Purpose: To report clinical presentations and factors affecting outcomes in rhino-orbital-cerebral mucormycosis following COVID-19. Methods: Retrospective multi-centric interventional case series of 58 eyes with rhino-orbital-cerebral mucormycosis. Demography, clinical parameters and management outcomes were noted. Factors affecting outcome and mortality were analyzed. Outcome was defined as favorable when complete resolution or stabilization without further progression of the infection was noted at last visit. Results: Mean age was 55 ± 11 years (median 56). The mean HbA1c value was 10.44 ± 2.84 mg% (median 10.5). The duration between the diagnosis of COVID-19 and rhino-orbital-cerebral mucormycosis was 16 ± 21 days (median: 8 days). Thirty-six eyes (62%) had no vision at presentation. Imaging revealed paranasal sinus involvement (100%), orbital apex involvement (41%), cavernous sinus involvement (30%), and central nervous system (CNS) involvement (33%). All the patients were treated with systemic Liposomal amphotericin-B and sinus debridement. Twenty-two eyes (38%) underwent exenteration. One eye underwent transcutaneous retrobulbar amphotericin-B. The mean follow-up duration was 5.62 ± 0.78 months (median 6). Favorable outcome was seen in 35 (60%) cases. Presence of uncontrolled diabetes ( p = 0.001), orbital apex involvement ( p = 0.04), CNS involvement ( p = 0.04), and history of steroid use ( p < 0.0001) resulted in unfavorable outcome. CNS involvement was the only factor predicting mortality ( p = 0.03). Mortality was seen in 20 (34%) patients. Conclusion: Over a third of patients with rhino-orbital-cerebral mucormycosis following COVID-19 have an unfavorable clinical outcome. Uncontrolled diabetes mellitus at presentation, involvement of the orbital apex, CNS, and the usage of steroids were associated with poorer outcomes. CNS involvement was a factor determining mortality.
Aim: To describe and review the clinical, radiological, and histopathological characteristics of an orbital perivascular epithelioid cell tumor (PEComa). Methods: A systematic review of clinical records, radiological investigations, microscopic features, and immunohistochemical characteristics was done. Results: A 9-year-old female child presented with a year-long history of a large orbital mass associated with painless, progressive proptosis of the right eye. Radiologically, a well-defined orbital mass was seen with no intracranial extension. Excision was performed and histopathological examination showed uniform epithelioid cells in nests separated by thin fibrovascular septae. The tumor cells stained positively for Human Melanoma Black-45, but negatively for desmin, S-100, smooth muscle actin, MyoD1, microphthalmia-associated transcription factor, vimentin, CD10, CD31, and CD34 with a low proliferation index of 5–7%. Based on the tumor’s morphological and immunohistochemical characteristics, a diagnosis of giant orbital PEComa was made. No recurrence was seen at the last follow-up. Conclusions: PEComas are uncommon mesenchymal neoplasms that have typical histological features, with an immunohistochemical profile of negativity for epithelial markers and positivity for melanocytic markers. For benign PEComas, complete excision is advised. However, since PEComas elsewhere in the body have been known to be malignant, a close follow-up of such cases is recommended.
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 followup 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.
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