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We report an autopsy case of 53-year-old male with poor controlled diabetes mellitus and hepatocellular carcinoma who developed rhino-orbito-cerebral mucormycosis. Initial complaints were epistaxis and headache followed by a sudden blindness, the 2nd through 7th cranial nerve palsy and diabetes inspidus. Laboratory data revealed that he had liver cirrhosis due to hepatitis C virus infection and diabetes mellitus. Head CT and MRI showed no significant findings. Eleven days after the onset, he died of subarachnoid hemorrhage. The postmortem examination revealed severe infiltration of numerous mucors in the sphenoid sinus, cavernous sinus and bilateral internal carotid arteries. Severe granulomatous vasculitis was seen in the cavernous portion of the bilateral internal carotid arteries. Thus, we considered that this case had been caused by the infiltration of mucors to the cavernous sinus, resulting in the obstruction of ophthalmic arteries. Rupture of the right internal carotid artery was seen at the branching portion of the ophthalmic artery, demonstrating the cause of his death. We would like to emphasize that rhino-orbito-cerebral mucormycosis should be ruled out if we examine a nondiagnostic case of diabetes mellitus or immunosuppressed disease associated with rapid multiple cranial nerve palsy following the orbital symptoms.
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PMID:[An autopsy case of rhino-orbito-cerebral mucormycosis associated with multiple cranial nerve palsy and subsequent subarachnoid hemorrhage]. 971 Nov 24

Rhino-orbital-cerebral mucormycosis (ROCM) is an acute, often fatal, fungal infection caused by members of the class Zygomycetes and the order Mucorales. The genus Rhizopus accounts for most cases of ROCM. The disease is characterized by fungal hyphal invasion of blood vessels resulting in thrombosis and infarction of the nasal, paranasal sinus, orbital, and cerebral tissues. The most commonly associated condition is diabetes mellitus; other associated conditions include immunocompromised states, renal disease, deferoxamine use, and acidotic states. Common clinical findings include rhinitis, periorbital and facial swelling, facial and mucosal necrosis, ophthalmoplegia, multiple cranial nerve palsies, facial pain, and headache. Definitive diagnosis is made by demonstration of fungal hyphae in tissue specimens. The mainstay of treatment is aggressive surgical debridement of infected tissue and administration of amphotericin B. ROCM has a mortality rate of 40-50%; 70% of survivors are left with residual defects. Early diagnosis and treatment are imperative in the successful management of patients afflicted with this devastating sight- and life-threatening disease.
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PMID:Rhino-orbital-cerebral mucormycosis: a review. 1204 3

To understand the demographic as well as clinical characteristics, and outcomes of patients with rhinocerebral mucormycosis in Taiwan, we retrospectively analyzed patients with this disease admitted to Chang Gung Memorial Hospital-Kaohsiung from 1988 through 2000. The 21 patients included 8 men (28%) and 13 women (62%). The median age was 60 years (range, 34-82 years). Twenty patients (95%) had underlying diabetes mellitus. The most common clinical feature at admission was ocular lesions, followed by headache, nostril lesions, and consciousness disturbance. Fifteen (94%) of 16 patients who received combined surgical debridement and therapy with amphotericin B survived, while only 1 (20%) of the 5 patients who received amphotericin B alone survived (p=0.004). The diagnosis of rhinocerebral mucormycosis was delayed in 4 patients (19%). Of the 16 patients who survived, 1 (6%) had delayed diagnosis, while of the 5 patients who died, 3 (60%) had delayed diagnoses (p=0.028). This series disclosed a higher proportion of patients with rhinocerebral mucormycosis in Taiwan had underlying diabetes mellitus, and ocular lesions were more frequent than nostril lesions at the time of admission. These results highlight the importance of the timely initiation of a combination of aggressive surgical debridement and treatment with amphotericin B in patients with rhinocerebral mucormycosis. Considering the high rate of delayed diagnosis, improved clinician's awareness of mucormycosis is extremely important and is in urgent need in Taiwan.
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PMID:Rhinocerebral mucormycosis in Taiwan. 1472 56

Invasive fungal sinusitis of the paranasal sinuses in a healthy immunocompetent person is uncommon. Isolated involvement of any paranasal sinus, particularly sphenoid sinus is rare. In this study, five immunocompetent patients who had no nasal complaints but obscure symptoms of headache and orbital symptoms such as diplopia, retro-orbital pain and loss of vision were diagnosed to be having fulminant fungal sinusitis of the sphenoid sinus. Three patients had aspergillosis and two patients had mucormycosis. These patients initially presented to neurologists and ophthalmologists because they had no ENT complaints. The diagnosis was made on endoscopy, radiology and histopathology. They were treated aggressively according to the standard protocols. The purpose of this paper is to bring to light the changing clinical spectrum of invasive fungal sinusitis. It can occur in immunocompetent patients and in the form of isolated sphenoid sinus involvement.
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PMID:Invasive fungal sinusitis of isolated sphenoid sinus in immunocompetent subjects. 1636 16

This is a report of 7 cases of mucormycosis infections in patients who had undergone transplantation and been admitted in the kidney transplant centre of Baqiyatallah Hospital in Tehran, Iran, from 2002 to 2005. We retrospectively reviewed the hospital records for demographic data, symptoms, diagnostic techniques and outcomes. Five patients were male and 2 female. The mean age of patients was 49.5 y. The time interval between transplantation and disease onset varied greatly (range: 1 month to 4 y). Patients' symptoms were fever (7 cases), respiratory distress (4 cases) and severe headache (3 cases). Suspected patients were evaluated by CT scan, BAL and biopsy and diagnosis confirmed by culture. The final diagnosis was pulmonary mucormycosis in 4 cases, rhino-cerebral mucormycosis in 2 cases and disseminated mucormycosis in 1 case. Despite early and intensive treatment with amphotericin B in all patients and extensive debridement in 3 cases, only 2 patients survived the disease. Mucormycosis is a potentially lethal complication after kidney transplantation. It could occur very early on or very late into the post-transplant period. Despite the results of other studies, the most frequent site of infection in our patients was the lungs.
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PMID:Mucormycosis after kidney transplantations: report of seven cases. 1765 47

Rhino-orbital-cerebral mucormycosis is a rare but often fatal opportunistic necrotizing infection of the sinuses, orbit, and brain caused by saprophytic fungi. It usually develops in patients with diabetes or immune system deficiency. In this study, imaging features in 3 patients with rhino-orbital-cerebral mucormycosis who presented with various symptoms and different cerebral involvements are discussed. Headache, blurred vision, fever, painful ophthalmoplegia, and cranial nerve involvement were among the clinical findings. Computed tomography and magnetic resonance imaging are the best imaging methods for assessing the extent of this disease. Relatively typical but nonspecific characteristics are bone destruction, vascular invasion, and central hypointensity in the paranasal sinuses or an intracranial mass that is revealed by T(2)-weighted magnetic resonance imaging. Imaging findings include cavernous sinus involvement, cerebral infarct, and intracerebral hemorrhage. Because of the invasive and fulminant nature of rhino-orbital-cerebral mucormycosis, successful treatment seems to be based on early diagnosis and on the management of underlying immunologic problems.
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PMID:Rhino-orbital-cerebral mucormycosis with different cerebral involvements: infarct, hemorrhage, and ophthalmoplegia. 1798 70

Invasive fungal sinusitis should be suspected in immunocompromised or diabetic patients who present with acute sinusitis, inflammation of nasal septal mucosa, unexplained fever or cough, or the orbital apex syndrome. Histopathological studies are required to differentiate among these syndromes. Acute (fulminant) invasive fungal sinusitis has been called mucormycosis, zygomycosis and fulminant invasive sinusitis. Fever, cough, crusting of nasal mucosa, epistaxis, and headache are the most common presenting symptoms. Histopathological studies show hyphal invasion of blood vessels, vasculitis with thrombosis, and tissue infarction. Reports of granulomatous invasive fungal sinusitis come primarily from Sudan, but also from India, Pakistan, and the United States. Patients usually present with proptosis, appear to be immunocompetent and are infected almost exclusively with A. flavus. Chronic invasive fungal sinusitis can be distinguished from the two other forms of invasive fungal sinusitis by its chronic course, dense accumulation of hyphae resembling a mycetoma, and association with the orbital apex syndrome, diabetes mellitus, and corticosteroid treatment. Biopsy and orbital exploration show vascular invasion by fungal elements and only a sparse chronic inflammatory infiltrate.
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PMID:Syndromes of invasive fungal sinusitis. 1865 20

Rhinocerebral mucormycosis is an invasive fungal sinusitis with a high mortality rate, especially in immunocompromised patients. A 70-year-old woman, with uncontrolled type 2 diabetes mellitus, presented with a one-month history of non-specific headaches associated with progressive swelling of her left eye. Computed tomography of the brain and orbits showed the extensive involvement of bilateral intranasal sinuses, orbits, extraocular muscle and soft tissues. The diagnosis of invasive mucormycosis was confirmed from a tissue biopsy taken from the internasal septum. Despite the extensive mucormycosis invasion, she was successfully treated with intranasal and systemic amphotericin B and minimal adjunctive intranasal sphenoidotomy.
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PMID:Invasive rhinocerebral mucormycosis with orbital extension in poorly- controlled diabetes mellitus. 1935 53

Fungal infections are rare but represent serious complications following organ transplantation. We present a case of mucormycosis primarily affecting the paranasal sinuses in a 51-year-old man with a kidney allograft. The patient presented with headache, left facial and orbital pain, nasal discharge, and elevation of serum creatinine 18 months after kidney transplantation. Laboratory tests revealed cyclosporine nephrotoxicity, cytomegalovirus infection, and prediabetes. Imaging findings were compatible with left maxillary, ethmoidal, and sphenoidal sinusitis. Diagnosis was made based on pathologic findings and detection of typical fungal hyphea in the infected tissues. The patient was successfully treated by discontinuation of cyclosporine and mycophenolate mofetil, initiation of systemic amphotericin B, and aggressive surgical debridement.
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PMID:Successful treatment of mucormycosis after kidney transplantation. 1937 32

The case of a 9-year-old boy is presented in this article who developed a rare fungal infection of central nervous system. The histopathologic examination has revealed mucormycosis. The diagnosis wasn't confirmed microbiologically as the culture and PCR were negativ. After the iv administered Amphotericin B lipid complex the MR images of the brain have improved. The mucormycosis classically develops in immunodeficient patients and presents an acute, fulminant, mostly lethal infection. This case is very unusual, because the chronic, isolated CNS mucormycosis has slowly developed in immuncompetent patient and only one symptom was the long existing headache. The aim of this paper is reporting the case history and to find out the possible way of infection.
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PMID:[Non-obstructive hydrocephalus internus with a rare pathogenesis--mucormycosis]. 1968 6


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