Imaging of COVID-19-associated craniofacial mucormycosis: a black and white review of the "black fungus".

A subset of diabetic COVID-19 patients treated with steroids, oxygen, and/or prolonged intensive care admission develop rhino-orbito-cerebral mucormycosis. Radiologists must have a high index of suspicion for early diagnosis, which prompts immediate institution of antifungal therapy that limits morbidity and mortality. Assessment of disease extent by imaging is crucial for planning surgical debridement. Complete debridement of necrotic tissue improves survival. Imaging features reflect the angioinvasive behaviour of fungal hyphae from the Mucoraceae family, which cause necrotising vasculitis and thrombosis resulting in extensive tissue infarction. Contrast-enhanced magnetic resonance imaging (MRI) is the imaging technique of choice. The classic "black turbinate" on contrast-enhanced imaging represents localised invasive fungal rhinosinusitis (IFRS). A striking radiological feature of disseminated craniofacial disease is non-enhancing devitalised and necrotic soft tissue at the orbits and central skull base. Sinonasal and extrasinonasal non-enhancing lesions in IFRS are secondary to coagulative necrosis induced by fungal elements. Multicompartmental and extrasinonasal tissue infarction is possible without overt bone involvement and caused by the propensity of fungal elements to disseminate from the nasal cavity via perineural and perivascular routes. Fungal vasculitis can result in internal carotid artery occlusion and cerebral infarction. Remnant non-enhancing lesions after surgical debridement portend a poor prognosis. Assessment for the non-enhancing MRI lesion is crucial, as it is a sole independent prognostic factor for IFRS-specific mortality. In this review, we describe common and uncommon imaging presentations of biopsy-proven rhino-orbito-cerebral mucormycosis in a cohort of nearly 40 COVID-19 patients.

Cerebrospinal fluid leak from lateral orbit during exenteration for mucormycosis.

We describe an elderly diabetic patient presenting with sudden onset right-sided proptosis and vision loss secondary to rhino-orbital mucormycosis and central retinal vascular occlusion. He underwent orbital exenteration that was complicated by intraoperative cerebrospinal fluid (CSF) leak from lateral orbital wall. The leak was surgically repaired and the patient recovered well. We postulate the cause of the CSF leak to be twofold: necrotic periorbital tissue due to mucormycosis rendering the thin bones susceptible to damage and second, intraoperative manipulation and dissection at the orbital apex with monopolar cautery and instruments. We describe measures taken to successfully repair the CSF leak and the possible precautions that can be taken to avoid it.

Cavernous sinus-orbital apex aspergillus infection in a diabetic patient: A case report.

RATIONALE: Cavernous sinus-orbital apex aspergillosis is a rare but serious complication of rhinosinusitis. Pathology results are scarce, and this condition is difficult to diagnose based on clinical and radiological results. PATIENT CONCERNS: A 64-year-old woman presented with cavernous sinus-orbital apex syndrome. Axial and sagittal T1 contrast-enhanced magnetic resonance imaging (MRI) showed a right orbital apex mass abutting the right posterior ethmoid sinus, sphenoid sinus, and cavernous sinus. DIAGNOSIS: Cavernous sinus and orbital apex aspergillosis. INTERVENTIONS: Functional endoscopic sinus surgery was performed, and a biopsy of the lesion tissue was submitted for examination, which showed the presence of aspergillus. OUTCOMES: One year after surgical debridement, antifungal, and anticoagulation treatments, the patient is still asymptomatic without recurrence. LESSONS: Early surgical debridement is crucial to confirm the diagnosis of cavernous sinus-orbital apex aspergillosis and prevent mortality. There is no evidence of negative effects of antibiotic and anticoagulation treatments. Despite controversy, among physicians, most opt to treat patients via anticoagulation therapy.

Complete surgical resection and short-term survival in acute invasive fungal rhinosinusitis.

BACKGROUND: Acute invasive fungal rhinosinusitis (AIFR) is a fulminant fungal infection seen in patients who are immunocompromised. Due to its rarity, there is little evidence regarding the appropriate antifungal treatment regimen, especially the degree of surgical intervention. OBJECTIVE: To assess factors that impact short-term survival in AIFR as defined by survival to hospital discharge and to develop a staging system to predict survival and complete surgical resection. METHODS: Fifty-four patients with histopathologically diagnosed AIFR who met inclusion criteria were identified between 1984 and 2014. Patient characteristics, disease extent, treatment modality, and short-term survival data were collected. Univariate analysis was performed to assess for factors associated with survival and increased likelihood of surgical resection. RESULTS: Of 52 patients with adequate documentation, 36 (69.2%) survived their hospital stay. Complete surgical resection was the only factor associated with improved survival (survival, 95.5%; p < 0.01). A surgical staging system was proposed to guide probability of complete resection and overall prognosis, with stage I disease limited to the nasal cavity, stage II involving the paranasal sinuses, stage III involving the orbit, and stage IV with skull base or intracranial extension. The χ2 analyses showed a decreased likelihood of complete surgical resection with stage III or IV disease compared with stage I (resection, 90.9%) (stage III resection, 37.5% [p = 0.01]; stage IV resection, 16.7% [p = 0.002]). There was a decreased likelihood of survival associated with increasing disease stage compared with stage I (survival, 100%) (stage II survival, 60% [p = 0.009]; stage III survival, 62.5% [p = 0.02]; stage IV survival, 54.6%, [p = 0.006]). CONCLUSION: Although further studies are needed to define specific treatment protocols, analysis of these data indicated that endoscopic sinus surgery with the goal of complete surgical resection may provide the best survival outcomes in select patients when complete surgical resection can be performed. Our staging system represents the first attempt to predict surgical success and prognosis in patients with AIFR.

Successful Treatment of Rhino-Orbital-Cerebral Mucormycosis in a Child With Leukemia.

Rhino-orbital-cerebral mucormycosis (ROCM) is a rare fulminant opportunistic fungal infection that despite relevant treatment has high mortality. We present a case of a 3-year-old girl with acute lymphoblastic leukemia and ROCM, who was treated successfully with excessive surgery, systemic antifungal treatment with amphotericin B (AmB), posaconazole, and terbinafine as well as hyperbaric oxygen. Surgery included, beside extracranial and intracranial removal of infected areas, endoscopic sinus and skull base surgery with local AmB installation and in addition placement of an Ommaya reservoir for 114 intrathecal administrations of AmB. In addition, we review the literature of ROCM in pediatric patients with hematological diseases.

Apical Orbital Aspergillosis Complicating Giant Cell Arteritis.

A 75-year-old woman with new onset headaches and left vision loss, temporal scalp tenderness, and jaw claudication was found to have biopsy-proven giant cell arteritis (GCA). Despite treatment and improvement with prednisone, she later developed left orbital apex syndrome, and an orbital biopsy revealed aspergillosis. After antifungal treatment, extraocular motility improved although vision in the left eye remained no light perception. Clinicians should be aware that fungal orbital apex disease may mimic or complicate steroid-treated GCA.

Granulicatella elegans Causing Periorbital Infection During Orthodontic Treatment.

Granulicatella elegans is a normal component of the oral flora and is an unusual causative agent of infective endocarditis. A case of G. elegans periorbital infection of the eyelid after dental treatment is reported. A healthy 35-year-old man presented with painful swelling of the left upper eyelid. He was empirically treated with oral amoxicillin for 1 week. He presented 3 months later with the same clinical features. G. elegans and Staphylococcus epidermidis were identified in bacterial cultures from wound aspirates. Probable relapse of periorbital infection was successfully treated with a 6-week course of oral amoxicillin. This is the first reported case of a non-bloodstream infection caused by G. elegans. Clinicians should be aware of G. elegans as an unusual causative agent of periorbital infection. Within the limitations of this case report, prolonged antibiotic therapy is recommended for a G. elegans periorbital infection to minimize the risk of relapse.

A Case of Orbital Histoplasmosis.

Histoplasma capsulatum var capsulatum is a dimorphic fungus endemic to the Ohio and Mississippi River Valleys of the United States. In this case report, a 33-year-old woman who presented with a right orbital mass causing progressive vision loss, diplopia, and facial swelling is described. Lateral orbitotomy with lateral orbital wall bone flap was performed for excisional biopsy of the lesion. The 1.5 × 1.8 × 2.3 cm cicatricial mass demonstrated a granulomatous lesion with necrosis and positive staining consistent with Histoplasma capsulatum var capsulatum infection. To the authors' knowledge, this is the first case of orbital histoplasmosis to be reported in the United States and the first case worldwide of orbital histoplasmosis due to Histoplasma capsulatum var capsulatum.

Alternaria-Associated Fungus Ball of Orbit Nose and Paranasal Sinuses: Case Report of a Rare Clinical Entity.

Alternaria-associated fungus ball of maxillar, ethmoidal paranasal sinuses, nasal cavity and orbit with bone erosion is extremely rare. Till recently, only two cases of this infection in immune competitive patients have been reported. We are herein describing the case of immune-competent woman who suffered of nasal congestion for 10 years. Patient was treated for tumor-like lesion in right maxillar sinus, where propagation in right nose cavity, right ethmoidal cells and right orbita was present. The organism that was seen in surgical removal of fungal debris by histological study, in using mycological testing, was proven as Alternaria alternata. Combination of surgical intervention and treatment with itraconazole eradicated fungal infection, and the disease was not relapsed in follow-up period of 2 years.

Post-operative endophthalmitis caused by Acremonium falciforme with orbital and extra-orbital involvement following combined cataract and glaucoma surgery: a case report.

INTRODUCTION: In this report, we describe an unusual case of post-operative Acremonium falciforme endophthalmitis with orbital and extra-orbital involvement following combined cataract and glaucoma surgery. CASE PRESENTATION: A 68-year-old Caucasian man with glaucoma presented with endophthalmitis characterized by pain, redness and impaired vision in the left eye fifteen days after combined cataract and filtering surgery. He subsequently underwent a pars plana vitrectomy, with vitreous sampling, silicone oil placement and intra-vitreal injection of antibiotics, but only after a second vitrectomy we identified Acremonium falciforme as the causative agent for the endophthalmitis. An antifungal systemic and topical therapy was started, but meanwhile the infection extended to orbital and peri-orbital tissues. Following these procedures, even if the eye went slowly in phthisis, we were able to limit the further extension and circumscribe the orbital and extra-orbital involvement. CONCLUSION: To our knowledge, this report is the first describe Acremonium falciforme endophthalmitis with orbital and extra-orbital involvement, following anterior segment combined surgery. Ophthalmologists and physicians should be aware of the extension risk of a fungal panophthalmitis, but also to potentially serious side effects related to systemic therapy.

[Two cases of allergic fungal rhinosinusitis with bone destruction].

We report herein two cases of allergic fungal rhinosinusitis accompanied by bone destruction of the adjacent nasal sinuses. The first case involved a 21-year-old man who presented with left exophthalmos. Computed tomography (CT) showed soft tissue lesions in the left paranasal sinuses and destruction of the left lamina papyraceae, as well as infiltration of the lesion into the orbit. In the second case, a 39-year-old man, CT showed bone destruction of the skull base and medial wall of the left orbit. In both cases, total serum immunoglobulin (Ig)E level was >1000 IU/mL and fungus-specific IgEs were increased. Fungal hyphae were identified within the mucus on histopathological examination in both cases; however, no fungal invasion of the mucosa was apparent. Final diagnosis was allergic fungal rhinosinusitis (AFRS) in both cases. AFRS is a relatively new disease concept that was proposed in the early 1980s, with disease characteristics very similar to eosinophilic rhinosinusitis. Occasionally, AFRS must be differentiated from malignant disease or invasive fungal rhinosinusitis, so an understanding of the clinical features is important.

Subcutaneous phaeohyphomycosis of the face presenting as rhinoentomophthoramycosis.

OBJECTIVES: Subcutaneous phaeohyphomycosis is the most common variety of phaeohyphomycosis and presents as asymptomatic or mildly painful, localized cysts, abscesses, or sometimes chromoblastomycosis-like lesions over the feet, legs, or hands in about 60-85% of cases. It usually afflicts adults with some degree of immunosuppression. METHODS: We report a 30-year-old man, who presented with features of recurrent sinusitis (episodes of fever, nasal stuffiness, paranasal sinus pain, and tenderness), mucosal hypertrophy of the maxillary sinuses, and mid-face swelling that was freely mobile and suggestive of rhinoentomophthoramycosis. He had no other associated illness. RESULTS: Histology suggested tumid lupus erythematosus. The diagnosis was based on Fonsecaea pedrosoi cultured from a biopsy specimen. CONCLUSIONS: Combination therapy with itraconazole and saturated solution of potassium iodide (SSKI) was more effective than itraconazole used alone.

Posaconazole pharmacokinetics in a 2-year-old boy with rhino-cerebral-orbital zygomycosis.

Posaconazole is a triazole antifungal agent used as adjuvant or salvage therapy for the treatment of zygomycosis, an invasive fungal infection associated with high mortality. Oral posaconazole absorption is highly variable. We describe the pharmacokinetics of oral posaconazole in a 2-year-old boy with rhino-cerebral-orbital zygomycosis. Seven days after induction therapy for acute lymphoblastic leukemia, he was brought to the emergency department because of left eyelid swelling and was admitted to the hospital. Zygomycosis was diagnosed 12 days later. After we conducted a literature search and consulted with antifungal drug experts, a triple-antifungal regimen consisting of liposomal amphotericin B, caspofungin, and posaconazole was started. Given the severity of the disease, we aimed for posaconazole plasma trough concentrations greater than 1.25 µg/ml; the dosage necessary to achieve this goal was posaconazole 200 mg 4 times/day. After a difficult 105-day stay in the hospital and stabilization of the fungal infection, the patient was discharged. Caspofungin was discontinued at time of discharge, but the patient continued to receive amphotericin B lipid complex 7.5 mg/kg/day intravenously and posaconazole 200 mg orally 4 times/day. This is one of the few case reports describing posaconazole pharmacokinetics in a child younger than 8 years. In patients with extensive zygomycosis, a triple-antifungal regimen, combined with therapeutic drug monitoring of posaconazole, may be helpful.

Clostridium septicum gas gangrene in the orbit: a case report.

Our report presents a case of Clostridium septicum gas gangrene in an unusual, orbital localization. The predisposing factors are typical: colon tumour and lymphatic malignancy. Most probably bacteria from the intestinal flora entered the bloodstream through the compromised intestinal wall and settled in the orbit resulting in the development of an abscess containing gas. At the site of the gas gangrene, an indolent B cell lymphoma was present. After surgery and antibiotic treatment, the patient healed from the C. septicum infection; but subsequently died as a consequence of the tumour.

Two cases of feline orbital aspergillosis due to Aspergillus udagawae and A. viridinutans.

Aspergillus udagawae and A. viridinutans are members of the section Fumigati; both cause invasive aspergillosis in humans. These two Aspergillus species are discriminated from A. fumigatus by molecular methods. Herein, we report two cases of feline orbital aspergillosis, one caused by A. udagawae and the other by A. viridinutans. To our knowledge, Case 1 represents the first reported case of treatment of A. udagawae with a high dosage of itraconazole, and Case 2 represents the first reported case of A. viridinutans infection associated with sarcoma. Identification of the etiologic agents of these cases was confirmed by comparative analyses of the sequences of ß-tubulin-encoding genes. With the spread of non-fumigatus aspergillosis, increasing emphasis should be placed on molecular identification of the infecting Aspergillus species and the use of in vitro drug susceptibility tests to ensure the selection of appropriate antibiotics.

Large primary intraorbital hydatid cyst.

The authors report a rare case of a large primary intraorbital hydatid cyst diagnosed in 3-year-old children who had presented with unilateral progressive nonpulsatile proptosis of the left eye, explored by computed tomography and magnetic resonance imaging.

[Five cases of invasive rhino-orbital-skull base aspergillosis with orbit apex syndrome initial presentation].

OBJECTIVE: Although rare, invasive rhino-orbital-skull base aspergillosis usually is fatal and necessitates prompt diagnosis, surgical intervention and antifungal treatment. METHOD: A case series of 5 patients, with review of treatments and outcomes. RESULT: Five patients initially presented with orbital apex syndrome without apparent rhino-sinus disease. Histologic examination revealed invasive aspergillosis. Four patients underwent transnasal endoscopic surgical exploration of rhino-orbital-skull base, and three of them simultaneously underwent optic nerve decompression. One patient underwent the rhinopharyngeal biopsy. Following surgical debridement and antifungal drug, Two patients achieved various degree of recovery, 1 patient (case 5) showed vision improvement, from counting fingers to 0.1 and symptom-free during 65 months follow-up. One patient (case 1) showed no recovery of vision from no light perception, but symptom-free during 43 months follow-up. Other three patients respectively died of massive intracranial spread (case 3), postoperative brainstem hemorrhage (case 2) and sepsis (case 4). CONCLUSION: Invasive sino-orbital-skull base aspergillosis often may resemble inflammatory conditions. Early diagnosis and surgical intervention are required to improve vision and survival. The endoscopic surgical exploration and biopsy often are necessary to rule out fungal sinus infection before considering steroid use, especially in immunocompromised individuals. The patients with invasive rhino-orbital-skull base aspergillosis usually have a poor prognosis.

Clival inflammation with cavernous sinus thrombophlebitis and orbital subperiosteal abscess--case report.

A 64-year-old woman presented with a very rare case of three infectious lesions, cavernous sinus thrombophlebitis, clival inflammation, and orbital subperiosteal abscess (SPA), manifesting as abducens palsy. An isolated non-specific mass in upper clivus was initially suspected to be derived from paranasal sinusitis. The clival lesion was approached by an endonasal transsphenoidal route and diagnosed as inflammation. However, progressive enlargement of an orbital mass was recognized, with eyelid erythema and swelling. Magnetic resonance imaging showed massive paranasal sinusitis and an intra-orbital mass, which was proved to be an orbital SPA by open surgery. Cavernous sinus thrombophlebitis might have been caused by primary paranasal sinusitis, and the origin of orbital cellulitis was suspected to be cavernous sinusitis based on the preoperative radiological findings. These unusual lesions should be kept in mind as one of the differential diagnoses.