A 64-yr-old man presented to the emergency room with right visual loss after a 5-day history of rhinorrhea, diplopia and severe headache. The body temperature was 36.5℃ and the white blood cell count was 9,100/mm3. He had a 10-yr history of poorly controlled type II diabetes mellitus. The initial blood sugar was measured and it was too high to register by a blood glucose test strip and it was decreased to 485 mg/dL after an IV infusion of normal saline. His right eye showed ptosis, mild eyelid swelling and total ophthalmoplegia. His visual acuity in the right eye was counting fingers (50 cm) and it was 20/40 in the left eye. The pupillary light reflex was lost with an afferent defect. The anterior chamber, the intraocular pressure and the fundi were normal. Endoscopic examination detected edematous mucosa, brown-colored fungal balls and there was a mucopurulent discharge from the sphenoethmoidal recess. The opthalomologic and nasal examinations of the left side showed no abnormal findings.\nComputed tomography showed unilateral sphenoethmoidal sinusitis along with signs of orbital invasion. Most of the orbital lesions were located at the retrobulbar area and the orbital apex, and inferior and medial rectus muscles were thickened. Even though there was bony erosion of the posterior wall of the sphenoid sinus, there was no evidence of intracranial extension. Increased signal intensities were noted at the same regions on brain MRI with gadolinium enhancement (). The initial clinical diagnosis was OAS due to invasive fungal sinusitis. Therefore, we immediately started systemic amphotericin B (50 mg/day for 21 days and 25 mg/day for 10 days) and insulin therapy to control his blood sugar. The patient was also started on intravenous ampicillin/sulbactam (6.0 g/day) and ceftriaxone sodium (2.0 g/day) to cover any possible bacterial infection.\nThe patient elected to undergo endoscopic sinus surgery under local anesthesia on the 3rd hospital day. The sphenoid sinus was filled with thick mucus and brown-colored matted fungal balls. All the fungal materials were removed using curettes and sinus irrigation. The mucosa around the ostium and the fungal materials were sent to the Department of Pathology for the determination of invasiveness.\nBy the 4th postoperative day (POD), there was no improvement and the visual acuity decreased from finger count to light perception. The patient complained of severe headache, nausea and vomiting; the follow-up computed tomography and cerebrospinal fluid (CSF) study showed no evidence of meningitis or intracranial invasion. The histopathology showed aspergillosis without evidence of tissue invasion. Therefore, empirical corticosteroid therapy with prednisolone (PDS, 55 mg for 7 days, 40 mg for 7 days, and 20 mg for 7 days) was started on the 9th POD. After the 3rd day of PDS therapy, his headache and ophthalmic symptoms began to improve. With the exception of the ophthalmoplegia, the ptotic upper eyelid completely recovered and the visual acuity improved to 20/100 by the day of discharge (the 28th POD). The patient's blood sugar was not well controlled with fluctuations being noted during the hospitalization. By 3 months, the right visual acuity improved to 20/63 and the extraocular muscle movement was completely normalized. After 8 months, there was no recurrence of the nasal and orbital symptoms.
A 59-year-old woman visited out-patient clinic of neurology because of 3 years intermittent sharp pain on left orbital. She was diagnosed as trigeminal neuralgia in the community hospital. Then, she was treated with Carbamazepine, Mecobalamin and acupuncture, but without significant relief. Our examination found tenderness at the exit of the left supraorbital nerve. MRI imagine demonstrated that partial empty sella and left sphenoid sinus. This patient was referred to the Otolaryngology Department. Paranasal sinus CT demonstrated inflammation of left sphenoid sinus. Patient was treated by transnasal endoscopic sphenoidotomy without antifungal therapy. Histopathological examination diagnosed left sphenoid sinus as SSFB. Follow-up of 2 month demonstrated no further recurrence of her headache.\nAll of the major clinical information in patients with SSFB was summarized in the Table .