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Pediatric Mucormycosis

  • Author: Meera Varman, MD; Chief Editor: Russell W Steele, MD  more...
 
Updated: Aug 25, 2011
 

Background

In this article, pediatric mucormycosis will be reviewed, such as infection involving immunosuppressed children; this disease has also been observed in neonates (especially premature infants), patients with burns, and children with a history of incidental trauma.

Mucormycosis usually refers to fungal infections in immunosuppressed hosts caused by ubiquitous molds found in organic matter and soil. Such molds belong to the order Mucorales.[1, 2, 3] The infections they cause manifest in the rhinocerebral, pulmonary, cutaneous (see the image below), gastrointestinal (GI), disseminated, and central nervous systems.

Black eschar on the skin of an immunocompromised p Black eschar on the skin of an immunocompromised patient.

Mucormycosis is often life threatening. Therefore, prompt diagnosis and institution of antifungal therapy are vital, as is appropriate management of the underlying disease process.

See also Pediatric Mucormycosis and Rhinocerebral Mucormycosis.

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Pathophysiology

The fungus of mucormycosis gains entry into the body through the nasopharynx. It can be inhaled into the lungs, or it can extend to the sinuses, orbit, and brain. The occurrence of mucormycosis depends on host immunity, but the mechanisms of increased susceptibility in certain hosts remain perplexing. Regardless of the anatomic site involved, characteristic histopathologic findings include angioinvasion with subsequent tissue infarction and necrosis leading to tissue destruction.[4]

The fungal pathogens that cause mucormycosis belong to the class Zygomycetes and the order Mucorales. Rhizopus species are the agents most commonly isolated in mucormycosis, followed by Rhizomucor species, Absidia corymbifera, Apophysomyces elegans, Cunninghamella bertholletiae, Mucor species, and Saksenaea vasiformis.[1, 2, 3]

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Etiology

Immunodeficiency

Risk factors for mucormycosis in adults and children include diabetes mellitus (especially with ketoacidosis), which is the underlying condition most commonly associated with this disease. This feature is probably due to diminished function of phagocytes at low pH.

Other individuals at risk include patients with malignancy, those with protein-calorie malnutrition, those with skin breakdown due to burns, those with trauma or who are undergoing surgery, those with acute and chronic renal disease, and those with hematologic disease who are receiving deferoxamine.

Patients with immunosuppression due to acquired immunodeficiency syndrome (AIDS), organ transplantation, neutropenia, or steroid therapy are also at risk.

Nosocomial infection

Hospital-acquired mucormycosis has been reported. In a review of 26 hospitalized, posttraumatic patients with mucormycosis, approximately 57% were females and two thirds had comorbidities (ie, diabetes mellitus, leukemia, immunosuppression); diabetes was noted in 6 patients.[5] In hospitalized patients with cannula, wound or occlusive dressings should be closely watched for erythema or necrosis.

Neonatal infection and prematurity

Neonates, especially those born prematurely, can be at risk. Unusual incidents of neonatal mucormycosis might have occurred when patients were exposed to contaminated surfaces, such as bandages, tongue depressors used as arm splints, or cardiac-monitor leads.[6]

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Epidemiology

In the United States, mucormycosis is most common in immunocompromised hosts, although cases in immunocompetent patients are also reported. Underlying diseases, such as diabetes mellitus and malignancy, are risk factors. Environmental spore exposure (from exposure to construction activity) has also led to clinical cases of mucormycosis. Other cases have been reported in patients with traumatic skin injury (eg, associated with the use of nonsterile adhesive tape or with use of tongue depressors as splints in neonates). Exposure to voriconazole, which is not active against mucormycosis, is noted to be a risk factor in patients with cancer.

No racial or sexual predilection is reported.However, most cases of mucormycosis occur in immunosuppressed adults. In a pooled review, Kline described 41 cases of rhinocerebral mucormycosis occurring in children and adolescents aged 2 months to 18 years.[7] About 49% of cases were found in patients with diabetes mellitus, and 15% of cases were found in those with leukemia. Four of the 41 children (10%) had no predisposing conditions.

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Prognosis

Survival rates largely depend on early diagnosis and treatment of mucormycosis as well as resolution of the patient's underlying condition. If the disease progresses and if the underlying condition remains uncontrolled, death usually ensues. The overall mortality rate in adults is 50%, though survival rates higher than this have been reported.

In neonates, these invasive fungal infections can be rapidly fatal; the time from clinical symptoms to death is in the range of 6 to 42 days.

Because the disease is rare and because therapy is not standardized, no studies aid in predicting patient outcomes.

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Contributor Information and Disclosures
Author

Meera Varman, MD Associate Professor, Department of Pediatrics, Section of Pediatric Infectious Diseases, Creighton University Medical Center

Meera Varman, MD is a member of the following medical societies: American Academy of Pediatrics, American Society for Microbiology, Infectious Diseases Society of America, Pediatric Infectious Diseases Society, Society for Healthcare Epidemiology of America

Disclosure: Received honoraria from phamaceutical companies for speaking and teaching; Received grant/research funds from phamaceutical companies for clinical trials research.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Mark R Schleiss, MD Minnesota American Legion and Auxiliary Heart Research Foundation Chair of Pediatrics, Professor of Pediatrics, Division Director, Division of Infectious Diseases and Immunology, Department of Pediatrics, University of Minnesota Medical School

Mark R Schleiss, MD is a member of the following medical societies: American Pediatric Society, Infectious Diseases Society of America, Pediatric Infectious Diseases Society, Society for Pediatric Research

Disclosure: Nothing to disclose.

Chief Editor

Russell W Steele, MD Clinical Professor, Tulane University School of Medicine; Staff Physician, Ochsner Clinic Foundation

Russell W Steele, MD is a member of the following medical societies: American Academy of Pediatrics, American Association of Immunologists, American Pediatric Society, American Society for Microbiology, Infectious Diseases Society of America, Louisiana State Medical Society, Pediatric Infectious Diseases Society, Society for Pediatric Research, Southern Medical Association

Disclosure: Nothing to disclose.

Additional Contributors

Gary J Noel, MD Professor, Department of Pediatrics, Weill Cornell Medical College; Attending Pediatrician, New York-Presbyterian Hospital

Gary J Noel, MD is a member of the following medical societies: Pediatric Infectious Diseases Society

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous authors Mary Carmen Y Mancao, MD, Christine A Reyes, MD, and Debra Whaley, MD, to the development and writing of the source article.

References
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  2. Sugar A. Agents of mucormycosis and related species. Mandell GL, Bennet JE, Dolin R, eds. Mandell, Douglas, and Bennett's Principles and Practice of Infectious Diseases. 7th ed. Philadelphia, Pa: Churchill Livingstone; 2009. Chapter 259.

  3. Wiedermann BL. Zygomycosis. Feigen RD, Cherry JD, Demmler-Harrison GJ, Kaplan SL, eds. Textbook of Pediatric Infectious Diseases. 6th ed. Philadelphia, Pa: Saunders; 2009.

  4. Frater JL, Hall GS, Procop GW. Histologic features of zygomycosis: emphasis on perineural invasion and fungal morphology. Arch Pathol Lab Med. 2001 Mar. 125(3):375-8. [Medline].

  5. Simbli M, Hakim F, Koudieh M, Tleyjeh IM. Nosocomial post-traumatic cutaneous mucormycosis: a systematic review. Scand J Infect Dis. 2008. 40(6-7):577-82. [Medline].

  6. Robertson AF, Joshi VV, Ellison DA, Cedars JC. Zygomycosis in neonates. Pediatr Infect Dis J. 1997 Aug. 16(8):812-5. [Medline].

  7. Kline MW. Mucormycosis in children: review of the literature and report of cases. Pediatr Infect Dis. 1985 Nov-Dec. 4(6):672-6. [Medline].

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  10. Dave SP, Vivero RJ, Roy S. Facial cutaneous mucormycosis in a full-term infant. Arch Otolaryngol Head Neck Surg. 2008 Feb. 134(2):206-9. [Medline].

  11. Scheinfeld N. A review of the new antifungals: posaconazole, micafungin, and anidulafungin. J Drugs Dermatol. 2007 Dec. 6(12):1249-51. [Medline].

  12. Ullmann AJ, Lipton JH, Vesole DH, Chandrasekar P, Langston A, Tarantolo SR, et al. Posaconazole or fluconazole for prophylaxis in severe graft-versus-host disease. N Engl J Med. 2007 Jan 25. 356(4):335-47. [Medline].

  13. Kaide CG, Khandelwal S. Hyperbaric oxygen: applications in infectious disease. Emerg Med Clin North Am. 2008 May. 26(2):571-95, xi. [Medline]. [Full Text].

  14. Garg PK, Gupta N, Gautam V, Hadke NS. Gastric zygomycosis: unusual cause of gastric perforation in an immunocompetent patient. South Med J. 2008 Apr. 101(4):449-50. [Medline].

  15. Goel S, Carter JE, Culpepper M, Kahn AG. Primary renal zygomycotic infarction mimicking renal neoplasia in an immunocompetent patient. Am J Med Sci. 2009 Oct. 338(4):330-3. [Medline].

  16. Kontoyiannis DP, Lionakis MS, Lewis RE, Chamilos G, Healy M, Perego C, et al. Zygomycosis in a tertiary-care cancer center in the era of Aspergillus-active antifungal therapy: a case-control observational study of 27 recent cases. J Infect Dis. 2005 Apr 15. 191(8):1350-60. [Medline].

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Black eschar on the skin of an immunocompromised patient.
Mucormycosis with broad, aseptate hyphae (hematoxylin and eosin, original magnification ×40).
Angioinvasion (hematoxylin and eosin, original magnification ×10).
Perineural invasion (hematoxylin and eosin, original magnification ×20).
 
 
 
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