- Email: [email protected]
Esophageal Mucormycosis
Video Journal and Encyclopedia of GI Endoscopy (2014) 1, 658–660
Available online at www.sciencedirect.com
journal homepage: www.elsevier.com/locate/vjgien
CLINICAL CASE REPORTS
Esophageal Mucormycosis$, $$ Benjamin Boatrighta, Shou-jiang Tanga,n, Zebedee J. Whatley IVa, Ruonan Wub, Julio Cespedesc, Feriyl Bhaijeec a
Division of Digestive Diseases, Department of Medicine, University of Mississippi Medical Center, 2500 North State Street, Jackson, MS 39216, USA b Department of Radiology, University of Mississippi Medical Center, 2500 North State Street, Jackson, MS 39216, USA c Department of Pathology, University of Mississippi Medical Center, 2500 North State Street, Jackson, MS 39216, USA Received 16 April 2013; received in revised form 24 May 2013; accepted 30 May 2013
KEYWORDS
Abstract
Esophagus; Mucor; Mucormycosis; Fungal infection; Endoscopy; Video
Mucormycosis is a rare invasive fungal infection with high mortality. It usually affects patients with poorly controlled diabetes, immunosuppression, or hematological malignancies. Gastroenterologists need to be aware of this rare infection because endoscopy can facilitate early diagnosis and prompt appropriate therapy. Here we describe a case of invasive esophageal mucormycosis that developed in a 63-year-old man with diabetes, acute promyelocytic leukemia, and prolonged leukopenia after chemotherapy. Upper endoscopy showed distal circumferential esophageal wall thickening with devitalization. The mucosa did not bleed after endoscopic biopsy. Histopathology confirmed mucormycosis. He was treated with various antifungal agents including echinocandins, fluconazole, and liposomal amphotericin B. Despite aggressive antifungal therapy and supportive care, the patient died 24 days later. & 2013 The Authors. Published by Elsevier GmbH. All rights reserved.
Video related to this article ☆
This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. ☆☆ The terms of this license also apply to the corresponding video. n Corresponding author. Tel.: +1 601 984 4540; fax: +1 601 984 4548. E-mail addresses: [email protected] (B. Boatright), [email protected] (S.-j. Tang), [email protected] (Z.J. Whatley IV), [email protected] (R. Wu), [email protected] (J. Cespedes), [email protected] (F. Bhaijee).
Video related to this article can be found online at http:// dx.doi.org/10.1016/j.vjgien.2013.05.002.
1.
Case report
A 63-year-old man with a previous history of diabetes, hypertension.
Received induction therapy with Daunorubicin, Cytarabine, and Tretinoin for acute promyelocytic leukemia.
2212-0971/$ - see front matter & 2013 The Authors. Published by Elsevier GmbH. All rights reserved. http://dx.doi.org/10.1016/j.vjgien.2013.05.002
Esophageal Mucormycosis
Subsequently he developed dysphagia, neutropenia, Candida
2.
bacteremia, pericarditis, and acute renal failure. Upper endoscopy revealed normal mucosa in the proximal esophagus. However, the distal esophagus showed massive changes with circumferential wall thickening and black discoloration due to devitalization. There was no bleeding after endoscopic biopsy sampling and devitalized fibrous filaments remained at the biopsy site. Histological work-up of targeted biopsies showed necrotic tissue with numerous fungal organisms, consistent with Candidiasis and mucormycosis with branching hyphae with vascular invasion. He was treated with broad spectrum antibiotics and various antifungal agents including echinocandins, fluconazole, and liposomal amphotericin B. Despite aggressive therapy and supportive care, the patient died 24 days later.
Materials
Diagnostic gastroscope (Olympus GIF-Q180, Olympus America, Center Valley, PA).
3.
Discussion
Zygomycoses are infections caused by fungi of the class Zygomycetes and include two orders: Entomophthorales and Mucorales [1–3]. Both are common fungi found in the soil and in dying plants. Entomophthoromycosis is a subcutaneous and mucocutaneous infections that affects immunocompetent hosts. Mucormycosis is an invasive infection that affects immunocompromised hosts. The pre-disposing conditions generally include uncontrolled diabetes, immunosuppression, or chemotherapy. Although Mucormycosis is a rare it ranks third behind Candidiasis and Aspergillosis. The hallmark of mucormycosis is significant angioinvasion with vessel thrombosis and tissue necrosis [1–3]. On histopathology, hyphae can be observed growing in and around vessels. Direct angioinvasion enhances hematogenous spread and results in thrombosis, ischemia, and tissue necrosis. Mucormycosis has been reported in the nasal sinuses, the lungs, the gastrointestinal (GI) tract, and the kidneys. Mortality varies with site of infection. In one large study, disseminated disease had 96% mortality, GI tract infection had 85% mortality, and pulmonary infection resulted in 76% mortality [4]. GI mucormycosis is very rare [1–7]. The stomach, colon, and ileum are the most commonly involved sites. Presenting symptoms are either nonspecific, such as abdominal pain, nausea and vomiting, or GI bleeding, or organ-specific. The diagnosis is confirmed by pathology: branching hyphae with vascular invasion. Clinical management includes systemic antifungal therapy and surgical debridement, if possible, to remove all dead and infected tissue. Mucormycosis has an extremely high death rate even when aggressive therapy and surgical intervention is performed. Because of the very high mortality rate despite aggressive surgical and antifungal therapy, physicians and the patient need to consider the operative risks in managing esophageal mucormycosis.
659 In conclusion, mucormycosis is a rare infection that occurs predominantly in immunocompromised patients. It can affect almost any part of the body and carries a high mortality rate. Endoscopy can facilitate early diagnosis and appropriate medical and surgical intervention.
4.
Scripted voiceover
Voiceover Text A 63 year old man with diabetes and acute promyelocytic leukemia was referred for dysphagia. Recently, he underwent induction chemotherapy therapy and developed neutropenia and Candida bacteremia. CT scan of the chest revealed distal esophageal wall thickening. On upper endoscopy, black discoloration involves distal esophagus associated with wall thickening and devitalization. The mucosa is intact but with luminal narrowing. There is absence of bleeding after endoscopic biopsy. Devitalized fibrous filaments remain at the biopsy site. The proximal esophagus appears normal. Histopathology reveals necrotic tissues with numerous fungal organisms, consistent with candidiasis and Mucormycosis. Despite aggressive antifungal therapy including amphotericin B, the patient died 24 days later. Mucormycosis affects the immunocompromised hosts. This invasive infection is associated with high mortality. The hallmark of mucormycosis is significant angioinvasion with vessel thrombosis and tissue necrosis. On histopathology, hyphae can be observed growing in and around vessels. Direct angioinvasion enhances hematogenous spread. GI mucormycosis is very rare. The stomach, colon, and ileum are the most commonly involved sites. Mortality varies with site of infection. In one large study, GI tract infection had 85% mortality. Endoscopy can facilitate early diagnosis and appropriate medical and surgical intervention.
Funding source No funding was available for this study and manuscript.
Conflict of interest All authors, Benjamin Boatright, Shou-jiang Tang, Zebedee J. Whatley IV, Ruonan Wu, Julio Cespedes, Feriyl Bhaijee have nothing to declare and have no conflict of interests.
References [1] Petrikkos G, Skiada A, Lortholary O, Roilides E, Walsh TJ, Kontoyiannis DP. Epidemiology and clinical manifestations of mucormycosis. Clin Infect Dis 2012;54(Suppl. 1):S23–34.
660 [2] Spellberg B, Edwards Jr J, Ibrahim A. Novel perspectives on mucormycosis: pathophysiology, presentation, and management. Clin Microbiol Rev 2005;18(3):556–69. [3] Prabhu RM, Patel R. Mucormycosis and entomophthoramycosis: a review of the clinical manifestations, diagnosis and treatment. Clin Microbiol Infect 2004;10(Suppl. 1):31–47. [Review]. [4] Roden MM, Zaoutis TE, Buchanan WL, Knudsen TA, Sarkisova TA, Schaufele RL, et al. Epidemiology and outcome of zygomycosis: a review of 929 reported cases. Clin Infect Dis 2005;41(5):634–53.
B. Boatright et al. [5] Margolis PS, Epstein A. Mucormycosis esophagitis in a patient with the acquired immunodeficiency syndrome. Am J Gastroenterol 1994;89(10):1900–2. [6] Singh N, Gayowski T, Singh J, Yu VL. Invasive gastrointestinal zygomycosis in a liver transplant recipient: case report and review of zygomycosis in solid-organ transplant recipients. Clin Infect Dis 1995;20(3):617–20. [Review]. [7] Corley DA, Lindeman N, Ostroff JW. Survival with early diagnosis of invasive gastric mucormycosis in a heart transplant patient. Gastrointest Endosc 1997;46:452–4.
Available online at www.sciencedirect.com
journal homepage: www.elsevier.com/locate/vjgien
CLINICAL CASE REPORTS
Esophageal Mucormycosis$, $$ Benjamin Boatrighta, Shou-jiang Tanga,n, Zebedee J. Whatley IVa, Ruonan Wub, Julio Cespedesc, Feriyl Bhaijeec a
Division of Digestive Diseases, Department of Medicine, University of Mississippi Medical Center, 2500 North State Street, Jackson, MS 39216, USA b Department of Radiology, University of Mississippi Medical Center, 2500 North State Street, Jackson, MS 39216, USA c Department of Pathology, University of Mississippi Medical Center, 2500 North State Street, Jackson, MS 39216, USA Received 16 April 2013; received in revised form 24 May 2013; accepted 30 May 2013
KEYWORDS
Abstract
Esophagus; Mucor; Mucormycosis; Fungal infection; Endoscopy; Video
Mucormycosis is a rare invasive fungal infection with high mortality. It usually affects patients with poorly controlled diabetes, immunosuppression, or hematological malignancies. Gastroenterologists need to be aware of this rare infection because endoscopy can facilitate early diagnosis and prompt appropriate therapy. Here we describe a case of invasive esophageal mucormycosis that developed in a 63-year-old man with diabetes, acute promyelocytic leukemia, and prolonged leukopenia after chemotherapy. Upper endoscopy showed distal circumferential esophageal wall thickening with devitalization. The mucosa did not bleed after endoscopic biopsy. Histopathology confirmed mucormycosis. He was treated with various antifungal agents including echinocandins, fluconazole, and liposomal amphotericin B. Despite aggressive antifungal therapy and supportive care, the patient died 24 days later. & 2013 The Authors. Published by Elsevier GmbH. All rights reserved.
Video related to this article ☆
This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. ☆☆ The terms of this license also apply to the corresponding video. n Corresponding author. Tel.: +1 601 984 4540; fax: +1 601 984 4548. E-mail addresses: [email protected] (B. Boatright), [email protected] (S.-j. Tang), [email protected] (Z.J. Whatley IV), [email protected] (R. Wu), [email protected] (J. Cespedes), [email protected] (F. Bhaijee).
Video related to this article can be found online at http:// dx.doi.org/10.1016/j.vjgien.2013.05.002.
1.
Case report
A 63-year-old man with a previous history of diabetes, hypertension.
Received induction therapy with Daunorubicin, Cytarabine, and Tretinoin for acute promyelocytic leukemia.
2212-0971/$ - see front matter & 2013 The Authors. Published by Elsevier GmbH. All rights reserved. http://dx.doi.org/10.1016/j.vjgien.2013.05.002
Esophageal Mucormycosis
Subsequently he developed dysphagia, neutropenia, Candida
2.
bacteremia, pericarditis, and acute renal failure. Upper endoscopy revealed normal mucosa in the proximal esophagus. However, the distal esophagus showed massive changes with circumferential wall thickening and black discoloration due to devitalization. There was no bleeding after endoscopic biopsy sampling and devitalized fibrous filaments remained at the biopsy site. Histological work-up of targeted biopsies showed necrotic tissue with numerous fungal organisms, consistent with Candidiasis and mucormycosis with branching hyphae with vascular invasion. He was treated with broad spectrum antibiotics and various antifungal agents including echinocandins, fluconazole, and liposomal amphotericin B. Despite aggressive therapy and supportive care, the patient died 24 days later.
Materials
Diagnostic gastroscope (Olympus GIF-Q180, Olympus America, Center Valley, PA).
3.
Discussion
Zygomycoses are infections caused by fungi of the class Zygomycetes and include two orders: Entomophthorales and Mucorales [1–3]. Both are common fungi found in the soil and in dying plants. Entomophthoromycosis is a subcutaneous and mucocutaneous infections that affects immunocompetent hosts. Mucormycosis is an invasive infection that affects immunocompromised hosts. The pre-disposing conditions generally include uncontrolled diabetes, immunosuppression, or chemotherapy. Although Mucormycosis is a rare it ranks third behind Candidiasis and Aspergillosis. The hallmark of mucormycosis is significant angioinvasion with vessel thrombosis and tissue necrosis [1–3]. On histopathology, hyphae can be observed growing in and around vessels. Direct angioinvasion enhances hematogenous spread and results in thrombosis, ischemia, and tissue necrosis. Mucormycosis has been reported in the nasal sinuses, the lungs, the gastrointestinal (GI) tract, and the kidneys. Mortality varies with site of infection. In one large study, disseminated disease had 96% mortality, GI tract infection had 85% mortality, and pulmonary infection resulted in 76% mortality [4]. GI mucormycosis is very rare [1–7]. The stomach, colon, and ileum are the most commonly involved sites. Presenting symptoms are either nonspecific, such as abdominal pain, nausea and vomiting, or GI bleeding, or organ-specific. The diagnosis is confirmed by pathology: branching hyphae with vascular invasion. Clinical management includes systemic antifungal therapy and surgical debridement, if possible, to remove all dead and infected tissue. Mucormycosis has an extremely high death rate even when aggressive therapy and surgical intervention is performed. Because of the very high mortality rate despite aggressive surgical and antifungal therapy, physicians and the patient need to consider the operative risks in managing esophageal mucormycosis.
659 In conclusion, mucormycosis is a rare infection that occurs predominantly in immunocompromised patients. It can affect almost any part of the body and carries a high mortality rate. Endoscopy can facilitate early diagnosis and appropriate medical and surgical intervention.
4.
Scripted voiceover
Voiceover Text A 63 year old man with diabetes and acute promyelocytic leukemia was referred for dysphagia. Recently, he underwent induction chemotherapy therapy and developed neutropenia and Candida bacteremia. CT scan of the chest revealed distal esophageal wall thickening. On upper endoscopy, black discoloration involves distal esophagus associated with wall thickening and devitalization. The mucosa is intact but with luminal narrowing. There is absence of bleeding after endoscopic biopsy. Devitalized fibrous filaments remain at the biopsy site. The proximal esophagus appears normal. Histopathology reveals necrotic tissues with numerous fungal organisms, consistent with candidiasis and Mucormycosis. Despite aggressive antifungal therapy including amphotericin B, the patient died 24 days later. Mucormycosis affects the immunocompromised hosts. This invasive infection is associated with high mortality. The hallmark of mucormycosis is significant angioinvasion with vessel thrombosis and tissue necrosis. On histopathology, hyphae can be observed growing in and around vessels. Direct angioinvasion enhances hematogenous spread. GI mucormycosis is very rare. The stomach, colon, and ileum are the most commonly involved sites. Mortality varies with site of infection. In one large study, GI tract infection had 85% mortality. Endoscopy can facilitate early diagnosis and appropriate medical and surgical intervention.
Funding source No funding was available for this study and manuscript.
Conflict of interest All authors, Benjamin Boatright, Shou-jiang Tang, Zebedee J. Whatley IV, Ruonan Wu, Julio Cespedes, Feriyl Bhaijee have nothing to declare and have no conflict of interests.
References [1] Petrikkos G, Skiada A, Lortholary O, Roilides E, Walsh TJ, Kontoyiannis DP. Epidemiology and clinical manifestations of mucormycosis. Clin Infect Dis 2012;54(Suppl. 1):S23–34.
660 [2] Spellberg B, Edwards Jr J, Ibrahim A. Novel perspectives on mucormycosis: pathophysiology, presentation, and management. Clin Microbiol Rev 2005;18(3):556–69. [3] Prabhu RM, Patel R. Mucormycosis and entomophthoramycosis: a review of the clinical manifestations, diagnosis and treatment. Clin Microbiol Infect 2004;10(Suppl. 1):31–47. [Review]. [4] Roden MM, Zaoutis TE, Buchanan WL, Knudsen TA, Sarkisova TA, Schaufele RL, et al. Epidemiology and outcome of zygomycosis: a review of 929 reported cases. Clin Infect Dis 2005;41(5):634–53.
B. Boatright et al. [5] Margolis PS, Epstein A. Mucormycosis esophagitis in a patient with the acquired immunodeficiency syndrome. Am J Gastroenterol 1994;89(10):1900–2. [6] Singh N, Gayowski T, Singh J, Yu VL. Invasive gastrointestinal zygomycosis in a liver transplant recipient: case report and review of zygomycosis in solid-organ transplant recipients. Clin Infect Dis 1995;20(3):617–20. [Review]. [7] Corley DA, Lindeman N, Ostroff JW. Survival with early diagnosis of invasive gastric mucormycosis in a heart transplant patient. Gastrointest Endosc 1997;46:452–4.