Top

Published in:

Open Access 01-12-2021 | Educational Review

Rhino-orbito-cerebral Mucormycosis: Pictorial Review

Authors: Vivek Pai, Rima Sansi, Ritesh Kharche, Sridevi Chaitanya Bandili, Bhujang Pai

Published in: Insights into Imaging | Issue 1/2021

Abstract

Mucormycosis (MCR) is a fulminant, potentially lethal, opportunistic fungal infection. Diabetes, immunocompromised states and elevated serum iron levels are the most important risk factors for contracting MCR infection. Recently, MCR co-infections have been observed in patients with COVID-19 disease owing to a complex interplay of metabolic factors and corticosteroid therapy. Rhino-orbito-cerebral mucormycosis (ROCM) is the most common clinical form of MCR infection and refers to infection of the nasal cavities, paranasal sinuses, neck spaces, orbits and intracranial structures. Sinonasal inoculation is typically the primary site of infection; the necrotising and angioinvasive properties of the fungus facilitate its spread into adjacent structures. In this review, we discuss the pertinent mycology and risk factors of MCR infection. The review also aims to acquaint the reader with the cross-sectional imaging appearances of ROCM and its complications. All the cases discussed in this pictorial essay are microbiologically and/or histopathologically proven cases of ROCM with concomitant COVID-19 infection.

Chung JH, Godwin JD, Chien JW, Pipavath SJ (2010) Case 160: pulmonary mucormycosis. Radiology 256(2):667–670PubMed

Therakathu J, Prabhu S, Irodi A, Sudhakar SV, Yadav VK, Rupa V (2018) Imaging features of rhinocerebral mucormycosis: a study of 43 patients. Egypt J Radiol Nucl Med 49(2):447–452

Agrawal R, Yeldandi A, Savas H, Parekh ND, Lombardi PJ, Hart EM (2020) Pulmonary mucormycosis: risk factors, radiologic findings, and pathologic correlation. Radiographics 40(3):656–666PubMed

Prakash H, Chakrabarti A (2019) Global epidemiology of mucormycosis. J Fungi 5(1):26

Ibrahim AS, Spellberg B, Walsh TJ, Kontoyiannis DP (2012) Pathogenesis of mucormycosis. Clin Infect Dis 54(1):16-S22

Rammaert B, Lanternier F, Poirée S, Kania R, Lortholary O (2012) Diabetes and mucormycosis: a complex interplay. Diabetes Metab 38(3):193–204PubMed

Spellberg B, Edwards J Jr, Ibrahim A (2005) Novel perspectives on mucormycosis: pathophysiology, presentation, and management. Clin Microbiol Rev 18(3):556–569PubMedPubMedCentral

Skiada A, Pavleas I, Drogari-Apiranthitou M (2020) Epidemiology and diagnosis of mucormycosis: an update. J Fungi 6(4):265

Salehi M, Ahmadikia K, Badali H, Khodavaisy S (2020) Opportunistic fungal infections in the epidemic area of COVID-19: a clinical and diagnostic perspective from Iran. Mycopathologia. https://doi.org/10.1007/s11046-020-00472-7CrossRefPubMedPubMedCentral

10.

Song G, Liang G, Liu W (2020) Fungal co-infections associated with global COVID-19 pandemic: a clinical and diagnostic perspective from China. Mycopathologia. https://doi.org/10.1007/s11046-020-00462-9CrossRefPubMedPubMedCentral

11.

Sarda R, Swain S, Ray A, Wig N (2021) COVID-19 associated mucormycosis: an epidemic within a pandemic. QJM. https://doi.org/10.1093/qjmed/hcab165CrossRefPubMed

12.

John TM, Jacob CN, Kontoyiannis DP (2021) When uncontrolled diabetes mellitus and severe COVID-19 converge: the perfect storm for mucormycosis. J Fungi 7(4):298

13.

Banerjee M, Pal R, Bhadada S (2021) Intercepting the deadly trinity of mucormycosis, diabetes and COVID-19 in India. Postgrad Med J. https://doi.org/10.1136/postgradmedj-2021-140537CrossRefPubMed

14.

Sen M, Lahane S, Lahane TP, Parekh R, Honavar SG (2021) Mucor in a viral land: a tale of two pathogens. Indian J Ophthalmol 69(2):244–252PubMedPubMedCentral

15.

Mallis A, Mastronikolis SN, Naxakis SS, Papadas AT (2010) Rhinocerebral mucormycosis: an update. Eur Rev Med Pharmacol Sci 14:987–992PubMed

16.

Wali U, Balkhair A, Al-Mujaini A (2012) Cerebro-rhino orbital mucormycosis: an update. J Infect Public Health 5(2):116–126PubMed

17.

Chikley A, Ben-Ami R, Kontoyiannis DP (2019) Mucormycosis of the central nervous system. J Fungi 5(3):59

18.

Munir N, Jones N (2007) Rhinocerebral mucormycosis with orbital and intracranial extension: a case report and review of optimum management. J Laryngol Otol 121(2):192–195PubMed

19.

Reid G, Lynch JP, Fishbein MC, Clark NM (2020) Mucormycosis. Semin Respir Crit Care Med 41(01):099–114

20.

Skiada A, Lass-Floerl C, Klimko N, Ibrahim A, Roilides E, Petrikkos G (2018) Challenges in the diagnosis and treatment of mucormycosis. Med Mycol 56(1):S93–S101PubMedCentral

21.

Honavar SG (2021) Code mucor: guidelines for the diagnosis, staging and management of rhino-orbito-cerebral mucormycosis in the setting of COVID-19. Indian J Ophthalmol 69(6):1361PubMedPubMedCentral

22.

Chamilos G, Lewis RE, Kontoyiannis DP (2008) Delaying amphotericin B-based frontline therapy significantly increases mortality among patients with hematologic malignancy who have zygomycosis. Clin Infect Dis 47:503–509PubMed

23.

Chan LL, Singh S, Jones D, Diaz EM Jr, Ginsberg L (2000) Imaging of mucormycosis skull base osteomyelitis. AJNR Am J Neuroradiol 21(5):828–831PubMedPubMedCentral

24.

Bhansali A, Bhadada S, Sharma A et al (2004) Presentation and outcome of rhino-orbital-cerebral mucormycosis in patients with diabetes. Postgrad Med J 80(949):670–674PubMedPubMedCentral

25.

DelGaudio JM, Swain RE Jr, Kingdom TT, Muller S, Hudgins PA (2003) Computed tomographic findings in patients with invasive fungal sinusitis. Arch Otolaryngol Head Neck Surg 129(2):236PubMed

26.

Safder S, Carpenter JS, Roberts TD, Bailey N (2009) The “Black Turbinate” sign: an early MR imaging finding of nasal mucormycosis. AJNR Am J Neuroradiol 31(4):771–774PubMed

27.

Horger M, Hebart H, Schimmel H et al (2006) Disseminated mucormycosis in haematological patients: CT and MRI findings with pathological correlation. Br J Radiol 79(945):88-e95

28.

Han Q, Escott E (2019) The black turbinate sign, a potential diagnostic pitfall: evaluation of the normal enhancement patterns of the nasal turbinates. AJNR Am J Neuroradiol 40(5):855–861PubMedPubMedCentral

29.

Gillespie MB, O’Malley BW Jr, Francis HW (1998) An approach to fulminant invasive fungal rhinosinusitis in the immunocompromised host. Arch Otolaryngol Head Neck Surg 124:520–526PubMed

30.

Ferry AP, Abedi S (1983) Diagnosis and management of rhino-orbitocerebral mucormycosis (phycomycosis): a report of 16 personally observed cases. Ophthalmology 90(9):1096–1104PubMed

31.

Yohai RA, Bullock JD, Aziz AA, Markert RJ (1994) Survival factors in rhino-orbital-cerebral mucormycosis. Surv Ophthalmol 39(1):3–22PubMed

32.

Slonimsky G, McGinn JD, Goyal N et al (2020) A model for classification of invasive fungal rhinosinusitis by computed tomography. Sci Rep 10(1):12591PubMedPubMedCentral

33.

Rumboldt Z, Castillo M (2002) Indolent intracranial mucormycosis: case report. AJNR Am J Neuroradiol 23(6):932–934PubMedPubMedCentral

34.

Peterson KL, Wong M, Canalis RF, Abemayor E (1997) Rhinocerebral mucormycosis: evolution of the disease and treatment options. Laryngoscope 107:855–862PubMed

35.

Nunes DM, Rocha AJ, Rosa Júnior MR, da Silva CJ (2012) “Black turbinate sign”: a potential predictor of mucormycosis in cavernous sinus thrombophlebitis. Arq Neuropsiquiatr 70(1):78PubMed

36.

Aribandi M, McCoy VA, Bazan C (2007) Imaging features of invasive and noninvasive fungal sinusitis: a review. Radiographics 27(5):1283–1296PubMed

37.

Gamba JL, Woodruff WW, Djang WT, Yeates AE (1986) Craniofacial mucormycosis: assessment with CT. Radiology 160(1):207–212PubMed

38.

Silverman CS, Mancuso AA (1998) Periantral soft-tissue infiltration and its relevance to the early detection of invasive fungal sinusitis: CT and MR findings. AJNR Am J Neuroradiol 19:321–325PubMedPubMedCentral

39.

Mossa-Basha M, Turan Ilıca A, Maluf F, Karakoç O, İzbudak I, Aygün N (2013) The many faces of fungal disease of the paranasal sinuses: CT and MRI findings. Diagn Interv Radiol 19:195–200PubMed

40.

Hosseini SMS, Borghei P (2005) Rhinocerebral mucormycosis: pathways of spread. Eur Arch Otorhinolaryngol 262(11):932–938PubMed

41.

Middlebrooks EH, Frost CJ, De Jesus RO, Massini TC, Schmalfuss IM, Mancuso AA (2015) Acute invasive fungal rhinosinusitis: a comprehensive update of CT findings and design of an effective diagnostic imaging model. AJNR Am J Neuroradiol 36(8):1529–1535PubMedPubMedCentral

42.

Chapman PR, Choudhary G, Singhal A (2021) Skull base osteomyelitis: a comprehensive imaging review. AJNR Am J Neuroradiol 42(3):404–413PubMed

43.

McLean FM, Ginsberg LE, Stanton CA (1996) Perineural spread of rhinocerebral mucormycosis. AJNR Am J Neuroradiol 17:114–116PubMedPubMedCentral

44.

Frater JL, Hall GS, Procop GW (2001) Histologic features of zygomycosis. Emphasis on perineural invasion and fungal morphology. Arch Pathol Lab Med 125:375–378PubMed

45.

Sravani T, Uppin SG, Uppin MS, Sundaram C (2014) Rhinocerebral mucormycosis: pathology revisited with emphasis on perineural spread. Neurol India 62(4):383–386PubMed

46.

Press GA, Weindling SM, Hesselink JR, Ochi JW, Harris JP (1988) Rhinocerebral mucormycosis: MR manifestations. J Comput Assist Tomogr 12:744–749PubMed

47.

Orguc S, Yücetürk AV, Akif Demir M, Goktan C (2005) Rhinocerebral mucormycosis: Perineural spread via the trigeminal nerve. J Clin Neurosci 12(4):484–486PubMed

48.

Dankbaar JW, Pameijer FA, Hendrikse J, Schmalfuss IM (2018) Easily detected signs of perineural tumour spread in head and neck cancer. Insights Imaging 9:1089–1095PubMedPubMedCentral

49.

Bae MS, Kim EJ, Lee KM, Choi WS (2012) Rapidly progressive rhino-orbito-cerebral mucormycosis complicated with unilateral internal carotid artery occlusion: a case report. Neurointervention 7(1):45–49PubMedPubMedCentral

50.

Anders UM, Taylor EJ, Martel JR, Martel JB (2015) Acute orbital apex syndrome and rhino-orbito-cerebral mucormycosis. Int Med Case Rep J 8:93–96PubMedPubMedCentral

51.

Chaulk AL, Do TH, Supsupin EP, Bhattacharjee MB, Richani K, Adesina OOO (2021) A unique radiologic case of optic nerve infarction in a patient with mucormycosis. J Neuroophthalmol. https://doi.org/10.1097/WNO.0000000000001179CrossRefPubMed

52.

Ghuman MS, Kaur S, Bhandal SK, Ahluwalia A, Saggar K (2015) Bilateral optic nerve infarction in rhino-cerebral mucormycosis: A rare magnetic resonance imaging finding. J Neurosci Rural Pract 6(3):403–404PubMedPubMedCentral

53.

Alsuhaibani AH, Al-Thubaiti G, Al Badr FB (2012) Optic nerve thickening and infarction as the first evidence of orbital involvement with mucormycosis. Middle East Afr J Ophthalmol 19(3):340–342PubMedPubMedCentral

54.

Mathur S, Karimi A, Mafee MF (2007) Acute optic nerve infarction demonstrated by diffusion-weighted imaging in a case of rhinocerebral mucormycosis. AJNR Am J Neuroradiol 28(3):489–490PubMedPubMedCentral

55.

Lee BL, Holland GN, Glasgow BJ (1996) Chiasmal infarction and sudden blindness caused by mucormycosis in AIDS and diabetes mellitus. Am J Ophthalmol 122(6):895–896PubMed

56.

Merkler AE, Duggal I, Kaunzner U et al (2016) Rapidly progressive bilateral optic nerve and retinal infarctions due to rhinocerebral mucormycosis and pseudoephedrine use. Neurol Clin Pract 6(6):549–552PubMedPubMedCentral

57.

Borruat FX, Bogousslavsky J, Uffer S, Klainguti G, Schatz NJ (1993) Orbital infarction syndrome. Ophthalmology 100(4):562–568PubMed

58.

Mazzai L, Anglani M, Giraudo C, Martucci M, Cester G, Causin F (2021) Imaging features of rhinocerebral mucormycosis: from onset to vascular complications. Acta Radiol. https://doi.org/10.1177/0284185120988828CrossRefPubMed

59.

Sandron J, Hantson Ph, Duprez T (2020) Intracranial brain parenchymal spread of mucormycosis through olfactory tract: a diffusion-weighted imaging-based concept. Acta Radiol Open 9(12):2058460120980999PubMedPubMedCentral

60.

Siegal JA, Cacayorinb ED, Nassif AS et al (2000) Cerebral mucormycosis: proton MR spectroscopy and MR imaging. Magn Reson Imaging 18(7):915–920PubMed

61.

Maheshwari S, Patil M, Shendey S (2019) Mucormycosis creeping along the nerves in an immunocompetent individual. J Radiol Case Rep 13(10):1–10PubMedPubMedCentral

62.

Pai V, Khan I, Sitoh YY, Purohit B (2020) Pearls and pitfalls in the magnetic resonance diagnosis of dural sinus thrombosis: a comprehensive guide for the trainee radiologist. J Clin Imaging Sci 10:77PubMedPubMedCentral

63.

Ma J, Jia R, Li J et al (2015) Retrospective clinical study of eighty-one cases of intracranial mucormycosis. J Glob Infect Dis 7(4):143–150PubMedPubMedCentral

Title: Rhino-orbito-cerebral Mucormycosis: Pictorial Review
Authors: Vivek Pai
Rima Sansi
Ritesh Kharche
Sridevi Chaitanya Bandili
Bhujang Pai
Publication date: 01-12-2021
Publisher: Springer International Publishing
Published in: Insights into Imaging / Issue 1/2021
Electronic ISSN: 1869-4101
DOI: https://doi.org/10.1186/s13244-021-01109-z

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Rhino-orbito-cerebral Mucormycosis: Pictorial Review

Abstract

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Please log in to get access to this content

Other articles of this Issue 1/2021

Low-grade serous epithelial ovarian cancer: a comprehensive review and update for radiologists

Tibial torsion analysis in computed tomography: development and validation of a real 3D measurement technique

Dual-energy CT angiography reveals high prevalence of perfusion defects unrelated to pulmonary embolism in COVID-19 lesions

Overuse-related instability of the elbow: the role of CT-arthrography

Lessons learned during implementation of MR-guided High-Intensity Focused Ultrasound treatment of uterine fibroids

Imaging of congenital lung diseases presenting in the adulthood: a pictorial review