Author(s): Correia, Filipe ; Pereira, Sílvia ; Toscano, Cristina ; Batista, Judite ; Sabino, Raquel ; Veríssimo, Cristina ; Lameiras, Ana Rita ; Donato, Mariana ; Escada, Pedro
Date: 2017
Persistent ID: http://hdl.handle.net/10400.18/5128
Origin: Repositório Científico do Instituto Nacional de Saúde
Subject(s): Mucormycosis; Rhizopus Microsporus; Diabetic Patient; Rhinocerebral Mycosis; Infecções Sistémicas e Zoonoses
Mucormycosis is a rare but life threatening invasive mycosis caused by members of the Mucorales order. It usually develops in an immunocompromised host, mainly diabetics, but also in hematologic malignancies or transplanted patients. The usual clinical presentation of this fungal sinusitis is a combined rhino-orbital-cerebral infection and, despite the advances in combining antifungal and surgical treatment, it remains a fatal human infection in most cases. Materials and Methods: Case report of a patient presenting with prostration and left eye exophthalmia and cellulitis, as a result of mucormycosis. We highlight the particularities of the surgical endoscopic debridement, microbiology and histologic results. Results: 68 years-old male, with no prior relevant history, presented with a 2-day history of polydipsia, polyuria, prostration, fever and dyspnea. Findings included left eye exophthalmia, chemosis and limitation on ocular movements. Laboratory results were compatible with an inaugural diabetic ketoacidosis and sinus and orbital- CT-scans showed a left side ethmoidalsinusitis, with postseptal cellulitis. Despite the metabolic correction and broad-spectrum antibiotic coverage, the neurologic status didn’t improve and brain scans showed a diffuse hypointense frontobasal area compatible with an ischemic stroke and intracranial extension of the inflammatory process. Urgent endoscopic surgical debridement of the sinus infection was accomplished through an endoscopic total ethmoidectomy, maxillary, sphenoidal and frontal sinusotomy. Intraoperatory findings included a scarce purulent discharge and a diffuse necrotic sinus mucosa. The histologic and microbiologic results were compatible with an invasive sinus mucormycosis: broad non-septate hyphae were observed on direct examination of sinus biopsy. On culture, macro and micro characteristics were compatible with Rhizopus arrhizus and amphotericin B was started in high doses. Identification confirmed by sequencing of genomic DNA fragments proved to be a Rhizopus microsporus. The initial post-operative period was favorable, with improvement on the left eye and sinus inflammatory signs but the neurologic status declined on the 4th day, with a huge hemorrhagic transformation of the frontal necrotic parenchyma ending in a fatal result. We present surgical images and video recording of the endonasal procedure, microbiology and histology pictures. Conclusions: Despite the aggressive therapy, the fatal closure is, unfortunately, a common result of mucormycosis. The angioinvasive feature of Mucormycetes, enhanced by the ketoacidic pro-growth environment, causes a purulent arteritis and thrombosis with resultant ischemia and infarction of tissues. Intracerebral hemorrhage probably resulted from mycotic aneurysms rupture. Early diagnosis, combined treatment and reversal of the immunosuppressive status remain the key points to a successful result.
