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October 07, 2021
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Outbreak of mucormycosis seen in India during COVID pandemic

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Mucormycosis is an opportunistic fungal infection that manifests in patients with low immunity. Although the fungus spores are ubiquitous in nature and found in the environment, fungal infections rarely occur in immunocompetent individuals.

Amar Agarwal
Amar Agarwal

In patients with low immunity, the spores are harbored in the lungs and nasal sinuses and proliferate further, leading to widespread tissue necrosis with devastating consequences. The disease has a predilection for patients with diabetes or immunodeficiency diseases and patients on steroids, immunomodulators and immunosuppressant drugs. During the COVID pandemic, a spurt of mucormycosis cases was seen in India, attributed to diabetes, steroids, overuse of zinc and vitamin tablets, and prolonged oxygen therapy. However, many patients presented with mucormycosis in the emergency department without a history of COVID or any other predisposing factors.

Presentation

Depending upon the organ involved, mucormycosis can be broadly categorized into rhinocerebral (most common), sino-orbital, pulmonary, cutaneous, gastrointestinal or disseminated mucor infection.

Priya Narang
Priya Narang

In rhinocerebral mucormycosis, following the invasion of the nasal mucosa, the fungal organism may further spread and involve the orbit or extend into the cranial cavity. The fungal hyphae can directly involve the blood vessels, leading to infarction and tissue necrosis. Therefore, the progression of mucormycosis might be rapid and aggressive in nature. Patients often presented with watering and congestion of the eyes and headache typically after 2 weeks of COVID infection. Few patients presented with an eschar that developed due to tissue necrosis on the palate, which is characteristic of maxillary sinus involvement. Orbital involvement frequently occurs after involvement of the ethmoidal sinus, which eventually leads to orbital apex syndrome and further extension to the cavernous sinus.

Proptosis of the left eyeball
Figure 1. Multislice CT scans. Proptosis of the left eyeball with diffuse heterogeneously enhancing altered signals in the left periorbital, preseptal, intraconal and retrobulbar regions predominantly involving the superior, medial and lateral compartments of the left orbit. Diffuse heterogeneously enhancing altered signals in the frontal, ethmoidal, left maxillary and left sphenoid sinuses, suggestive of diffuse pansinusitis.

Source: Amar Agarwal, MS, FRCS, FRCOphth, and Priya Narang, MS

Investigations

Imaging with MRI forms one of the major investigative approaches to diagnose fungal infection. It also helps to delineate the extent of tissue involvement and guide the debridement procedure for eventual surgery. The sinus and the infected area demonstrate lack of enhancement in the sinus mucosa as well as the affected tissue along with fluid level in the sinuses and destruction of bone. Tissue biopsy and histopathological analysis help to confirm the clinical diagnosis.

Postoperative status with removal of left turbinate
Figure 2. Postoperative status with removal of left turbinate and medial wall of left maxillary sinus.

Treatment

The initial medical line of treatment comprises infusion of antifungal drugs amphotericin B (lipid based) or posaconazole. Surgical debridement is recommended in the early stages to limit the spread of fungal infection as systemic medications are often not effective due to destruction of blood vessels and necrosis in the affected area. Aggressive surgical debridement should be performed as early as possible, and it is a rule to debride until fresh bleeding is observed in the affected area as necrosed tissues do not bleed. Orbital exenteration is often performed in advanced cases to limit the mortality rate.

Prompt diagnosis and surgical debridement form the cornerstone of therapy for mucormycosis. Cosmetic disfigurement often leads to emotional trauma in young patients. Rehabilitation and psychological counseling should be considered for these extreme cases.