Cerebro-Sino-Orbital Mucormycosis: Case Report of Novel Biopsy Technique and Review of Literature

Introduction: We present an usual case of a patient with multiple myeloma status-post autologous peripheral blood stem cell transplant diagnosed with cerebro-sino-orbital mucormycosis on pathologic examination of specimens taken during cerebral angiography for acute internal carotid artery occlusion, averting open cranial biopsy.

Case: A 56-year-old man presented with left retrobulbar pain eight months duration following diagnosis of multiple myeloma, while undergoing peripheral blood stem cell harvest for autologous transplant. Initial investigation showed normal vision and benign ophthalmologic evaluation. CT and MRI showed small enlargement of the left optic canal with enhancement.

Following successful engraftment, his vision began to decline. Repeat MRI showed extension of left orbital apex enhancement into proximal left optic nerve and ventral cavernous sinus. His condition progressed rapidly, within three days he lost light perception and plans were made for open cranial biopsy.

The day before surgery, he suffered left MCA stroke with occlusion of the proximal left ICA. Following cerebral angiogram and stenting, catheter tip was sent to pathology for analysis given the suspicion for debris. Pathology returned showing hyphae and he was started on intravenous amphotericin. He decided to transition to home hospice rather than pursue further aggressive treatment.

Discussion: Disseminated intracranial fungal infections occur predominantly in immunocompromised hosts via nasal mucosa and paranasal sinuses, but can present with primary cerebro-orbital symptoms. They are associated with intravascular thrombosis and invasion. Aggressive surgical debridement of intracranial disease remains of questionable benefit, especially in the setting of intravascular invasion. Endovascular techniques have been used in the past to treat stenosis in the setting of invasive fungal infections. To our knowledge, this is the first case of intracranial Rhizomucor diagnosed via cerebral arteriography. This averted potentially morbid radical skull base dissection.

Conclusion: Early diagnosis and treatment require multidisciplinary approach in cases of cerebro-sino-orbital mucormycosis. Cerebral angiography can be considered an adjunct in appropriately selected patients, yielding another avenue for intracranial diagnosis and aid the skull base surgeon in surgical decision making and operative planning.