Rhino-cerebral mucormycosis in a paediatric patient affected by acute lymphoblastic leukemia: a case report

Rhino-cerebral mucormycosis in a paediatric patient affected by acute lymphoblastic leukemia: a case report. Aim. Mucormycosis, or zygomycosis, is an uncommon disease caused by fungi of the order Mucorales. It consists of an opportunistic infection, which affects patients with severe immune deficiencies or uncontrolled metabolic disorders, mainly diabetes mellitus. The hyphae penetrate through the oral and nasal mucosae and subsequently invade the blood vessels, thus causing thrombosis, ischemia and necrosis of the hard and soft tissues. This work was aimed at describing a case of rhino-cerebral mucormycosis occurred in a paediatric patient affected by acute lymphoblastic leukemia (ALL).Methods. A 12-year-old female patient affected by ALL, treated according to the Italian Association of Paediatric Hematologic Oncology protocol (2009), referred to the Complex Operating Unit of Odontostomatology with 10-15 days severe facial pain and swelling in correspondence of the left cheek, fever, yellow-greenish discharge from the nose and the mouth, teeth loss and mobility, headache, epileptic seizures and a slight esophtalmo. Clinical oral examination showed: rapid expansion of hard hemipalate and maxillary alveolar process with multiple oral ulcers, bone exposure, fistulas and a sinusal defects through which intrasinusal necrotic material was detected. Ortopantomography (OPT) and Computed Tomography (CT) with 3D reconstruction revealed extensive destruction of the involved maxillary sinus with necrosis extended to the nasal cavity, left ethmoidal cells, orbital floor and maxillary alveolar process; MRI showed multiple hyperintense lesions in the frontal, occipital and temporal encephalic regions, suggesting a cerebral abscess. Rhinoendoscopy revealed greenish discharge, which resulted negative for fungi at microbiological examination, and a pale, oedematous and necrotic mucosa, which was removed and sent for histopathological examination, which confirmed the diagnosis of mucormycosis. The patients were accordingly treated with intravenous L-Amphotericin B therapy at a daily dosage of 7,5 mg/Kg for two weeks before surgery which resulted in complete regressions of all encephalic lesions, thus avoiding the necessity of cerebral abscess drainage. The persisting disease and the increasing soft tissue involvement called for surgery. Using upper vestibular and subciliary incisions, a maxillary resection extending into the ipsilateral ethmoidal and orbital regions was performed, with removal of the orbital walls and ethmoidal cells. Osteotomies were carried out with piezoelectrical tools. Due to the removal of a large of the orbital floor and the paediatric age of the patient, the inferior and medial orbital walls were reconstructed using poli-p-dioxanon (PDS) foil. The postsurgical medical treatment consisted of intravenous L-amphotericin B therapy in a daily dosage of 3mg/ Kg until hospital resignation, followed by systemic oral therapy with Posaconazole at the dosage of 200 mg four times daily. Local medical therapy was carried out by using puffs of iodine solution through the orosinusal defect, once a day for one month. Results. Medical therapies, both local and systemic, and conservative surgical treatment allow the complete healing after one year follow-up. The patient was temporary rehabilitated with an obturator prosthesis waiting for a complete reconstruction at the end of the patient development. Conclusion. Given the lethal nature of the disease, the authors underline the importance of early diagnosis and of a multidisciplinary approach in order to undertake correct surgical and medical treatments, while keeping the underlying disease under control.