Introduction: A sharp upsurge in the number of coronavirus disease-associated mucormycosis cases was noted during the second wave of coronavirus disease in India. The fungal hyphae spread from the nasal mucosa, orbit to the brain, hence otherwise called rhino-orbito-cerebral mucormycosis (ROCM). Prompt diagnosis and early initiation of treatment with amphotericin B, aggressive surgical debridement of the PNS, and orbital exenteration, where indicated, are essential for successful outcome. This study is done to enumerate the clinical, radiological, and histopathological findings of post-exenterated specimens of ROCM. Methods: This is a non-randomized cross-sectional study conducted at a tertiary care center. Ten post-exenterated specimens were examined histopathologically for the involvement of the central retinal artery, superior ophthalmic vein (SOV), optic nerve, muscle, and orbital fat showing necrosis. Clinical findings of these 10 patients were studied retrospectively for visual loss, proptosis, extraocular movements for muscle involvement, scleral necrosis, and fundus findings for artery or vein occlusions. Preoperative magnetic resonance imaging (MRI) findings like the number of orbital quadrants showing soft tissue thickening or tissue enhancement, diffusion restriction in the optic nerve, soft tissue enhancement seen at the orbital apex or superior orbital fissure, loss of flow void in the left internal carotid artery, enlargement of the SOV containing filling defect on post-contrast images were noted. Results: Optic nerve involvement was seen in 100% patients clinically, 70% on MRI, and 50% on histopathological examination (HPE). Muscle involvement was seen in 100% patients clinically, 90% on MRI, and 80% on HPE. None of the patients had scleral necrosis clinically. Scleral tenting was seen in 20% patients on MRI and 20% on HPE. Clinically, central retinal artery involvement was seen in 40%, and cilioretinal artery involvement was seen in 10%. MRI could not give much information on artery involvement. Clinically, none of them had central retinal vein occlusion. 20% had an enlarged SOV seen on MRI. HPE showed vascular involvement in 90% of the specimens. Conclusion: Clinical, radiological, and histopathological findings play a major role in the diagnosis, staging, and follow-up of ROCM.

Journal Section:
Research Article
Keywords:
Exenterated specimens, Histopathological examination, Magnetic resonance imaging, Mucormycosis
1.
Kwon-Chung KJ. Taxonomy of fungi causing mucormycosis and entomophthoramycosis (zygomycosis) and nomenclature of the disease: molecular mycologic perspectives. Clin Infect Dis. 2012 Feb;54(Suppl 1):S8–15.
https://doi.org/10.1093/cid/cir864
2.
Sen M, Lahane S, Lahane TP, Parekh R, Honavar SG. Mucor in a viral land: a tale of two pathogens. Indian J Ophthalmol. 2021 Feb;69(2):244–52.
https://doi.org/10.4103/ijo.IJO_3774_20
3.
Wali U, Balkhair A, Al-Mujaini A. Cerebro-rhino orbital mucormycosis: an update. J Infect Public Health. 2012 Apr;5(2):116–26.
https://doi.org/10.1016/j.jiph.2012.01.003
4.
Mallis A, Mastronikolis SN, Naxakis SS, Papadas AT. Rhinocerebral mucormycosis: an update. Eur Rev Med Pharmacol Sci. 2010 Nov;14(11):987–92.
5.
Chikley A, Ben-Ami R, Kontoyiannis DP. Mucormycosis of the central nervous system. J Fungi. 2019 Jul 8;5(3):59.
https://doi.org/10.3390/jof5030059
6.
Munir N, Jones NS. Rhinocerebral mucormycosis with orbital and intracranial extension: a case report and review of optimum management. J Laryngol Otol. 2007 Feb;121(2):192–5.
https://doi.org/10.1017/S0022215106003409
7.
Reid G, Lynch JP3rd, Fishbein MC, Clark NM. Mucormycosis. Semin Respir Crit Care Med. 2020 Feb;41(1):99–114.
https://doi.org/10.1055/s-0039-3401992
8.
Sen M, Honavar SG, Bansal R, Sengupta S, Rao R, Kim U, et al. Epidemiology, clinical profile, management, and outcome of COVID-19-associated rhino-orbital-cerebral mucormycosis in 2,826 patients in India -Collaborative OPAI-IJO Study on Mucormycosis in COVID-19 (COSMIC), Report 1. Indian J Ophthalmol. 2021 Jul;69(7):1670–92.
https://doi.org/10.4103/ijo.IJO_1565_21
9.
Sreshta K, Dave TV, Varma DR, Nair AG, Bothra N, Naik MN, et al. Magnetic resonance imaging in rhino-orbital-cerebral mucormycosis. Indian J Ophthalmol. 2021 Jul;69(7):1915–27.
https://doi.org/10.4103/ijo.IJO_1439_21
10.
Hayreh SS, Zimmerman MB. Fundus changes in central retinal artery occlusion. Retina. 2007 Mar;27(3):276–89.
https://doi.org/10.1097/01.iae.0000238095.97104.9b
11.
Flaxel CJ, Adelman RA, Bailey ST, Fawzi A, Lim JI, Vemulakonda GAet al. Retinal and ophthalmic artery occlusions preferred practice pattern®. Ophthalmology. 2020 Feb;127(2):P259–87.
https://doi.org/10.1016/j.ophtha.2019.09.028
12.
Wani N, Jehangir M, Lone P. Rhino-orbito-cerebral mucormycosis: magnetic resonance imaging. Indian JOtol. 2015;21(3):215–8.
https://doi.org/10.4103/0971-7749.159700
13.
Shah K, Dave V, Bradoo R, Shinde C, Prathibha M. Orbital exenteration in rhino-orbito-cerebral mucormycosis: a prospective analytical study with scoring system. Indian J Otolaryngol Head Neck Surg. 2019 Jun;71(2):259–65.
https://doi.org/10.1007/s12070-018-1293-8
14.
Herrera DA, Dublin AB, Ormsby EL, Aminpour S, Howell LP. Imaging findings of rhinocerebral mucormycosis. Skull Base. 2009 Mar;19(2):117–25.
https://doi.org/10.1055/s-0028-1096209
15.
Ferry AP, Abedi S. Diagnosis and management of rhino-orbitocerebral mucormycosis (phycomycosis). A report of 16 personally observed cases. Ophthalmology. 1983;90(9):1096–104.
https://doi.org/10.1016/s0161-6420(83)80052-9
16.
Downie JA, Francis IC, Arnold JJ, Bott LM, Kos S. Sudden blindness and total ophthalmoplegia in mucormycosis. A clinicopathological correlation. J Clin Neuro Ophthalmol. 1993;13(1):27–34.
17.
Mathur S, Karimi A, Mafee MF. Acute optic nerve infarction demonstrated by diffusion-weighted imaging in a case of rhinocerebral mucormycosis. AJNR Am J Neuroradiol. 2007;28:489–90.
18.
Badakere A, Patil-Chhablani P. Orbital apex syndrome: a review. Eye Brain. 2019;11:63–72.
https://doi.org/10.2147/EB.S180190
19.
Alsuhaibani AH, Al-Thubaiti G, Al Badr FB. Optic nerve thickening and infarction as the first evidence of orbital involvement with mucormycosis. Middle East Afr J Ophthalmol. 2012;19(3):340–2.
https://doi.org/10.4103/0974-9233.97957
20.
Kohn R, Hepler R. Management of limited rhino-orbital mucormycosis without exenteration. Ophthalmology. 1985;92(10):1440–4.
https://doi.org/10.1016/s0161-6420(85)33844-7
21.
Sarkies N. Traumatic optic neuropathy. Eye. 2004;18(11):1122–5.
https://doi.org/10.1038/sj.eye.6701571
22.
Tsutsumi S, Ono H, Ishii H. Central retinal artery delineation using magnetic resonance imaging. Surg Radiol Anat. 2022 May;44(5):727–32.
https://doi.org/10.1007/s00276-022-02947-2
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