube vascularized by the right gastroepiploic artery that was connected with the proximal esophagus in the upper mediastinum. The postoperative course was severely protracted by the ongoing sepsis and development of a multiorgan failure. The patient never recovered and died on postoperative day 17.
Histologic examination of the specimen revealed transmural ulceration at the esophagogastric junction with concomitant severe mediastinitis. Within that ulceration, large masses of fungal hyphal elements were found (Fig 2). Additional microbiological analysis identified C. albicans and Candida norvegensis. These Candida species were also detected in the sputum, vagina, and pleural cavities.
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Fig 2. Histologic specimen of the esophageal wall. Arrowheads show large masses of fungal hyphal elements.
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Comment
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Candida represents a commensal fungus of different mucous membranes in healthy individuals. The esophagus is colonized in as much as 25% of normal individuals [4]. However, invasive infections causing extensive tissue necrosis and ulceration predominantly occur in immunocompromised patients after major surgical procedures or prolonged intensive care unit stays, and also in patients with organ transplantations [3]. It has only been partially elucidated which factors may contribute to promote progression from simple colonization to invasive disease [5]. Competent T-cell immune response is a crucial protecting mechanism against cutaneous, vaginal, and oral Candidiasis in patients with AIDS who are known to develop oral Candidiasis in as much as 70% [6]. Resistance to systemic fungal infection is closely associated with normal mononuclear phagocytes and neutrophil response [5]. Moreover, it has been hypothesized that depending on specific preexisting host deficiencies, different forms of Candidiasis may occur. The invasiveness of different Candida strains induces various types of diseases and host responses accordingly [2, 5].
Transmural invasive Candida infection remains an extremely rare cause of esophagointestinal perforation. By thoroughly reviewing the current English literature, we could only find 4 patients with esophageal perforation that were histologically confirmed to be caused by transmural Candida infection [7, 8]. Jones and colleagues [8] reported severe necrotizing Candida esophagitis in 2 insulin-dependent diabetic patients after renal transplantation. The first patient was a 34-year-old woman who died 6 months after transplantation. She had ulcerative Candida esophagitis develop 2 weeks postoperatively. Despite amphotericin B treatment she had an esophagomediastinal fistula develop, and she died after sudden massive hematemesis. Autopsy disclosed a ruptured mycotic aneurysmal of a peripheral pulmonary artery. The second patient was a 40-year-old human who had characteristic Candida esophagitis develop 35 days after transplantation. An esophagram taken 66 days after the operation showed a fistula between the esophagus and the main stem of the left bronchus. Despite treatment with amphotericin B, decompressive gastrostomy, esophagostomy above the fistula, and dissection below the fistula, the patient became septic and died 3 months after transplantation. An autopsy showed a septal myocardial infarction as the cause of death [8]. Both patients developed invasive Candida esophagitis in the early posttransplant course. Despite prophylactic oral nystatin treatment, esophageal perforation could not be prevented.
Another two cases of nonfatal esophageal perforation related to invasive Candidiasis were reported by Gaissert and colleagues [7]. The first patient (a 10-year-old boy with Down syndrome) underwent chemotherapy for acute B-cell lymphocytic leukemia when he had esophageal necrosis develop. The second patient (a 27-year-old autistic human) underwent diagnostic esophagogastroscopy for esophageal Candidiasis. Delayed perforation of the esophageal wall occurred 3 months later at the biopsy location. Emergency esophagectomy and cervical esophagostomy were performed. Moreover, systemic antifungal therapy was started. Both patients survived and revealed a good long-term outcome after restoration of gastrointestinal continuity by using a gastric conduit.
In our patient, a preexisting esophageal colonization with Candida can be assumed. Temporary incarceration of the paraesophageal hernia probably caused a mucosal damage at the gastroesophageal junction. Transmural ulceration by invasive Candidiasis was therefore facilitated by the impaired mucosal defense. At the time of delayed diagnosis, esophageal perforation was advanced and extensive mediastinitis was already established.
Successful treatment of invasive Candida infection complicated by esophageal perforation requires both long-term systemic antifungal therapy and emergency surgical intervention. Surgery must include esophageal resection, extensive necrosectomy, and drainage of the pleural cavities and mediastinum. Correction of the patient’s immunosuppressive drugs should be attempted. Because there are only a very few cases that have been reported so far, no established treatment regimen has been validated and long-term outcome probably remains poor.
In conclusion, esophageal perforation due to invasive Candidiasis is an extremely rare complication, although its true incidence may be underestimated. Despite aggressive medical and surgical treatment, the patient’s prognosis remains poor.
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References
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- Goldstein LA, Thompson WR. Esophageal perforation a 15-year experience Am J Surg 1982;143:495-502.[Medline]
- Bernhardt J, Herman D, Sheridan M, et al. Adherence and invasion of Candida albicans strains using in vitro models of esophageal Candidiasis J Infect Dis 2001;184:1170-1175.[Medline]
- Dean DA, Burchard KW. Surgical perspective on invasive Candida infections World J Surg 1998;22:127-134.[Medline]
- Andersen LI, Frederiksen HJ, Appleyard M. Prevalence of esophageal Candida colonization in a Danish populationspecial reference to esophageal symptoms, benign esophageal disorders, and pulmonary disease. J Infect Dis 1992;165:389-392.[Medline]
- Calderone RA, Fonzi WA. Virulence factors of Candida albicans
Trends Microbiol 2001;327:35.
- Fidel Jr PL, Vazquez JA, Sobel JD.
Candida glabratareview of epidemiology, pathogenesis, and clinical disease with comparison to C albicans
. Clin Microbiol Rev 1999;12:80-96.[Abstract/Free Full Text]
- Gaissert HA, Breuer CK, Weissburg A, et al. Surgical management of necrotizing Candida esophagitis Ann Thorac Surg 1999;67:231-233.[Abstract/Free Full Text]
- Jones JM, Glass NR, Belzer FO. Fatal Candida esophagitis in two diabetics after renal transplantation Arch Surg 1982;117:499-501.[Abstract/Free Full Text]
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Abstract
Introduction
