Mucormicosis rino-órbito-cerebral crónica: una patología poco frecuente / Chronic rhino-orbital-cerebral mucormycosis: an uncommon pathology

Resumen La mucormicosis rino-órbito-cerebral (ROC) crónica es una patología poco frecuente, con un número reducido de casos publicados en la literatura, cuyas manifestaciones son muy diversas e inespecíficas. El tratamiento se basa en la experiencia de casos y series de casos. Las herramientas terapéuticas incluyen el uso de antifúngicos endovenosos y orales por tiempo prolongado, asociado o no a debridamiento quirúrgico amplio, pudiendo requerir incluso exenteración orbitaria. Presentamos a continuación un caso de mucormicosis ROC crónica, junto con las dificultades para su diagnóstico y manejo, en el que destaca el enfrentamiento multidisciplinario. Dada la poca frecuencia de esta enfermedad, nos parece relevante difundirlo.

Abstract Chronic rhino-orbital-cerebral mucormycosis is a rare condition with a small number of cases that have been published, whose manifestations are very diverse and nonspecific. The treatment is based on case series experiences. Therapeutic options include the use of long-term intravenous and oral antifungals, associated or not with extensive surgical debridement, and may even require orbital exenteration. We present below a case of chronic rhino-orbital-cerebral mucormycosis with the challenge of diagnosis and management in which multidisciplinary work is fundamental. Since it is an uncommon pathology, it seems relevant to share the information.

An Emergent Entity: Indolent Mucormycosis of the Paranasal Sinuses. A Multicenter Study

Abstract Introduction Indolent or chronic mucormycosis is a rare entity that affects both immunosuppressed and immunocompetent individuals. Additionally, its clinical evolution is nonspecific and there is no standardized treatment for this condition. Objective To describe the clinical characteristics and management of patients with indolent mucormycosis. Methods In the project of study with chart review in the Interinstitutional secondary care centers, patients with evidence of indolentmucormycosis, defined as pathological confirmation of nasal/paranasal sinus mucormycosis for more than 1 month, were included. All patients underwent complete laboratory workup, imaging studies, surgical treatment and adequate follow-up. No evidence of disease status was defined when patient had subsequent biopsies with no evidence of mucormycosis. Results We included seven patients, three female and four male subjects. The mean age was 53.14 years. Four patients were immunosuppressed and three immunocompetent. Among the immunosuppressed patients three had diabetes and one had dermatomyositis. The symptomswere nonspecific: facial pain/headache, mucoid discharge and cacosmiawere the ones most frequently reported. Maxillary sinus involvement was present in all patients. Two immunosuppressed subjects received amphotericin. Posaconazole was the only treatmentinoneimmunosuppressedpatient. Allimmunocompetent patientshadsingleparanasal sinus disease and received only surgical treatment. All patients are alive and free of disease. Conclusion Indolent mucormycosis is a new and emerging clinical entity in immunosuppressed and immunocompetent patients. Single paranasal sinus disease is a frequent presentation and should not be overlooked as a differential diagnosis in these patients. Immunocompetent patients should only be treated surgically. (AU)

Tricodisplasia espinulosa asociada a síndrome mielodisplásico e inmunosupresión / Trichodysplasia spinulosa: report of one case

Trichodysplasia spinulosa is a rare disease that occurs in the setting of immunosuppression, associated with tolerogenic therapy used in allograft recipients or patients with hematologic malignancies. Clinically, it is characterized by a centrofacial cutaneous eruption of erythematous papules with a central keratinous spicule, often associated with variable degrees of alopecia. Histologic findings are characteristic, and electron microscopy reveals the presence of trichodysplasia spinulosa associated polyomavirus. We report a 47-year-old woman with idiopathic autoimmune pancytopenia refractory to diverse immunosuppressant regimens, with clinical and pathologic findings compatible with the disease, in whom complementary studies were required to exclude other differential diagnoses.

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)

Mucormicosis rinosinusal por mucor hiemalis en una paciente con leucemia mieloide aguda / Rhinosinusal mucormycosis by mucor hiemalis in a patient with acute myeloid leukemia

Se reporta un caso clínico de una paciente femenina de 41 años, con antecedentes de leucemia mieloide aguda (LMA) en remisión. Estudiada por hematología, se confirmó recaída de LMA M4. Se inició quimioterapia. La paciente evolucionó con pancitopenia severa. Presentó dos episodios de neutropenia febril, el primero fue asociado a un absceso glúteo que se trató con antibacterianos, y el segundo a compromiso rinosinusal y úlcera necrótica de punta nasal, columela, tabique, cornete inferior izquierdo y paladar duro. Debido a la clínica e imá- genes radiológicas, se sospechó mucormicosis, por lo que se realizó cirugía con debridación extensa y se inició tratamiento antimicótico con anfotericina B desoxicolato. El cultivo de tejido informó abundante desarrollo de Mucor hiemalis. Se mantuvo pancitopénica durante aproximadamente un mes, siendo diariamente evaluada por un equipo multidisciplinario. Se hicieron varios aseos quirúrgicos, en el último se encontró tejido vital. La paciente completó diez días con anfotericina B desoxicolato y posteriormente se hizo traslape a posaconazol oral. Se realizó mielograma de control que evidenció remisión completa de recaída de LMA. Se dio de alta a su domicilio al día 40 de hospitalización, con hemograma adecuado y tratamiento oral con posaconazol para completar 6 semanas en total.

We report a case of a 41-years-old female patient with a history of acute myeloid leukemia (AML) in remission. Hematology studies confirmed relapse of AML M4. Chemotherapy was started. The patient developed severe pancytopenia. She presented two episodes of febrile neutropenia, the first one was associated with a gluteal abscess that was treated with antibacterials, and the second to rhinosinusal involvement and necrotic ulcer of nasal tip, columella, septum, left inferior turbinate and hard palate. Due to clinical and radiological imaging, mucormycosis was suspected, so surgery was performed with extensive debridement and antifungal treatment with amphotericin B deoxicholate was initiated. Tissue culture reported abundant development of Mucor hiemalis. She remained pancytopenic for approximately one month, being evaluated daily by a multidisciplinary team. Several surgical were made, finding vital tissue in the last perform. The patient completed ten days with amphotericin B deoxicholate and later was overlapped to oral posaconazole. A control myelogram was performed, showing complete remission of AML. She was discharged to her home at day 40 of hospitalization, with adequate blood count and oral treatment with posaconazole to complete 6 weeks in total.

Abdominal mucormycosis in a child: a case report

Abstract A 2-year-old Brazilian female child from the countryside in Bahia State presented with pain in the right flank of the abdomen, accompanied by a daily fever for about 2 weeks before admission. A large mass in the abdomen was resected by the surgical team. The biopsies revealed the mass was an intra-abdominal mucormycosis. However, the diagnosis was late, and despite treatment (amphotericin B) initiation, the patient eventually died.

Gastrointestinal mucormycosis: a success story and appraisal of concepts

Mucormycosis is an opportunistic, life-threatening fungal infection caused by fungi of the class Zygomycetes. The disease has traditionally been reported in immunocompromised patients, premature infants, diabetics, transplant recipients, prolonged use of corticosteroids or in condition associated with increased availability of serum iron such as acidosis or deferoxamine administration. The infection is progressive and associated with a high mortality unless treatment is initiated promptly. The number of cases of gastrointestinal mucormycosis indexed on PubMed over the past 2 decades has shown an alarming rise. Moreover, the infection is being increasingly reported in patients without the traditional risk factors. We report successful management of an immunocompetent child with gastrointestinal mucormycosis who responded to aggressive treatment with surgical debridement and antifungal agents. The fungicidal activity of colistin [polymyxin E] has also been highlighted

Recurrent pulmonary mucormycosis after lobectomy in a non-smoking patient without predisposing risk factors

Pulmonary mucormycosis is a very rare clinical condition in patients without underlying risk factors. A limited number of cases have been reported in predominantly elderly patients; history of smoking appears to be a common feature. A case of non-smoking male who developed pulmonary mucormycosis with the longest reported follow-up is presented. In addition, this is also the first reported case with disease recurrence after lobectomy (two years) in an immunocompetent host. Treatment with an additional lobectomy and amphotericin B was successful in this patient.

Mucormicose: estudo clínico / Mucormycosis: clinical study

A mucormicose é uma infecção fúngica oportunista, habitualmente fulminante, que acomete, principalmente, imunodeprimidos. Pode apresentar-se sob as formas rinocerebral, pulmonar, gastrintestinal, cerebral, cutânea, subcutânea e disseminada. O diagnóstico é estabelecido a partir da biopsia de tecido com envio de material para cultura de fungos, podendo-se usar exames de imagem para quantificar a área acometida. O tratamento é cirúrgico, devendo ser associado à anfotericina B.

Murcomicosis is a fungal opportunistic infection that is usually fatal, which attacks, essentially, immunodeficient people. It usually presents itself in: rinocerebral, pulmonary, gastrointestinal, cerebral, cutaneous, subcutaneous and disseminated forms. The diagnosis is established by tissue biopsy after fungal culture, and image exams. Can be used to calculate the surgical treatment should be associated with anfotericin B.

Successful treatment of mucormycosis after kidney transplantation

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

Mucormicosis: infección necrotizante como complicación de dermolipectomía abdominal / Mucormycosis: necrotizing infection as a complication of abdominal dermolipectomy

Las infecciones necrotizantes de los tejidos blandos varían en su presentación clínica, pero en general son de curso grave y alta mortalidad. El cuadro clínico incluye: fiebre, celulitis, edema, crepitación, necrosis y sepsis; con frecuencia existe un antecedente o traumático quirúrgico. Como hallazgo operatorio se encuentra necrosis de la piel y tejido subcutáneo, con o sin mionecrosis. El tratamiento es una inmediata y amplia debridación acompañada con antibioticoterapia de amplio espectro. Se presenta un caso clínico de infección necrotizante de tejidos blandos por mucor, en una mujer con antecedente de abdominoplastia y lipoescultura con progresión crítica y evolución fatal. La mucormicosis es una patología agresiva, cuyo diagnostico clínico no es fácil por lo cual debe ser considerada seriamente por el cirujano plástico.

Terapéutica de apoyo en la mucormicosis / Therapeutic support in mucormycosis

La mucormicosis es una infección infrecuente, causada por hongos oportunistas, pertenecientes al orden de los mucorales, cuyo sello es la invasión vascular por hifas, que conduce a la trombosis arterial. Generalmente afecta a pacientes con una enfermedad de base y produce una infección invasiva, frecuentemente fulminante. A continuación analizamos el caso de un paciente de 13 años con leucemia linfática aguda y neutropenia secundaria a la quimioterapia, cuyo cuadro clínico se presentó con cefalea fiebre y rinorrea. Las imágenes evidenciaron un compromiso de la pared lateral del seno esfenoidal. Fue tratado con Anfotericina B liposomal, factor de crecimiento de neutrófilos y resección quirúrgica en dos oportunidades con buena respuesta clínica y radiológica.

Mucormicosis rinocerebral: caso clínico / Rhinocerebral mucormycosis: clinical case

La mucormicosis es una enfermedad poco frecuente, causada por hongos del orden de los mucorales. Suele presentarse en pacientes con algún grado de inmunodepresión. La forma rinocerebral afecta principalmente a pacientes con cetoacidosis diabética. El cuadro clínico corresponde al de una sinusitis invasiva, con un curso generalmente fatal y el tratamiento es médico-quirúrgico. Se presenta un caso clínico con revisión de la literatura

Mucormicosis de la nariz y los senos paranasales: Diagnóstico, pronóstico y tratamiento / Mucormycosis of the nose and paranasal sinuses: Diagnosis, prognosis and treatment

Las micosis de la nariz y los senos paranasales han sido clasificadas en invasiva y no invasiva, incluyendo esta última a la variedad alérgica micótica. El comportamiento clínico de la infección depende de la interacción entre la competencia inmunológica del paciente y la patogenicidad del hongo. Se realizó un estudio clínico para evaluar las características de pacientes con mucormicosis de la nariz y senos paranasales con o sin involucro cerebral. Se presentan cuatro casos de mucormicosis pasosinusal, los cuales fueron diagnosticados y tratados en el periodo de 1994-1997; estos casos representaron el 3.3 por ciento de los 120 de sinusitis crónica tratados quirúrgicamante en el Departamento de Otorrinolaringología del Hospital General "Dr. Manuel Gea González". A todos los pacientes se les dio tratamiento multidisciplinario, incluyendo insulina para corrección de su enfermedad de base ya que todos eran diabéticos, anfotericina B por vía intravenosa y debridación quirúrgica temprana y amplia de los tejidos necróticos, siendo este último el factor más importante para la supervivencia de los pacientes. Revisamos el espectro clínico de la enfermedad y se analizan los casos antes mencionados

Mucormicosis rinocerebral por Rhizopus sp / Cerebral mucormycosis by Rhizopus sp

Masculino de 24 años de edad con diabetes mellitus insulino-dependiente descompensada, cetoacidosis y mucormicosis rinocerebral por Rhizopus sp. Se trató en forma multidisciplinaria con control metabólico, anfotericina B y extensa debridación del área necrótica. El resultado fue satisfactorio; se planeó reconstrucción quirúrgica