The surgical management of rhinocerebral mucormycosis

Background: Rhinocerebral mucormycosis is a rare, rapidly progressive life threatening opportunistic fungal disease that usually occurs in immunocompromised patients. Aim: The aim of the study is to present a case series of six immunocompromised patients who were diagnosed with rhinocerebral mucormycosis, review the diagnostic criteria and treatment approach. Patients and materials: Six patients were treated in our department between the years 2005-2010. Their diagnostic criteria, surgical treatment and mortality rate are analyzed and discussed. Results: All six immunocompromised patients suffered from a primary hematological malignancy and received chemotherapy to treat their primary disease. Symptoms such as pain mimicking sinusitis, facial swelling, oral or dental pain, and fever were found in most patients. The diagnosis was based on both clinical signs and a biopsy for microbiological culture and histological examination. All patients underwent aggressive surgical resection and were treated simultaneously with anti fungal therapy. Four patients died from their primary illness. One patient died due to uncontrolled spreading of mucormycosis and one patient, the youngest and with the most extensive form of the disease (brain invasion) survived and clinically recovered with no evidence of recurrent disease following the surgical management. Conclusions: Rhinocerebral mucormycosis is a rapidly progressing disease with a high mortality rate, which requires immediate surgical and medical intervention. It seems from the data presented that the presence of mucormycosis is an ominous sign in immunocompromised patients. The extent of the disease is of less prognostic value, since the only patients in our series who survived had the most extensive disease, yet his primary haemato-oncological disease was under control. Controlling the underlying disease with early diagnosis and aggressive surgical intervention appears to be the most important factor for survival.