Acute invasive fungal rhinosinusitis
Acute invasive fungal rhinosinusitis is an angioinvasive infection of the sinonasal mucosa by Mucorales species or Aspergillus in immunocompromised or diabetic ketoacidosis patients that causes vascular thrombosis and tissue necrosis. It is a surgical and infectious disease emergency requiring same-day biopsy, emergent debridement, and high-dose liposomal amphotericin B, with mortality up to 60-80% even with therapy.
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What this article covers
- Overview
Angioinvasive fungal emergency of the sinonasal tract: infection of the nasal and paranasal sinus mucosa by Mucorales (Rhizopus arrhizus, Mucor species) or Aspergillus species that invade vessel…
- Epidemiology
Third most common invasive fungal infection in immunocompromised persons: ranks behind candidiasis and aspergillosis in frequency but carries among the highest mortality of the invasive mycoses.
- Risk factors
Diabetic ketoacidosis: the classic risk factor; acidosis impairs neutrophil function and favors fungal growth, and rhinocerebral mucormycosis is a recognized life-threatening complication of DKA.
- Pathophysiology
Spore inhalation and mucosal inoculation: inhaled spores deposit on nasal and sinus mucosa and germinate in the susceptible host rather than being cleared by neutrophil defenses.
- Clinical features
Facial pain, headache, and fever in a high-risk host: the earliest, most common symptoms; their appearance with DKA, neutropenia, or transplantation should trigger urgent evaluation rather than routine sinusitis management.
- Differential diagnosis
Bacterial acute rhinosinusitis: purulent discharge and facial pressure without necrotic eschar or orbital signs; responds to antibiotics.
- Diagnosis
The diagnosis is confirmed by biopsy showing angioinvasive fungal hyphae on histopathology; there is no formal clinical criteria set or scoring system for this disease.
- Management
First, immediately reverse the predisposing condition in parallel with all other steps: correct DKA with insulin and fluids, reduce immunosuppression where feasible, stop deferoxamine, and promote…
- Complications
Death: mortality remains as high as 60% to 80% even with aggressive combined surgical and antifungal therapy.
- Prognosis
Mortality remains 60% to 80% despite modern combined therapy: driven by speed of diagnosis and surgery, extent of disease at presentation, and reversibility of the underlying immune defect.
- Prevention
Aggressive glycemic control to prevent diabetic ketoacidosis: the single most common precipitant of rhinocerebral mucormycosis; any diabetic patient admitted in DKA should have a nasal and oral…
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Related articles
- Mucormycosis
Mucormycosis is a rapidly progressive, angioinvasive mold infection caused by Rhizopus and Mucor species that thrive in diabetic ketoacidosis, neutropenia, and iron-overload states. Diagnosis rests on biopsy showing broad, nonseptate hyphae in necrotic tissue, and first-line treatment combines urgent surgical debridement with high-dose liposomal amphotericin B, stepped down to posaconazole or isavuconazole; mortality remains 60 to 80 percent.
- Aspergillosis
Invasive aspergillosis is an angioinvasive mold infection of neutropenic and immunosuppressed hosts that begins in the respiratory tract and disseminates hematogenously, diagnosed with CT, galactomannan, and biopsy, and treated first-line with voriconazole.
- Diabetic ketoacidosis
Diabetic ketoacidosis is a life-threatening hyperglycemic crisis of near-absolute insulin deficiency, most common in type 1 diabetes, producing an anion-gap ketoacidosis from unrestrained lipolysis and osmotic diuresis from severe hyperglycemia. It is diagnosed by hyperglycemia, ketonemia, and an anion-gap metabolic acidosis, and treated with intravenous isotonic saline, an intravenous regular insulin bolus and infusion, and aggressive potassium repletion guided by frequent electrolyte monitoring.
- Cavernous sinus thrombosis
Septic thrombosis of the cavernous sinus from spreading facial, sinus, or orbital infection, marked by fever, proptosis, chemosis, and cranial nerve palsies that classically become bilateral. It is a neuro-ophthalmic emergency treated with immediate broad-spectrum IV antibiotics, source control, and consideration of anticoagulation.
- Orbital cellulitis
Orbital cellulitis is infection of the fat and muscle posterior to the orbital septum, most often from spreading sinusitis, that causes proptosis, painful ophthalmoplegia, and vision loss and can progress to cavernous sinus thrombosis. It is a vision- and life-threatening emergency treated with admission, blood and tissue culture, orbital/sinus CT or MRI, and immediate empiric IV vancomycin plus ceftriaxone with ophthalmology and ear, nose, and throat (ENT) involvement.
- Neutropenic fever
Neutropenic fever is an oncologic emergency defined as a single oral temperature of 38.3 C or higher, or 38.0 C sustained for 1 hour, with an ANC of 500/microL or lower, most often during the chemotherapy nadir. Management is empiric antipseudomonal beta-lactam therapy within 60 minutes, with vancomycin, antifungal therapy, and growth factors added only for specific indications.
- Neutropenia
Neutropenia is a reduced absolute neutrophil count that raises infection risk sharply once the count falls below 500/microL, most often from chemotherapy or another marrow-suppressing or idiosyncratic drug. Diagnosis rests on a repeat CBC with differential and smear review followed by a directed search for cause, and management centers on stopping the offending agent, treating any underlying disease, and using G-CSF and neutropenic precautions when the count is low or the patient is febrile.
- Brain abscess
Brain abscess is a focal, ring-enhancing suppurative infection of the brain parenchyma most often from contiguous sinus, ear, or dental spread or hematogenous seeding, diagnosed by contrast MRI and image-guided aspiration and treated with predisposing-source-directed empiric antibiotics plus drainage of abscesses larger than 2.5 cm.
- Acute rhinosinusitis
Acute rhinosinusitis is inflammation of the nasal cavity and paranasal sinuses lasting up to 4 weeks, presenting with purulent nasal discharge plus nasal obstruction or facial pain/pressure/fullness. Most cases are viral and treated with acetaminophen/ibuprofen plus nasal saline irrigation alone; a minority are bacterial, identified by persistence beyond 10 days, a biphasic worsening course, or severe onset, and treated with amoxicillin-clavulanate 875/125 mg PO every 12 hours for 5 to 7 days.
- Nasal polyps
Nasal polyps are benign, typically bilateral outgrowths of edematous nasal mucosa driven by chronic mucosal inflammation, most often arising with chronic rhinosinusitis, aspirin-exacerbated respiratory disease, or cystic fibrosis. Unilateral polyps or a mass that does not respond to treatment must be biopsied to exclude tumor.