Abstract
Purpose
Rhino-orbital-cerebral mucormycosis (ROCM) is a rare opportunistic infection with a high mortality despite relevant treatment.
Observations
A 3-year-old girl under treatment for acute lymphoblastic leukemia developed periorbital swelling, ophthalmoplegia and a necrotic palatal lesion during a period of neutropenia. Imaging revealed sinusitis, pre- and postseptal cellulitis. The disease later progressed to cerebral involvement and orbital apex syndrome with complete ophthalmoplegia, ptosis and loss of vision. The patient was treated with systemic antifungal therapy, hyperbaric oxygen and extensive surgery. This included orbital exenteration, skull base resection, cerebral debridement with placement of an Ommaya reservoir for intrathecal administrations of amphotericin B (AmB) and in addition endoscopic sinus surgery with local AmB installation. Chemotherapy was safely continued after resolution of the ROCM and the patient remains in complete remission after 5 years.
Conclusion and importance
Patients with ROCM can be cured with aggressive multimodality treatment, including surgical intervention, even if in myelosuppression.
1
Introduction
Rhino-orbital-cerebral mucormycosis (ROCM) is an aggressive, rare and opportunistic infection typically seen in immunosuppressed or poorly controlled diabetes patients. Mucormycosis is caused by fungi of the order Mucorales (class Zygomycetes) which are ubiquitous in nature and intrinsically resistant to several antifungal agents. The fungus invades the sinonasal mucosa and may spread to the orbit via the ethmoidal sinuses and lamina papyracea. Based on the ability for angioinvasion, the fungal hyphae may extend to the cerebrum along the ophthalmic vessels or the cavernous sinus. The vascular invasion results in thrombosis causing infarction and necrosis of the involved tissues leading to decreased tissue penetration of systemic antifungal therapy. Treatment thus usually consists of systemic antifungals together with aggressive surgical debridement of infected tissue and reversal of predisposing conditions. Hyperbaric oxygen (HBO) may be given as adjuvant therapy. , ROCM typically presents in a rapidly fulminant manner with headache, fever, mucosal necrosis and ophthalmological findings such as ophthalmoplegia, proptosis, chemosis, decreased visual acuity, periorbital swelling and pain. Despite early diagnosis and aggressive treatment, the disease carries a high mortality. Individualized multidisciplinary approaches are therefore recommended. We report the successful treatment of ROCM in a 3-year-old child under treatment for acute lymphoblastic leukemia (ALL).
1.1
Case report
A 3-year-old girl was diagnosed with pre-B ALL and treated according to the Nordic Society for Pediatric Haematology and Oncology ALL-2008 protocol. After 4 weeks of induction therapy, she obtained complete remission. Six weeks after diagnosis (after completing 16 days of antibiotics due to an episode of febrile neutropenia with no recognized focus), the patient developed right-sided periorbital swelling and fever (defined as the first day of mucormycosis infection) ( Fig. 1 ). The prophylactic fluconazole treatment was supplemented with broad-spectrum antibiotics and chemotherapy was paused. On day 4, the periorbital swelling had progressed to the left eye and fusidic acid eye drops were initiated. Examination on day 6 revealed hyperemia of the conjunctiva, chemosis and mildly restricted extraocular movements of the right eye. On day 7, the patient presented with areactive mydriasis, decreased abduction of the right eye and a white-brown lesion of the hard palate. Computed tomography (CT) revealed opacification of the right maxillary sinus and ethmoidal cells, 2-mm proptosis, edema of the periorbital soft tissue and enlargement of the rectus muscles of the right side consistent with pre- and postseptal cellulitis ( Fig. 2 A). The findings were suspected to be due to a fungal infection and fluconazole was switched to liposomal amphotericin B (L-AmB) and voriconazole. Examination on day 8 revealed resistance to retropulsion of the globe, possible loss of light perception of the right eye and an increase in anisocoria. The dilated right pupil was interpreted as a possible reversible affection of the third cranial nerve at the orbital apex. The patient underwent right-sided endoscopic sinus surgery with an opening to the maxillary sinus and ethmoid bulla. Microscopic examination of biopsies from the palate and nasal cavity revealed no sign of fungi. Canthotomy and medial wall orbital decompression were not performed due to the risk of further spread of the infection. During the following two days, there was progression to orbital apex syndrome with complete ophthalmoplegia, ptosis and visual loss of the right eye. Betaxolol eye drops were started on the right eye to improve the circulation of the optic nerve. Serum Aspergillus galactomannan antigen test was negative and blood, faeces and nasal swab cultures were negative for bacteria and fungi.
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