A 12-year-old girl was seen for a 3-day history of diplopia, right-sided otalgia, and right-sided face numbness that had progressed during the past 24 hours to blindness and proptosis in the right eye. The medications she had been taking included azathioprine and prednisolone for autoimmune hepatitis, diagnosed 3 months earlier.
Physical examination showed right-sided facial numbness, ophthalmoplegia, a fixed dilated right pupil, and diminished visual acuity in her right eye. There was prominent right-sided facial droop with tongue deviation to the right. Preliminary laboratory workup showed that the patient was in diabetic ketoacidosis. Ear, nose, and throat examination showed nonviable-appearing tissue in the right nasal cavity. Magnetic resonance imaging of her head revealed nonviable tissue involving the right middle and inferior turbinates (Figure, E). The patient underwent an immediate nasal biopsy with debridement of the nasal mucosa. The debrided tissue was found to be dusky and nonviable and did not bleed during debridement. The tissue biopsy findings were consistent with a diagnosis of mucormycosis (Figure, A-D). She was given intravenous amphotericin B to which oral posaconazole and intrathecal amphotericin B were added during the next week. The initial follow-up magnetic resonance images showed progression of the disease with significant intracranial extension (Figure, F). She continued antifungal drug therapy and follow-up in the clinic; 6 months after diagnosis, she showed remission of the disease, without any new neurologic deficits, with magnetic resonance imaging showing regression in the size of abnormally enhanced areas seen previously.
Shown here are photomicrographs and magnetic resonance images of the lesion. A, Frozen section biopsy demonstrating occlusion of a vessel by ribbonlike, nonseptate hyphae (arrow). Note the surrounding mixed inflammatory response. B, Same slide under higher magnification showing vascular occlusion by nonseptate hyphae (arrow) branching at right angles. C, Biopsy showing invasive, nonseptate fungal hyphae (arrows) in a nerve fascicle. D, Periodic acid–Schiff stain distinguishing the elongated, pink fungal hyphae (arrow) from the lighter connective tissue background. E, Postgadolinium T1-weighted axial magnetic resonance image showing nonenhancing tissue (arrow) in the right nasal cavity compatible with necrotic mucosa. F, Similar technique showing abscesses appearing as ring-enhancing lesions in the right medial temporal lobe and right cerebellopontine angle. Also note occlusion of the right internal carotid artery (arrow) and thrombosis of the right cavernous sinus, although there was no radiographic evidence of cerebral infarction.
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