CASE REPORT: RHINORBITAL MUCORMYCOSIS AND DESFERRIOXAMINE – REVISITING AN OLD FOE IN PATIENTS RECEIVING DIALYSIS

Dr Rebecca Hong1,2, Dr Catherine Brumby1,2, Professor  Lawrence P McMahon1,2

1Department of Renal Medicine, Box Hill Hospital, Eastern Health, Melbourne , Australia , 2Eastern Health Clinical School, Monash Univeristy, Melbourne, Australia

Background

Mucormycosis is a highly virulent and rare invasive fungal infection seen in immunocompromised patients, diabetics, and patients with iron overload or on maintenance dialysis. Mucorales thrive in iron-rich environments and specific use of intravenous (IV) desferrioxamine markedly increases risk of infection and mortality. Mucormycosis is now rarely seen in dialysis patients since introduction of oral iron chelators (deferasirox), widespread ESA use reducing iron overload, and less prevalent aluminium toxicity.

Case Report

A 66-year-old female on dialysis for 12 years due to ADPKD with previous transfusion-dependent aplastic anaemia was receiving long-term IV desferrioxamine due to deferasirox intolerance for chronic iron overload. She presented with sudden-onset of unilateral facial pain and headache. Examination and investigations were unremarkable aside from left-sided cheek numbness and a C-reactive protein of 135. Broad-spectrum IV antibiotics were commenced, but over 24 hours she developed a complex ophthalmoplegia, proptosis, diplopia, and eventual full visual loss. Repeat CT scan was unremarkable aside from enlargement of the left medial and inferior rectus muscles. A presumptive diagnosis of orbital mucormycosis was made. IV liposomal amphotericin B was commenced and she underwent debridement and orbital exenteration. Histopathology confirmed mucorales. Despite further debridement and IV antifungal therapy, an MRI three days post-exenteration demonstrated multifocal cerebral ischemia from angioinvasive rhinorbital mucormycosis. Haemodialysis was withdrawn due to poor prognosis and she died 11 days after initial presentation.

Conclusion

Although Mucormycosis is rare, clinicians should carry a high index of suspicion in at-risk patient groups due the absence of clinical signs at presentation, and rapid progression to an often fatal outcome despite aggressive treatment. Importantly, desferrioxamine use and iron overload confer a markedly increased risk to dialysis patients.


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