Unilateral blindness following sino-orbito-cranial aspergillosis in a diabetic

A 56-year-old diabetic female presented with acute onset blindness with ptosis and complete ophthalmoplegia of the right eye. CT scan of the head showed soft tissue swelling of the right orbital cavity contents with proptosis and the CSF examination revealed sterile pleocytosis. Ipsilateral sinus mucosal histology showed inflammatory changes on H&E staining and fungal staining with methenamine-silver showed typical fungal hyphae of aspergillosis. The infection cleared with a course of IV amphotericin B however blindness and ophthalmoplegia of the right eye were permanent.

Keywords: Aspergillosis, invasive, mycosis, sinocranial

Sinocranial aspergillosis is seen by far the most with skull-base syndromes ^[1] in temperate countries in both the immunocompetent and immunocompromised especially diabetics. ^[2] Delayed diagnosis causes high mortality and early treatment improves outcome. ^[3] Corticosteroid treatment masks symptoms, delays diagnosis, and potentiates the infection. ^[4] Voriconazole is currently the drug of choice and oral itraconazole is a useful second-line agent. ^[5],[6] The authors detail a rare case of sino-orbito-cranial aspergillosis with acute onset painful loss of vision, complete ophthalmoplegia, and proptosis in a diabetic woman with CSF pleocytosis demonstrating retrograde central nervous system involvement. Despite treatment ophthalmoplegia and blindness were irreversible. The authors were compelled to use amphotericin B in treatment due to economic constraints. This case highlights the importance of suspicion of possible mycoses in patients presenting with skull-base syndromes which is the key determinant of early diagnosis, treatment with minimal residual disability.

A 56-year-old diabetic female presented with acute onset painful loss of vision, squint, and proptosis of the right eye, which was preceded by fever and ipsilateral headache of 3 weeks. Her past medical history revealed completely treated pulmonary tuberculosis 8 years previously.

High fever spikes were documented. Right eye examination revealed ptosis, chemosis, complete opthalmoplegia, proptosis, and corneosclereal insensitivity. Visual acuity in the right eye was 0/60 and the pupil was fixed and nonreactive. Ophthalmoscopy revealed an ischemic right-sided optic fundus. The left eye was normal except for an early cataract with a visual acuity of 36/60 improving to 6/6 with spectacles. Facial sensation was preserved. Meningism was absent. Other systems were normal.

The ESR and CRP were elevated at 108 mm/h and 8.4 mg/dl respectively. The full blood count revealed hemoglobin of 10.5 g/dl, total count of 19,300/mm ³ , differential leukocyte count of polymorphs 93%, lymphocytes 5%, and eosinophils 1%, the platelets were adequate on peripheral smear. The blood picture showed neutrophils with toxic changes. CT scan of the head showed edema of soft tissue surrounding the right eye, and the ipsilateral maxillary, ethmoidal sinuses. Regional MRI confirmed CT findings with better resolution [Figure 1]. Lumbar puncture showed a lymphocyte predominant CSF pleocytosis with a total count of 140 cells/mm ³ of which 120 cells/ cu mm ³ were lymphocytes. CSF proteins were mildly elevated at 53 mg/dl. CSF staining was negative for organisms. Serum proteins and creatinine levels were normal and the urine had no active sediments. Fasting blood sugar on regular medications was 256 mg/dl. Chest x-ray was normal. Maxtoux, CSF PCR for mycobacteria, C ANCA, P ANCA, ANA, and HIV antibody were all negative. Bacterial culture of blood and CSF came negative. Fiberoptic rhinoscopy and multisite biopsies of the sinuses were taken for studies and broad-spectrum intravenous antibiotics, CAT 1 antituberculosis drugs and high dose steroids were commenced in the interim.

Sinus mucosal histology showed inflammatory changes on H&E staining and fungal staining with methenamine-silver showed fungal hyphae confirming aspergillosis [Figure 2].

Intravenous amphotericin B was commenced while the other drugs were discontinued. Tight glycemic control was also achieved. Clinical improvement of constitutional symptoms was seen within 3 days, closely followed by resolution of chemosis and proptosis. Radiological resolution of the pathology was confirmed post-treatment. Ophthalmoplegia and blindness of the right eye was however permanent. She was counseled regarding the permanent complications of the disease to her right eye.

Invasive aspergillosis is usually acquired by inhalation commonly involves the lungs; however sinocranial involvement is also seen. ^[1] Skull-base syndrome is the most common sinocranial presentation, followed by features of intracranial space occupying lesion rarely a stroke-like presentation. ^[7]

Acute onset painful unilateral blindness is alarming for both the patient and the physician. Infectious and noninfectious causes need consideration. Infectious etiopathogensis includes sinusitis causing optic neuritis, orbital apex syndrome, and cavernous sinus infections. ^[8] Painful ophthalmoplegia occurs due to inflammation of the optic nerve or the contents of the orbital cavity.

Sinocranial aspergillosis has been described from countries with temperate climates in immunocompetent individuals and immunocompromised patients. ^[2] Diabetes is a predisposing risk factor for opportunistic infections including mycoses .

Fever and incorrect diagnosis are associated with high mortality. Early medical management with or without surgical intervention is necessary to improve outcome. ^[3] Retrograde involvement of the cavernous sinus is a serious complication associated with unfavorable outcome and needs very aggressive management. Some presentations, such as optic nerve involvement, can respond to systemic corticosteroids, leading to delays in diagnosis, and possibly iatrogenic potentiation of the infectious process. ^[4]

Amphotericin B had been the drug of choice for invasive aspergillosis until studies demonstrating better responses and improved survival with fewer severe side effects resulted in the preferential use of voriconazole. Oral itraconazole is a useful second-line agent. ^[5],[6] The authors were compelled to use amphotericin B in this patient due to economic constraints.

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1755-6783.98632

[Figure 1], [Figure 2]