1 Case Presentation

A 39-year-old male patient with a medical history of autologous bone marrow transplantation 2 months ago due to relapse of a B-cell Hodgkin’s Lymphoma, was admitted to Haematology Department with symptoms of worsening nasal congestion, purulent nasal discharge, persistent high fever, midfacial pain, and hard palate numbness. An urgent ENT assessment was requested. Nasal endoscopy revealed oedematous nasal mucosa, purulent nasal discharge, and black crust formation inside the right nostril. Multiple swabs, along with tissue samples, were collected for microbiological cultures and histopathological examination. Intraoral examination revealed a black ulcer with irregular margins over the right side of the hard palate, approximately 2 × 2 cm2 in dimensions. Cranial nerve function was unremarkable. An urgent contrast-enhanced CT scan of the head and paranasal sinuses was performed, showing right maxillary sinus opacification with bone erosion (Fig. 5.1). Laboratory studies revealed elevated ESR and neutropenia. Blood cultures were also obtained. An urgent sinus MRI (Figs. 5.2 and 5.3) was performed, and the patient was taken to theatre for surgical debriment of the necrotic tissue, middle meatal antrostomies and biopsies under general anesthesia. A high dose of Amphotericin B was administered intravenously. The suspected diagnosis of mucormycosis, a subtype of Acute Invasive Fungal Sinusitis was confirmed.

Fig. 5.1
figure 1

CT scan of the paranasal sinuses

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Fig. 5.2
figure 2

T2-weighted MRI

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Fig. 5.3
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T1-weighted MRI

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2 Background Knowledge

Acute Invasive Fungal Rhinosinusitis is a rare and extremely aggressive disease with high morbidity and mortality (50–80%). It is related in the vast majority of cases with immunosuppression and especially malignancy, chemotherapy, uncontrolled diabetes, autoimmune disorders, and organ transplantation. There are several saprophytic fungi associated with acute invasive fungal rhinosinusitis. These fungi are inhaled and deposited in the airway, causing local or generalized inflammation to the immunocompromised patients. The most common are Mucor, Rhizopus, Rhizomucor, Absidia, Mortierella, Apophysomyces species and Aspergillus fumigatus, which is also related to the chronic form of invasive fungal sinusitis. Histopathological studies demonstrate mucosal invasion, vasculitis, arterial and venous thrombosis, and eventually, tissue necrosis. As it represents a potentially fatal and rapidly evolving disease, early diagnosis is of great importance.

3 Clinical Approach

3.1 Diagnosis

The clinician should be aware of the disease and its clinical manifestations in cases of immunosuppressed or immunocompromised patients, presenting with persistent high fever, severe headache and symptoms of rhinosinusitis. The clinical approach involves a thorough evaluation of the nasal cavities and paranasal sinuses, oral and pharyngeal mucosa, including endoscopy. Dark ulcers with eschar formation and swollen mucosa on the septum, turbinates and palate, accompanied by nasal discharge and severe headache, are pathognomonic of the disease. Microbiology samples for bacteria and fungi should be taken. Tissue samples for culture and histopathologic examination should also be collected with great care because of the risk of bleeding, especially in patients with low platelet count. Cranial nerve function should be assessed because of the risk of intracranial invasion. Signs of ophthalmoplegia and/or exophthalmos with decreased pupillary responses indicate extension beyond paranasal sinuses to the orbit. Signs of cavernous sinus thrombosis are indicative of extensive disease. Urgent brain—sinus contrast CT scan should be performed. Bone erosions, soft tissue edema, mucosa thickening and vessel invasion may be noticed. MRI is more useful when intracranial, intraorbital or extension to the adjacent tissues is suspected.

3.2 Treatment

Once a rapidly evolving and possibly fatal disease, treatment should also be aggressive and effective. Urgent surgical resection with tissue debriment and complete disease removal, with simultaneous treatment of the underlying causes such as uncontrolled diabetes, neutropenia and immune system deficiency. In cases of early diagnosis with the disease isolated to the nasal cavity, without adjacent tissue invasions, surgical approach is restricted to extended endoscopic sinus surgery procedures (ethmoidectomy, medial maxillectomy, etc.). Sometimes extensive surgical procedures are required, such as transoral maxillectomy and orbital exenteration. Preoperative platelet transfusion should be considered in patients with low platelet count because of the risk of bleeding. High doses of Amphotericin B or Lipid Formulation of Amphotericin B should be administered intravenously as soon as the diagnosis of invasive fungal sinusitis is suspected.

3.3 Follow-up

As already mentioned, acute invasive fungal rhinosinusitis is related to high morbidity and mortality rates. Antifungal medication should be continued for an extended period after surgery. Those who remain disease-free require close monitoring due to the possibility of recurrence of the disease. Several specialists may be involved in the follow-up, such as ENT and Oral&Maxillofacial surgeons due to possible facial deformities after extensive surgeries, Hematologists in cases of hematologic malignancies, Clinical Oncologists, Immunologists, Endocrinologists in cases of diabetes etc.

Summary and Author’s Comments

  1. 1.

    Acute Invasive Fungal Rhinosinusitis is a rare, extremely aggressive disease with high morbidity and mortality rates (50–80%).

  2. 2.

    Clinicians should be aware of the disease in cases of immunocompromised patients, presenting with persistent high fever, rhinosinusitis symptoms with nasal discharge, severe headache, black crust—eschar formation in the nasal cavity.

  3. 3.

    Intraorbital extension, opthalmoplegia, cavernous sinus thrombosis and cranial nerve involvement are signs of extensive disease.

  4. 4.

    Urgent wide surgical resection combined with high doses of antifungal medication and treatment of underlying conditions is the treatment of choice.