Introduction

Mucormycosis is an aggressive opportunistic fungal infection. The fungus that causes the disease is ubiquitous in nature and is found in soil and on decaying vegetation.1 Because the fungus is so widespread, humans are exposed to it on a regular basis. The spores of the fungus are inhaled through the mouth and nose, but infection rarely occurs in a person with an intact immune system because macrophages phagocytize the spores. However, an immunocompromised individual is unable to mount an effective immune response against the inhaled spores; thus, germination and hyphae formation occur and infection develops, most commonly in the sinuses.2 They may into the orbit or brain parenchyma, causing sino-orbital and/or rhino-orbital-cerebral mucormycosis, respectively.3

A drastic spike of cases of mucormycosis have been seen during this COVID-19 pandemic. Because mucormycosis may involve the orbit and other ocular structures, the ophthalmologist may be the first physician to see a patient with this highly morbid condition.

Materials and Methods

We performed a prospective, Interventional study on 40 patients admitted in mucor ward of a tertiary centre who had mucormycosis and concurrent or past history of COVID-19 infection. Patients with any symptoms or signs suspicious of mucormycosis were evaluated at presentation with a detailed history, clinical signs, ENT, ophthalmic, and neurological examination to assess the extent of disease followed by diagnostic nasal endoscopy and KOH mount was sent.4 Investigations for the treatment plan include CT-PNS, MRI (Brain+Orbit+PNS) and histhopathology. Wherein Histopathology was useful for confirming the diagnosis and radiological imaging to know the extent of spread of the disease and help in surgical plan.5

Results

Clinical presentation

Ocular signs and symptoms encountered in 23 patients (57.5%). Rest 17 patients showed ophthalmic examination within normal limits though they had ENT or dental symptoms or signs. Varied Ocular symptoms and signs seen are listed below:

Table 2

Ocular signs and symptoms

Symptoms and Signs	Number out of 40	%
1. Orbital Pain	2	5
2. Decreased vision	2	5
3. No PL	9	22.5
4. Lid Oedema	5	12.5
5. Ptosis	13	32.5
6. Conjunctival Chemosis	6	15
7. RAPD	8	20
8. Movement Restriction	5	12.5
9. Complete Ophthalmoplegia	9	22.5
10. Eschar	2	5
11. Proptosis	10	25
12. CRAO	2	5

Figure 1

Left eye of patient showing proptosis, conjunctival chemosis & eschar

Imaging

15 (37.5%) out of the total patients showed normal orbital findings on imaging. Imaging of 7 patients could not be due to low general health condition (ventilated or tracheostomised) who eventually succumbed. 18 Imaging films showed orbital involvement which are enlisted as follows:

Table 3

Percentage of various findings seen on MRI Orbit

Findings	Number out of 18	%
Extraconal medial wall fat stranding	6	33.3
Extraconal inferior wall fat stranding	6	33.3
Retrobulbar Space	4	22.2
Bulky EOM	3	16.6
Orbital Apex	2	11.1
Both Intraconal + Extraconal fat stranding	2	11.1
Extraconal lateral wall fat stranding	1	5.5
Orbital Cellulitis	1	5.5

Figure 2

MRI (Brain+Orbit+PNS) coronal view (A) & axial view (B) showing extensive fat stranding in right orbit

Discussion

Mucormycosis is amongst the most fulminant form of Zygormycosis caused by Mucorales species of the phylum Zygomycota.6

The studies for pathogenesis of the infection show that there are alterations in cell-mediated immunity, such as chemotaxis, phagocytosis and cytokine secretion in diabetics. Thus T-cells (CD4+ and CD8+) that produce cytokines such as interleukin (IL) 4, IL-10, IL-17 and interferon-gamma (IFN-γ) and damage the fungal hyphae lack in such patients leading to pre-disposition to infection.7 Also procoagulant state and vasoconstriction lead to ischaemia and hence necrosis

Early sign and symptoms of mucormycosis include fever, sinusitis, nasal discharge, epistaxis, orbital and periorbital pain, nasal mucosal ulceration, crusting, and necrosis.8 A black eschar over skin, nasal mucosa, or palate discoloration are characteristic of mucormycosis. Decreased vision, proptosis, periorbital edema, and complete external ophthalmoplegia are the most common ophthalmic features. Other ophthalmic signs and symptoms include ptosis, chemosis, congestion, internal ophthalmoplegia, and corneal anesthesia.9 Sudden blindness can occur due to central retinal artery occlusion, thrombosis of posterior ciliary arteries, infarction of the intraorbital part of optic nerve, or direct fungal invasion of the intracranial part of the optic nerve or optic chiasm.10 It can also present less commonly as a painless orbital apex syndrome without any signs of orbital cellulitis.11 From the orbit, the infection can spread to brain through cribriform plate and orbital apex. Invasion of the cavernous sinus and cavernous part of carotid artery can lead to carotid occlusion, cerebral infarction, intracranial aneurysm/hemorrhage, fungal meningitis, mycotic abscess, and, eventually, death.12

A scoring system was devised by a team of experienced ENT surgeons and Ophthalmologists in from prior experience in managing mucormycosis.13

The scoring system is based on 3 main criteria, namely:

Severity marked as :

If the total score,

For Conservative Management, Luna and colleagues studies show that they have treated a patient with orbital mucormycosis by direct irrigation of amphotericin B into the muscle cone. 14

Conclusion

Mucormycosis is a severe, emergent and fatal infection requiring multidisciplinary management. Early diagnosis and urgent antifungal treatment associated to surgery with joint care are of extreme importance for successful eradication of infection and for patient survival and decrease residual morbidities. In order to achieve this, screening of patients with high risk factors should be done. Simple tests like vision, pupil, ocular motility and sinus tenderness can be a part of routine physical evaluation in Covid-19 hospitalised patients and home quarantined patients should be made aware of the early symptoms.

Source of Funding

None.

Conflict of Interest

The authors declare no conflict of interest.