Jamur Journal

Fungal rhinosinusitis refers to a broad group of conditions caused by fungal infections of the
paranasal sinuses.
Fungus Ball
In this condition, an isolated paranasal sinus is completed filled with a ball of fungal debris, most
frequently in the maxillary sinuses. Patient symptoms include fullness, pressure and discharge.
Treatment for a fungus ball requires surgery for complete removal of all fungal elements. The
prognosis is good.
Allergic Fungal Rhinosinusitis

Allergic fungal rhinosinusitis (AFRS) is characterized by it the sinus secretions, which have a
characteristic golden-yellow color and have a consistency like rubber cement. These secretions
contain proteins from degranulated eosinophils (a type of inflammatory cell) plus some fungal
elements. Patients often will have received multiple treatments (including steroids) for chronic
rhinosinusitis before the diagnosis of AFRS is confirmed. Many AFRS patients also have asthma.
Endoscopic sinus surgery is required for diagnosis and mechanical cleansing of the sinuses, but
surgery must be combined with long-term medical management. Medical sinus infection treatments
include systemic and topical corticosteroids and antifungals as well as antibiotics for bacterial
infection.
Acute Fulminant Fungal Rhinosinusitis

Acute fulminant invasive rhinosinusitis (also know as rhinocerebral mucormycosis or simply “mucor”)
occurs when fungal organisms invade the sinus tissues in patients who are immunosuppressed.
Classically, these patients have suppressed immune systems due to chemotherapy administered for
cancer treatment, or they have diabetes which leads to immune suppression. In the early stages,
patients have an area of necrotic tissue (i.e., dead tissue due to invasion by the fungus) within the
sinuses, but within hours, it can rapidly progress to eye and brain involvement. Prognosis is poor.
Emergency surgery is necessary to confirm the diagnosis and to mechanically remove all dead
tissue. Systemic antifungal treatment is also provided. If possible, the efforts to reverse the
underlying immune suppression should be initiated.
Chronic Invasive Fungal Rhinosinusitis

In chronic invasive fungal rhinosinusitis, the process of invasion of the sinus tissues occurs over a
period of weeks or months, rather than hours. Many patients with this relatively rare condition have
subtle abnormalities in their immune system due to diabetes or chronic steroid use. Patients can
present with eye swelling and blindness. Urgent surgery is necessary to confirm the diagnosis and to
remove all involved tissues. Again, systemic antifungal treatments are also critically important.
Granulomatous Fungal Rhinosinusitis

The onset of granulomatous fungal rhinosinusitis is also gradual. This condition is characterized by a
specific long-term inflammatory response, known as granulomatous inflammation to fungal
organisms that have invaded the sinus tissues. Almost all cases occur in the Sudan and neighboring
countries.
FUNGAL SINUSITIS
Devyani Lal, MD.
INTRODUCTION
Fungus is ubiquitous, present in all our surroundings and the air we inhale. Most healthy people do
not react to the presence of fungus due to a functioning immune system. However, in rare instances,
fungus may cause inflammation in the nose and the sinuses. Fungal sinusitis can come in many
forms, differing in pathology, symptoms, course, severity and the treatment required. It is broadly
classified into invasive and non-invasive types.
A simplified classification of fungal sinusitis is as follows:
A. Non-invasive fungal sinusitis
i. Fungus ball
ii. Allergic fungal sinusitis
iii. Non-allergic fungal sinusitis
B. Invasive fungal sinusitis
i. Acute invasive fungal sinusitis
ii. Chronic invasive fungal sinusitis
iii. Granulomatous invasive fungal sinusitis
NON-INVASIVE FUNGAL SINUSITIS

Fungus Ball: This is a non-invasive form of fungal sinusitis. In essence, there is an overgrowth of
fungal elements in the sinuses. Most commonly molds such as Aspergillus are responsible. The
most commonly involved sinuses are the maxillary and the sphenoid sinuses, where the fungus finds
favorable conditions such as warmth and humidity for growth. Sometimes, bacteria can cause super-
added infection in the sinus affected by the fungus ball. Typically, only a single sinus is involved, and
the disease has a classic appearance on CT or MRI scans. Treatment involves removal of the
fungus ball through endoscopic sinus surgery. Usually a peanut-butter like appearance of the fungal
ball is noted. Most patients have excellent results from surgery, and may not require any further
treatment.
Allergic Fungal Sinusitis (AFS): Allergic Fungal Sinusitis (AFS): Patients with allergy to
certain fungi may develop allergic fungal sinusitis. Common fungi belonging to the Dematiaceous
family are usually involved in AFS. These include Alternaria, Bipolaris and Curvularia species. The
presence of fungus in the sinuses causes an allergic response, resulting in production of allergic
mucin and nasal polyps. Usually, the disease affects more than one sinus on one side. However, all
sinuses on both sides may be involved in severe cases. Patients have a typical appearance on nasal
endoscopy with the presence of allergic mucin and polyps. Allergy testing to fungi is positive. Sinus
CT scans also have a typical appearance. Tissue examination under the microscope shows allergic
mucin containing fungal elements without tissue invasion. Treatment involves endoscopic sinus
surgery to clear polyps and allergic mucin, and to restore the ventilation and drainage of sinuses.
This has to be combined with aggressive medical therapy with corticosteroids which can be used
nasally and/ or systemically. Patients may also benefit from treatment of allergy
with immunotherapy (allergy shots or drops) and antihistamines. Anti-fungal treatment is usually not
required, as it is the reaction to the fungus that needs to be modulated. However, in severe recurrent
disease, anti-fungal therapy may be needed.
Non-allergic fungal sinusitis: In some instances, mucin and fungus may be identified in
patients with sinusitis in the absence of any allergy to fungus. Fungus may also be found in the
sinuses of patients that have had previous surgery. Whether these fungi are innocent bystanders or
are the cause of sinus disease is currently under investigation and a subject of great debate.
INVASIVE FUNGAL SINUSITIS

Acute Invasive Fungal Sinusitis: This is the most dangerous and life-threatening form of
fungal sinusitis. Fortunately, it is very rare, and usually only affects severely immunocompromised
patients (people whose immune systems don’t work properly). These include patients with leukemia,
aplastic anemia, uncontrolled diabetes mellitus, and hemochromatosis. Patients undergoing anti-
cancer chemotherapy or organ/ bone-marrow transplantation are especially susceptible. Aspergillus
or members of the class Zygomycetes (Mucor, Rhizopus) are the most frequent causative agents.
The disease has an aggressive course, with fungus rapidly growing through sinus tissue and bone to
extend into the surrounding areas of the brain and eye. Endoscopically, (meaning when we look with
a small scope in the nose) areas of dead tissue and eschar are noted. Microscopic examination
shows invasion of blood vessels by the fungus, causing tissue to die. Treatment involves a
combination of aggressive surgical and medical therapy. Repeated surgery may be necessary to
remove all dead tissue. Medications such as anti-fungal drugs and those that help restore the
immune status of the patient are key to improving survival, as this disease is frequently fatal.
Chronic invasive fungal sinus: Unlike acute invasive fungal sinusitis whose typical course is
less than 4 weeks (and can actually progress over hours and days), chronic invasive fungal sinusitis
is a slower destructive process. The disease causes rare vascular invasion, sparse inflammatory
reaction and limited involvement of surrounding structures. It is usually seen in patients with AIDS,
diabetes mellitus or chronic corticosteroid treatment. The disease most commonly affects
the ethmoid and sphenoid sinuses, but may involve any sinus. The typical time course of the disease
is over 3 months. Tissue cultures show fungus in over half the patients, and Aspergillus fumigatus is
the most commonly grown fungus. Treatment involves surgery in combination with medical therapy
(anti-fungal drugs and measures to restore the patient’s immune system).
Granulomatous invasive fungal sinusitis: This form of fungal sinusitis is rare in the United
States. It is usually seen in patients from Sudan, India, Pakistan and Saudi Arabia. Patients have
normal immune status. The disease has a relatively slow time course over 3 months, and patients
present with an enlarging mass in the cheek, orbit, nose, and sinuses. Microscopically, it is
characterized by formation of granulomas, and this differentiates it from chronic invasive fungal
sinusitis. Aspergillus flavus is usually the causative organism. Treatment may involve surgery in
combination with antifungal agents.
CONCLUSION
There are many forms of fungal sinusitis. A complete evaluation by your rhinologist will help to
determine if you have a form of fungal sinusitis and how it needs to be treated, as some forms of
fungal sinusitis have distinctly different medical and surgical treatments.

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Fungal rhinosinusitis refers to a broad group of conditions caused by fungal infections of the

Allergic Fungal Rhinosinusitis

Acute Fulminant Fungal Rhinosinusitis

Chronic Invasive Fungal Rhinosinusitis

Granulomatous Fungal Rhinosinusitis

A simplified classification of fungal sinusitis is as follows:

A. Non-invasive fungal sinusitis

ii. Allergic fungal sinusitis

iii. Non-allergic fungal sinusitis

B. Invasive fungal sinusitis

i. Acute invasive fungal sinusitis

ii. Chronic invasive fungal sinusitis

iii. Granulomatous invasive fungal sinusitis

NON-INVASIVE FUNGAL SINUSITIS

INVASIVE FUNGAL SINUSITIS

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