Mucormicosis de cabeza y cuello: actualización en el diagnóstico y manejo médico-quirúrgico. Una revisión narrativa / Mucormycosis of the head and neck: update on diagnosis and medical-surgical management. A narrative review

La mucormicosis, es una patología de baja preva- lencia, rápidamente progresiva y de alta mortalidad que engloba un amplio espectro de infecciones del tipo opor- tunistas, causada por hongos de la familia Mucoraceae, Lichtheimiaceae, Thamnidiaceae, Cunninghamellaceae, Syncephalastraceae y Radiomycetaeae. Actualmente es la tercera causa de infección fúngica invasiva, posterior a la candidiasis y aspergilosos, siendo su presentación clínica más frecuente la rinocerebral de origen paranasal, cuyo síntoma característico es la rinosinusitis aguda bacteriana con proyección a los dientes antrales, de rápido avance y fatalidad. En esta revisión se emplearon resultados extraídos ma- nualmente de artículos indexados en las bases de datos MED- LINE y EBSCO a raíz de la búsqueda de los términos mu- cormycosis, oral surgery y patient care management con el objetivo de entregar una visión actualizada de la literatura, respecto al diagnóstico y tratamiento de la mucormicosis de cabeza y cuello (AU)

Mucormycosis is a low-prevalence, rapidly progres- sive and high-mortality pathology that encompasses a wide spectrum of opportunistic infections caused by fungi of the Mucoraceae, Lichtheimiaceae, Thamnidiaceae, Cunningha- mellaceae, Syncephalastraceae, and Radiomycetaeae. It is currently the third cause of invasive fungal infection, after candidiasis and aspergillosis, with its most frequent clinical presentation being rhinocerebral of paranasal origin, whose characteristic symptom is acute bacterial rhinosinusitis with projection to the antral teeth, with rapid progression and fatality. In this review, manually extracted results from articles indexed in the MEDLINE and EBSCO databases were used following the search for the terms mucormycosis, oral sur- gery and patient care management with the aim of providing an updated view of the literature regarding the diagnosis and treatment of mucormycosis of the head and neck

Mucormicosis periostomal posoperatoria, a propósito de un caso / Mucormycosis periostomal: case report

Resumen Introducción: La mucormicosis en una enfermedad infrecuente y oportunista que afecta, principalmente, a pacientes inmunocomprometidos. Pocas veces se han reportado casos de afectación periostomal. Clínicamente puede ser confundida con otras patologías, pudiendo tener una evolución fulminante, por lo que un adecuado y pronto diagnóstico son necesarios para una instauración precoz del tratamiento. Caso Clínico: Se presenta el caso de una paciente de 62 años inmunocomprometida, que tras complicaciones quirúrgicas evoluciona con mucormicosis periostomal de la pared abdominal. A pesar de un tratamiento quirúrgico con múltiples resecciones de tejido asociado a antifúngico local y sistémico, la paciente fallece, concordante a la letalidad expresada en la literatura.

Introduction: Mucormycosis is a rare and opportunistic disease that mainly affects immunocompromised patients. Few cases of peristomal involvement have been reported. Clinically it can be confused with other pathologies and may have a fulminant evolution, so an adequate and prompt diagnosis is necessary for an early establishment of treatment. Clinical Case: We present the case of a 62-year-old immunocompromised patient who, after surgical complications, evolves with periostomal mucormycosis of the abdominal wall. Despite surgical treatment with multiple tissue resections, associated with local and systemic antifungal agents, the patient died, consistent with the lethality expressed in the literature.

Mucormicosis rino-órbito-cerebral de origen dental / Rhino-orbito-cerebral mucormycosis from dental origin: Case report

Resumen La mucormicosis es una infección aguda causada por hongos oportunistas pertenecientes al orden de los mucorales, que afecta principalmente a pacientes diabéticos e inmunosuprimidos. Se reporta el caso de un hombre diabético de 63 años de edad, que se extrajo una pieza dental por sus propios medios y, posteriormente, desarrolló una mucormicosis rino-órbito-cerebral con afección cutánea y palatina. La especie aislada mediante cultivos micológicos fue Rhizopus sp.

Abstract Rhino-orbito-cerebral mucormycosis from dental origin is an acute infection caused by opportunistic fungi belonging to the order of Mucorales, which affects mainly diabetic and immunocompromised patients. We report the case of a 63-year old diabetic man who performed a dental extraction on himself by his own means and subsequently developed a rhino-orbito-cerebral mucormycosis with cutaneous and palatal affection. The species isolated in the mycological culture was Rhizopus sp.

Cutaneous mucormycosis

Abstract Cutaneous mucormycosis is an emerging fungal infection caused by opportunistic fungi of the phylum Glomeromycota. It is frequent in poorly controlled diabetic patients and individuals with immunosuppression. It is usually acquired by direct inoculation through trauma. The clinical presentation is nonspecific, but an indurated plaque that rapidly evolves to necrosis is a common finding. Diagnosis should be confirmed by demonstration of the etiological agent and new molecular diagnostic tools have recently been described. It is an invasive life-threatening disease and in order to improve survival, a prompt diagnosis and multidisciplinary management should be provided. The treatment of choice is amphotericin B, but new azoles, such as posaconazole and isavuconazole, must be considered.

Fulminant auricular mucormycosis in a diabetic patient

Human mucormycosis is an atypical fungal infection that commonly affects the skin, but rarely the auricular region. A 32-year-old diabetic woman, agricultural worker, was admitted with swelling, redness and mild signs of epidermolysis of the left ear, associated with intense pain, facial paralysis and septic signs. The ear cellulitis evolved into necrosis of the same region on the following day. Surgical debridement was performed and antimycotic therapy was started with poor response. The patient died in 48h. Culture was confirmatory for Rhizopus sp. (AU)

Identification of Mucorales From Clinical Specimens: A 4-Year Experience in a Single Institution

Mucormycosis, a fatal opportunistic infection in immunocompromised hosts, is caused by fungi belonging to the order Mucorales. Early diagnosis based on exact identification and multidisciplinary treatments is critical. However, identification of Mucorales fungi is difficult and often delayed, resulting in poor prognosis. This study aimed to compare the results of phenotypic and molecular identification of 12 Mucorales isolates collected from 4-yr-accumulated data. All isolates were identified on the basis of phenotypic characteristics such as growth rate, colony morphology, and reproductive structures. PCR and direct sequencing were performed to target internal transcribed spacer (ITS) and/or D1/D2 regions. Target DNA sequencing identified five Lichtheimia isolates, two Rhizopus microsporus isolates, two Rhizomucor pusillus isolates, one Cunninghamella bertholletiae isolate, one Mucor fragilis isolate, and one Syncephalastrum racemosum isolate. Five of the 12 (41.7%) isolates were incorrectly identified on the basis of phenotypic identification. DNA sequencing showed that of these five isolates, two were Lichtheimia isolates, one was Mucor isolate, one was Rhizomucor isolate, and one was Rhizopus microspores. All the isolates were identified at the species level by ITS and/or D1/D2 analyses. Phenotypic differentiation and identification of Mucorales is difficult because different Mucorales share similar morphology. Our results indicate that the molecular methods employed in this study are valuable for identifying Mucorales.

Mucormicosis cutánea en un paciente inmunocomprometido / Cutaneous mucormycosis in an immunocompromised patient

Mucormycosis is an increasingly emerging life-threatening infection and is an important cause of morbidity and mortality in patients with leukemia. We present the case of a 4-year-old boy with an acute lymphoblastic leukemia complicated by severe and prolonged neutropenia during induction chemotherapy, who developed cutaneous mucormycosis of the elbow. Direct microscopy with KOH and the histopathologic observation of the skin revealed fungal hyphae without septations. The cultures were positive for Rhizopus microsporus var oligosporus. The patient was treated succefully with amphotericin B during 40 days, surgical debridement and dermoepidermic graft. Early recognition and prompt intervention with combined medical and surgical treatment may improve the outcome. The most common management strategy in survivors involves a combination of antifungal therapy with amphotericin B and surgical debridement.

La mucormicosis es una enfermedad emergente grave, producida por hongos saprófitos del orden Mucorales, que afecta fundamentalmente a pacientes inmunocom-prometidos. La forma cutánea se origina por inoculación de esporas dentro de la dermis con el subsecuente desarrollo de una lesión tipo ectima, generalmente única y de evolución rápidamente progresiva, por las características angioinvasoras del hongo, que determina amplias zonas de infartos y necrosis en los tejidos. Presentamos el caso de un paciente con cuatro años de edad, con diagnóstico de leucemia linfoblástica aguda, en quimioterapia de inducción, que cursó con neutropenia profunda y prolongada, presentando una lesión cutánea en el codo compatible con ectima gangrenoso. Recibió tratamiento antimicrobiano y antifúngico, asociado a un aseo quirúrgico. El cultivo para hongos demostró crecimiento de Rhizopus microsporus var oligosporus, y la histología concluyó presencia de hifas no septadas. El estudio de extensión descartó compromiso óseo, sinusal y cerebral. Completó 40 días de terapia antifúngica con anfotericina B deoxicolato, evolucionando satisfactoriamente. Posteriormente requirió injerto dermo-epidérmico. Si bien esta patología es infrecuente, debemos sospecharla en pacientes con neoplasias hematológicas, para establecer un diagnóstico etiológico oportuno, ya que el tratamiento contempla el desbridamiento quirúrgico precoz asociado a antifúngicos sistémicos, siendo de elección anfotericina B.

Necrotizing (malignant) otitis externa: an unusual localization of mucormycosis.

Malignant otitis externa (MOE) is a severe infection of external auditory canal and skull base. A 17-year-old diabetic girl was admitted with diabetic ketoacidosis. Cellulitis of her right ear occurred on the second day of hospitalization and a black necrotic scar in the same region appeared on the next day. The lesion rapidly invaded to right side of neck and surrounding tissue of the patient. Therefore, antimycotic therapy was started. Unfortunately the patient died on seventh day of hospitalization because of probably extensive fungal invasion. Physicians should suspect MOE connected to mucormycosis especially in patients with cutaneous lesions of ear unresponsive to antibiotic therapy.

Cutaneous zygomycosis: necrotising fascitis due to Saksenaea vasiformis.

Saksenaea vasiformis is an emerging human pathogen, most often associated with cutaneous or subcutaneous lesions following trauma. This is the report of a case of subcutaneous zygomycosis from which Saksenaea vasiformis was isolated on culture. As the patient developed acute interstitial nephritis, amphotericin B could not be administered in full dose. Surgical debridement was carried out, but the patient deteriorated gradually and died. To the best of our knowledge, this is the first reported case of Zygomycosis due to Saksenaea vasiformis from Visakhapatnam.

Fascitis necrosante por Apophysomyces elegans, moho de la familia mucoraceae, en paciente inmunocompetente / Necrotizing fasciitis in an immunocompetent patient caused by Apophysomyces elegans

Se presenta la historia de un niño de 7 años de edad, politraumatizado por accidente automovilístico, que falleció a consecuencia de infección por Apophysomyces elegans, un hongo de la familia Mucoracea. La invasión fue progresiva, inicialmente se observó una lesión puntiforme en la región lumbar izquierda que progresó a fascitis necrosante y, posteriormente, afectó la zona lumbar, los glúteos y el flanco derecho. El tratamiento antimicótico resultó inefectivo y el paciente falleció 8 semanas después de su accidente. Se presenta, además, una revisión de los casos atribuidos a este hongo

Rhino-orbito-cerebral mucormycosis. A retrospective analysis of clinical features and treatment outcomes.

OBJECTIVE: The conventional management of rhino-orbito-cerebral (ROC) mucormycosis includes control of metabolic abnormality, administration of amphotericin B and surgery that spans simple sinus clearance, radical debridement and orbital exenteration. Recent literature includes anecdotal descriptions of successful treatment with conservative management of involved orbits. We evaluated the clinical features and outcome of treatment for the different stages of ROC mucormycosis. METHOD: In this retrospective case series, 34 case records of patients with a histopathological diagnosis of ROC mucormycosis treated between 1992 and 2000 were reviewed. Three clinical stages and three treatment groups were identified. Patients with limited sino-nasal disease (Clinical stage I) underwent sino-nasal debridement (Treatment group A). Patients with limited rhino-orbital disease (Clinical stage II) underwent either sino-nasal debridement alone (Treatment group A) or orbital exenteration in addition to sino-nasal debridement (Treatment group B). Patients with rhino-orbito-cerebral disease (Clinical stage III) did not undergo any surgical procedure (Treatment group C). Thirty-three patients received intravenous amphotericin B. Outcome for each group was measured as "Treatment success" (disease free, stable patient with metabolic abnormality under control) and "Treatment failure" (progression of disease with worsening general condition or mortality due to the disease). RESULTS: Uncontrolled diabetes in 30 (88.2%) of 34 patients was the commonest underlying disease and 16 (53.3%) of 30 diabetics had ketoacidosis. Chronic renal failure (n = 4), hepatic disease (n = 3) and idiopathic thrombocytopenia (n = 1) were the other underlying diseases. Eleven patients had stage I disease, 16 patients had stage II disease and seven patients had stage III disease. All 11 patients with stage I disease received treatment A; of 16 patients with stage II disease, 7 received treatment A and the remaining with stage III disease received treatment B; 7 patients with stage II disease received treatment C. Ten of 11 patients (91%) with stage I disease had treatment success. In patients with stage II disease, 7 of 7 (100%) with treatment A and 1 of 9 (11.1%) with treatment B had treatment success. All seven patients with stage III disease had treatment failure. CONCLUSION: Debridement of the sinuses is necessary in all cases of rhino-orbito-cerebral mucormycosis. Diagnosis in the early stage needs a high degree of suspicion. There is a definite role for retention of orbits in patients whose metabolic derangement is rapidly controlled and orbital involvement is non-progressive.

Panophthalmitis due to rhizopus in an AIDS patient: a clinicopathological study.

Various opportunistic infections in the eye have been reported earlier in AIDS. We report a case of panophthalmitis in an AIDS patient where the eviscerated tissue on histopathologic and microbiologic examination showed the fungus Rhizopus.

Hepatic and small bowel mucormycosis after chemotherapy in a patient with acute lymphocytic leukemia

Mucormycosis is a rare but invasive opportunistic fungal infection with increased frequency during chemotherapy-induced neutropenia. The clinical infections due to Mucor include rhinocerebral, pulmonary, cutaneous, gastrointestinal and disseminated diseases. The first two are the most common diseases and all entities are associated with a high mortality rate. Still hepatic involvement of Mucor is rarely reported. We experienced a case of hepatic and small bowel mucormycosis in a 56-year-old woman after induction chemotherapy for B-cell acute lymphocytic leukemia. Initial symptoms were a high fever unresponsive to broad spectrum antibiotics and pain in the left lower abdominal quadrant. It was followed by septic shock, deterioration of icterus and progressively elevated transaminase. An abdominal CT demonstrated multiple hypodense lesions with distinct margins in both lobes of liver and pericolic infiltration at small bowel and ascending colon. Diagnosis was confirmed by biopsy of the liver. The histopathology of the liver showed hyphae with the right-angle branching, typical of mucormycosis. The patient was managed with amphotericin B and operative correction of the perforated part of the small bowel was performed. However, the patient expired due to progressive hepatic failure despite corrective surgery and long-term amphotericin B therapy.

Zigomicose nasofacial no Estado do Pará: registro de dois casos / Nasofacial zygomycosis in the state of Pará: the registration of 2 cases

São descritos dois casos de zigomicose nasofacial, procedentes de Belém, PA. O achado indica não ser tão rara, como se poderia supor, pela falta de registros, a ocorrência dessa micose na Região Norte do Brasil. Em ambos os pacientes, o tratamento pelo cetoconazol determinou involução rápida das manifestações clínicas, principalmente a obstrução nasal. Com o presente relato, sobe para cinco o número de casos da infecção referidos até agora do Estado do Pará.

Two new cases of nasofacial zygomycosis from the state of Pará, Brazil, are reported. Both cases were treated with ketoconazole and the response to the drug was considered to be good; the patients improved rapidly and the nasal obstruction was the first manifestation to disappear. As five cases have been described in recent years from the state of Pará, this form of zygomycosis can no longer be considered as a rare disease in northern Brazil.