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Rhinocerebral Mucormycosis with Perineural Spread Pattern

  • Background:
    • Invasive fungal rhinosinusitis in immunocompromised patients has a classically reported high (80-90%) mortality. CNS is involved through direct invasion, along perineural spread or through hematogenous spread.
  • Relevant Clinical Information:​
    •  Patients are neutropenic or diabetic patients with poor metabolic control. In this case the patient was immunosuppressed due to diabetes and renal transplant.
       
  • Key Diagnostic Features:
    • CT: Sinus opacification with bone erosion and adjacent fat/soft tissue stranding. Bone erosion can be subtle (as in this patient’s case) or even absent as angioinvasive fungi may extend along small vessels and vasa nervorum. 
    • MR can better depict intracranial and intraorbital extension. Fungal sinus secretions can have a low T2 signal. It is the exam of choice for perineural spread, cavernous sinus involvement, meningeal extension, and intraparenchymal abscess. 
    • Fungal hyphae invade vessel walls and vascular complications such as stenosis, septic thrombosis, or pseudoaneurysm can be seen. 
       
  • Differential Diagnoses:
    • Complicated non-fungal rhinosinusitis: immunocompetent patients. Bone erosions are less common. They show air-fluid levels and sinusal mucosal enhancement (no evidence of mucosal necrosis). 
    • Sinonasal squamous cell carcinoma: Solid mass with bone destruction. Can also show perineural spread. 
    • Sinonasal lymphoma: more solid and homogeneous mass in nasal cavity. Can also be hypointense in T2 and show perineural spread. May be indistinguishable from SCC.
    • Wegener's granulomatosis/Granulomatosis with polyangiitis: Bilateral, usually symmetric chronic sinus inflammatory changes and septal perforation. Extra sinusal involvement is seen later in the disease.
       
  • Treatment:
    • ​Prompt diagnosis of invasive fungal sinusitis allows treatment as soon as possible. The outcome may be poor. Treatment consists of aggressive surgical debridement and antifungal therapy.
August 16, 2018

A 54-year-old diabetic woman with immunosuppressive therapy due to renal transplant. She had a previous dental abscess treated with oral antibiotics for 2 weeks. Progressive, right-sided facial swelling and proptosis required surgical malar debridement with poor clinical response. She was transferred to a referral hospital 10 days later.

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