Black Fungus(Mucormycosis) Nepali Treatment Guidelines and More Details.

BACKGROUND

 Mucormycosis is a potentially lethal, angioinvasive fungal infection predisposed by diabetes mellitus, corticosteroids and immunosuppressive drugs, primary or secondary immunodeficiency, hematological malignancies and hematological stem cell transplantation, solid organ malignancies and solid organ transplantation, iron overload, etc
 Mucor is a fungus which is normally present in the environment and in soil. It causes disease only when immunity is critically low.
 Mucormycosis is not a new disease. It is known to occur in patients with low immunity such as in uncontrolled diabetes, post-transplant, and some cancer treatments
 It is a rare but serious infection which can be life threatening.
 Mucor infection may occur during active COVID-19 infection or a few weeks after recovery from Covid-19.
 It is not a contagious disease

Causative Agents

 Mucormycosis (previously called zygomycosis) is a serious but rare fungal infection caused by a group of molds called mucormycetes.

RISK FACTORS

  1. COVID-19 infection (Active or Post COVID)
  2. Steroid therapy: High dose and early initiation of therapy in treatment of COVID
  3. Uncontrolled Diabetes
  4. Irrational use of broad-spectrum antibiotics
  5. Chronic Kidney Disease
  6. Immunodeficiency conditions: Neutropenia, hematological malignancies, stem cell transplants, and organ transplant patients on immunosuppressants.
  7. Elevated free iron levels, deferoxamine
  8. Inappropriate use of immunosuppressants like Tocilizumab
  9. Living in dusty and damp area, stagnant area without proper ventilation
    10.Dehydration

PRESENTATION

 Any age or sex but commonly middle-aged people
 Usually 2-4 weeks of Covid-19 symptom onset. However, can appear at 10-60 days or during active covid infection

SYMPTOMS AND SIGNS

Rhino-orbito-cerebral Mucormycosis

Common Early Symptoms
 Unilateral facial pain; Focal in cheek, retro- orbital pain
 Swelling, redness around the eyes and nose: progressive anesthesia felt over cheek region or nasal mucosa
 Nasal congestion
 Bloody/black nasal discharge
 Blurring of vision, double vision
 Fever, malaise
 Dental pain or loosening of teeth

Symptoms in later stage
 Facial swelling
 Ptosis (closure of eyelids)
 Proptosis (swelling of the eyeballs)
 Diplopia, Restricted eye movements
 Chemosis
 Facial skin discolouration
 Palatal blackish discolouration or ulcer
 Other neurological symptoms, Seizure, altered sensorium , Hemiplegia (Contralateral)

Pulmonary mucormycosis

 Refractory fever, non-productive cough, progressive dyspnea, pleuritic chest pain.
Gastro-intestinal mucormycosis
 Fever, Bleeding per rectum, mass like lesion, perforation (late stage)
Cutaneous and soft tissue:
 Erythema, induration, then black eschar, muscle pain with deeper involvement
Disseminated mucormycosis:
 Symptoms vary as per site of involvement, mostly associated with pneumonia

SAMPLING FOR LAB:

 Histopathology (In 10%Formalin) – for presence of fungus
 KOH mount (in Normal Saline) – for presence of fungus
 Fungal culture (in Normal Saline) – for type of fungus

TREATMENT

  1. All mucormycosis cases should be managed in a separate ward.
  2. In any immunocompromised patient with suspected mucormycosis, immediate treatment initiation is strongly recommended.
  3. Treatment of comorbid illness/ blood sugar control & of Covid illness
    The two mainstays of treatment:
    Medical treatment with Amphotericin B and aggressive surgical debridement is the mainstay of treatment.

Antifungal:
 Injection Liposomal Amphotericin B is the drug of choice and to be started after the diagnosis is confirmed because it is effective and less nephrotoxic.
Liposomal Amphotericin B ( L-AmB)
 Rhino-orbital -5 mg/kg/ day given as infusion in 5% Dextrose over 2 hours
 Intracranial – 10 mg/kg/ day
 Amphotericin B deoxycholate ( D-AmB)
 1 – 1.5 mg/kg/d to a total dose of 2 to 4 g (Maximum cumulative dose is 5-7 gm)
 Minimum 10-14 days weeks post debridement but may need to be given for up to 6 weeks if progressive
Step down to oral Posaconazole 300mg BD on day 1 and then OD for at least 3 months

Note: Watch for toxicity: Serum creatinine, potassium and magnesium need to be monitored. In renal impairment cumulative dose needs to be reduced. Total cumulative dose: 5-7 gm) *No role of Fluconazole, voriconazole, and itraconazole as primary treatment or as prophylaxis

SURGICAL DEBRIDEMENT

(Functional endoscopy and sinus surgery and/or orbital clearance) at the earliest possible
 reduces the disease burden,
 allows better penetration of intravenous drugs,
 limits further spread of the disease
 allows intraoperative diagnosis with characteristic necrotic tissue and provides sample for histopathological and microbiological confirmation
 Glycemic control should be urgently undertaken in Diabetics.
Treatment duration:
 Mucormycosis usually requires long term treatment therapy ranging from weeks to months. If an immune defect is resolved e.g. diabetes is controlled, neutropenia definitively resolved, immunosuppression can be tapered or stopped, therapy can be continued until resolution of signs and symptoms and improvement in radiological signs. Source : EDCD, nepal

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