eMedicine Specialties > Ophthalmology > Infectious Disease

Mucormycosis

Author: Kimberly G Yen, MD, Assistant Professor of Ophthalmology, Department of Ophthalmology, Cullen Eye Institute, Baylor College of Medicine
Coauthor(s): Michael T Yen, MD, Associate Professor of Ophthalmology, Department of Ophthalmology, Division of Ophthalmic Plastic, Lacrimal, and Orbital Surgery, Cullen Eye Institute, Baylor College of Medicine
Contributor Information and Disclosures

Updated: Dec 31, 2008

Introduction

Background

Mucormycosis is an aggressive, opportunistic infection caused by fungi in the class of Phycomycetes, first described in 1885 by Paltauf. The genera most commonly responsible for mucormycosis usually are Mucor or Rhizopus.

Orbitorhinocerebral mucormycosis, the most common type, generally occurs in conjunction with sinus or nasal involvement.1 Mucormycosis also may affect other parts of the body, including the lungs, GI tract, or skin.

Most cases of mucormycosis are acute surgical emergencies; however, several cases of a more chronic, indolent form have been reported, with signs and symptoms developing over 4 weeks.

Pathophysiology

The spores of these fungi are ubiquitous and gain entrance to the human body through the mouth and the nose. Individuals who are immunocompetent will phagocytize these spores; therefore, they do not develop the disease. In individuals who are immunocompromised, germination and hyphae formation occur, and this allows the organism to invade the patient's blood vessels. Mucormycosis is described almost exclusively in patients with compromised immune systems or metabolic abnormalities.

The spores attach to the nasal or oral mucosa where massive spore formation occurs; then, the fungus directly invades the blood vessels. Spread occurs when it invades the nasal cavity and maxillary sinuses. Extension to ethmoid sinuses can lead to orbital involvement. Intracranial spread can occur through the ophthalmic artery, superior fissure, or cribriform plate. Areas of ischemic infarction and necrosis are seen in the infected tissue. The fungi invade the blood vessel lumina and cause thrombosis through inflammatory occlusion.

Frequency

United States

Exact frequency is unknown but is higher in patients with immune compromise or metabolic abnormalities.2 Of those patients with mucormycosis, 50-75% have poorly controlled diabetes mellitus and ketoacidosis. Diabetic patients are predisposed to mucormycosis because of the decreased ability of their neutrophils to phagocytize and adhere to endothelial walls. Furthermore, the acidosis and hyperglycemia provide an excellent environment for the fungus to grow.

Mortality/Morbidity

Despite advances in diagnosis and treatment, a high mortality still exists for this disease. Death may occur within 2 weeks if untreated or unsuccessfully treated.

  • Until the 1950s, this disease almost always was a fatal disease. Permanent residual effects of the disease occur up to 70% of the time. These effects include blindness and cranial nerve defects.
  • Mortality rates of 30-70% are quoted in the literature, with mortality higher in older series. The mortality rate in diabetic patients appears to be lower than in nondiabetic patients and in patients with intracerebral involvement. Patients who have been treated with amphotericin B and who have had orbital exenterations are more likely to survive.
  • In a meta-analysis by Yohai et al, it was believed that the survival rate declines when interval from diagnosis to treatment is longer than 6 days.3

Race

No racial predisposition is known.

Sex

No sex predisposition is known.

Age

No age predisposition is known.

Clinical

History

  • Early diagnosis and treatment is the key. This diagnosis requires a high degree of clinical suspicion.4,5,6,7
  • Common symptoms include the following:
    • Orbital and facial pain
    • Sinusitis
    • Headache
    • Fever
    • Visual changes
    • Nasal discharge or stuffiness

Physical

  • External examination may reveal the following8 :
    • Periorbital and facial swelling with signs of orbital cellulitis (eg, proptosis, ophthalmoplegia)9
    • Fever
    • Change in mental status
    • Necrotic tissue can be seen on the nasal turbinates, septum, and palate and may look like a black eschar.
  • Ocular examination may reveal the following:
    • Decreased vision
    • Afferent pupillary defect
    • Conjunctival chemosis
    • Proptosis and periorbital edema
    • Decreased ocular motility/partial or total ophthalmoplegia
    • Orbital apex syndrome

Causes

  • Mucormycosis is an aggressive, opportunistic infection caused by fungi in the class of Phycomycetes. The genera most commonly responsible for mucormycosis usually are Mucor or Rhizopus.
  • Patients with immunosuppression and/or metabolic abnormalities are at risk. Other patients at risk include the following:
    • Patients on long-term antibiotics, steroids, or cytotoxic therapy
    • Patients with chronic renal failure or liver problems
    • Patients with transplants
    • Patients with cancer
    • Patients with HIV
    • Patients with malnutrition or acidosis, especially diabetic patients with ketoacidosis (historically considered patients at highest risk)

More on Mucormycosis

Overview: Mucormycosis
Differential Diagnoses & Workup: Mucormycosis
Treatment & Medication: Mucormycosis
Follow-up: Mucormycosis
Multimedia: Mucormycosis
References
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References

  1. Gilbard SM, Della Rocca RC. Paranasal sinus disease and the orbit. In: Nesi FA, et al, ed. Smith's Ophthalmic Plastic and Reconstructive Surgery. 2nd ed. 1998:896-930.

  2. Rocha G, Garza G, Font RL. Orbital pathology associated with diabetes mellitus. Int Ophthalmol Clin. Spring 1998;38(2):169-79. [Medline].

  3. Yohai RA, Bullock JD, Aziz AA, et al. Survival factors in rhino-orbital-cerebral mucormycosis. Surv Ophthalmol. Jul-Aug 1994;39(1):3-22. [Medline].

  4. Guevara N, Roy D, Dutruc-Rosset C, et al. Mucormycosis--early diagnosis and treatment. Rev Laryngol Otol Rhinol (Bord). 2004;125(2):127-31. [Medline].

  5. Turunc T, Demiroglu YZ, Aliskan H, et al. Eleven cases of mucormycosis with atypical clinical manifestations in diabetic patients. Diabetes Res Clin Pract. Nov 2008;82(2):203-8. [Medline].

  6. Nithyanandam S, Jacob MS, Battu RR, et al. Rhino-orbito-cerebral mucormycosis. A retrospective analysis of clinical features and treatment outcomes. Indian J Ophthalmol. Sep 2003;51(3):231-6. [Medline].

  7. Bhansali A, Sharma A, Kashyap A, et al. Mucor endophthalmitis. Acta Ophthalmol Scand. Feb 2001;79(1):88-90. [Medline].

  8. Arndt S, Aschendorff A, Echternach M, et al. Rhino-orbital-cerebral mucormycosis and aspergillosis: differential diagnosis and treatment. Eur Arch Otorhinolaryngol. Jan 2009;266(1):71-6. [Medline].

  9. Doty CI, Lucchesi M. Mucormycosis manifesting as proptosis and unilateral blindness. Acad Emerg Med. Aug 2000;7(8):944-6. [Medline].

  10. Strasser MD, Kennedy RJ, Adam RD. Rhinocerebral mucormycosis. Therapy with amphotericin B lipid complex. Arch Intern Med. Feb 12 1996;156(3):337-9. [Medline].

  11. Tarani L, Costantino F, Notheis G, et al. Long-term posaconazole treatment and follow-up of rhino-orbital-cerebral mucormycosis in a diabetic girl. Pediatr Diabetes. Sep 17 2008;[Medline].

  12. Reed C, Bryant R, Ibrahim AS, et al. Combination polyene-caspofungin treatment of rhino-orbital-cerebral mucormycosis. Clin Infect Dis. Aug 1 2008;47(3):364-71. [Medline].

  13. Luna JD, Ponssa XS, Rodriguez SD, et al. Intraconal amphotericin B for the treatment of rhino-orbital mucormycosis. Ophthalmic Surg Lasers. Aug 1996;27(8):706-8. [Medline].

  14. Seiff SR, Choo PH, Carter SR. Role of local amphotericin B therapy for sino-orbital fungal infections. Ophthal Plast Reconstr Surg. Jan 1999;15(1):28-31. [Medline].

  15. Hargrove RN, Wesley RE, Klippenstein KA, et al. Indications for orbital exenteration in mucormycosis. Ophthal Plast Reconstr Surg. Jul-Aug 2006;22(4):286-91. [Medline].

  16. Pelton RW, Peterson EA, Patel BC, et al. Successful treatment of rhino-orbital mucormycosis without exenteration: the use of multiple treatment modalities. Ophthal Plast Reconstr Surg. Jan 2001;17(1):62-6. [Medline].

  17. Hargrove RN, Wesley RE, Klippenstein KA, et al. Indications for orbital exenteration in mucormycosis. Ophthal Plast Reconstr Surg. Jul-Aug 2006;22(4):286-91. [Medline].

  18. Croce A, Moretti A, D'Agostino L, et al. Orbital exenteration in elderly patients: personal experience. Acta Otorhinolaryngol Ital. Aug 2008;28(4):193-9. [Medline].

  19. Lari AR, Kanjoor JR, Vulvoda M, et al. Orbital reconstruction following sino-nasal mucormycosis. Br J Plast Surg. Jan 2002;55(1):72-5. [Medline].

  20. Dhiwakar M, Thakar A, Bahadur S. Improving outcomes in rhinocerebral mucormycosis--early diagnostic pointers and prognostic factors. J Laryngol Otol. Nov 2003;117(11):861-5. [Medline].

  21. Fairley C, Sullivan TJ, Bartley P, et al. Survival after rhino-orbital-cerebral mucormycosis in an immunocompetent patient. Ophthalmology. Mar 2000;107(3):555-8. [Medline].

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Further Reading

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Keywords

mucormycosis , Mucor, rhinocerebral mucormycosis, rhino-orbital cerebral mucormycosis

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Contributor Information and Disclosures

Author

Kimberly G Yen, MD, Assistant Professor of Ophthalmology, Department of Ophthalmology, Cullen Eye Institute, Baylor College of Medicine
Kimberly G Yen, MD is a member of the following medical societies: Association for Research in Vision and Ophthalmology
Disclosure: Nothing to disclose.

Coauthor(s)

Michael T Yen, MD, Associate Professor of Ophthalmology, Department of Ophthalmology, Division of Ophthalmic Plastic, Lacrimal, and Orbital Surgery, Cullen Eye Institute, Baylor College of Medicine
Michael T Yen, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Ophthalmic Plastic and Reconstructive Surgery, and Association for Research in Vision and Ophthalmology
Disclosure: Nothing to disclose.

Medical Editor

Ron W Pelton, MD, PhD, Private Practice, Colorado Springs, Colorado
Ron W Pelton, MD, PhD is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, American Society of Ophthalmic Plastic and Reconstructive Surgery, Colorado Medical Society, Utah Medical Association, and Wilderness Medical Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Simon K Law, MD, PharmD, Assistant Professor of Ophthalmology, Jules Stein Eye Institute; Chief of Section of Ophthalmology Surgical Services, Department of Veterans Affairs Healthcare Center, West Los Angeles
Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, and Association for Research in Vision and Ophthalmology
Disclosure: Nothing to disclose.

Managing Editor

Mark T Duffy, MD, PhD, Consulting Staff, Division of Oculoplastic, Orbito-facial, Lacrimal and Reconstructive Surgery, Green Bay Eye Clinic, BayCare Clinic; Medical Director, Advanced Cosmetic Solutions, A BayCare Clinic
Mark T Duffy, MD, PhD is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, American Society of Ophthalmic Plastic and Reconstructive Surgery, Sigma Xi, and Society for Neuroscience
Disclosure: Allergan - Botox Cosmetic Consulting fee Consulting; Quest medical - lacrimal balloons Honoraria Speaking and teaching; Ortho-Neutrogenia Consulting fee Consulting

CME Editor

Lance L Brown, OD, MD, Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri
Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences
Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology
Disclosure: Nothing to disclose.

 
 
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