Local control of rhino-orbito-cerebral mucormycosis dramatically impacts survival.

Surgery and antifungals are the reference standard for rhino-orbito-cerebral mucormycosis (ROCM) treatment. The impact of local control on survival of 22 consecutive ROCM adults was studied on day 90: none vs. 75% died, respectively, with or without local control (p <0.0001). Hence, repeated surgical procedures are recommended to achieve local control of ROCM.

Chronic invasive sinus aspergillosis in immunocompetent hosts: a geographic comparison.

PURPOSE: To investigate potential differences in clinical presentation, histopathology, and outcomes of chronic invasive sinus aspergillosis (CISA) based on geographic region and species of Aspergillus isolated. MATERIALS AND METHODS: A retrospective analysis of published cases of CISA with a comparison of North American and worldwide cases comprised a systematic search of the English language literature. Thirty-four articles were identified detailing 15 North American and 76 global cases of CISA with cranio-cerebral extension in clinically immunocompetent patients. RESULTS: North American patients with CISA were older, had a more rapidly progressive course, and appeared to have higher rates of treatment failure and mortality. Anatomic distribution and presenting symptoms were similar between the two groups. North American cases were mostly due to A. fumigatus, while A. flavus was the predominant pathogen worldwide. While granulomatous inflammation was a rare observation in North American cases, it was seen in the majority of cases worldwide. CISA due to A. fumigatus was encountered in older adults, was associated with a chronic inflammatory response, an accelerated clinical course, and a trend toward treatment failure and higher mortality. Patients with A. flavus were younger, demonstrated granulomatous inflammation, and pursued an indolent, clinically responsive course. CONCLUSION: Observed differences in clinical presentation, histopathology, and outcome might involve a complex interplay between the human host, Aspergillus species, and local climatic conditions.

Rhino-orbital-cerebral mucormycosis and aspergillosis: differential diagnosis and treatment.

In immunocompromised patients, symptoms and the pathogen spectrum of sinusitis are frequently atypical. If progressive loss of vision occurs, an infection of the anterior skull base or nasal sinuses should be considered. We report on four patients with orbit-associated symptoms. CT-imaging revealed bony defects in sinus borders to orbits or endocranium. In all the cases immediate surgical drainage was performed because complications following sinusitis were suspected. Histopathological diagnosis revealed two cases of aspergillosis and mucormycosis. The possibility of opportunistic infections by saprophytic fungi must be taken into account in immunocompromised patients, as they may endanger both vision and survival. Immediate diagnosis and therapy are essential. Nowadays, therapeutic success can be achieved due to advances in antimicrobial therapy, hyperbaric oxygen therapy and treatment of the underlying disease. Radical procedures like orbital exenteration must be considered in all cases. The current state of diagnostics, therapy and prognosis is discussed based on these case reports and the recent literature.

Nasal polyposis: is there an inheritance pattern? A single family study.

BACKGROUND: Nasal Polyposis (NP) is defined as a chronic inflammatory disease of sinonasal mucosa leading to diffuse formation of benign polyps. Although family histories are frequently suggested in medical literature, no specific study focused on this point has been reported. The purpose of this study is to determine whether a hereditary factor could be implied for NP in a family where several members were affected. We included 99 members of this family. METHODS: All patients were assessed for conditions known to be associated with the development or presence of NP. Concerning NP, patients were screened with a validated questionnaire and selected patients had a medical examination by an Ear, Nose and Throat practitioner. RESULTS: Thirteen patients had a personal history of NP without asthma, aspirin intolerance, Churg Strauss syndrome, cystic fibrosis, Young's syndrome, bare lymphocyte syndrome, or primary ciliary dyskinesia. Within this family, 19.7% of those older than 17 years were affected by NP, as compared with the national French prevalence of 2.1%. CONCLUSIONS: Regarding the pedigree, we discuss different modes of inheritance. The presence of consanguineous unions in this family suggests the possibility of a common ancestor and thus a recessive autosomal mode of inheritance.

Rhinocerebral mucormycosis in a boy with recurrent acute lymphoblastic leukemia: long-term survival with systemic antifungal treatment.

Rhinocerebral mucormycosis is rare in hematologic malignancies and usually leads to death within weeks. In contrast, chronic rhinocerebral mucormycosis takes a slowly progressive course and has not been reported in hematologic malignancies in children so far. The authors report the long-term survival of a boy with rhinocerebral mucormycosis in a relapse of acute lymphoblastic leukemia after allogeneic cord blood transplantation. The disease started acutely but took a chronic course thereafter. No surgical debridement was performed because of extensive involvement of the sinuses, orbits, and cerebrum. His long-term survival of 15 months is attributed to the long-range administration of liposomal amphotericin B, early neutrophil recovery, and slow progression of the relapsing acute lymphoblastic leukemia.

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.

[Complications with permanent damage in endonasal paranasal sinus operations--more frequent in experienced surgeons?]. / Komplikationen mit bleibenden Schäden bei endonasalen Nasennebenhöhlenoperationen--häufiger bei erfahrenen Operateuren?

BACKGROUND: The rate of serious complications in endonasal sinus surgery has not gone down although optical aids are widely used nowadays. Are serious complications caused more often by unexperienced or experienced surgeons using a microscope and/or endoscope? METHODS: We defined serious complications as follows: death, persistent neurological deficits or permanent loss of vision, and injury to the internal carotid artery. Two different studies were made: the first consecutive 300 interventions of 6 sinus surgeons were evaluated. Sixteen malpractice cases were analysed regarding the experience of the surgeon. RESULTS: In 9 out of 16 malpractice cases serious complications were attributable to experienced surgeons, five to moderately experienced surgeons, and only two to an inexperienced surgeon (although he had extensive experience in external sinus surgery). There were 6 deaths, 6 neurologic defects, 2 visual disorders, and 2 injuries to the internal carotid artery without any sequelae. In 9 cases the serious complications were related to injury of the internal carotid artery, in five cases to perforation of the skull base. Twice the orbital wall was penetrated. In 1800 procedures performed by 6 surgeons, no serious complications were encountered. There were only lesions of the periorbit (n = 33) or CSF leaks (n = 8) without any permanent damage to the patient. CONCLUSIONS: Even an experienced surgeon must always keep in mind that serious complications can occur in sinus surgery. One must constantly be alert to the possibility of anatomical variants or specific pathologic findings.

Craniofacial zygomycosis caused by Apophysomyces elegans.

A fatal case of craniofacial zygomycosis caused by Apophysomyces elegans in a 52-year-old man was diagnosed by the presence of broad aseptate, branched hyaline hyphae in tissue from paranasal sinuses and surrounding areas, and isolation of the fungus from the same tissue. The patient suffered from idiopathic myelofibrosis as underlying disease, he was thrombocytopenic and was mildly hyperglycaemic. The infection represents the second case of craniofacial zygomycosis due to A. elegans.

Patient survival factors in paranasal sinus mucormycosis.

Analysis of 170 cases of paranasal sinus mucormycosis collected from the literature and 9 cases of our own revealed a 50% mortality for this disease. When analyzed according to decade, survival has increased to 70% in the cases reported from 1970-1979. There were no significant differences between the survivors and the fatalities when evaluated according to age, sex, laterality, or radiographic findings. There was a markedly poorer prognosis for those patients with hemiplegia, facial necrosis, and nasal deformity. The underlying disease was an important determinant of survival: 75% of patients with no systemic disease, 60% of diabetics, and 20% of patients with other disorders survived. Surgical debridement or radical resection and the use of amphotericin B significantly increased survival. Their combination further enhanced survival, especially in the diabetic.