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Mucormycosis is a serious fungal infection caused by fungi in the order of Mucorales. Orbital mucormycosis occurs more frequently in rhino-orbital, sino-orbital, and rhino-orbito-cerebral forms of the disease, while isolated orbital mucormycosis is much less common. Herein, we present four cases of immunocompetent children who developed primary cutaneous mucormycosis, which subsequently invaded and progressed to orbital mucormycosis following direct traumatic injury caused by pecking from Acridotheres tristis (Common Myna). Given the low prevalence of orbital mucormycosis in healthy children, an unknown source of infection and delayed diagnosis followed by late therapeutic interventions could result in life-threatening conditions and serious sequelae.

Purpose: There has been a sudden increase in the number of rhino-orbital mucormycosis cases, primarily affecting patients recovering from COVID-19 infection. The local health authorities have declared the current situation an epidemic. In this study, we assess the role of exenteration in preventing disease progression and improving survival in patients with rhino-orbital mucormycosis., Methods: The patients undergoing exenteration were grouped into the exenteration arm and those denying exenteration were grouped into the nonexenteration arm. The patients were followed at 1 month and 3 months. The 6-month survival data were collected telephonically. Continuous data were presented as Mean ± SD/Median (IQR) depending on the normality distribution of data, whereas the frequency with percentages was used to present the categorical variables. Kaplan-Meier survival curves were created to estimate the difference in survival of patients with exenteration in rhino-orbital mucormycosis versus those without exenteration., Results: A total of 14 patients were recruited for our study based on the inclusion and exclusion criteria. All the patients were qualified for exenteration; however, only eight patients underwent exenteration and six patients did not consent to exenteration. At the end of 3 months in the exenteration group, four (50%) patients died. Two patients died within a week of exenteration, whereas two patients died after 2 weeks of exenteration. The deaths in the first week were attributed to septic shock and the deaths happening beyond 2 weeks were attributed to severe meningitis. The Kaplan-Meier survival analysis showed the cumulative probability of being alive at 1 month in the exenteration arm to be 85%, and it decreased to 67% by 53 days and subsequently remained stable until the end of 3 months., Conclusion: The Kaplan-Meier survival analysis did not show a survival benefit of exenteration at 3 months and 6 months in COVID-associated rhino-orbital mucormycosis., (Copyright © 2023 Copyright: © 2023 Indian Journal of Ophthalmology.)

Background: Rhino-orbital-cerebral mucormycosis (ROCM) is a rare, opportunistic, angio-invasive, and fatal infection caused by mold fungi of the genera Rhizopus, Mucor, and Rhizomucor. The global incidence of ROCM is 0.005-1.7 per million, with a fatality rate of 46%. Early diagnosis and treatment are crucial for this disease, as a delay of one week can increase the mortality rate to 66%., Case: A 32-year-old male, a known case of coronavirus disease 2019 (COVID-19) for the past 15 days from Janakpur, Dhanusha, Nepal presented to the emergency department of Dhulikhel Hospital with a sudden onset of blurred vision in the left eye, left-sided ocular pain, and nasal bleeding for the last three days., Observations: The patient was suspected of post-COVID-19 mucormycosis, and a nasal swab for potassium hydroxide (KOH) mount showed hyphae in microscopy. Intravenous and retrobulbar liposomal amphotericin B were administered as medical therapy, along with surgical debridement. A multidisciplinary approach was necessary for the treatment., Conclusion: A long-term, multimodal treatment approach involving combined antifungal drug therapy (intravenous liposomal amphotericin B and retrobulbar amphotericin B), and timely surgical debridement leads to an improvement in both short-term and long-term outcomes., (© NEPjOPH.)

Background: Rhino-orbital-cerebral mucor mycosis (ROCM) is a relatively rare opportunistic infection caused by the Mucorales species. While ROCM suggests involvement of the paranasal sinuses, orbit and brain ROM (rhino-orbital-Mucormycosis) stands for the fungal invasion in sinuses and orbit sans cerebral involvement. In India with the outbreak of the second COVID wave and the delta variant of the virus, there has been a steep increase in this opportunistic fulminant fungal infection, named COVID-associated Mucor mycosis (CAM). The most critical question in orbital management is when to go ahead with an exenteration. Our study aims to design a pertinent minimal invasive surgical protocol for surgeons to manage such cases based on our surgical experience and mitigate the need for exenteration and save the eyes wherever possible., Methods: The study is a retrospective analysis of patients of ROM with and without brain involvement, who underwent minimal surgical management between March 2021 to March 2022 along with their follow-up., Results: There were 184 eyes of 148 patients diagnosed with CAM. The mean age was 51.7 years with a male predominance of 103 (70%). All patients developed ROM following the COVID-19 infection and the duration between diagnosis of COVID-19 and ROM was 36 ± 23 days. 18 cases (12%) were bilateral. 76 eyes (41%) had no vision at the presentation. Imaging revealed paranasal sinus involvement (100%), orbital apex involvement (61%), cavernous sinus involvement (53%), and central nervous system (CNS) involvement (47%). All the patients (100%) were treated with systemic Liposomal amphotericin-B and sinus debridement. Endoscopic debridement of the orbital disease was performed in 45 (30.4%) cases, 15(8.1%) eyes underwent exenteration and were later rehabilitated with a customized ocular prosthesis, 103 (56%) eyes underwent transcutaneous retrobulbar amphotericin-B. At a mean follow-up of 13.1 months; the complete resolution was seen in 25 (17%) cases, the residual stable lesion was seen in 77(52%) of the cases and new lesions were developed in 13(9%) of the cases. Mortality was seen in 33 (22%) patients and all of them had CNS involvement., Conclusions: Systemic and protocol-based management can save the life and salvage the eyes., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Method: Medical Records of cases with orbital fungal infection from January 2000 to December 2019 were reviewed., Results: The study included 45 patients. Mucormycosis was proven in 32 (71.1%) and aspergillosis in 13 (28.9%) patients. Thirty-five (77.8%) patients had predisposing factors mainly uncontrolled diabetes. Twenty-seven (60%) patients presented with orbital apex syndrome, 10 (22.2%) with orbital cellulitis and 8 (17.7%) with proptosis. The prognosis was generally poor (31.1% mortality, 8.88% exenteration and 11.1% self-evisceration). The proposed algorithm composed of six atypical clinical presentations mainly acute orbital apex and fulminating orbital cellulitis plus any radiological or microbiological findings., Conclusion: The proposed algorithm markedly reduced the complications after 5 years of the study. Once fungal orbital infection is suspected, treatment should start immediately.

Background: Mucormycosis is a potentially lethal, angioinvasive fungal infection caused by the Mucoracea family comprising Mucor, Rhizopus, and Absidia species. It is commonly associated with uncontrolled diabetes mellitus, the use of corticosteroids, immunosuppressive drugs, and Covid-19 infection. The occurrence of mucormycosis in an immunocompetent patient is rare. Also, only a few case reports have been published where patients developed mucormycosis with associated malarial infection., Case Presentation: A young female presented with a 3-weeks history of painful swelling and outward protrusion of the right eye with complete loss of vision. She had a history of P.vivax malaria two weeks before her ocular symptoms. On ocular examination, there was proptosis and total ophthalmoplegia with loss of corneal sensations in the right eye. Hematological examination revealed normocytic normochromic anemia and thrombocytopenia. MRI was suggestive of right-sided pansinusitis and orbital cellulitis with right superior ophthalmic vein thrombosis and bulky cavernous sinus. Nasal biopsy was negative for fungal culture. An emergency surgical debridement of all the sinuses was done with right orbital exenteration. Histopathology confirmed the diagnosis of mucormycosis and the patient improved post-operatively on systemic antifungals., Conclusion: Such an association of mucormycosis with malaria infection is rarely reported in the literature and is hypothesized to be a result of immunosuppression caused by malaria. Also, emphasis is laid upon having a high index of suspicion for fungal infection in the setting of pansinusitis even if the risk factors are absent. We hereby report a case of rhino-orbital mucormycosis following P.vivax malaria in a 20-year-old female with anemia and thrombocytopenia., (© 2022. The Author(s).)

Purpose: To explore the various globe salvaging treatment strategies for patients with coronavirus disease 2019-associated mucormycosis (CAM)., Methods: This Was a Prospective: interventional analytical study conducted at a Medical College in rural India. A total of 84 patients of CAM admitted between May 2021 and August 2021 were enrolled for the study. Patients with histologically proven CAM with clinical and/or radiological evidence of orbital involvement were divided into three treatment categories based on the site and extent of the lesion. Re-assessment was performed after 7 days. For patients who worsened with the primary approach, orbital exenteration was considered based on a Sion Hospital Scoring System. A novel approach to intra-orbital anti-fungal therapy, site-centered peri-bulbar injection of amphotericin B (SCPeriAmB), was also explored. All the patients were followed up for at least 3 months. Convenience sampling with descriptive statistics was used., Results: Six patients had to finally undergo exenteration by the end of the study period. The rest of the patients were reported to be stable or improved. No mortalities were reported on delaying the exenteration. No adverse events were noted in patients who were given SCPeriAmB., Conclusion: Globe salvaging treatment options should be advocated as a primary approach in patients with CAM. Site-centered peri-bulbar injections can be considered as an approach for delivering intra-orbital anti-fungal therapy in selected patients., Competing Interests: None

Purpose: Following COVID-19 infection a rising count of rhino-orbito-cerebral mucormycosis is observed, requiring orbital exenteration, a disabling lifetime affecting surgery. One of the potential interventions for globe salvage in these patients is retrobulbar injections of amphotericin B. This study was conducted to review protocols, outcomes, and side effects of retrobulbar injection of amphotericin B in patients with COVID-19 associated rhino-orbito-cerebral mucormycosis (CAM)., Methods: The PubMed, Scopus, Web of Science, and Embase databases were searched using a comprehensive string of relevant keywords. All English studies with the confirmed diagnosis of CAM infection were included. We excluded all studies in which retrobulbar injection of amphotericin B was not implemented in any of the patients or there was a lack of clarified and detailed data about this procedure among participants., Results: A total of 647 cases had a history of retrobulbar injection(s) of amphotericin B in 13 reviewed studies with 3,132 subjects of CAM. The most common protocol was the retrobulbar injection of 1 ml of 3.5 mg/ml liposomal amphotericin B for 3 doses daily or on alternate days. We discerned that the globe salvage rate was 95.0% in eyes with a history of retrobulbar injection(s). The total rate of orbital exenteration was 14.9%, regardless of the history of retrobulbar injection of the drug. Other outcomes of this intervention were vision salvage and reduced major ophthalmic complaints, including pain, swelling, chemosis, ptosis, and ophthalmoplegia. The side effects of this intervention were not serious, and most of them were transient. They included swelling at the injection site, restriction of ocular motilities, exacerbation of orbital inflammation, and even intensification of visual impairment in a few cases., Conclusions: Retrobulbar injection of amphotericin B should be considered a nearly safe and protective intervention against orbital exenteration in patients with CAM. It may also be effective in saving vision. Since the effectiveness of orbital exenteration in the survival of patients is not ascertained, retrobulbar injections can be considered an alternative intervention., Competing Interests: The authors have no financial or conflicts of interest to disclose., (Copyright © 2022 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Purpose: To report two cases of COVID-19 under treatment with a corticosteroid; in one case rhino-orbitocerebral mucormycosis and in another one rhino-orbital mucormycosis developed., Case Presentation: A 40-year old woman and a 54-year old man with severe COVID-19 underwent corticosteroid therapy for immune-related lung injuries. The first case presented with a bilateral visual loss and complete ophthalmoplegia of the right eye. The second case presented with vision loss, proptosis, orbital inflammation, and complete ophthalmoplegia on the left side. Histopathologic, nasal endoscopic examinations, and radiologic findings confirmed mucormycosis in both patients. The patients denied orbital exenteration and were managed with systemic amphotericin B and daily endoscopic sinus debridement and irrigation with diluted amphotericin B. Because of the intracranial space involvement, the first case died. The second case was successfully managed surgically and medically., Conclusion: Rhino-orbital/cerebral mucormycosis may be developed in COVID-19 patients under treatment with corticosteroid, and requires prompt diagnosis and management.

Background: Mucormycosis is an emerging aggressive mold infection. This study aimed to assess the outcome of hospitalized adults with rhino-orbito-cerebral mucormycosis (ROCM). The secondary objective was to identify prognostic factors in this setting., Methods: This study was an international, retrospective, multicenter study. Patients' data were collected from 29 referral centers in 6 countries. All qualified as "proven cases" according to the EORTC/MSGERC criteria., Results: We included 74 consecutive adult patients hospitalized with ROCM. Rhino-orbito-cerebral type infection was the most common presentation (n = 43; 58.1%) followed by rhino-orbital type (n = 31; 41.9%). Twenty (27%) had acquired nosocomial bacterial infections. A total of 59 (79.7%) patients (16 in combination) received appropriate antifungal treatment with high-doses of liposomal amphotericin B. Fifty-six patients (75.7%) underwent curative surgery. Thirty-five (47.3%) required intensive care unit admission (27; 36.5% under mechanical ventilation). Hospital survival was 56.8%, being reduced to 7.4% in patients with invasive mechanical ventilation. A multivariate binary backward logistic regression model identified confusion at admission (OR 11.48), overlapping hospital-acquired infection (OR 10.27), use of antifungal treatment before diagnosis (OR 10.20), no surgical debridement (OR 5.92), and the absence of prior sinusitis (OR 6.32) were independently associated with increased risk for death., Conclusion: Today, ROCM still has high mortality rate. Improving source control, rational therpy, and preventing nosocomial infections may improve survival in this severe infection., (Copyright © 2022. Published by Elsevier B.V.)

Objective: To document and analyse demographic data, clinical presentation, possible interventions for early clinical detection and management of post-COVID-19 rhino-orbito-cerebral mucormycosis (ROCM)., Method: 32 patients having history of SARS-CoV-2 infection with features of ROCM were observed in terms of their history, presenting features, clinical, microbiological examination, type of surgical intervention, surgical sites of involvement which were subsequently analyzed., Results: The mean (± S.D.) age of patients was 57 ± 13 years. All patients were diabetic. Mean (± S.D.) time of onset of ROCM symptoms, since onset of COVID-19 symptoms was 18 (± 4) days. 12.5% patients were fully vaccinated. 78.1% patients received steroid therapy; 28.1% received high flow nasal oxygen. 87.5% patients had blurring of vision, 65.62% headache, 59.37% cheek and eyelid swelling, 50% proptosis, 46.87% ophthalmoplegia, 40.62% ptosis, 40.62% loss of sensation over cheek, 25% orbital pain. Examination of specimen with KOH mount revealed Mucor spp. in all patients. 87.5% patients underwent endoscopic sinus surgery with debridement with/without orbital clearance; 56.25% maxillectomy; 25% orbital exenteration. 87.5% patients had paranasal sinus involvement, 43.75% orbit sparing orbital apex, 68.75% orbit with orbital apex. 81.25% patients had involvement of pterygopalatine fossa±infratemporal fossa. 50% patients had disease in Vidian canal and pterygoid wedge. 25% of patients had involvement of palate and 56.25% cheek and eyelid soft tissues., Conclusion: A judicious COVID treatment protocol, high index of suspicion, close monitoring of high-risk patients and early institution of treatment can prevent case severity and reduce mortality., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: To assess whether a modified treatment ladder algorithm incorporating transcutaneous retrobulbar amphotericin B (TRAMB) for invasive fungal rhino-orbital sinusitis can reduce the risk of exenteration without compromising survival., Design: Retrospective, comparative clinical study with historical control subjects., Methods: Fifty consecutive patients with biopsy-proven invasive fungal sinusitis and radiographic evidence of orbital involvement were evaluated at a single tertiary institution from 1999-2020. TRAMB was incorporated as part of the treatment algorithm in 2015. Demographics, underlying immune derangement, infective organism, ophthalmic examination, surgical care, and survival were compared in a quasiexperimental pre-post format, dividing patients into a pre-2015 group and a post-2015 group. Risk of exenteration and mortality were the primary outcomes., Results: Baseline characteristics did not differ significantly between the 2 groups. Nearly all patients underwent a surgical intervention, most commonly functional endoscopic sinus surgery with debridement. TRAMB was administered to 72.7% of the post-2015 group. Exenteration was more common in the pre-2015 group (36.4% vs 9.1% [95% confidence interval {CI} 5.2-48.8]; P = .014), while mortality was similar (40.0% vs 36.7% [95% CI -22.1 to 29.3]; P = .816). After adjusting for potential confounders, patients treated after 2015 were found to have lower risk of exenteration (relative risk 0.28 [95% CI 0.08-0.99]; P = .049) and similar risk of mortality (relative risk 1.04 [95% CI 0.50-2.16]; P = .919)., Conclusion: Compared with historical control subjects, patients with invasive fungal rhino-orbital sinusitis who were treated with a modified treatment ladder algorithm incorporating TRAMB had a lower risk of disfiguring exenteration without an apparent increase in the risk of mortality., (Published by Elsevier Inc.)

Purpose: Second wave of COVID-19 pandemic was associated with an unprecedented rise in cases of mucormycosis, treatment of which has been challenging owing to the availability and side effects associated with amphotericin., Methods: All patients presenting with rhino-orbital cerebral mucormycosis (ROCM) following COVID-19 infection between April 2021 to June 2021 were included in this retrospective interventional study. Primary objective was to assess the clinical response with combination of intravenous liposomal amphotericin B (4-5 mg/kg/day) and saturated solution of potassium iodide (SSKI) given orally along with surgical debridement., Results: Twenty-five patients of ROCM were treated with the regimen. Mean age and fasting blood sugar levels were 53.48 years and 239.64 mg/dL respectively. All patients had history of intake of steroids with a mean daily dose of 86.39 mg of prednisolone equivalent. 88% of patients had a "proven" diagnosis of mucormycosis. Cultures were positive in 52% of patients with Rhizopus arrhizus as the predominant species. The mean daily dose of amphotericin received was 268 mg/day with a mean duration of 9.52 days. Mean daily dose of SSKI was 2.57 g. 21 patients (84%) had stabilization of disease at week 8 and achieved cure at the end of treatment whereas the mortality rate was 16%. Factors that significantly affected outcome were eye and central nervous system (CNS) involvement on presentation., Conclusion: SSKI, with its remarkably low cost and safety profile, makes it a potential adjuvant drug that may help achieve the twin benefits of shortened duration and dose of LAMB., (Copyright © 2021. Published by Elsevier Inc.)

Purpose: To report and validate an objective staging system for COVID-related Rhino-Orbito-Cerebral mucormycosis (ROCM)., Methods: Proof of concept of a new ROC staging system was devised for COVID-related ROCM. The staging system was based on data points drawn from a comprehensive ophthalmic examination, diagnostic endoscopy, and appropriate imaging of orbit, paranasal sinuses and brain in post-COVID ROCM patients to formulate the final staging expressed as R 0-3 O 0-3 C 0-3 . Each stage in the sinus, orbit and brain was graded into three levels of severity of disease. Progressive stages necessitated more active and urgent intervention and poorer prognosis. Finally, ten consecutive ROCM patients were independently staged by two blinded clinicians by examining clinical photographs and radiological images and the results matched for concordance., Results: Each component of the staging: Rhino, Orbital, and Cerebral were graded 0-3 based on severity. Each stage was matched with a recommended management plan. The final ROC grading by two independent clinicians showed a high degree of concordance - maximum for orbit grade (100%) followed by brain (90%) and sinuses (80%). Overall R 0-3 O 0-3 C 0-3 staging had 70% concordance. Extrapolation of final ROC staging of two clinicians showed 60% match. When we allowed for one-point difference in the ROC staging system the concordance between two clinicians improved to 100% and the management plan matched in 90%., Conclusion: The proposed ROC staging system based on objectively defined clinico-radiological criteria of the three components of COVID-related ROCM is simple-to-use and has high concordance when validated by independent clinicians. The staging helps devise a management plan and has prognostic value.

Background: Invasive mucormycosis (IM) is a life-threatening fungal infection occurring mostly in solid organ transplant (SOT) recipients, patients with hematological malignancies, and diabetes. A sudden spurt of mucormycosis has been reported in severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) pandemic in India; however, there is little data about coronavirus disease 2019 (COVID-19) associated mucormycosis (CAM) in kidney transplant recipients (KTRs)., Methods: We describe the clinical presentations, risk factors, treatment and outcomes of 11 mucormycosis cases in KTRs post-COVID-19 infection from February 2020 to June 2021 at a single center in India., Results: Mucormycosis was seen in 11/102 (10.7%) KTRs during the pandemic. Six patients had mild disease and rest five had moderate disease. Seven patients had pre-existing diabetes mellitus and four developed new onset hyperglycemia after receiving steroids for COVID-19 infection. All had poorly controlled sugars at the time of presentation. Most common presentation was rhino-orbital-cerebral mucormycosis (ROCM) in 10/11 (89%) patients and one has pulmonary mucormycosis. All patients received combination of amphotericin B and surgical debridement/excision of affected tissue followed by posaconazole prophylaxis. Nine patients recovered, however two patients succumbed to their illness after median of 14 (7-21) days from diagnosis. One patient developed acute T-cell-mediated rejection during the course of recovery. At last follow up, the mean serum creatinine was 2.05 mg/dl as compared to 1.4 mg/dl at presentation., Conclusions: IM is a common fungal infection in transplant recipients in India after COVID-19. Early diagnosis and prompt treatment with combination of surgical debridement and liposomal amphotericin B are key to better outcomes in CAM. Judicious use of steroids and control of hyperglycemia is key to avoid flaring up of the fungal infection., (© 2021 Wiley Periodicals LLC.)

Competing Interests: The authors declare that they have no conflicts of interest to disclose.

Purpose: To assess the outcome and response of transcutaneous retrobulbar injection of amphotericin B (TRAMB) in post-coronavirus disease of 2019 (COVID-19) rhino-orbito-cerebral mucormycosis (ROCM) and to establish a scoring system in guiding treatment modalities., Methods: An interventional, prospective study was done on 82 eyes of post-COVID-19 ROCM from May 2021 to July 2021. A comprehensive multi-departmental evaluation along with detailed ophthalmic examination, laboratory investigations, and radiological examination was done. Scoring points were given to each symptom, sign, and radiological features of orbit and the total score was taken. Based on these scores, severity of disease was grouped into A, B, and C corresponding to mild, moderate, and severe orbital ROCM. One milliliter of reconstituted liposomal amphotericin B was given to all patients every alternate day as three doses. Efficacy of these injections was assessed in all groups, even though other treatment modalities like orbital debridement and exenteration were considered for moderate and severe cases. Patients were followed up for a period of 8 weeks., Results: Out of 82 eyes, symptomatic improvement was seen in a major proportion (72%) of patients. A statistically significant improvement in scores was noted in group A (93% improved) with a P value of 0.002, while 68.4% showed improvement in group B (P-value- 0.0001). Group C with severe disease showed minimal improvement in post-injection scores of 41% (P-value 0.086), necessitating surgical intervention. No serious adverse effect of the drug or procedure was noted., Conclusion: Significant improvement in scores of groups A and B highlights TRAMB as an effective and safe treatment modality in mild to moderate ROCM. It is an effective adjunct in severe cases, along with other interventions. Also, the scoring system helps in assessing the severity and guiding in management strategies., Competing Interests: None

Purpose: To describe the role of localized debridement and instillation of amphotericin B for the management of orbital mucormycosis post COVID-19 infection with a view to avoid exenteration., Methods: The records of all patients with orbital mucormycosis post COVID-19 infection in the last 6 months from December 2020 to June 2021 were evaluated, and ten patients were identified who were successfully managed with localized debridement, that is, removing the fungal tissue and necrotic material and amphotericin B gel instillation locally. MRI scan was used to identify the area of fungal infiltration and presence of necrotic material. Early surgery in the form of transconjunctival orbitotomy was performed for disease in the infraorbital fissure area, and superior transcutaneous lid crease approach was employed for disease in the superomedial orbit or medial orbit. Most patients had lid edema, ptosis, and proptosis; this resolved with the medication. Systemic antifungals were given and the follow-up ranged from 1 to 5 months., Results: The ptosis, proptosis, and lid edema subsided in all, except in one patient who had residual ptosis and in one who had residual ophthalmoplegia. Vision deficit did not occur in any patient. All patients were successfully discharged on oral antifungal medication., Conclusion: Localized clearance of the fungal tissue and the necrotic material is a good option to avoid exenteration in cases of orbital mucormycosis, avoiding disfigurement and mental trauma to the patient., Competing Interests: None

Purpose: To evaluate our patients with rhino-orbito-cerebral mucormycosis according to a new scoring system and to compare those who underwent orbital exenteration persuant to a new threshhold., Methods: Mucormycosis is a fungal disease that can be of acute onset, aggressive, and angioinvasive. Aggressive surgical debridement, long-term systemic antifungal therapy, and treatment of underlying predisposing factors are essential to the treatment. However, orbital exenteration is a very difficult decision to make, particularly in patients with orbital involvement, because there is little information in the literature, resulting in limited support for making this aggressive surgical decision. In this study, our 43 cases of mucormycosis were evaluated in terms of orbital exenteration using a scoring system (including clinical signs and symptoms, ophthalmoscopic findings, and radiologic results) developed by Shah et al., which establishes indications for orbital exenteration in mucormycosis., Results: According to our study, if the threshold score for exenteration is 19.0, the sensitivity was 100% and specificity was 97%, providing better results than the 23.0 threshold score determined by the reference study for exenteration. When these two score thresholds were compared, there was a statistically significant difference., Conclusion: We believe that this scoring system may be beneficial to use for orbital exenteration in patients with mucormycosis. Prospective studies in large case series are required to determine the most appropriate threshold score., (Copyright © 2021 Elsevier Masson SAS. All rights reserved.)

Purpose: To report a series of 13 immunocompetent patients who developed new-onset uncontrolled diabetes mellitus (DM) following COVID-19 infection and presented as rhino-orbital mucormycosis (ROM)., Method: Retrospective study., Results: A total of 127 patients of COVID-19 Associated Mucormycosis (CAM) were evaluated at four centres in India. All patients underwent endoscopic sinus debridement surgery and received systemic amphotericin-B therapy. Five patients (5/13; 38.4%) received retrobulbar amphotericin-B injections. Orbital exenteration was performed in advanced orbital involvement or progression of orbital disease in spite of maximal medical therapy. In his cohort, 13/127 (10.2%) patients presented with new onset DM, where one patient had bilateral disease. The mean age was 35.9 years (range: 20-51 years) and the mean duration from diagnosis of COVID-19 to the diagnosis of mucormycosis was 14.2 days. While 7/13 (53.8%) of the patients received systemic corticosteroids during the course of their treatment for COVID-19, six patients received no steroids or immunomodulators. The mean follow-up period was 9.2 weeks (range: 3-18 weeks) following discharge. Life salvage was possible in 100% of the cases. While overall globe salvage was possible in 42.8% (6/14 eyes), the globe could be preserved in 4/5 patients who received retrobulbar amphotericin-B injections., Conclusions: Those involved in the care of COVID-19 patients should be aware about the possibility of recent-onset DM, even in patients without a history of corticosteroid therapy. Rarely, recent-onset DM following COVID-19 may present as rhino-orbital mucormycosis, which requires aggressive surgical and medical intervention.

Objective: To determine the prevalence, risk factors, and elaborate our experiences with diagnosis and treatment of patients with mucormycosis, enabling a better understanding of the disease and its management. Methods: This is a case series of patients with Covid-19 associated with Rhino-orbital-cerebral mucormycosis, managed in our tertiary care center from April 2021 to June 2021. Results: Six cases of Covid-19 associated with Rhino-orbital-cerebral mucormycosis have been analyzed in the study. The mean age of patients was 40.67 years with a male preponderance (83.3%). The most common complaint was headache (100%), while a minority (33%) came with ocular complaints. All the patients either had a previous history of diabetes mellitus or developed increased blood sugar levels following Covid infection, and were kept on insulin to control their blood sugar levels. 4 patients (66.67%) had a history of corticosteroid use during Covid-19 hospitalization. Treatment included intravenous liposomal Amphotericin B (100%), functional endoscopic sinus surgery (66.67%), maxillectomy (33.33%) and transcutaneous retrobulbar liposomal Amphotericin B (33.33%). Amphotericin B induced nephrotoxicity, which was seen in 1 patient (16.67%). Mortality occurred in only one patient (16.67%), 25 days following successful surgery. Conclusion: Diabetes Mellitus is the most important predisposing factor for the development of Covid-19 associated Rhino-orbital-cerebral mucormycosis. Early presentation, prompt diagnosis and timely initiation of treatment with liposomal Amphotericin B and surgical debridement along with strict blood sugar control can lead to a favorable outcome. However, regular follow-up and monitoring of serum electrolytes and kidney profile must be ensured for such patients., (© The Authors.Romanian Society of Ophthalmology.)

Purpose: To present the different clinical manifestations of rhino-orbital mucormycosis (ROM) co-infection in severe COVID-19 patients., Study Design: Prospective observational clinical study METHODS: Among 32,814 patients hospitalized with the diagnosis of COVID-19 between March 2020 and December 2020 in our center, eleven microbiologically confirmed ROM co-infection cases in severe COVID-19 patients were evaluated., Results: There were nine men and two women with a mean age of 73.1 ± 7.7 years. Eight patients had uncontrolled type 2 diabetes with a mean diagnosis duration of 12.1 ± 4.4 years. All patients had COVID-19-associated acute respiratory distress syndrome and received corticosteroids. The mean time interval between COVID-19 diagnosis and ROM diagnosis was 14.4 ± 4.3 days. Seven patients (63.6%) had orbital apex syndrome, and four patients (36.4%) presented with orbital cellulitis. Endophthalmitis was detected in 54.5% of patients, and two of these patients developed retinoschisis. CT scan/MRI revealed sino-orbital involvement in all patients, and three of these had cerebral involvement at initial presentation. All patients received intravenous and retrobulbar liposomal amphotericin B and had undergone radical debridement of involved sinuses. Intravitreal liposomal amphotericin B injected in patients with endophthalmitis. Despite all measures, 63.6% of patients expired., Conclusions: Severe COVID-19 is associated with a significant incidence of ROM with higher mortality rates due to immune dysregulation and the widespread use of steroids. Physicians should be aware of the possibility of this infection in patients with COVID-19. An aggressive multidisciplinary approach can help to reduce mortality.

Purpose: COVID-19-associated rhino-orbital-cerebral mucormycosis (ROCM) has reached epidemic proportion during India's second wave of COVID-19 pandemic, with several risk factors being implicated in its pathogenesis. This study aimed to determine the patient demographics, risk factors including comorbidities, and medications used to treat COVID-19, presenting symptoms and signs, and the outcome of management., Methods: This was a retrospective, observational study of patients with COVID-19-associated ROCM managed or co-managed by ophthalmologists in India from January 1, 2020 to May 26, 2021., Results: Of the 2826 patients, the states of Gujarat (22%) and Maharashtra (21%) reported the highest number of ROCM. The mean age of patients was 51.9 years with a male preponderance (71%). While 57% of the patients needed oxygen support for COVID-19 infection, 87% of the patients were treated with corticosteroids, (21% for > 10 days). Diabetes mellitus (DM) was present in 78% of all patients. Most of the cases showed onset of symptoms of ROCM between day 10 and day 15 from the diagnosis of COVID-19, 56% developed within 14 days after COVID-19 diagnosis, while 44% had delayed onset beyond 14 days. Orbit was involved in 72% of patients, with stage 3c forming the bulk (27%). Overall treatment included intravenous amphotericin B in 73%, functional endoscopic sinus surgery (FESS)/paranasal sinus (PNS) debridement in 56%, orbital exenteration in 15%, and both FESS/PNS debridement and orbital exenteration in 17%. Intraorbital injection of amphotericin B was administered in 22%. At final follow-up, mortality was 14%. Disease stage >3b had poorer prognosis. Paranasal sinus debridement and orbital exenteration reduced the mortality rate from 52% to 39% in patients with stage 4 disease with intracranial extension (p < 0.05)., Conclusion: : Corticosteroids and DM are the most important predisposing factors in the development of COVID-19-associated ROCM. COVID-19 patients must be followed up beyond recovery. Awareness of red flag symptoms and signs, high index of clinical suspicion, prompt diagnosis, and early initiation of treatment with amphotericin B, aggressive surgical debridement of the PNS, and orbital exenteration, where indicated, are essential for successful outcome.

Mucormycosis is an aggressive and deadly fungal infection, which is invariably associated with an immunocompromised patient. Mucormycosis in the head and neck region presents as skeletal necrosis, with or without soft tissue involvement. Early identification and treatment with combination of surgical debridement and parenteral antifungal therapy is critical for a favourable outcome. This paper reports an unusual presentation of mucormycosis, mimicking a localised sino-orbital pathology involving the infraorbital subcutaneous tissue and the maxillary sinus, in a 35 years old immunocompetent man. Despite aggressive antifungal therapy and surgical management, the course of disease was fatal, reiterating the high mortality associated with mucormycosis., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

Orbital apex syndrome (OAS) can be a rare, but severe complication of an odontogenic infection and has high morbidity and mortality. Antibacterial drugs are typically an appropriate treatment choice, but the most severe cases are fungal in nature and pose a tough challenge to the clinician. The aim of this study was to determine the predisposing factors, specific aspects in its management and the appropriate treatment strategy in order to improve patient outcome. A systematic review was conducted using PubMed, PubMed Central, Web of Science, and Scopus up to February 2020, based on the associations between dental extraction or infections and OAS. Of 721 papers found, 18 articles were considered eligible and presented in total 21 cases (13 fungal and eight bacterial infections). The information was organized into a diagnostic and treatment algorithm which included data extracted both from the included cases and updated literature of treatment efficacy studies. Immunosuppression (uncontrolled diabetes mellitus and chemotherapy) was found as an important predisposing factor particularly for fungal infections. In these cases, we suggest that early simultaneous approaches, including aggressive surgical procedures and systemic administration of amphotericin B, result in a better outcome. In conclusion, medical intervention success depends on aggressive treatment and multidisciplinary teamwork.

Introduction: Rhino-orbital-aspergillosis (ROA) is a rare but serious disease in immunocompetent patients. Diagnosis is often delayed due to the absence of specific clinical symptoms. We describe the case of a patient who presented initially with ROA which spread progressively to the right ethmoid-sphenoid sinuses and then to the brain., Observation: A 61-year-old patient with a history of well-controlled diabetes presented with a sudden severe decrease in right visual acuity. Cerebral MRI showed the presence of an infiltrate in the right orbital apex extending to the homolateral cavernous sinus without any cerebral involvement. A diagnosis of right orbital myositis was made and corticosteroid therapy was started. His symptoms worsened progressively leading to quasi-blindness. A new MRI showed the development of right sphenoid-ethmoid osteolytic lesions. A fungal aetiology was suspected and tests for fungal biomarkers found a β-(1-3)-D-glucan level of 99pg/ml but negative galactomannan. An ethmoid biopsy was performed for histological and mycological investigations, including the detection of Aspergillus DNA by qPCR. qPCR was positive and culture resulted in the isolation of multi-sensitive Aspergillus fumigatus. Treatment was initiated with voriconazole. Due to persistence of blindness and the appearance of a lesion extending to the right frontal lobe, surgical excision was performed followed by antifungal treatment for a total duration of 1year. The patient is currently stable, but has persistence of blindness in the right eye., Conclusion: Invasive ROA is a rare but serious disease in immunocompetent patients which should be evoked in the differential diagnosis of a tumour or vasculitis. Early diagnosis is essential for optimal management., (Copyright © 2020. Published by Elsevier Masson SAS.)

Background/objectives: To report the spectrum of fungal infections involving the orbit encountered in an Australian subtropical population with respect to presentation, host risk factors, involved pathogens, treatment and outcomes., Subjects/methods: A retrospective chart review was performed on all adult patients with orbital mycosis treated by the senior author (TJS) from 1986 to 2017 in a tertiary setting., Results: Thirty cases of fungal infection involving the orbit were included in this case series. Of these, 26 patients had invasive disease and four patients had non-invasive disease. Causative organisms included mucormycosis (16), aspergillus (8) and other fungi (7). Common risk factors included haematological disorders or malignancy, neutropenia, corticosteroid use and diabetes mellitus. Mucormycosis in three immunocompetent patients was caused by Apophysomyces elegans. Orbital apex syndrome was observed in approximately one third of patients at initial ophthalmological assessment. Amphotericin B was used in most cases of mucormycosis, while there was a more varied spectrum of anti-fungal use in other fungal infections. Seven patients with mucormycosis proceeded to orbital exenteration with a survival rate of 43%. No patients with other orbital fungal infections were exenterated., Conclusions: Orbital mycoses are not only opportunistic but true pathogenic infections. While initial symptoms may be varied, the development of orbital apex syndrome should raise suspicion for this condition, regardless of patient immune status or age. Survival and visual outcomes are often poor with invasive disease. Multidisciplinary team management with early orbital specialist involvement is essential.

Competing Interests: As an epidemiology advisor of the Editorial Board, HKL Yuen was not involved in the peer review process of the article. The other author has disclosed no conflicts of interest.

Aim: To report the frequency and factors affecting patients', globe and vision survivals in rhino-orbito-cerebral mucormycosis (ROCM)., Methods: This is a retrospective study of 63 patients (79 eyes) with biopsy-proven ROCM at a university hospital 2008-2016. Systemic and ophthalmic manifestations, imaging, management and final outcomes were recorded. Globe survival was defined as no exenteration and vision survival as final visual acuity of light perception and more., Results: Mean age was 55.5 (SD 12.9) years with no gender preference. Diabetes was the most common underlying disease (68.3%). Patient survival was observed in 57.1 % (36/63). Presence of frozen eye (OR 4.6), nasal mucosal involvement (OR 7.3) and shorter duration of antifungal therapy (OR 1.03) were significantly associated with lower patient survival. Exenteration did not significantly change the survival. Globe survival was detected in 43% (34/79). Higher white blood cell (WBC) count was associated with a lower globe survival (p=0.02). Vision survival was observed in 25.3% (20/79) in whom younger age was significantly associated with a worse vision survival., Conclusion: Patient, globe and vision survivals were 57%, 43% and 25%, respectively. Exenteration did not affect the patients' survival. While frozen eye and nasal mucosal involvement were significantly associated with a lower survival, higher WBC count significantly increased the risk of exenteration., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Purpose: To report our experience in the diagnosis and management of invasive fungal infections with orbital involvement in children from a subtropical population., Methods: The medical records of children (<18 years of age) with orbital mycosis and treated by the senior author (TJS) from 1995 to 2017 in multiple pediatric tertiary centers were reviewed retrospectively., Results: Six patients (aged 12 weeks to 15 years) were included in this series. Four patients had confirmed infection with isolated pathogens, including mucormycosis (3) and Exserohilum (2). One patient rapidly deteriorated and died before biopsy could be performed; however, the patient was presumed to have invasive fungal disease. Four patients had underlying hematological malignancy, and 1 presented in diabetic ketoacidosis. Orbital apex syndrome was observed in one patient. All patients received liposomal amphotericin B and five underwent at least one debridement surgery. One patient proceeded to orbital exenteration and survived. The overall survival rate was 67%., Conclusions: Orbital mycoses can affect children of all ages. Immunocompromised patients are particularly at risk, and mortality rates are high. In a subtropical population, these infections may be caused by a different spectrum of fungi compared to other climate zones. We believe extensive surgical debridement, including exenteration may still be necessary in the management of this disease in a young population, particularly if there is extensive orbital involvement., (Copyright © 2019 American Association for Pediatric Ophthalmology and Strabismus. All rights reserved.)

Competing Interests: There are no conflicts of interest.

Objective: Invasive sino-orbital fungal infections in immunocompetent patients are a rare clinical entity; the diagnosis and management of which is challenging. We present a large case series of invasive sino-orbital fungal granulomas in patients without pre-existing systemic immunocomprimising condition., Design: Retrospective case series., Participants: Twenty cases of invasive sino-orbital/naso-orbital fungal granulomas in immunocompetent individuals., Methods: We retrospectively analyzed all patients with orbital fungal granuloma who were treated at a tertiary referral eye center in South India between January 2005 and December 2012. Histopathologic confirmation of tissue invasion by fungal elements and presence of granulomatous inflammation was established in all cases included in the study., Main Outcome Measures: Relief of patient symptoms, resolution/no progression of disease on orbital imaging, ocular, and vision salvage were the treatment outcomes studied., Results: Twenty patients (11 male and 9 female) were studied. Mean age of patients was 47.4 years ranging from 24-65 years. Aspergillus was the causative fungus in 18 cases while 2 were cases of mucormycosis. Surgical debulking of the orbital disease was performed in 7 patients. Exenteration was performed in 2 patients to prevent spread to the CNS. Medical therapy consisted of oral itraconazole in all patients and intravenous amphotericin B was administered in 2 patients. Average duration of medical therapy required to achieve relief from symptoms was 6-8 months. Recurrences are common and long-term follow-up is essential., Conclusions: Orbital fungal infections are challenging in terms of both diagnosis and treatment. Debulking along with prolonged antifungal therapy seems to be effective in controlling the infection.

A 61-year-old man with well-controlled diabetes mellitus type 2, cirrhosis from hepatitis C, alcohol abuse, and portal hypertension presented with painful vision loss and left orbital swelling. Imaging showed diffuse orbital, perineural, and pachymeningeal inflammation. He was initially diagnosed with neurosarcoidosis. However, cerebrospinal fluid analysis revealed central nervous system lymphoma, and lacrimal gland biopsy showed fungal organisms consistent with mucormycosis. The authors describe a case of Mucorales infection lacking sinonasal involvement and discuss the differential diagnosis and management of patients presenting with orbital and central nervous system inflammation from this uncommon fungal infection.

Mucormycosis is a rare clinical entity, often affect immunocompromised patients. It is an emergency situation and has poor prognosis. Prompt diagnosis with tissue biopsy, local control of the disease by aggressive surgical debridement and appropriate systemic antifungal treatment improve the prognosis and survival of the patients. Treatment of mucormycosis needs antifungal agents such as Amphotericin B and wide surgical debridement. Early diagnosis and treatment is often needed for survival of the patients. We describe a rare case of mucormycosis affecting facio-orbital area without involving sinon-nasal cavity., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

A 55-year-old man presented with unilateral orbital inflammation and no light perception vision. Imaging revealed infiltrative enhancement of the optic nerve, orbit, and intracranial tissue. The case was suspicious for invasive fungal disease, but ultimate workup and orbital biopsy revealed granulomatosis with polyangiitis. The patient's inflammation resolved with corticosteroid and rituximab therapy. Granulomatosis with polyangiitis is a systemic vasculitis that can mimic a number of orbital pathologies.

Rationale: Orbital apex syndrome is a complex clinical disorder featuring a collection of cranial nerve deficits characterized by impairment of the extraocular muscles, the ophthalmic branch of the trigeminal nerve, and even the optic nerve. Sino-orbital aspergillosis is rare but aggressive infection. Surgical resection accompanied by antifungal medication is advised currently., Patient Concerns: We report a 61-year-old woman diagnosed as aspergilloma presenting with the characteristic manifestations and imaging features of orbital apex syndrome., Diagnoses: Paranasal sinus tumor was misdiagnosed initially according to magnetic resonance imaging of the orbit. Finally aspergilloma was diagnosed by pathologic report., Interventions: The anti-fungal medication, voriconazole, was administered immediately. Surgical excision was also done due to the poor response to medical treatment., Outcomes: Postoperative follow-up showed no recurrence of aspergillosis but the vision was lost permanently., Lessons: Invasive sino-orbital aspergillosis as an aggressive disease with highly invasive patterns and it may be misdiagnosed as tumors. To achieve better prognosis and survival, clinicians should be aware of this distinct manifestation.

Purpose: To report a case of complete ophthalmoplegia and blindness in sino-orbital mucormycosis., Case Report: A 68-year-old woman with history of diabetes and breast cancer presented with sudden onset of fever, orbital pain and blindness in the right eye. The patient was found to have no light perception, complete ophthalmoplegia, chemosis, corneal ulcer and optic nerve atrophy. In rhinologic examination, necrosis was noticed. Brain and orbit magnetic resonance imaging showed diffuse sinusitis with orbital involvement. The result of the clinical examination was reported as sino-orbitalmucormycosis. The diagnosis was confirmed by pathological specimens. The patient underwent extensive surgical debridement and systemic antifungal treatment. Despite treatment, visual acuity did not recover., Conclusion: The sino-orbital form of mucormycosis is a rare and insidious fungal infection. Ocular findings may range from orbital pain to ophtalmoplegia and blindness such as in our case. Black scar tissues seen on the nasal mucosa are pathognomonic. Delay in treatment due to late presentation and complications were major determinants in ocular prognosis and survival outcome.

Purpose: To describe the non-exenteration management of sino-orbital fungal infection, a life-threatening condition for which orbital exenteration is generally considered a first-line treatment., Methods: A retrospective case series is presented of 7 orbits in 6 consecutive patients admitted and treated at 2 major metropolitan tertiary teaching hospitals in Sydney, New South Wales, Australia., Results: Seven orbits in 6 consecutive patients with sino-orbital fungal infection were treated conservatively with surgical debridement and intravenous antifungal agents. Four patients were immunosuppressed and the other 2 patients were otherwise healthy. All presented with pain, proptosis, or loss of vision. Causative organisms found were Mucormycoses, Aspergillus, and Scedosporium prolificans. Exenteration was avoided in all patients as part of their planned management and 5 patients, including 1 with bilateral disease, survived their disease without exenteration. Medical treatment included intravenous liposomal amphotericin B or voriconazole. A single immunosuppressed patient deteriorated and as a last resort, exenteration was performed, but this made no difference to his clinical course and in retrospect could have been avoided as he died of multiple cerebral metastases diagnosed shortly after his deterioration., Conclusion: The authors recommend that patients with sino-orbital fungal disease preferably be treated conservatively, without orbital exenteration.