Abstract:Sinonasal mucormycosis in the immunocompromised pediatric population is an uncommon but potentially fatal disease. A high index of suspicion is of utmost importance to decrease its related morbidity and mortality.
“…In total, 210 case reports were identified from the literature search and reviewed, of which 175 contained sufficient clinical data to be analysed. These were derived from a total of 140 papers (some papers contained multiple case reports) . Larger case series were usually excluded due to a lack of chronological data (refer to Figure for PRISMA flow diagram).…”
Although overall survival rates have not improved, survival in patients with renal disease were better, potentially due to the introduction of liposomal amphotericin B which is less nephrotoxic. Prompt recognition of ROCM, reversal of predisposing co-morbidities and aggressive medical treatment remain the cornerstone of managing this highly aggressive disease.
“…In total, 210 case reports were identified from the literature search and reviewed, of which 175 contained sufficient clinical data to be analysed. These were derived from a total of 140 papers (some papers contained multiple case reports) . Larger case series were usually excluded due to a lack of chronological data (refer to Figure for PRISMA flow diagram).…”
Although overall survival rates have not improved, survival in patients with renal disease were better, potentially due to the introduction of liposomal amphotericin B which is less nephrotoxic. Prompt recognition of ROCM, reversal of predisposing co-morbidities and aggressive medical treatment remain the cornerstone of managing this highly aggressive disease.
“…Hypoenhancement and near-normal T2 signal in invasive fungal disease due to microvascular invasion of hyphae and subsequent necrosis in the absence of an immune response sufficient to cause substantial mucosal edema, as seen in this case, are suggestive of AIFRS; this is distinct from the diffuse enhancement and T2 hyperintensity typically seen in other inflammatory diseases [12, 13]. A pattern of nasal cavity, orbital, ethmoid, and maxillary involvement is highly suggestive [14].…”
Gorlin Syndrome (GS), also known as nevoid basal cell carcinoma syndrome, is a rare autosomal dominant condition characterized by developmental abnormalities and predisposition to certain neoplasms. Acute invasive fungal rhinosinusitis (AIFRS) is an uncommon clinical entity characterized by high morbidity and mortality. In immunocompromised patients, computed tomography plays a critical role in screening for suspected AIFRS. However, due to the association between exposure to ionizing radiation and subsequent development of malignancies in patients with GS, patients with GS and suspected AIFRS present a unique and challenging clinical scenario. We present a case of a pediatric patient with GS and acute lymphocytic leukemia (ALL) diagnosed with AIFRS; to the best of our knowledge, it is the only case described in the literature.
“… 3 Four immunocompromized patients with nasal sinuses mucormycosis undergoing multiple aggressive debridement were described by Rassi et al ; in the postoperative course one child died of the disease, one developed persistent unilateral blindness, one was lost to follow-up and the last one was cured with no sequelae. 4 Other single-center pediatric cases have recently been described, including a seven-year-old girl with ALL and rhino-cerebral mucormycosis, two children with acute leukemia and intraoral mucormycosis and four children with ALL and cutaneous mucormycosis; all patients were successfully treated with amphotericin B and surgical debridement. 5-8 Further case reports describe a child with acute leukemia and isolated muscular mucormycosis, one with ALL and fatal gastrointestinal mucormycosis, a nine-year-old boy with ALL and isolated hepatic mucormycosis requiring surgical excision and combined liposomal amphotericin B and posaconazole therapy.…”
Mucormycosis is an uncommon but severe fungal infection, typically observed in immunocompromized patients. We report a case of acute lymphoblastic leukemia complicated by rhino-oculo-cerebral mucormycosis in a pediatric patient. Combination lipid polyene-echinocandin therapy, along with surgical debridement appeared to be effective. Nevertheless, a severe relapse occurred during posaconazole prophylaxis; antifungal therapy, hemimaxillectomy and suspension of chemotherapy were performed. Although mucormycosis is a frequently lethal infection, prompt diagnosis and aggressive treatment can be successful even in cases of relapse.
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