PMC6180894_01
The patient's orbital area photographs show left periorbital oedema and swelling on the dorsal aspect of the root of the nose (a), lower conjunctival oedema with mild proptosis of the mucosa and a fixed and dilated pupil (b).
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On examination (Figure 1a,b), there was left periorbital oedema, swelling on the dorsal aspect of the root of the nose and lower conjunctival oedema with mild proptosis of the mucosa.
(a-c) Axial and coronal CT images of the paranasal sinuses and orbits demonstrate mild mucosal thickening of the left maxillary (c) and left ethmoid (a, b) sinuses, proptosis of the left globe, retro-orbital fat stranding, soft tissue thickening along the medial wall of the orbit and subtle bony erosion in the left lamina papyracea (a), bony defect in the alveolar margin of the maxilla at the root of the left incisor tooth (b) and an oroantral fistula (c).
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CT scan of the paranasal sinuses (Figure 2a-c) demonstrated mild mucosal thickening of the left maxillary and ethmoid sinuses, bony defect in the alveolar margin of the maxilla at the root of the left incisor tooth and an oroantral fistula.
Axial T
1 weighted images show fat stranding in the retro-orbital, orbital apex and left cavernous sinus regions with minimal proptosis of the left globe (a). Axial T
2 weighted fat-saturated images (b, c) show extensive fat stranding in the retro-orbital region, ill-defined isointense to hypointense soft tissue along the medial wall of the left orbit and intraconal retro-orbital space extending to the orbital apex. On short tau inversion-recovery coronal images (d), the extraocular muscles appear oedematous. Left internal carotid artery flow void is small in calibre with adjacent soft tissue thickening in the left cavernous sinus (a-c). A small focal hyperintense collection is seen in the left preseptal space (c, d).
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 T 2 weighted fat-saturated axial images (Figure 3a,b) and short tau inversion-recovery coronal images (Figure 3d) demonstrated extensive fat stranding in the retro-orbital region; ill-defined isointense to hypointense soft tissue along the medial wall of the left orbit and the intraconal retro-orbital space, which extended to the orbital apex.
Diffusion-weighted images show restricted diffusion in the two intraorbital lesions (including the encased optic nerve) with very low apparent diffusion coefficient values (0.45 x 10-3 mm2 s-1). The preseptal lesion does not show any restriction of diffusion (a, b). Multiple small acute infarcts are seen in the left corona radiata and left parietal lobe with apparent diffusion coefficient value similar to the orbital lesions (0.407 x 10-3 mm2 s-1) (c-e).
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Diffusion-weighted images (Figure 4a-e) showed restricted diffusion in the two intraorbital lesions (including the encased optic nerve) with low apparent diffusion coefficient values (0.45 x 10-3 mm2 s-1).
Susceptibility weighted imaging demonstrates blooming in the two intraorbital images (a). MR angiography demonstrates narrowing of the left internal carotid artery from its origin, involving both the intracranial and the extracranial portions (b).
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Susceptibility weighted imaging (Figure 5a) demonstrated blooming in the two intraorbital images. MR angiography (Figure 5b) demonstrated narrowing of the left internal carotid artery from its origin, involving both the intracranial and extracranial portions.
Post-contrast T
1 weighted fat-saturated axial, sagittal and coronal images demonstrate three peripherally enhancing lesions in the left orbit. A 15 x 8 x 10 mm lesion is seen in the extraconal space on the medial aspect of the orbit subperiosteally, displacing the medial rectus laterally with subtle enhancement in the left ethmoid sinus (a). A 33 x 18 x 15 mm intraconal retro-orbital lesion communicates with the lesion along the medial wall and extends to the orbital apex and left cavernous sinus, with the intracranial component measuring 7 mm (a, c). The left optic nerve is encased by the intraconal lesion with enhancement in the distal part of the optic nerve close to insertion at the globe (a, b). The enhancement is seen to extend along the left internal carotid artery, which has a small calibre with thickened wall (a-d). There is also an inferior extension into the left pterygopalatine fossa. The third lesion is seen in the lower preseptal space measuring 23 x 7x 9 mm, and shows signal intensity similar to fluid with peripheral enhancement (b).
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Post-contrast fat-saturated T 1 weighted images (Figure 6a-d) demonstrated three peripherally enhancing lesions in the left orbit. A 15 x 8 x 10 mm lesion was seen in the extraconal space on the medial aspect of the orbit subperiosteally, displacing the medial rectus laterally with subtle enhancement in the left ethmoid sinus (Figure 6a). A 33 x 18 x 15 mm intraconal retro-orbital lesion was seen communicating with the lesion along the medial wall and extending to the orbital apex and the left cavernous sinus, with the intracranial component measuring 7 mm (Figure 6a,c). The left optic nerve was encased by the intraconal lesion with enhancement in the distal part of the optic nerve close to its insertion into the globe (Figure 6a,b). The enhancement was seen to extend along the left internal carotid artery, which had a small calibre with a thickened wall (Figure 6a-d). There was also inferior extension into the left pterygopalatine fossa (Figure 6b).
Haematoxylin and eosin staining of tissue samples from the left orbit and orbital apex show broad, aseptate, obtuse-angle branching hyphal elements amid necrotic tissue when viewed with 40x magnification (a). They also show epithelioid granuloma with giant cells and lymphocyte predominant inflammatory cells when viewed with 10x magnification (b). The presence of fungal elements is confirmed with periodic acid-Schiff staining when viewed with 10x magnification (c).
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Tissue samples from the left orbit and orbital apex on haematoxylin and eosin staining showed necrotic tissue with inflammatory cells composed of neutrophils, lymphocytes, plasma cells, histiocytes and a few giant cells amid which were seen broad, aseptate, obtuse-angle branching hyphal elements (Figure 7a,b). The presence of fungal elements was confirmed with periodic acid-Schiff staining (Figure 7c).