endoscopic surgical treatment of rhino-sinusal mucormycosis

Conditions: Rhinocerebral Mucormycosis

Summary: radical endoscopic transnasal surgery associated with medical treatment with liposomal amphotericine b may increase the local control of rhino-sinusal mucormycosis and the survival rate. the objective of this study is to evaluate the local control rate and survival rate at 3 months after radical endoscopic transnasal surgery extended towards the skull base in association with antifungal therapy and early surgical reevaluation of the extent of the disease.

introduction : treatment of rhino-sinusal mucormycosis remains a challenge because of the severity of the disease with high mortality rates. mainly involving diabetic and immunodeficient patients, first clinical presentation is a common infection of the sinuses which can extend towards deep spaces of the face, orbits, the skull base and the brain. the mortality rates range from 20 to 50%, up to 80% in case of cerebral extension. through transnasal endoscopic surgery extended to the skull base. hypothesis : we hypothesize that infected tissues are devascularized and because of that antifungal therapies can hardly reach areas of infected tissues. radical endoscopic transnasal surgery associated with medical treatment with liposomal amphotericine b may increase the local control of the disease and the survival rate. clinical evaluation of the extent of the disease within the first 7 days following initial surgery may represent a prognosis factor. methods : first national mutlicentric and multidisciplinary cohort on rhino-sinusal mucormycosis radiological staging for evaluation of the extent of the disease using ct scan, mri and pet scan at initial stage before surgery radical surgery and/or endoscopic transnasal surgery extended towards the skull base associated with liposomal amphotericine b medical treatment. at day 7 new radiological evaluation and second surgical look to adapt surgical resection of infected tissues, perform biopsies to search for mycormycosis, biofilms and dosage of the concentration of liposomal amphotericin b inside tissues. new radiological and surgical looks in case of absence of local control obtained on the second look and further. study of the response rate by an endoscopic \& scan follow-up (with biopsies for anatomopathological et mycological studies) at 1 month, 3 months, 6 months \& one year. pet scan initially \& at 3 months. number of subjects included : phase ii type trial (exclusion of a zero hypothesis of survival rate \<50% ), n= 23 patients total length of the study 4 years inclusion period time : 3 years time of participation per patient : 1 year number of center and/or participating departments: 24 mean number of patients included per year and per center : 0 to 3 patients

Eligibility criteria:
inclusion criteria: * man or woman, over 18 years old, presenting a mucormycosis on a sinus biopsy showing large filaments with non or little septae compatible with a mucoral or with a positive culture for a mucoral on a sinus sample associated to clinical head \& neack anomalies (endoscopic) \& scans compatible with a mucormycosis previous to the inclusion and this whatever the patient incumbent pathology. * patient treated by liposomal amphotericin b or just before being treated * signature of informed consent : * by the patient if he is able to express their will * by the family or close, if the patient is unable to consent * inclusion in emergency clause is possible ( cpp agreement ) if the patient is incapacitated , no close is present and that the surgery is urgent * person affiliated to a health security system (beneficiary) exclusion criteria: * pregnancy, breastfeeding * disseminated mucormycosis (involvment of one site distant from the head and neck) * known hypersensitivity to a polyene * absence of documentation of mucormycosis (histological, mycological) * contraindication to the completion of the surgery as provided in this protocol * patient is the subject of a guardianship or tutelage measure

Keywords: rhinocerebral mucormycosis surgery fungal infection survival