#26: Rhino-Orbital Mucormycosis in Immunocompromised Children: Treatment Outcomes of Three Cases
Authors: Valdés G , Martínez M , Morayta A 
Published: March 2021

Abstract

Background
Mucormycosis is an aggressive opportunistic fungal infection of the family Mucoraceae, including the genera Mucor, Absidia, and Rhizopus. It is the third most common cause of invasive fungal infection, with low incidence, but high mortality (50–90%), and it usually presents in immunocompromised hosts. The fungus spores are ubiquitous in nature and are found in soil, air, and on decaying vegetation. Individuals get infected following inhalation of spores, ingestion, or contamination of wounds. Rhino-orbito-cerebral mucormycosis is the most common form of illness in children. An early diagnosis and a multidisciplinary approach to treatment are necessary to prevent mortality.

Methods
We present a case series of three immunocompromised children with rhino-orbital mucormycosis in Nacional Medical Center “20 de Noviembre” from 2015 to 2019. We describe time to diagnosis, start of antifungal therapy, surgery involvement, and patient outcomes.

Results
Patient 1 was a 9-year-old girl with aplastic anemia who developed right palpebral swelling (day 1 of initial symptoms) and was initially diagnosed with preseptal cellulitis. On day 5, a necrotic area appeared in the right inner canthus. Paranasal sinus CT scan showed opacified ethmoidal sinus. Liposomal amphotericin B therapy was started. At day 7, surgical debridement was performed. At day 18, the patient died and the culture of the debrided tissue showed Mucor ramosissimus. Patient 2 was a 15-year-old boy with acute lymphoblastic leukemia who developed a necrotic area in right side of the nose and palate (day 1). Liposomal amphotericin B therapy was initiated. At day 3 and 6, surgery was performed. At day 8, cultures resulted in Rhizopus oryzae, and treatment with caspofungin was added. He had progression of the infection, requiring multiple interventions. Antifungal therapy consisted of 75 days with amphotericin B and 67 days with caspofungin, with resolution. At day 152, he had a event of neutropenia and fever and died of septic shock Patient 3 was a 16-year-old girl with acute lymphoblastic leukemia who developed a necrotic lesion on the palate and right side of the nose (day 1). Direct examination of the lesion showed hyaline, non-septated hyphae. Amphotericin B therapy was initiated and surgery was performed at day 3. By day 7, there was good clinical evolution and resolution the infection. She died at day 12 because of intestinal bleeding and hypovolemic shock.

Conclusions
The diagnosis and treatment of mucormycosis remains a challenge. Clinical suspicion should be high in patients with risk factors, and early identification and prompt treatment with antifungals and surgical debridement can reduce mortality and improve the prognosis. The poor outcomes of the patients in this case series were mainly due to complications of the underlying disease and not because of mucormycosis. However, in the first case, delayed diagnosis and treatment might have contributed to the unfavorable outcome. The treatment of choice is liposomal amphotericin B. In case 2, a second antifungal therapy included caspofungin due to isolation of R. oryzae, sensitive to echinocandins. Posaconazole can be considered as an alternative option, but it is not available at our institution. Early identification of clinical manifestations and early multidisciplinary treatment with surgical services and antifungal are needed to eliminate the infection. Risk factors must be modified to increase patient survival.