MUCORMYCOSIS IN The Name Of God

BACKGROUND Mucormycosis refers to rare, severe opportunistic infection with fungi of the order Mucorales. Hence the name “ Mucormycosis “

Systemic Zygomycosis (Mucormycosis) An acute and rapidly developing, less commonly chronic, infection of debilitated patients. Depending on the portal of entry, the disease involves the rhino-facial-cranial area, lungs, gastrointestinal tract, skin or less commonly other organ systems. The infecting fungi have a predilection for invading vessels of the arterial system, causing embolization and subsequent necrosis of surrounding tissue. A suppurative, pyrogenic reaction is elicited; granuloma formation is not frequently encountered. Distribution: World-wide. . Text slide. 3

ORGANISMS Rhizopus species are the most common causative organisms. Other less frequent species include:  Rhizomucor,  Absidia,  Cunninghamella,  Saksenaea, and  Apophysomyces

PATHOPHYSIOLOGY Mucorales fungi are ubiquitous environmental organisms. The major route of infection is by inhalation of spores. Ingestion or traumatic inoculation are recorded Humans are resistant to disease, but immuno-compromised hosts are at risk.

PATHOPHYSIOLOGYcont. Once the spores begin to grow, fungal hyphae invade blood vessels, producing tissue infarction, massive necrosis with bone destruction. Thus producing the life-threatening, invasive, rhinocerebral, and other organ-centered manifestations.

RISK FACTORS Diabetes mellitus esp. with ketoacidosis Steroid therapy Neutropenea, HIV patients Hematologic and solid malignancies Malnourished individuals, especially children Desferoxamine therapy and all causes of iron overload Burn victims susceptible to cutaneous MM BM transplant recipients Persons in Renal Failure Intravenous drug abusers (at risk for cerebral MM)

FREQUENCY MM is extremely rare,one center showed it was present in 0.7% of patients at autopsy. Rhinocerebral disease is the most common form, hence the name “Zygomycosis”. Others include pulmonary, cutaneous, gastrointestinal, and disseminated diseases. Very rare cases occur in immunocompetent patients, usually after traumatic inoculation.

MM is found in patients of a wide age range. SEX There is equal sex distribution, but pulmonary MM shows a male-to-female ratio of 3:1

MORTALITY/ MORBIDITY Mucormycosis has a very high mortality rate reaching 50 to 80% even with treatment. Pulmonary and gastrointestinal diseases have higher mortality rate due to late diagnosis. Rhinocerebral disease causes significant morbidity in patients who survive because treatment requires extensive facial surgery.

CLINICAL PICTURE

Mucormycosis is distinguished by its fulminant course with evidence of extensive tissue necrosis.

RHINOCEREBRAL MM Presents early with unilateral, retro-orbital headache, proptosis, epistaxis and nasal stuffiness progressing to black discharge. Late symptoms are diplopia and visual loss indicating invasion of the orbital nerves and vessels.

RHINOCEREBRAL MM cont. Signs of rhinocerebral MM are characteristic and must be recognized promptly. Signs are progressive with discharge of black pus from the necrotic palatine or nasal eschars.

479. Rhinocerebral zygomycosis showing involvement of the palate caused by Apophysomyces elegans. 15

RHINOCEREBRAL MM cont. A reduced conscious state or hemiplegia denotes brain involvement. Cavernous sinus and ICA thrombosis can occur reflecting the vascular tropism of the fungus. Late symptoms are indicative of poor prognosis

GASTROINTESTINAL MUCORMYCOSIS The presentation is nonspecific, with abdominal pain, distension, nausea, and vomiting. Stomach Observed in severely malnourished patients

PULMONARY MUCORMYCOSIS Presents nonspecifically with fever, dyspnea, and cough. By comparison, signs of pulmonary and GI MM are nonspecific, which leads to difficulty in diagnosis.

CUTANEOUS MUCORMYCOSIS Manifests as cellulitis that progresses to dermal necrosis and black eschar formation

DISSEMINATED MUCORMYCOSIS Accounts for 9% cases of MM and it carries high mortality. Presents with non-specific clinical symptoms and ante-mortem diagnosis is either difficult or made late which leads to rapid dissemination and death. Renal, hepatic and peritoneal involvement are also seen frequently in disseminated disease.

MUCORMYCOSIS IN HEALTHY PERSONS ??? !!!! The underlying factors for increased pathogenicity of Mucorales in apparently healthy patient may be: 1. Alteration of bacteria flora due to preceding antibiotics or 2. Sepsis induced altered immune response. History of trauma is common.

DIAGNOSIS

The diagnosis is dependant on direct histologic examination of scrapings or biopsies of involved tissue. Suggestive evidence of the disease could be found on imaging studies. Fungal cultures are occasionally positive, and blood cultures are not helpful. No serologic methods or skin tests are adequate at present for diagnosis of MM.

IMAGING CT EXAMINATION

IMAGING SPIRAL CT

IMAGING MRI

IMAGING CT CHEST

IMAGING X RAY CHEST

RHINOSCOPY

BIOPSY Pathognomonic picture of broad, irregular, nonseptate, right-angled, branching hyphae are demonstrated by H&E or fungal stains.

Vascular invasion is characteristic with neutrophil infiltrate BIOPSY Vascular invasion is characteristic with neutrophil infiltrate MM: HIGH POWER

TREATMENT

1. MEDICAL CARE A.Correction of the underlying abnormality:  Diabetic ketoacidosis requires insulin & correction of acidosis  Neutropenia requires use of CSF and withdrawal of cytotoxic CH  Wean glucocorticosteroids  Interrupt desferoxamine B. Prompt institution of IV amphotericin B therapy is critical to survival.

Amphotericin B Is the only antifungal agent with proven efficacy in 7. High doses are required, and nephrotoxicity may result. The lipid formulations of amphotericin B allows for very high doses while protecting renal function.

2. SURGICAL CARE Debridement of necrotic tissue is mandatory for survival. In rhinocerebral disease, surgical care includes drainage of the sinuses, excision of the eye, other orbital contents, and involved brain.

2. SURGICAL CARE cont. In pulmonary disease, excise lesions if they are localized to a single lobe. In cutaneous or GI diseases, excise lesions entirely.

Finally, mucormycosis carries an extremely poor prognosis Finally, mucormycosis carries an extremely poor prognosis. Because of the rapidity with which this disease progresses, prompt diagnosis and aggressive therapy are essential.

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