Fungal Infections of the Sinonasal Tract and Their Differential Diagnoses.

Fungal rhinosinusitis is a broad group of diseases that includes noninvasive and invasive forms with overlapping clinical presentations. While most cases of fungal rhinosinusitis follow an indolent clinical course, surgical pathologists play a crucial role in early identification of life-threating subtypes, specifically invasive fungal rhinosinusitis. This review describes fungal infections of the sinonasal tract and their histopathologic mimickers. Clinical, gross, and microscopic features that are important for diagnosis, as well as available ancillary studies, are discussed.

Orbital apex syndrome secondary to acute invasive fungal rhinosinusitis diagnosed by transnasal endoscopic biopsy of the optic canal:A case report.

Orbital apex syndrome (OAS) is a complex condition characterized by visual loss, diplopia, and eye pain that occurs secondary to several pathological processes involving the orbital apex. We report a case of acute invasive fungal rhinosinusitis (AIFRS) associated with OAS. A 76-year-old man with left-sided visual loss, diplopia, palpebral ptosis, and headache was diagnosed with OAS secondary to Tolosa-Hunt syndrome and received systemic corticosteroid therapy from his neurologist. Owing to persistent symptoms, we opened the optic canal using a transnasal endoscopic approach for a surgical biopsy of the orbital apex lesions. Histopathological evaluation revealed numerous Aspergillus organisms in the biopsied granuloma. After surgical debridement, he received a 12-month course of voriconazole, and no recurrence of AIFRS occurred during 8-year follow-up. Patients with OAS may occasionally be prescribed corticosteroids because the clinical manifestations of AIFRS-induced OAS are similar to those observed in OAS secondary to Tolosa-Hunt syndrome, especially no nasal symptoms which is known to respond to corticosteroid therapy. Because both AIFRS-induced OAS and OAS secondary to Tolosa-Hunt syndrome induce ophthalmoplegia, proptosis, eye pain, it is sometimes difficult to differentiate these two diseases in early stage. However, corticosteroid therapy causes exacerbation of fungal infection in patients with AIFRS-induced OAS resulting in delayed accurate diagnosis and poor prognosis. AIFRS is associated with a high mortality rate ; therefore, transnasal endoscopic biopsy of orbital apex lesions before corticosteroid administration is recommended in patients with OAS. J. Med. Invest. 71 : 310-313, August, 2024.

A Rare Case of Fungal Granuloma Confined to the Nasal Septum.

Granulomatous lesions in the nasal sinuses are associated with a variety of diseases, including immune disorders such as sarcoidosis, vasculitis, immunoglobulin G4 (IgG4)-related diseases, malignant lymphomas, and microbial infections. Here, we report a rare case of fungal granuloma that occurred exclusively within the nasal septum. The patient presented to the Department of Surgery with the chief complaint of nasal obstruction associated with nasal septal deviation. A bulge was found below the right nasal septum. Initially, it was diagnosed as mucosal swelling associated with rhinitis, and surgery was performed. A granulomatous lesion with bone destruction was found under the mucosa of the nasal septum, which led to the diagnosis of fungal granulation based on postoperative pathology. Though bacterial and fungal infections of the nasal septum are occasionally observed, this is the first reported instance of a fungal granuloma confined to the nasal septum. Infection within the nasal septum, although rare, should also be considered as a differential diagnosis for morphological abnormalities of the nasal septum.

Invasive phaeohyphomycosis co-infection with Alternaria spp. and Curvularia spp. in a neutropenic host.

Phaeohyphomycoses are infections caused by dark-walled dematiaceous fungi. Alternaria and Curvularia are two genera of dematiaceous molds known to cause invasive fungal rhinosinusitis, particularly in immunocompromised patients. Co-infection with two dematiaceous fungi is rarely reported in the literature. This report describes a case of biopsy proven invasive fungal rhinosinusitis with Alternaria spp. and Curvularia spp. co-infection in a neutropenic host. The infection characteristics, microbiologic findings, and treatment are described.

Number of sinus procedures reduces inpatient mortality for invasive fungal rhinosinusitis.

OBJECTIVES: To evaluate surgical outcomes of invasive fungal rhinosinusitis MATERIALS AND METHODS: The National Inpatient Sample Database (2000-2015 Q3) was queried for patients with a diagnosis of aspergillosis and/or mucormycosis and a diagnosis of acute sinusitis using the International Classification of Diseases, Ninth Edition. Factors associated with inpatient mortality were then identified with multivariate logistic regression. RESULTS: 514 adult patients with a median age of 57.0 years were identified, of which 231 (44.9 %) underwent sinus surgery. Surgical patients had a longer length of stay (17.0 vs 9.0 days, p < 0.001) and higher total charges ($139,762.00 vs $57,945.00, p < 0.001). The number of sinus procedures was associated with reduced odds of inpatient mortality (OR 0.69; p < 0.001) in multivariate analysis. Hypertension (OR 0.34, p = 0.002) and chronic kidney disease (OR 0.23, p = 0.034) were associated with reduced odds of inpatient mortality. Total number of procedures (OR 1.24; p = 0.002), mucormycosis (OR 2.75, p = 0.002), age (OR 1.03, p = 0.006) and acid-base disorders (OR 2.85, p = 0.012) were associated with increased odds of inpatient mortality. CONCLUSION: This represents the first large scale study to evaluate outcomes for invasive fungal rhinosinusitis. These findings suggest the odds of inpatient mortality decrease with greater extent of sinus surgery performed. The potentially protective roles of hypertension and chronic kidney disease should be evaluated in future research.

Invasive fungal rhinosinusitis by Fusarium proliferatum/annulatum in a patient with acute myeloid leukemia: A case report and review of the literature.

Antifungal prophylaxis with a mold-effective agent has led to a substantial decrease in invasive infections caused by Aspergillus spp. in the management of patients with acute myeloid leukemia undergoing induction chemotherapy. However, difficult-to-treat infections caused by other molds, such as Fusarium, Lomentospora, and Scedosporium species may still complicate the neutropenic period. Here, we present a case of a 23-year-old woman with acute myeloid leukemia who developed a breakthrough invasive fungal rhinosinusitis caused by Fusarium proliferatum/annulatum on posaconazole prophylaxis. The infection was diagnosed using clinical, microbiological, and radiological criteria and the isolate was identified using Matrix Assisted Lazer Desorption Ionization Time of Flight Mass Spectrometry (MALDI-TOF MS) and sequencing. We searched Pubmed with "Fusarium proliferatum", "Fusarium annulatum", "immunosuppression AND fusariosis", "rhinosinusitis AND Fusarium proliferatum" and summarized the English literature for similar rhinosinusitis cases infected with the same pathogen.

Acute invasive fungal rhinosinusitis (AIFRS) - A histopathological analysis of expanding spectrum of fungal infections in backdrop of COVID-19 pandemic.

Context: Acute invasive fungal rhinosinusitis (AIFRS) is an aggressive infection affecting immunocompromised patients and carries a high morbidity and mortality. It is commonly seen in immunocompromised patients, mainly in uncontrolled diabetes, malignancy, acquired immunodeficiency syndrome, and so on. However, there has been an exponential increase in the incidence of AIFRS in relation to recent coronavirus disease 2019 (COVID-19) infection. Aims: We present this study to assess histomorphological features of fungal infections in the background of COVID-19 era. Materials and Methods: The study includes interpretation of 34 biopsies of suspected AIFRS in post COVID-19 patients. The demographic details like patients age, sex, diabetic status, COVID-19 status, and history of steroid intake were collected. All specimens were stained with hematoxylin and eosin and PAS stain. Detailed microscopic examination including the presence of fungal hyphae in the tissue, characterization of inflammatory response, presence of tissue invasion, angioinvasion, and necrosis was noted for each case. Results: Thirty-four biopsy specimens from various sites - nasal cavity, maxillary sinus, ethmoid sinus, and so on - were studied. The mean age of the patients with AIFRS was 52.68 years. The dominant fungi were Mucorales in 31 (91.3%), Aspergillus and Mucorales in 1 (2.9%), a combination of Mucorales and Candida identified in 1 (2.9%) case, and Candida alone in 1 case (2.9%). Bony invasion and perineural invasion were observed in 5 cases (14.7%) and 1 (2.9%) case, respectively. Conclusion: Histopathological examination plays an essential role in the diagnosis and appropriate management of the patients. Histopathological features including characterization of fungi, angioinvasion, and bone invasion may provide information on rare dreaded infections in post-COVID-19 patients for possible prognostic characteristics on histology.

A Rare Case of Chronic Rhinosinusitis With Elevated Levels of Immunoglobulin G4.

Chronic rhinosinusitis is a condition characterized by inflammation of the paranasal sinuses causing several symptoms, including facial pain, anosmia, nasal drainage, and obstruction for a minimal duration of three months. It is a commonly occurring disease and is diagnosed through direct visualization or the detection of inflammation on a CT scan. A mucosal tissue biopsy typically reveals stromal fibrosis and an increase in submucosal glands, and infiltration of mixed mononuclear cells, neutrophils, and eosinophils may also be present but typically makes up less than 10% of the total cells. T helper type 2 releasing cytokines, interleukins (IL-5 and IL-13), as well as histamine, are frequently found in high concentrations in polyp tissue. We report a case of rare chronic rhinosinusitis. The patient's specimen shows a very high number of immunoglobulin G4 (IgG4)-positive plasma cells.

Neurosarcoidosis - A diagnostic challenge?

Background: Sarcoidosis is an inflammatory granulomatous multisystem disease with an unknown etiology. Neurosarcoidosis is a cryptogenic neuroinflammatory manifestation of sarcoidosis. Objective: This article aims at better understanding of one of the rarer diseases whose diagnosis may be difficult leading to delay in definitive management of the patient. Method: We describe a case of neurosarcoidosis with initial presentation similar to acute invasive fungal rhinosinusitis, whose diagnosis was challenging and delayed owing to the presenting symptoms. Conclusion: The diagnosis of neurosarcoidosis becomes challenging when it presents as isolated clinical neurological symptoms. We want to highlight the variable nature of neurosarcoidosis and its consideration as diagnosis after excluding other common infectious and inflammatory conditions.

Pediatric Invasive Fungal Rhinosinusitis: A Comprehensive Analysis of Prognostic Factors for Survival.

OBJECTIVE: Pediatric invasive fungal rhinosinusitis (IFS) is a devastating infection that manifests almost exclusively in immunocompromised children. The goal of this work was to determine which clinical features carry prognostic value for survival. METHODS: A retrospective review of children with a histopathological diagnosis of IFS was performed at an academic tertiary care institution from 1990 to 2021. Clinical variables were collected to generate survival and life-table estimators at 6-months and 1-year. RESULTS: Eighteen patients were included in this analysis, with a mean age of 9.8 years (range, 1-17 years). Most children were neutropenic (n = 15, 83.3%), with acute lymphoblastic leukemia (n = 10, 55.6%) representing the most common primary diagnosis. A mean of 3.2 operations (range 1-7 operations) was performed per patient for either mucormycosis (n = 10, 55.6%) or aspergillosis (n = 8, 44.4%). The mean time to absolute neutrophil count recovery was 65.8 days (range 20-137 days), with a 6-month and 1-year survival rate of 47.6% and 41.7%, respectively. Gross total resection (p = 0.006, p < 0.001), number of antifungals (p = 0.0004, p = 0.0003), and total operation number (p = 0.0032, p = 0.0035), served as positive prognostic factors for 6-month and 1-year survival. Conversely, altered mental status (p = 0.0026), cerebral involvement (p = 0.0010), cranial neuropathies (p < 0.0001), hyperglycemia (p = 0.0445, p = 0.0208), and intensive care unit status (p = 0.0013) served as negative prognostic factors for 6-month and 1-year survival. CONCLUSION: Several key elements were identified and found to play a vital role in influencing survival for pediatric IFS. Early diagnosis, prompt medical therapy, and aggressive surgical intervention remain at the forefront in the treatment of this complex opportunistic infection. LEVEL OF EVIDENCE: 4 Laryngoscope, 133:1239-1250, 2023.

Diagnostic and therapeutic strategies of acute invasive fungal rhinosinusitis.

Acute invasive fungal rhinosinusitis (AIFR) is a rare disease, but the prognosis is by no means ideal. Pathologically, fungal infection is not only located in the sinus cavity, but also invades the sinus mucosa and bone wall, the surrounding structures and tissues such as the orbit and anterior skull base are often compromised and are accompanied with intracranial and extracranial complications. Despite decades of efforts, acute invasive fungal rhinosinusitis remains a devastating disease, the mortality of the disease continues to hover around 50%. The main impediments to improving the prognosis of acute invasive fungal rhinosinusitis are the difficulties of early diagnosis and the rapid reversal of immune insufficiency. Moreover, aggressive surgery combined with systemic antifungal therapy are significant positive prognostic factors as well. Progress and standardization of AIFR treatment protocols have been limited by the scarcity of the disease and the absence of published randomized studies. Therewith, how to improve the therapeutic outcome and reduce the mortality rate has always been a challenging clinical discussion. We have summarized the relevant case series and literature from the recent years, management with optimal diagnostic and curative strategies are reviewed.

Gracin's Syndrome, a Rare Clinical Challenge in the Setting of Invasive Mucormycosis: A Systematic Review.

Garcin's Syndrome is a rare pathology involving multiple cranial nerves in the setting of invasive mucormycosis, usually in immunocompromised patients. Owing to its extremely high mortality rate, clinician should have a high suspicion for diagnosis. This article presents a rare case of Rhino-Orbito-Cerebral Mucormycosis with Garcin's syndrome in a 33-years old male along with a discussion of previously reported cases. The case is discussed in light of scant contemporary literature on the cited subject. A thorough search using the keywords Garcin's Syndrome, Invasive Mucormycosis, Rhino-orbital, Rhino-cerebral mucormycosis, was conducted on Pubmed/MEDLINE, Google scholar, LILACS, medRxiv and Google. A total of six reported cases found in international literature published between 2000 and 2020 were reviewed and analyzed. Garcin's Syndrome is associated with a high mortality rate. In our review, of the total seven patients, only three survived, bringing the collective mortality to 42.85%. Patients had multiple cranial nerve involvement leading to long term sequelae. Our case showed the unique finding of VIII nerve involvement. Early intervention helped in patient survival and better functional outcome. This literature review highlights the distinct clinical nature of the presentation of disease and the importance of prompt diagnosis and early management in the reversal of complications in an otherwise potentially fatal disease.

A Multi-Institutional Database Review of Orbital Complications and Survival Outcomes in Adult Patients with Invasive or Non-Invasive Fungal Rhinosinusitis.

BACKGROUND: Invasive fungal rhinosinusitis (IFS) with orbital complications has remained a challenging disease over the past few decades. Only a few studies have been conducted to investigate the factors associated with orbital complications in fungal rhinosinusitis (FRS). We aimed to review the characteristics between IFS and non-invasive fungal rhinosinusitis (NIFS) and determine clinical factors associated with orbital complications and overall survival. METHODS: A multi-institutional database review study was conducted using the Chang Gung Research Database (CGRD) from January 2001 to January 2019. We identified FRS patients using International Classification of Diseases diagnosis codes and SNOMED CT. We categorized patients into IFS and NIFS groups and analyzed the demographic data, underlying diseases, clinical symptoms, laboratory data, image findings, fungal infection status, and survival outcomes. RESULTS: We included 1624 patients in our study, with 59 IFS patients and 1565 NIFS patients. The history of an organ or hematopoietic cell transplantation had a significant prognostic effect on the survival outcomes, with surgical intervention and high hemoglobin (Hb) and albumin levels recognized as positive predictors. Posterior ethmoid sinus involvement, sphenoid sinus involvement, facial pain, blurred vision, and periorbital swelling were risk factors of orbital complications. CONCLUSIONS: In NIFS patients, orbital complications were found to be associated with old age, a high WBC count, high blood glucose, and a high CRP level. For the risk factors of orbital complications in IFS patients, posterior ethmoid sinus involvement, sphenoid sinus involvement, facial pain, blurred vision, and periorbital swelling were recognized as predictors. Among IFS patients, a history of organ or hematopoietic cell transplantation was a risk factor for poor survival, while, conversely, surgical intervention and high Hb and albumin levels were related to improved survival. As predictors of orbital complications in IFS patients, posterior ethmoid sinus involvement, sphenoid sinus involvement, facial pain, blurred vision, and periorbital swelling upon the first visit should raise attention, with close monitoring.

Orbital Complications of Acute Invasive Fungal Rhinosinusitis: A New Challenge in the COVID-19 Convalescent Patients.

Purpose: Increased incidence of acute invasive fungal rhinosinusitis (AIFR) in the setting of COVID-19 is undeniable. This can be attributed to its effect on innate immunity and extensive use of corticosteroids. The goal of our study was to assess the orbital complications of AIFR and its management in the COVID-19 convalescent patients. Methods: Our longitudinal prospective study included 45 patients with orbital complications of AIFR in recently recovered COVID-19 patients. We performed otorhinolaryngological, ophthalmological, and neurological examinations to monitor the manifestations of the disease. Computed tomography and contrast enhanced magnetic resonance imaging were performed to detect the extent of infection. Antifungal medications, surgical intervention, and general condition management were all provided to all the patients. Results: We reported pre-septal cellulitis, orbital cellulitis, and orbital apex syndrome in 18, 13, and 10 patients, respectively. Four patients had cavernous sinus thrombosis. Mucormycosis and Aspergillus species were detected in 80% and 11.11% of our patients, respectively, while the mixed infection was found in 8.88% of our patients. Diabetes mellitus was the most common cause of immunocompromise (95.55% of our patients). Orbital pain and ophthalmoplegia were the most common ocular manifestations, followed by proptosis and relative afferent pupillary defect. All patients underwent surgical intervention, except for one patient who was unfit for surgery. One patient had orbital exenteration. The ophthalmological manifestations were reversible in cases of orbital and pre-septal cellulitis. The overall survival rate was 66.67%. Conclusion: Early diagnosis and treatment of AIFR can decrease the morbidity and mortality rate of affected patients.

Fourteen cases of acute invasive fungal rhinosinusitis: is there a place for less aggressive surgical treatment?

PURPOSE: Acute invasive fungal rhinosinusitis (AIFRS) is a rare, fulminant, angio-invasive infection with high mortality rate. Treatment of AIFRS includes aggressive surgical debridement combined with antifungal agents and treatment of the underlying disease. The aim of this study is to report our experience with this often fatal disease and estimate the long-term survival of AIFRS patients with more conservative surgery. METHODS: A retrospective study was performed on immunocompromised adults with AIFRS from January 2011 to December 2020. The diagnosis was histologically confirmed by tissue fungus invasion. We analyze epidemiological and microbiological findings, the underlying disease and discuss our patients' treatment plan and long-term survival. RESULTS: Fourteen patients were included in the study. Mean age was 53.8 ± 18.9 years. The underlying disease was hematologic malignancy in nine patients, diabetes mellitus in three, aplastic anemia in one, and renal/liver failure in one. Leukopenia was not significantly correlated with outcome or survival. Histological and culture findings revealed that Mucor/Rhizopus were the causative organisms in nine cases. All patients received systemic antifungal therapy. Surgery was performed in nine patients (endoscopic for all patients, combined in three with an external approach). Overall survival was 35.7% as five patients survived, two who underwent surgery, and three who were treated non-surgically. Nine patients died, two from AIFRS after central nervous system involvement and seven from their primary disease, free of fungi. Older individuals and patients with hematologic malignancies had a worse outcome. CONCLUSION: Early surgical debridement, antifungal agents, and treatment of the underlying disease remain the cornerstones of AIFRS management. Prognosis is overall defined by the underlying disease and in some selected cases, medical treatment alone could be a reasonable option.

Magnetic Resonance Imaging Prognostic Findings for Visual and Mortality Outcomes in Acute Invasive Fungal Rhinosinusitis.

PURPOSE: To identify initial, preintervention magnetic resonance imaging (MRI) findings that are predictive of visual and mortality outcomes in acute invasive fungal rhinosinusitis (AIFRS). DESIGN: Retrospective cohort study. PARTICIPANTS: Patients with histopathologically or microbiologically confirmed AIFRS cared for at a single, tertiary academic institution between January 2000 and February 2020. METHODS: A retrospective review of MRI scans and clinical records of patients with confirmed diagnosis of AIFRS was performed. For each radiologic characteristic, a modified Poisson regression with robust standard errors was used to estimate the risk ratio for blindness. A multivariate Cox proportional hazards model was used to study AIFRS-specific risk factors associated with mortality. MAIN OUTCOME MEASURE: Identification of initial, preintervention MRI findings associated with visual and mortality outcomes. RESULTS: The study comprised 78 patients (93 orbits, 63 with unilateral disease and 15 with bilateral disease) with AIFRS. The leading causes of immunosuppression were hematologic malignancy (38%) and diabetes mellitus (36%). Mucormycota constituted 56% of infections, and Ascomycota constituted 37%. The overall death rate resulting from infection was 38%. Risk factors for poor visual acuity outcomes on initial MRI included involvement of the orbital apex (relative risk [RR], 2.0; 95% confidence interval [CI], 1.1-3.8; P = 0.026) and cerebral arteries (RR, 1.8; 95% CI, 1.3-2.5; P < 0.001). Increased mortality was associated with involvement of the facial soft tissues (hazard ratio [HR], 4.9; 95% CI, 1.3-18.2; P = 0.017), nasolacrimal drainage apparatus (HR, 5.0; 95% CI, 1.5-16.1; P = 0.008), and intracranial space (HR, 3.5; 95% CI, 1.4-8.6; P = 0.006). Orbital soft tissue involvement was associated with decreased mortality (HR, 0.3; 95% CI, 0.1-0.6; P = 0.001). CONCLUSIONS: Extrasinonasal involvement in AIFRS typically signals advanced infection with the facial soft tissues most commonly affected. The initial, preintervention MRI is prognostic for a poor visual acuity outcome when orbital apex or cerebral arterial involvement, or both, are present. Facial soft tissues, nasolacrimal drainage apparatus, intracranial involvement, or a combination thereof is associated with increased mortality risk, whereas orbital soft tissue involvement is correlated with a reduced risk of mortality.

Management of Fungal Rhinosinusitis: Experience From a Tertiary Care Centre in North India.

Introduction Fungal rhinosinusitis (FRS) has increased over the past few decades due to the rampant use of antibiotics, steroids, immunosuppressive drugs, increased incidence of HIV and uncontrolled diabetes. The current study reviews the types, clinical presentation, microbiology, histopathology and outcomes related to FRS in a tertiary care center in North India. Methods We retrospectively reviewed the clinical and follow-up records of patients diagnosed with FRS over three years. The data reviewed included clinical workup, ophthalmological profile, comorbidities, immunological status, radiological investigations, intraoperative and histopathological findings, treatment and follow-up records. In addition, we performed a descriptive analysis of the reviewed data. Results The study consisted of 30 FRS patients (16 male, 14 female). In that, 77% of cases were of allergic FRS, while fungal ball, chronic invasive, chronic granulomatous and acute invasive FRS represented 3%, 10%, 3% and 7% cases, respectively. The most common presentation in non-invasive forms was nasal obstruction, nasal discharge, hyposmia and polyposis, while it was facial pain and headache in the invasive varieties. After appropriate medical and surgical management through endoscopic sinus surgery, the recurrence rate in non-invasive and invasive fungal sinusitis was 16.6% and 20.8%, respectively. There was nil mortality at a minimum of one year of follow-up. Conclusion The non-invasive forms of FRS are common and have a relatively mild course. Early medical and surgical intervention and management of the underlying comorbidities are the key factors in managing invasive FRS. Close follow-up after surgery is also necessary for the timely detection and management of recurrences.

Changing Trends in the Survival of Immunosuppressed Children with Invasive Fungal Rhinosinusitis.

BACKGROUND: Acute invasive fungal rhinosinusitis has been associated with high mortality rates. OBJECTIVE: We aimed to explore the contribution of novel detection and treatment methods on the outcome of immunosuppressed children with acute invasive fungal rhinosinusitis. METHODS: The records of all children with a hematologic or oncologic disease who developed acute invasive fungal rhinosinusitis between 2005-2020 were reviewed. RESULTS: Thirty-four patients were included. Aspergillosis and mucormycosis were diagnosed in 20 patients (59%) and 12 patients (35%), respectively. Panfungal polymerase chain reaction (PCR) was associated with a change of treatment in 36% of patients. A more aggressive surgical approach as well as treatment with liposomal amphotericin B and novel antifungals were adopted in recent years. Overall, 26% of patients died of disease, however no disease-specific death occurred since 2012. Diagnosis using panfungal PCR (p = .04) and treatment with novel antifungal medications (p = .017) were significantly associated with disease-specific survival. CONCLUSION: Enhanced fungal detection using panfungal PCR and treatment with novel antifungal agents, combined with rapid diagnosis and treatment, aggressive surgical approach and better control over the underlying oncological disease, may significantly improve the outcome of immunosuppressed children with acute invasive fungal rhinosinusitis.

Clinical predictors of invasive fungal rhinosinusitis in a tertiary pediatric hospital.

OBJECTIVES: Invasive fungal rhinosinusitis (IFRS) is a potentially fatal disease that affects the severely immunocompromised and requires aggressive treatment. The objective of this study is to better describe predictors of biopsy positivity in patients at high risk of IFRS at a pediatric hospital. METHODS: This was a single-center case-control study of 36 patients (37 total biopsies) ≤ 21 years old with one of five high-risk oncologic/hematologic diagnoses who underwent operative endoscopy for clinical suspicion for IFRS. IFRS positivity was defined histologically. Collected information included patient demographics, primary diagnosis, oncologic relapses, time from diagnosis to biopsy, clinical characteristics, and endoscopic findings. These data were used to create a simple predictive scoring system. RESULTS: 17 patients had biopsy-proven IFRS (IFRS(+)) for an overall incidence of 2.1% in the designated high-risk population. Average time from most recent oncologic development (diagnosis, relapse, or hematopoietic stem-cell transplant) to biopsy in the IFRS(+) group was 2.09 months (SD = 2.26), and 7.28 months in the IFRS(-) group (SD = 9.17) (p = 0.009). Clinical characteristics did not differentiate between IFRS(+) and IFRS(-). Bedside endoscopy performed poorly, as it was interpreted as normal in 42.8% of IFRS(+) and 53.8% of IFRS(-). In contrast, the presence of any positive endoscopic finding intra-operatively was highly specific for IFRS(+) (94%) with moderate sensitivity (70%), and the difference in rate of positivity between groups was statistically significant (p < 0.001). CONCLUSION: Operative endoscopy with biopsy remains the gold-standard to rule-out IFRS in the setting of high clinical suspicion. Time elapsed from most recent oncologic development to clinical concern for IFRS may influence the likelihood of disease, though this requires further study. Clinical symptoms and bedside endoscopy were not predictive and should be used with caution in decision-making.

Scedosporium apiospermum invasive rhinosinusitis in an elderly patient: diagnosis and treatment.

Scedosporium apiospermum is a ubiquitous organism present in the environment and is rarely identified in rhinosinusitis. We report a case of invasive rhinosinusitis with Scedosporium apiospermum which made a definite diagnosis by metagenomic next-generation sequencing (mNGS) from a biopsy sample. The resection of the Scedosporium apiospermum pathological mass was performed with low-temperature plasma radiofrequency ablation. Six months of continuous oral voriconazole treatment was followed. The patient was asymptomatic with no signs of recurrence during the next 1-year follow-up.