Rhino-Orbital-Cerebral-Mucormycosis: The Importance of Urgent Assessment
Antony C Boynes1,2*, Ruben Schuringa1,3, Doron G Hickey1,2, Gemma Reynolds1,3, Jwu Jin Khong 1,2
1 Austin Health, 145 Studley Rd, Heidelberg, VIC 3084, Australia
2 The Royal Victorian Eye and Ear Hospital, 32 Gisborne St, East Melbourne, VIC 3002, Australia
3 The University of Melbourne, Parkville, VIC 3010, Australia
*Corresponding author : Antony C Boynes, Royal Victorian Eye and Ear Hospital, 32 Gisborne Street, East Melbourne, VIC 3002, Australia
Received Date: 12 January 2023
Accepted Date: 30 January 2023
Published Date: 01 February 2023
Citation : Boynes AC, Schuringa R, Hickey DG, Reynolds G, Khong JJ (2023) Rhino-Orbital-Cerebral-Mucormycosis: The Importance of Urgent Assessment. Ann Case Report. 8: 1148. DOI:https://doi.org/10.29011/2574-7754.101148
Abstract
A case report highlighting the importance of early assessment and intervention in treating ROCM. This paper is particularly relevant as we are seeing a concerning increase in the incidence of ROCM associated to COVID-19.
A 67 year-old female had a delayed diagnosis of ROCM confirmed with radiological and microbiological findings. Her predisposing risk factors included end-stage renal disease, aplastic anaemia with neutropenia, and iron overload requiring deferoxamine infusions. Management included surgical debridement of paranasal sinuses, orbital exoneration and systemic antifungal treatment. The disease progressed despite this management, with the development of multifocal fungal cerebritis and superimposed ischaemic infarcts. The patient died five days after the cessation of treatment. ROCM is a life-threatening emergency. ROCM should be considered in all at risk patients who present with common sinus symptoms and disproportionate facial pain, especially when associated with red flags such as ophthalmoplegia, vision loss and facial numbness.
Introduction
This case report adds to current knowledge by highlighting less well-known risk factors for poor prognosis in rhino-orbital-cerebral-mucormycosis (ROCM) such as end-stage renal failure and deferoxamine infusions. It describes recent changes in the evidence-based management of ROCM including topical amphotericin B, which is gaining increasing popularity. This report is particularly relevant as we are seeing a concerning increase in the incidence of ROCM associated to COVID-19. Written patient consent was provided and this report adhered to the tenets of the Declaration of Helsinki.
Case Presentation
A 67-year-old female presented to an Emergency Department with two days of left-sided facial pain and headache. She was diagnosed with acute-on-chronic sinusitis based on computerized tomography (CT) and treated with oral Augmentin duo (500/125mg BD). Her medical history included end-stage renal disease secondary to polycystic kidney disease, requiring haemodialysis; transfusion-dependent aplastic anaemia and associated iron overload, treated with deferoxamine infusions; and left middle cerebral artery (MCA) aneurysm clipping. She suddenly deteriorated two days later whilst an inpatient, developing left ptosis, proptosis, periorbital discolouration, ophthalmoplegia, and an absent corneal reflex. Her best corrected visual acuity (BCVA) was 6/9 in both eyes. A CT showed enlargement of the left medial and inferior rectus muscles alongside early orbital fat stranding and left maxillary and ethmoidal sinus opacification. Her medical care was transferred to our hospital with a provisional diagnosis of orbital cellulitis. On the initial ophthalmology assessment, her BCVA was 6/6 in the right eye and light perception in the left eye. She had a left relative afferent pupillary defect and complete ptosis. Examination revealed 3mm of proptosis in the left eye and complete ophthalmoplegia (Figure 1). In addition, reduced sensation in the V1 distribution had resulted in an absent corneal reflex. The posterior segment examination in both eyes was grossly normal. Otorhinolaryngology assessment identified necrotic tissue underlying the sphenoid and ethmoid sinuses, and throughout the left turbinate’s. Magnetic resonance imaging (MRI) showed inflammatory changes compatible with acute invasive fungal sinusitis involving the left optic nerve. There was significant artefact over the cavernous sinus due to previous MCA aneurysm clipping (Figure 2). A provisional diagnosis of mucormycosis was made, and she was commenced on intravenous amphotericin, ceftazidime and flucloxacillin. That same day, paranasal sinus debridement and orbital exploration were performed, during which extensive tissue necrosis including the left turbinate’s, maxillary/ethmoid sinuses, internal maxillary artery, extraconal fat and medial rectus were noted. Intraoperative tissue samples were consistent with mucor species showing angioinvasive fungal hyphae with extensive necrosis extending to the orbital apex (Figure 3). A diagnosis of ROCM was made, as was a decision to proceed to left orbital exoneration with amphotericin onlay at the orbital apex and orbital fissures, alongside further debridement of left paranasal sinuses. Postoperatively she experienced ongoing fever and became increasingly confused. A postoperative MRI showed features consistent with multifocal fungal cerebritis with superimposed ischaemic infarcts. As this represented a terminal event, active treatment was withdrawn, and the patient died five days later.
Figure 1: Patient photograph on initial ophthalmology assessment with evidence of left ptosis, proptosis and periorbital discolouration.
Figure 2: Pre-operative MRI brain/orbits (A) T1 coronal view showing opacification of the left maxillary and ethmoid sinuses as well as thickening of the inferior and medial rectus muscles (white arrows). (B) MRI brain/orbits T1 axial view showing inflammatory changes involving the intraconal optic nerve and thickening of the left medial rectus muscles. Image quality at the cavernous sinus and orbital apex is degraded by artefact related to middle cerebral artery metallic clip (dashed arrow).