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.

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.

[Mucormycosis: four case reports]

Mucormycosis is a rare and invasive fungal infection, but frequently fatal when it occurs. It commonly affects patients with diabetes mellitus. The aim of this study is to assess the clinical presentation, radiological findings, management and prognosis of mucormycosis. This retrospective study was conducted in the department of infectious diseases of Rabta hospital between January 1988 and December 2004 and included patients hospitalized for mucormycosis confirmed by mycological and/or histological findings. The study is about four diabetic patients with mucormycosis [3 men and a woman]. Three of them had diabetic ketoacidosis at the time of diagnosis. The infection was sinusal in 2 cases and rhinocerebral in the other two cases. Treatment consisted in systemic amphotericin B combined with surgical debridement in 3 cases. A fatal outcome was noted in 2 cases. Mucormycosis remains a severe infectious disease in diabetic patients. Early diagnosis and treatment is mandatory for a successful management of this infection

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.