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Management of CNS mucormycosis in the pediatric patient.

Rhinocerebral mucormycosis (RM) is a rare, rapidly progressive disorder caused by fungi from the Mucoraceae family. With extensive central nervous system involvement, this disease is uniformly fatal within weeks. Mucormycosis normally presents in poorly controlled diabetics, intravenous drug abusers and immunocompromised patients. Many have advocated radical surgical resection (i.e. exenteration of the cavernous sinus with carotid sacrifice and en bloc resection) with administration of amphotericin B. We present a case of mucormycosis involving the paranasal sinuses and cranial base in a pediatric patient who experienced long-term survival with a more limited resection. We also present a review of the relevant literature. A 14-year-old diabetic male presented with RM with involvement of the bilateral frontal lobes, right basal ganglia and temporal lobe. Additionally, there was involvement of the sphenoid sinus and right cavernous sinus with extension into the posterior fossa along the course of the trigeminal nerve and encasement with narrowing of the right carotid artery. The patient underwent sinus endoscopy with debridement of necrotic fungal tissue and bone. This was followed by craniotomy with evacuation of bifrontal, right temporal and basal ganglia abscesses in such a way that all abscess cavities communicated. An Ommaya reservoir was placed into the largest cavity. The patient continued to receive intrathecal and intravenous antibiotics as well as hyperbaric oxygen therapy. The patient was clinically and radiographically free of disease 1 year after diagnosis. While invasive RM is generally a fatal disease, this rare disorder can be treated successfully without radical resection, particularly if multimodality treatment options are implemented.

Adolescent↗

Pulmonary mucormycosis diagnosed by fine needle aspiration cytology. A case report.

BACKGROUND: The usefulness of fine needle aspiration cytology (FNAC) in the diagnosis of lung lesions is well documented. Fungal lesions are among nonneoplastic lesions of the lung in which FNAC has proven a useful technique in both immunocompromised and immunocompetent patients. These include cryptococcosis, aspergillosis, histoplasmosis and coccidiodomycosis. Pulmonary mucormycosis, an aggressive fungal infection, is rarely diagnosed on FNAC. We report a case of isolated pulmonary mucormycosis diagnosed on FNAC. CASE: A 62-year-old renal transplant recipient with diabetes mellitus and hypertension, asymptomatic for four months, presented with tachypnea, generalized malaise and weakness. Radiologic studies showed an enlarging, cavitating lesion in the right lung. Computed tomography-guided fine needle aspiration performed on the lung lesion showed fungal profiles with broad, ribbonlike, aseptate hyphae with right-angled branching consistent with the Zygomycetes class of fungi, which includes Rhizopus and Mucor species. Fungal cultures confirmed the presence of Rhizopus. The patient underwent right pneumonectomy, was placed on liposomal amphotericin B therapy and discharged with good pulmonary status and stable kidney function. CONCLUSION: FNAC is a useful technique in the diagnosis of pulmonary mucormycosis.

Biopsy, Needle↗

Rhinocerebral mucormycosis treated with 32 gram liposomal amphotericin B and incomplete surgery: a case report.

BACKGROUND: Mucormycosis (or zygomycosis) is the term for infection caused by fungi of the order Mucorales. Mucoraceae may produce severe disease in susceptible individuals, notably patients with diabetes and leukemia. Rhinocerebral mucormycosis most commonly manifests itself in the setting of poorly controlled diabetes, especially with ketoacidosis. CASE PRESENTATION: A 31-year-old diabetic man presented to the outpatient clinic with the following signs and symptoms: headache, periorbital pain, swelling and loss of vision in the right eye. On physical examination his right eye was red and swollen. There was periorbital cellulitis and the conjunctiva was edematous. KOH preparation of purulent discharge showed broad, ribbonlike, aseptate hyphae when examined under a fluorescence microscope. Cranial MRI showed involvement of the right orbit, thrombosis in cavernous sinus and infiltrates at ethmoid and maxillary sinuses. Mucormycosis was diagnosed based on these findings. Amphotericin B (AmBisome(R); 2 mg/kg.d) was initiated after the test doses. Right orbitectomy and right partial maxillectomy were performed; the lesions in ethmoid and maxillary sinuses were removed. The duration of the liposomal amphotericin B therapy was approximately 6 months and the total dose of liposomal amphotericin B used was 32 grams. Liposomal amphotericin B therapy was stopped six months later and oral fluconazole was started. CONCLUSIONS: Although a total surgical debridement of the lesions could not be performed, it is remarkable that regression of the disease could be achieved with medical therapy alone.

Adult↗

Chronic mucormycosis.

Chronic rhinocerebral mucormycosis, as distinct from the acute fulminating form, has been previously unreported in the otolaryngology literature. We have observed two cases of chronic rhinocerebral mucormycosis with osteomyelitis and bony sequestration at 8 months and 2 years after apparent control of initial infection. In addition to the accepted management of early diagnosis, surgical debridement, an appropriate medical therapy, patients who have been successfully treated for acute and iulminating mucormycosis need long-term, close follow-up with further debridement should any chronic infection become evident.

Aged↗

Rhinocerebral mucormycosis: results of aggressive surgical debridement and amphotericin B.

Rhinocerebral mucormycosis is a fulminant, devastating fungal disease. The use of amphotericin B, combined with aggressive surgical debridement, has increased survival rates from approximately 20% to 70%. Eleven patients with this rare disease have been treated over the past 6 years. Therapy with amphotericin B was begun in all patients as soon as the diagnosis of mucormycosis was suspected. Seven patients underwent aggressive surgical debridement; all seven had disease involving the sphenoethmoidal complex, requiring external ethmoidectomy with or without sphenoidotomy. Six patients had maxillary sinus disease, requiring Caldwell-Luc antrostomy or maxillectomy. Three patients required orbital exenteration. Surgery was performed within 24 hours of diagnosis on all but two of our patients. Despite prompt diagnosis, aggressive surgical debridement, therapy with amphotericin B, and correction of metabolic acidosis, our mortality rate was 82%. Rhinocerebral mucormycosis remains a rapidly progressive, frequently fatal disease.

Adolescent↗

Clinical signs of orbital ischemia in rhino-orbitocerebral mucormycosis.

Mucormycosis is the most acutely fatal fungus infection of man (Ferry and Abedi). The most common clinical type of infection is rhino-orbitocerebral mucormycosis. Prompt recognition of the clinical picture is essential if the appropriate urgent management is to be instituted without delay. The presence of black eschar in the region of the nasal passages, palate, midface, and orbit is the best-recognized clinical sign alerting the clinician to the diagnosis. Black eschar is, however, a feature in only a minority of these patients at the time of presentation. This paper discusses other clinical signs, particularly orbital ischemia, which should suggest the diagnosis. The clinical presentation of orbital ischemia in mucormycosis includes proptosis, total external and internal ophthalmoplegia, and early blindness. A lax, nontender periorbital puffiness, which does not feel warm to the examiner's touch, is typical. Proptosis and chemosis, if present, are mild. These signs are compared with those of pyogenic orbital cellulitis, with which the condition might most easily be confused.

Adult↗

The clinical manifestations of cardiac mucormycosis.

The manifestations of cardiac mucormycosis may dominate the clinical picture of disseminated mucormycosis. These manifestations include myocardial infarction, congestive heart failure, conduction system disease, valvular imcompetence and pericarditis. The development of such manifestations in a febrile compromised host with one or more predisposing factors should prompt consideration of disseminated mucormycosis in the differential diagnosis and initiation of appropriate diagnostic and therapeutic strategies.

Aged↗

Clinical predictors of mucormycosis in children with type 1 diabetes mellitus.

BACKGROUND: Mucormycosis is an acute, often rapidly fatal fungal infection that is an important cause of death in young patients with diabetes mellitus (DM) who have poor glycemic control and acute ketosis. Despite the importance of this condition, the clinical predictors of mucor infection are not well defined. METHODS: A retrospective chart review was carried out for five patients, age 14-19 years, diagnosed with mucormycosis at the University of Florida between 1984 and 1999. For the three patients for whom adequate information was available, two randomly selected, age-matched case controls were selected and compared to affected patients for the following variables: average HbA1c concentrations, number of missed clinic appointments, and the number of hospital and Diabetes Project Unit (DPU) admissions. RESULTS: HbA1c levels were similar in affected patients and controls, indicating glycemic control is not the principal factor predisposing to mucor infection. African-American race and poor compliance, as evidenced by poor clinic attendance, risk-taking behaviors and high admission rate to the DPU, were predictors of increased susceptibility to infection with mucormycosis.

Adolescent↗

Pulmonary mucormycosis: radiologic findings in 32 cases.

OBJECTIVE: The purpose of this study was to characterize the radiologic manifestations of pulmonary mucormycosis with clinical and pathologic correlation. MATERIALS AND METHODS: Clinical records, pathology reports, chest radiographs, and CT scans of 32 cases of pathologically proven pulmonary mucormycosis were retrospectively reviewed. RESULTS: The study group included 20 males and 12 females with a mean age of 47 years old. Clinical data were available for 29 patients. Signs and symptoms included fever (n = 23), cough (n = 21), bloody sputum (n = 9), dyspnea (n = 7), and chest pain (n = 6). Four patients were asymptomatic. Most patients were either immunocompromised (n = 20) or had diabetes mellitus (n = 9). Sputum or bronchoalveolar lavage cultures showed no growth in 17 of 18 cases. Diagnoses were confirmed at surgery or autopsy in all cases. Abnormalities seen on chest radiographs included lobar (n = 15) or multilobar (n = 6) consolidation, solitary (n = 7) or multiple (n = 1) masses, and solitary (n = 3) or multiple (n = 2) nodules. Cavitation was seen on chest radiographs in 13 patients, and intracavitary masses were seen in four. Associated radiographic findings included hilar (n = 3) or mediastinal (n = 3) adenopathy and unilateral (n = 6) or bilateral (n = 3) pleural effusion. CT in 19 patients revealed these significant additional findings: splenic (n = 1) or renal (n = 1) involvement, bronchial occlusion (n = 1), extrapulmonary invasion (n = 1), and pulmonary artery pseudoaneurysm (n = 1). CONCLUSION: In our study, pulmonary mucormycosis typically was manifested in immunocompromised or diabetic patients by consolidation on chest radiographs; cavitation was seen in 40% of patients. CT revealed significant unsuspected abnormalities in 26% of patients. Definitive diagnosis required pathologic demonstration of the organism in affected tissue because cultures from our patients rarely showed growth.

Diabetes Mellitus↗

Rhinocerebral mucormycosis caused by Apophysomyces elegans.

Mucormycosis is an uncommon fungal disease and one of the most fulminant infections known. This is the second report of rhinocerebral mucormycosis caused by Apophysomyces elegans, a newly recognized genus and species classified in the family Mucoraceae. The patient was a 54-year-old man being treated for a severe sinus infection with antibiotics and oral steroids. Recovery occurred in our patient after prompt surgical debridement and drainage of his maxillary sinuses. This case fits the reported characteristics of other A. elegans infections including warm climate, intimate contact with the soil, and an incubation period measured in days. Several reported cases indicate A. elegans can cause mucormycosis in immunocompetent individuals with no underlying medical problems.

Amphotericin B↗

Endoscopic sinus surgery for rhinocerebral mucormycosis.

Rhinocerebral mucormycosis is a fulminant, often fatal, disease. Aggressive surgical debridement has been considered an important part of treatment. Traditionally, an external or transantral approach has been the classic method. Recently, endoscopic sinus surgery (ESS) has been tried on several occasions to reach the goal of radical resection. Since 1991, ESS has been used to treat 9 rhinocerebral mucormycosis patients in our department. Among them, ESS was the only surgical procedure in six patients. The other three patients were treated by ESS combined with a transantral procedure. As a result, eight patients (88.9%) have survived the disease. One patient died 5 days after ESS because of an internal carotid artery occlusion. We conclude that ESS can be used to treat rhinocerebral mucormycosis alone or in combination with the traditional surgical procedures. It has the advantage of less operative morbidity and greater operative accuracy.

Adult↗

Mucormycosis in patients with renal failure.

Nine fatal cases of systemic mucormycosis observed in association with renal failure are described. Four patients were hospitalized for chronic renal failure as a consequence of chronic glomerulonephritis, myeloma kidney, chronic pyelonephritis, and polycystic kidney disease, respectively; and five patients presented with acute renal failure. The underlying causes in three of these five patients were gentamycin nephrotoxicity, acute gastroenteritis, and allograft rejection, respectively, and in the remaining two, acute renal failure was the result of extensive renal vascular and parenchymal invasion by mucor hyphae. Tissue invasion with mucormycosis was documented during life in two patients and at autopsy in seven patients. The infection was disseminated in five patients, and isolated pulmonary and rhinocerebral involvement occurred in two patients each. Our observations have shown that patients with renal failure are prone to develop mucormycosis, which carries a grave prognosis if therapy is not instituted in time.

Acute Kidney Injury↗

Disseminated neonatal gastrointestinal mucormycosis: a case report and review of the literature.

Mucormycosis of the gastrointestinal tract is a rare and fatal infection of neonates and children. This report describes a case of disseminated mucormycosis originating in the gastrointestinal tract of a premature infant of 26 weeks gestational age. The antemortem impression was that of necrotizing enterocolitis, diagnosed at 15 days of age, followed by death at 22 days. However, autopsy revealed extensive colonization and invasion of the small intestine and colon by mucormycosis, with blood and peritoneal fluid cultures positive for Mucorales species but negative for bacteria. There was associated hematogenous and adjacent spread of organisms to other viscera.

Diagnosis, Differential↗

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.

Female↗

[Mucormycosis in paranasal sinuses].

Paranasal sinus mucormycosis is a rare and often fatal condition that occasionally occurs in patients with debilitating disease. Five mucormycosis cases with paranasal sinus involvement are reported. Two also complicated diabetes mellitus. These diabetic patients rapidly developed meningitis after the onset of symptoms. One case was soon operated upon and necrotic masses with fungus were excised. Despite i.v. amphotericin B administration, two patients lost their consciousness in a few days, relapsed into coma and died. In these cases, the infection originated from the ethmoidal sinus and directly spread towards the orbit and brain. The remaining three cases with mucormycosis had operations after the initial diagnosis of sinusitis. All of the three patients are alive and well. The combination of an excisional operation and antifungal therapy resulted in favorable response. High resolution CT scanning is valuable both in planning treatment and monitoring the response to therapy.

Adult↗

Mucormycosis presenting as palatal perforation.

Mucormycosis is an opportunistic fungal infection that is caused by normally saprobic organism of the class Zygomycetes. The main form of mucormycosis are pulmonary and rhinocerebral. Rhinocerebral mycormycosis typically starts in the maxillary antrum, particularly in poorly controlled diabetics. Invasion of surrounding tissue can cause necrotizing ulceration of palate with a blackish slough and exposure of bone. A case of mucormycosis presenting as palatal performation is discussed in this article.

Antifungal Agents↗

A fatal case of gastric mucormycosis and diabetic ketoacidosis.

OBJECTIVE: To describe a case of gastric mucormycosis in conjunction with severe bleeding in a young woman, which occurred after an episode of ketoacidosis and had a fatal outcome. METHODS: We present a case report, including detailed clinical and pathologic findings in a woman with gastric mucormycosis associated with severe bleeding. RESULTS: A 17-year-old woman sought medical assistance for diabetic ketoacidosis and severe epigastric pain. Chest radiography showed normal findings, and blood and urine cultures were negative for bacterial growth. Endoscopy disclosed an extensive ulcerated lesion involving the greater curvature and posterior wall of the stomach. Biopsy specimens demonstrated the presence of invasive mucormycosis. Treatment with amphotericin B was initiated, but severe persistent gastrointestinal bleeding resulted in the patient's demise. CONCLUSION: In conjunction with diabetic ketoacidosis, severe infection by Mucor has been reported mainly in the rhino-orbital area. Although uncommon, the gastrointestinal tract can also be involved. In the case described, severe hemorrhage ensued and caused the patient's death.

Abdominal Pain↗

Endoscopic sinus surgery in the management of mucormycosis.

This is a report of the use of endoscopic sinus surgery in the management of three patients diagnosed with rhino-orbital or rhino-orbito-cerebral mucormycosis. A retrospective review was performed of the clinical examinations and imaging studies of three patients who underwent endoscopic sinus surgery as part of their therapy for mucormycosis. In addition to endoscopic surgery, all patients had aggressive control of underlying risk factors (diabetes mellitus, immunosuppression) and prolonged intravenous amphotericin B therapy. All three patients survived and avoided orbital exenteration. In selected patients with rhino-orbito-cerebral mucormycosis, endoscopic techniques can play a valuable role in diagnosis and management.

Adult↗