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Allergic fungal rhinosinusitis a low power of eosinophilic (allergic) mucin showing a layered appearance of mucin admixed with inflammatory cells and debris (Hematoxylin and eosin; original magnification ×10). b Eosinophilic mucin showing collections of eosinophils and sloughed epithelial cells (Hematoxylin and eosin; original magnification ×50). c Eosinophilic mucin showing eosinophils singly and in cluster (Hematoxylin and eosin; original magnification ×50). d Eosinophilic mucin showing Charcot–Leyden crystals (Hematoxylin and eosin; original magnification ×200)
Source publication
Fungal rhinosinusitis (FRS) comprises a spectrum of disease processes that vary in clinical presentation, histologic appearances, and biological significance. FRS can be acute or chronic and is most commonly classified as non-invasive or invasive based on whether fungi have invaded into tissue. This manuscript will review the pathologic classificat...
Citations
... As infecções causadas por fungos da ordem Mucorales, gênero Rhizopus, Mucor, Litcheimia, Cunninghamella, Rhizomucor, Apophysomyces, e Saksenaea são incomuns, mas são significativas por sua natureza agressiva, pelo desafio diagnóstico e alta mortalidade (40 -100%), apesar dos antifungicos e terapia cirurgica atualmente disponíveis. 3,4,5 Arnold Paltauf relatou o primeiro caso histologicamente comprovado na Universidade de Graz, Áustria, em 1885. O termo "Zigomicose" foi instituído, em 1976, descrevendo qualquer infecção fúngica invasiva causada por espécies do antigo filo Zygomycota. ...
... 6 Apresentando-se nas formas rino-orbital, rinocerebral, pulmonar, cutânea ou subcutânea, gastrointestinal ou disseminada. 2,4 Nosso caso em questão, contempla um paciente com mucormicose rinocerebral (MC), com acometimento em base de crânio. A MC pode afetar os seres humanos em todas as faixas etárias. ...
Apresentação do Caso: Masculino, 62 anos, agropecuarista, foi admitido com cefaleia frontal, pulsátil e diária. Tomografia de Crânio (TC) sugerindo sinusopatia esfenoidal inflamatória aguda associada a fungo. Realizado tratamento clínico em serviço de origem, não especificado, com melhora parcial. Retorno da cefaleia associada a diplopia e estrabismo convergente de olho esquerdo. Nova TC com formação expansiva sólida infiltrativa na base do crânio preenchendo esfenoide esquerdo com destruição da parede, obliterando recesso esfenoetmoidal ipsilateral e captação do contraste. Realizada cirurgia transesfenoidal, mediante hipótese de cordoma. Anatomopatológico: colonização fúngica, suspeita de mucormicose. Após investigação sorológica e de comorbidades, concluiu imunocompetência. Tratado com Anfotericina B e Micafugina. Segue em acompanhamento, sem sinais de recidiva da infecção. Discussão: A mucormicose rinocerebral é uma infecção fúngica invasiva, rara e grave. Apresenta-se com comprometimento de seios paranasais, comumente maxilar, raramente acometendo esfenoide, seio cavernoso, órbitas e cavidade craniana. Incomum nos imunocompetentes. Os sinais e sintomas são febre, edema periorbital ou facial, diminuição da acuidade visual, oftalmoplegia e cefaleia. Considerações finais: Mucormicose rinocerebral é incomum em indivíduos sem comprometimento imunológico. Entretanto, esse caso demonstra a necessidade de suspeição diagnóstica para detecção precoce e tratamento imediato devido à elevada mortalidade. A associação entre terapêutica cirúrgica e clínica é decisiva para o prognóstico.
... They help prevent the recurrence. 6,7 The genus of dematiaceous fungi which cause allergic fungal sinusitis (AFS) includes Bipolaris, Curvularia, Alternaria, Fusarium, Aspergillus. AFRS usually presents with progressive nasal obstruction and multiple nasal polyps. ...
Allergic fungal rhinosinusitis (AFRS) is one of the most common form of fungal sinusitis. It is a hypersensitivity reaction to fungal infection. The patients are either atopic or immunocompetent. Such patients suffer from rhinosinusitis. The allergic mass is expanding in nature and this results in bony remodelling. It also involves the adjacent structures. Ophthalmological complications occur when the mass involves orbit. These complications include diplopia, telecanthus, proptosis, malar flattening, epiphora, asthenopia and even visual loss.
The diagnosis can be made using radiological imaging. Histopathological examination is needed to confirm the diagnosis. The treatment of AFRS includes both surgical and medical therapy. This case report demonstrates a rare presentation of the non-invasive AFRS with bilateral proptosis with hypertelorism. The patient showed a drastic improvement after endoscopic sinus surgery, oral anti fungal medication, oral steroids and nasal saline irrigation.
... In contrast, invasive fungal rhinosinusitis, for example, is a known lethal disease with high mortality and morbidities, and orbital involvement is one of the critical complications [15,16]. The mechanism of ophthalmologic disorder was considered as the rapid invasion of blood vessels by hyphae, inducing luminal thrombosis and finally causing tissue necrosis [17]. In addition, bacterial rhinosinusitis and fungal balls are more common for sphenoid inflammatory diseases in clinical practice compared to invasive fungal rhinosinusitis [3]. ...
Background and Objectives: Isolated sphenoid rhinosinusitis may have devastating consequences such as orbital complications due to its anatomical contiguity with vital structures. This study aimed to identify patients with isolated sphenoid inflammatory diseases at high risk for developing orbital complications and requiring aggressive management through investigation of the clinical and computed tomography (CT) characteristics of patients with isolated sphenoid rhinosinusitis. Materials and Methods: The medical records of patients who underwent endoscopic sinus surgery between 2005 and 2022 were retrospectively reviewed. Patients with isolated sphenoid rhinosinusitis were identified based on a manual review of the clinical and histopathological findings. Participants’ clinical and CT features were reviewed. Results: Among the 118 patients with isolated sphenoid rhinosinusitis, 15 (12.7%) developed orbital complications, including diplopia, extraocular motility limitation, ptosis, and visual impairment. Headaches and facial pain occurred significantly more frequently in patients with orbital complications than in those without orbital complications (p < 0.001). Patients with diabetes mellitus or malignant neoplasms were more likely to develop orbital complications than those without these comorbidities (p < 0.05). Bony dehiscence on CT images was significantly more common in patients with orbital complications than in those without. In the regression analysis, diabetes mellitus (OR, 4.62), malignant neoplasm (OR, 4.32), and bony dehiscence (OR, 4.87) were significant predictors of orbital complications (p < 0.05). Conclusions: Headaches and facial pain are the most common symptoms of isolated sphenoid rhinosinusitis. Orbital complications of isolated sphenoid rhinosinusitis are more common in patients with comorbidities such as diabetes mellitus or malignancy or in those with bony dehiscence on CT images.
... Viêm xoang do nấm (VXDN) là tình trạng viêm kéo dài với sự hiện diện của nấm gây tổn thương niêm mạc xoang và tổ chức xung quanh. Tiến triển của VXDN thường chậm, diễn ra từ từ nhưng lâu ngày bệnh có thể lan rộng và gây các biến chứng nguy hiểm như tạo khối choán chỗ trong xoang, phá hủy xương thành xoang, xâm lấn cơ quan lân cận, đặc biệt là hốc mắt và sọ não gây viêm não, màng não do nấm [3]. ...
Mục tiêu: Đánh giá giá trị của cắt lớp vi tính (CLVT) đa dãy đối với chẩn đoán viêm xoang do nấm (VXDN). Đối tượng và phương pháp: Nghiên cứu mô tả được thực hiện tại Bệnh viện Đại học Y Hà Nội từ tháng 01/2022 đến tháng 7/2023 trên 70 bệnh nhân viêm mũi xoang mạn tính được khám lâm sàng, nội soi mũi, chụp CLVT đa dãy xoang, sau đó được phẫu thuật nội soi xoang và xét nghiệm nấm sau mổ. Các tổn thương xoang trên CLVT được đối chiếu với kết quả xét nghiệm nấm sau mổ nhằm đánh giá độ nhạy (Sn), độ đặc hiệu (Sp), giá trị dự báo dương tính (PPV), giá trị dự báo âm tính (NPV) của CLVT đối với chẩn đoán VXDN. Kết quả: VXDN được chẩn đoán trên 60/70 bệnh nhân, chiếm 86% trong đó có 46/60 bệnh nhân là u nấm xoang, chiếm 76,7%, số còn lại là VXDN xâm nhập mạn tính. Dựa trên các dấu hiệu tổn thương xoang một bên, đám mờ xoang, vôi hóa trong đám mờ và tổn thương đặc/tiêu xương thành xoang, CLVT đa dãy có Sn, độ Sp, PPV, NPV đối với chẩn đoán VXDN lần lượt là 98,3%, 70%, 95,2% và 87,5%. Kết luận: Tuy còn hạn chế về cỡ mẫu, nghiên cứu cho thấy CLVT đa dãy là phương pháp không xâm lấn có giá trị cao đối với chẩn đoán VXDN.
... 7 This disease primarily affects young immunocompetent atopic patients. 8 The symptoms of AFRS are comparable to those of CRS with nasal polyposis, since all patients with AFRS have nasal polyposis. Patients with early disease may present with nasal congestion or obstruction, anosmia, and/or postnasal drip. ...
... Invasive fungal rhinosinusitis differs from AFRS in that the fungi invade blood vessels through the paranasal sinus mucosa, bone, and dura, leading to thrombosis and infarction. 8 obstruction, nasal discharge, and hyposmia. Endoscopy revealed bilateral nasal polyps, and nonenhanced CT revealed extensive sinonasal polyposis with expansion of the sphenoid and ethmoid sinuses with bone thinning (Figure 1). ...
... Early diagnosis can be difficult, and difficulties in accessing specialized healthcare can further delay diagnosis and management. 8 Neuroimaging studies are warranted in patients with clinically suspected compressive optic neuropathy or ophthalmoplegia. Because of the sensitivity and specificity of modern neuroimaging, a negative scan essentially rules out compression as the cause of vision loss, and cavernous sinus thrombosis or a cavernous sinus fistula is considered based on the findings. ...
Allergic fungal rhinosinusitis (AFRS) is a subtype of chronic noninvasive sinusitis accounting for 7.8% (0.2%-26.7%) of all chronic rhinosinusitis cases. A definitive diagnosis is usually made after sinus surgery. Successful treatment requires a combination of surgical and medical management. Although orbital involvement is relatively common, reports on optic neuropathy and acute vision loss are limited. Herein, we present a series of 3 patients with AFRS who presented with acute visual loss as the chief complaint. All 3 patients were otherwise healthy adults in their early 20s with extensive nasal polyps on endoscopic nasal examination and bone erosion in the bilateral orbits and lateral wall of the sphenoid sinus on the affected side on imaging. One of the 3 patients had bilateral cranial nerve IV defects in addition to cranial nerve III defects. All patients underwent endoscopic sinus surgery with orbital decompression and were followed up postoperatively by both otolaryngology and ophthalmology services with endoscopic and radiologic evaluation. Unfortunately, no meaningful improvement in vision was observed in any patient despite successful nerve decompression. Prompt diagnosis and early medical and surgical intervention are warranted to prevent complications in patients with AFRS with orbital extension.
... Fungal infection can develop if there are blockages of sinuses and nasal passages, like mucocele. Aspergillus sp. is the most likely pathogen to be cultured from the lesion, 7 one of the colonized fungal species in the sinonasal mucosa. ...
Mucocele is a benign, expansile, and oppressive lesion, more common in the frontal and ethmoid sinus and less in the maxillary sinus. Sinus mucocele mainly causes cheek swelling pain and nasal obstruction. In some cases, the paranasal mucocele grows large enough to compress periorbital structures and lead to impaired vision. Generally, mucocele is full of simple mucus, but pathogens can be found if co-infected, which means a poor prognosis. Functional endoscopic sinus surgery is an effective treatment for this disease. Here, the authors report a case that a mucocele occurred in the maxillary sinus, and a fungal ball was also found during the operation, which is a result of Paecilomyces farinosus co-infection.
... Fungal rhinosinusitis is broadly divided into invasive and non-invasive and the invasive type is further classified into three subtypes: Acute invasive, chronic invasive, and chronic granulomatous invasive forms. [11][12][13] Invasive fungal sinusitis is defined by the presence of fungal hyphae within the mucosa, submucosa, bone, or blood vessels of the PNSs. [11] AIFRS occurs in immunocompromised patients, predominantly in those belonging to one of the following two categories: One group is patients with diabetic ketoacidosis or uncontrolled diabetes. ...
... e other is those with an immunocompromised state with neutropenia due to causes such as chemotherapy, bone marrow transplant, steroid therapy, or immunosuppressive therapy for organ transplant. [3,[11][12][13][14] Secondary infections have been documented in patients affected with COVID-19 infection. [15,16] While a majority of these have been bacterial and fungal pulmonary infections, the COVID-19 pandemic also saw an unprecedented incidence of AIFRS cases in epidemic proportions. ...
Objectives
The aim of the study was to evaluate the magnetic resonance imaging (MRI) features of acute invasive fungal rhinosinusitis (AIFRS) at presentation and on follow-up imaging when patients receive treatment with systemic antifungal therapy and surgical debridement.
Material and Methods
This is a retrospective analysis of imaging data from a cohort of patients diagnosed with AIFRS during the second wave of COVID-19 in single tertiary referral hospital in South India between March 2021 and May 2021 ( n = 68). Final diagnosis was made using a composite reference standard which included a combination of MRI findings, clinical presentation, nasal endoscopy and intraoperative findings, and laboratory proof of invasive fungal infection. Analysis included 62 patients with “Definite AIFRS” findings on MRI and another six patients with “Possible AIFRS” findings on MRI and laboratory proof of invasive fungal infection. Follow-up imaging was available in 41 patients.
Results
The most frequent MRI finding was T2 hypointensity in the sinonasal mucosa (94%) followed by mucosal necrosis/loss of contrast-enhancement (92.6%). Extrasinosal inflammation with or without necrosis in the pre-antral fat, retroantral fat, pterygopalatine fossa, and masticator space was seen in 91.1% of the cases. Extrasinosal spread was identified on MRI even when the computed tomography (CT) showed intact bone with normal extrasinosal density. Orbital involvement (72%) was in the form of contiguous spread from either the ethmoid or maxillary sinuses; the most frequent presentation being orbital cellulitis and necrosis, with some cases showing extension to the orbital apex (41%) and inflammation of the optic nerve (32%). A total of 22 patients showed involvement of the cavernous sinuses out of which 10 had sinus thrombosis and five patients had cavernous internal carotid artery involvement. Intracranial extension was seen both in the form of contiguous spread to the pachymeninges over the frontal and temporal lobes (25%) and intra-axial involvement in the form of cerebritis, abscesses, and infarcts (8.8%). Areas of blooming on SWI were noted within the areas of cerebritis and infarcts. Perineural spread of inflammation was seen along the mandibular nerves across foramen ovale in five patients and from the cisternal segment of trigeminal nerve to the root exit zone in pons in three patients. During follow-up, patients with disease progression showed involvement of the bones of skull base, osteomyelitis of the palate, alveolar process of maxilla, and zygoma. Persistent hyperenhancement in the post-operative bed after surgical debridement and resection was noted even in patients with stable disease.
Conclusion
Contrast-enhanced MRI must be performed in all patients with suspected AIFRS as non-contrast MRI fails to demonstrate tissue necrosis and CT fails to demonstrate extrasinosal disease across intact bony walls. Orbital apex, pterygopalatine fossa, and the cavernous sinuses form important pathways for disease spread to the skull base and intracranial compartment. While cerebritis, intracranial abscesses, and infarcts can be seen early in the disease due to the angioinvasive nature, perineural spread and skull base infiltration are seen 3–4 weeks after disease onset. Exaggerated soft-tissue enhancement in the post-operative bed after debridement can be a normal finding and must not be interpreted as disease progression.
... FRS is classified into invasive and non-invasive forms. Non-invasive FRS includes allergic and fungal ball, whereas invasive FRS includes acute, chronic, and granulomatous [1,3]. This is based on type of inflammatory tissue reaction and duration of disease. ...
... Some fungal forms may grow rapidly invading vital structures (orbit, nasal cavity, and vessel wall) especially in the head and neck region. Their rapid diagnosis is essential for initiating timely treatment and reducing morbidity and mortality [3]. ...
... There are other techniques for the diagnosis of fungal infection, such as in situ hybridization (ISH) to detect ribosomal ribonucleic acid (rRNA) of fungi, immunoassay to detect fungal antigens, genomic amplification, and serology to detect immunoglobulins against fungi [9]. These are expensive, not readily available especially in developing countries, and have decreased utility in necrotic tissue [3,4,8]. ...
Fungal rhinosinusitis (FRS) is a relatively common, but often misdiagnosed disease of paranasal sinuses. The FRS is classified into invasive and non-invasive forms. The non-invasive form includes fungal ball and allergic FRS, and invasive form includes acute invasive FRS, chronic invasive FRS, and granulomatous FRS. Invasive fungal infections are associated with high morbidity and mortality, hence requiring urgent medical and surgical intervention. The histomorphology can help identify certain fungal organisms that cannot be cultured or are rarely visible in exudates. The morphologic diagnosis of tissue invasive and non-invasive fungal infection is essential for appropriate treatment. We analyzed cases of rhinosinusitis from 2017 to 2019 in Pathology Department at a tertiary care cancer hospital, Lahore, Pakistan. All clinical information was retrieved from patient records. Paraffin-embedded tissue blocks were stained with hematoxylin and eosin (H&E), special Grocott methenamine silver stain (GMS), and periodic acid Schiff stain (PAS) according to standard protocol. They were reviewed by two pathologists blinded by fungus status. A total of 169 cases of rhinosinusitis were reviewed. FRS comprised 146 (86.4%) of them. The mean age of patients with FRS was 32.8±14 years. The male:female ratio was 1.4:1. Maxillary sinus was the main site of involvement in 39 (27%) FRS cases. Aspergillus was identified in 117 (80.1%) cases of FRS. The culture reports were available in 44/146 (30.14%) FRS cases. They were negative in 22/44 (50.0%), and Aspergillus species were isolated in 18/44 (40.9%) cases of FRS. There were 84 (57.5%) cases of non-invasive FRS and 59 (40.4%) cases of invasive FRS. Among invasive FRS, there were 56 (38.4%) chronic granulomatous FRS cases including mixed patterns. Majority cases, 54 (96.4%), of chronic granulomatous FRS showed a unique crowded giant cell pattern comprising of foreign body and Langhans type giant cells. These giant cells were arranged closely forming irregular non-caseating granulomas surrounded by lymphocytes and fibrosis. Interestingly, the giant cells were scattered haphazardly without forming a granuloma as well. Fungal organisms were identified in all 56 cases of chronic granulomatous FRS. Histologically, predominant organism was Aspergillus in 48 (85.7%) on GMS and PAS stain. Our study observed a unique crowded giant cell pattern, which is a hallmark of invasive fungal infection. If pathologists are familiar with this unique pattern, they can make a quick and accurate diagnosis on histology. The physician can start antifungal treatment timely for better prognosis.
... Fungal rhinosinusitis (FRS) is a growing cause of rhinosinusitis across the world. 1 As a clinical entity, FRS encompasses three different types: acute invasive FRS, granulomatous invasive FRS, and chronic invasive FRS (CIFRS). 2 While the acute invasive variant has been covered extensively in prior literature and is associated with a high risk of mortality, the chronic invasive category has received less attention but is still equivalently critical to diagnose and treat promptly due to a high mortality rate. 3 CIFRS is defined as a slowly growing destructive process (>4 weeks) of any paranasal sinus with vascular invasion, inflammatory reaction, and involvement of other local structures. ...
Key Clinical Message
Early identification and management of chronic invasive fungal rhinosinusitis (CIFRS) is key to optimizing outcomes. A missed diagnosis can result in permanent vision loss, chronic facial pain, or death. We present a case of CIFRS and literature review.
Abstract
This case report presents a 56‐year‐old female with CIFRS involving orbital and facial complications. The patient experienced delayed diagnosis despite multiple ED visits for sinusitis with progressive facial pain and ocular deficits not alleviated with antibiotics, emphasizing the importance of early identification and maintaining high clinical suspicion for CIFRS. Prompt recognition, initiation of antifungal therapy, and aggressive surgical debridement were crucial for preventing disease progression and improving the patient's quality of life.
... AFRS is believed to be a complex immune reaction involving humoral and cellular responses as part of type 1 and type 3 hypersensitivity reaction characterized by the presence of eosinophilic mucin and Charcot-Leyden crystals with noninvasive fungal hyphae within the nasal cavity. [1][2][3] Inflammation in AFRS produces a thick nasal discharge that may obstruct paranasal sinus drainage, and mucocele-like expansion may occur, which can affect adjacent structures, including the visual pathway. This process occurs due to compression of the neural structure or its blood supply rather than invasion of the dura. ...
Visual loss is a rare manifestation of allergic fungal rhinosinusitis (AFRS). We report a case of an adult male who was diagnosed with AFRS and who presented during the COVID-19 pandemic lockdown with sudden-onset complete vision loss and a lack of recovery after surgical and medical management. We reviewed the literature on reported cases of AFRS complicated by visual loss to identify factors associated with visual outcomes. We found 50 patients who were diagnosed with acute visual loss due to AFRS, with an average age of 28 ± 14 years. Complete and partial recovery after surgical intervention were reported in 17 and 10 cases, respectively. However, the absence of vision improvement was reported in 14 of the cases. Early diagnosis and prompt intervention can return vision back to normal. However, delayed presentation, complete loss of vision, and acute onset of visual loss are associated with worse outcomes.
