title: Chronic orbital and calvarial fungal infection with Apophysomyces variabilis in an immunocompetent patient.
abstract: Apophysomyces is a rare fungal organism causing rhino-orbito-cerebral mycotic infections with high morbidity and mortality, typically in immunocompetent individuals. Several cases of Apophysomyces elegans orbital disease have been reported. Herein, we report a case of Apophysomyces variabilis infection involving the orbit, sinuses, and calvarium in an immunocompetent 74-year-old woman, with a review of the literature. Unlike prior cases of A. elegans classic rhino-orbito-cerebral infection, our case included diffuse calvarial lytic lesions and overlying soft tissue nodules, but without parenchymal intracranial involvement. There was radiographic and clinical evidence of infarction of the orbital contents and cavernous sinus thrombosis. Anastomoses between the superior orbital (ophthalmic) vein and diploic veins of the calvarium are believed to be primarily responsible for the unusual mode of spread on the extradural surface of the brain. Although the patient stabilized without definitive surgical intervention, her disease slowly and intermittently progressed for over a year after presentation, requiring multiple courses of antifungal treatment.
authors.affiliation: Department of Ophthalmology, Harvard Medical School, Boston, Massachusetts, USA; Department of Ophthalmology, Massachusetts Eye & Ear Infirmary, Boston, Massachusetts, USA.
authors.forename: Natalie
authors.initials: N
authors.lastname: Wolkow
authors.affiliation: Department of Ophthalmology, Harvard Medical School, Boston, Massachusetts, USA; Department of Ophthalmology, Massachusetts Eye & Ear Infirmary, Boston, Massachusetts, USA; Department of Ophthalmology, David G. Cogan Laboratory of Ophthalmic Pathology, Massachusetts Eye & Ear Infirmary, Boston, Massachusetts, USA. Electronic address: Fred_Jakobiec@meei.harvard.edu.
authors.forename: Frederick A
authors.initials: FA
authors.lastname: Jakobiec
authors.affiliation: Department of Ophthalmology, Harvard Medical School, Boston, Massachusetts, USA; Department of Ophthalmology, Massachusetts Eye & Ear Infirmary, Boston, Massachusetts, USA; Department of Ophthalmology, David G. Cogan Laboratory of Ophthalmic Pathology, Massachusetts Eye & Ear Infirmary, Boston, Massachusetts, USA.
authors.forename: Anna M
authors.initials: AM
authors.lastname: Stagner
authors.affiliation: Department of Ophthalmology, Harvard Medical School, Boston, Massachusetts, USA; Department of Radiology, Massachusetts Eye & Ear Infirmary, Harvard Medical School, Boston, Massachusetts, USA.
authors.forename: Mary E
authors.initials: ME
authors.lastname: Cunnane
authors.affiliation: Division of Infectious Disease, Department of Medicine, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA.
authors.forename: Anne L
authors.initials: AL
authors.lastname: Piantadosi
authors.affiliation: Division of Infectious Disease, Department of Medicine, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA.
authors.forename: Nesli
authors.initials: N
authors.lastname: Basgoz
authors.affiliation: Department of Ophthalmology, Harvard Medical School, Boston, Massachusetts, USA; Division of Ophthalmic Plastic Surgery, Department of Ophthalmology, Massachusetts Eye & Ear Infirmary, Boston, Massachusetts, USA.
authors.forename: Daniel
authors.initials: D
authors.lastname: Lefebvre
mesh_terms: D000368:Aged; D000935:Antifungal Agents / Q000627:therapeutic use; D003646:Debridement / Q000379:methods; D019468:Disease Management; D015821:Eye Infections, Fungal / Q000453:epidemiology / Q000382:microbiology / Q000628:therapy; D005260:Female; D014943:Global Health; D006801:Humans; D016867:Immunocompromised Host; D009017:Morbidity; D009090:Mucorales / Q000302:isolation & purification*; D009091:Mucormycosis / Q000453:epidemiology / Q000382:microbiology / Q000628:therapy; D013508:Ophthalmologic Surgical Procedures; D009916:Orbital Diseases / Q000453:epidemiology / Q000382:microbiology / Q000628:therapy
chemical_list: D000935:Antifungal Agents
keywords: Apophysomyces; Saksenaea; amphotericin B; calvarial lytic lesions; diploic venous system; fungus; mucor; posaconazole; rhino-orbito-cerebral mucormycosis