A 68-year-old male patient, presented to us with history of progressive loss of vision in the right eye and persistent right-sided retro-orbital pain and headache for 2 months which started immediately after the cataract surgery of the right eye (Phacoemulsification under topical anaesthesia using lignocaine 2% eye drops) with intra-ocular lens implantation. The pre-operative visual acuity was 6/60 in the right eye and 1/60 in the left eye. The patient reported initial improvement in vision, but it was followed by rapid deterioration, for which he attended his ophthalmologist, who diagnosed it as a case of optic neuritis, as a complication of cataract surgery. He was started on oral methyl-prednisolone. There was no improvement in the symptoms and then the patient attended our out-patient department clinic. He had no history of diplopia, redness, watering, proptosis or floaters. There was no history of trauma or symptoms suggestive of meningitis or encephalitis. There was no history of cough, dyspnoea or haemoptysis.\nOn examination, the patient was conscious and oriented, with non-tender paranasal sinuses and bilaterally palpable temporal arteries. Neurological examination revealed complete right-sided ophthalmoplegia with mydriasis, hypoalgesia and diminished right corneal reflex. Ophthalmological examination revealed right and left visual acuity of 3/60 and 1/60, respectively. Right pupil was dilated and fixed, whereas the left pupil was reactive to light. Fundoscopy revealed oedema and pallor of right disc, whereas the left disc was normal.\nLaboratory investigations revealed haemoglobin of 12 g/dl (Normal:12–16 g/dl) and fasting blood sugar of 105 mg/dl (normal: 60–100 mg/dl). Serum angiotensin-converting enzyme and thyroid function tests were within their normal limits. Serum immuno-inflammatory markers were within normal limits. He was negative for HIV, hepatitis B and C and had normal levels of cluster of differentiation (CD)-4 and CD-8.\nThe first T1-weighted axial magnetic resonance imaging (MRI) of brain (Fig. A) showed minimal soft tissue at the right orbital apex which was abutting the right-sided optic nerve suggestive of OAS. In view of clinical examination and MRI report, a presumptive diagnosis of pseudotumor (inflammatory versus infectious aetiology) was made and the patient was started on ceftriaxone and vancomycin in meningitis dosage with tapering course of dexamethasone. Even after 10 days of this treatment, there was no improvement in symptoms. Instead, the visual acuity in the right eye decreased to perception of light only with development of right-sided proptosis.\nRepeat T1-weighted axial MRI scan of head (Fig. B) revealed an increase in the size of the mass with bony erosion of sphenoid sinus which was suggestive of infectious aetiology. Biopsy of the lesion was deferred in view of the proximity of the mass to the major neuro-vascular bundle in the orbital apex and cavernous sinus and the major risk involved in the procedure relative to its yield. The patient was empirically started on liposomal amphotericin-B in view of possibility of fungal infection.\nEven after 21 days of liposomal amphotericin-B, there was further increase in proptosis and the visual acuity in right eye diminished to ‘No light perception’ with increased intensity of peri-orbital and retro-orbital pain. A repeat T1-weighted axial MRI of head (Fig. C) revealed further destruction of the local anatomic sites with increase in mass lesion to 19 × 16 × 14 mm. Serum galactomannan antigen level was 0.5 index (Borderline).\nBy virtue of its size and extension, the mass was now accessible for biopsy. Under general anaesthesia, endoscopic navigation-assisted biopsy was taken from seven different sites. Review of histological specimen from posterior ethmoid mucosa, posterior ethmoid tissue near optic nerve and sphenoid sinus mucosa (Fig. ) showed fungal hyphae (Fig. A and B) morphologically compatible with Aspergillus species.\nHe was subsequently started on injectible voriconazole which was continued for 10 days. Now the patient showed dramatic improvement in his headache which resolved completely in 5 more days. Right eye movements began. The patient was then discharged on oral voriconazole.\nThe patient was completely free of symptoms with completely resolved ophthalmoplegia with repeat T2-weighted axial MRI revealing decrease in the size of the mass (Fig. D), after 7 months of oral voriconazole. But unfortunately, the patient had no recovery of vision in his right eye.
An 86-year-old female with medical comorbidities including well-controlled type 2 diabetes (HbA1c 5.6 %) on oral therapy and glaucoma who presented with 2 weeks of right ear pain, right frontal headache and right facial pain just lateral to the nose. The patient also described painful extraocular movements (EOMs) on the right, decreased vision, and thick purulent drainage from the right nasal cavity. Vitals were normal with a blood pressure of 132/70, heart rate of 78, respiratory rate of 14, oxygen saturation of 97 % on room air, and temperature of 36.7 degrees Celsius. Examination was significant for hand motion vision, severely restricted right eye EOMs with ptosis, and excyclotorsion. Computerized tomography of the head and orbits demonstrated soft tissue thickening and indistinctness of fat in the region of the right optic canal and right orbital apex, possibly representing extension of paranasal inflammatory disease to an unusual bone defect in the right lateral wall of the right sphenoidal sinus (). Additionally, there was associated right anterior ethmoidal mucosal disease and complete opacification of the right maxillary antrum. Magnetic resonance imaging (MRI) of the brain without and with intravenous (IV) contrast showed right-sided paranasal sinus infection with transsphenoidal phlegmonous extension into the right orbital apex and cavernous sinus consistent with orbital apex syndrome (, ). Ophthalmology and otolaryngology (ENT) were consulted and performed a right endoscopy of the nasal cavities. This revealed purulence from the right middle meatus obstructing the view of the sphenoid. Nasal swab was sent for bacterial culture. Infectious disease was consulted who recommended IV vancomycin, cefepime, and metronidazole in addition to dexamethasone.\nThe patient underwent surgery on hospital day three. ENT completed a broad endoscopic sinus debridement including right total ethmoidectomy, frontal sinusotomy, sphenoidotomy, tissue biopsy, and washout of orbital apex with tissues sent for bacterial and fungal cultures. Tissue from the right naris and right ethmoid sinus grew methicillin-susceptible Staphylococcus aureus (MSSA). Initial pathology at frozen section examination revealed mixed acute and chronic inflammation at all biopsy sites. The patient’s antibiotics were narrowed to IV cefazolin on hospital day 5. On hospital day 6, one colony of Aspergillus fumigatus grew from a culture of the right orbital apex tissue. The pathology revealed fungal hyphae consistent with Aspergillus spp. and related molds, with tissue invasion in the orbital apex (, ). She was started on IV voriconazole. She was discharged to a skilled nursing facility after a 9-day hospital course with antimicrobials including oral cefdinir for a 4-week course and oral voriconazole with a planned treatment course of at least 12 weeks. At two-week follow-up with infectious disease, a repeat MRI brain showed worsening right ethmoid and sphenoid sinus fungal infection. Intravenous caspofungin was added to her regimen for a two-week course of therapy with clinical improvement. She underwent further debridement with rigid endoscopy at two, four, and six weeks after diagnosis with mostly crusted blood products and no evidence of necrotic tissue. Ophthalmologic exam ultimately demonstrated improvement in visual acuity to 20/50 with full EOMs.