Rhinology Fungal Disease Flashcards

1
Q

Describe the cause of allergic fungal sinusitis.

A

A noninvasive fungal sinusitis arising from an allergic
response (type I hypersensitivity) to sinonasal fungal
exposure

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2
Q

Describe the criteria presented by Bent and Kuhn
(Otolaryngology HNS, 1994) for the diagnosis of
allergic fungal rhinosinusitis.

A

Bent and Kuhn allergic fungal rhinosinusitis criteria:
● Type I hypersensitivity to mold allergens (history or
formal allergy testing)
● Eosinophilic mucin with Charcot-Leyden crystals
● Fungal hyphae without invasion into soft tissue
● NP
Characteristic imaging
● CT: Hyperdense central mucin surrounded by a rim of

hypointensity with speckled areas of increased attenu-
ation resulting from ferromagnetic fungal elements.

Unilateral > bilateral. May have bony expansion of the
paranasal sinuses
● MRI: T1 and T2 show central hypointensity surrounded by
hyperintensity and T2 may show a central void.

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3
Q

What are the characteristics of eosinophilic mucin?

A

Necrotic inflammatory cells
● Eosinophils
● Charcot-Leyden crystals
● Fungal hyphae

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4
Q

What fungi are commonly implicated in allergic

fungal rhinosinusitis?

A

Alternaria, Aspergillus, Bipolaris, Curvularia, Cladosporium,
and Dreschlera

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5
Q

When evaluating a patient with NP, eosinophilic
mucin on examination, and a history of atopy to
inhaled mold allergens, what comorbid condition
must also be investigated?

A

Asthma

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6
Q

During the workup for allergic fungal rhinosinusitis,
what procedures and/or diagnostic tests are
recommended?

A

● Allergy testing for fungi-specific IgE (skin or blood tests)
● Endoscopy for assessment and procurement of mucin
specimen
● Pathologic analysis of eosinophilic mucin for fungal stains
and possible culture
● May consider total serum IgE
● Strongly consider CT without contrast

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7
Q

What term was proposed by Ponikau (Mayo
Clinic Proceedings, 1999) to describe patients
with CRS and fungal hyphae in eosinophilic mucin
but no evidence of type I hypersensitivity
reactions on allergy testing?

A

Eosinophilic fungal rhinosinusitis

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8
Q

What is the recommended treatment for allergic

fungal rhinosinusitis?

A

● Endoscopic surgery
● Possibly systemic or topical antifungals
● Consider systemic or topical steroids
● Nasal saline irrigations

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9
Q

What are the two categories of fungal

rhinosinusitis?

A

● Invasive

● Noninvasive

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10
Q

Name the subtypes of noninvasive fungal

rhinosinusitis.

A

● Fungus ball (old terms no longer recommended: myce-
toma, aspergilloma)
● Allergic fungal rhinosinusitis
● Saprophytic fungal infestation

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11
Q

Fungus balls most frequently form in an isolated
paranasal sinus as a mass of fungal hyphae with
associated inflammatory debris and no evidence
of mucosal invasion in immunocompetent patients
and are found incidentally or manifest with
associated symptoms. What is the most common
fungus that is isolated?

A

Aspergillus fumigatus

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12
Q

Define the distribution of paranasal sinus fungus

balls.

A

Maxillary > sphenoid > ethmoid > frontal sinuses

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13
Q

Paranasal fungus balls exhibit what imaging

characteristics?

A

● Complete or subtotal opacification, usually of a single
sinus
● Osteal thickening or sclerosis
● Noncontrast CT shows hyperattenuating lesion with
punctate calcifications. The fungus ball is hypointense on
T1-weighted and T2-weighted images owing to the
absence of free water.
● Calcifications and paramagnetic metals generate areas of
signal void on T2-weighted images.

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14
Q

How are paranasal sinus fungus balls treated?

A

Surgical debridement and postoperative irrigations

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15
Q

What type of noninvasive fungal rhinosinusitis
often has an asymptomatic or foul-smelling fungal
colonization of mucous crusts after previous sinus
surgery?

A

Saprophytic fungal colonization

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16
Q

What are the three types of invasive fungal

rhinosinusitis?

A

● Acute invasive
● Chronic invasive
● Chronic granulomatous

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17
Q

An immunocompromised patient has
sudden-onset and rapidly progressive periorbital
or facial swelling and/or ophthalmoplegia, neutro-
penic fever, and a nonpainful intranasal ulcer or

eschar most likely has developed what disease
process?

A

Acute invasive fungal rhinosinusitis (old terms that are no

longer recommended: fulminant, necrotizing)

18
Q

True or false. Acute invasive fungal rhinosinusitis is
defined as an invasive fungal infection that can
take up to 4 weeks to develop.

A

True

19
Q

What patient populations are at highest risk for
development of acute invasive fungal
rhinosinusitis?

A
Immunocompromised:
● Hematologic malignancy
● Hematopoietic stem cell transplant
● Diabetes mellitus
● Advanced HIV
● Immunodeficiency
● Chemotherapy induced neutropenia
● Solid-organ transplant
● Corticosteroids
20
Q

What are the most common initial symptoms
associated with acute invasive fungal
rhinosinusitis?

A

Fever, facial pain,* nasal congestion, decreased sensation
over malar regions,* and epistaxis, which may develop
change in vision and mentation
*Important complaint that warrants attention.

21
Q

What are the most common physical examination
findings suggestive of acute invasive fungal
sinusitis?

A

Early: Pale, boggy mucosa, petechiae, or areas of ischemia
Late: Black eschar, sloughing mucosa, gross hyphae,
decreased sensation. Mucosa does not bleed.

22
Q

What areas of the sinonasal cavities most
commonly manifest acute invasive fungal
rhinosinusitis?

A

The middle turbinate is reported to be the most common,
followed by the septum and inferior turbinate. The palate
and oral cavity must also be evaluated.

23
Q

What findings on CT scan would be suggestive of

invasive fungal sinusitis?

A

● Unilateral sinus involvement (more common than bilat-
eral)
● Severe soft tissue edema of the nasal cavity mucosa
(turbinates, lateral nasal wall and floor, septum)
● Paranasal sinus mucoperiosteal thickening
● Bone erosion
● Facial soft tissue swelling
● Retroantral fat pad thickening (CT or MRI)
● Orbital invasion
● Intracranial invasion

24
Q

When should an MRI be ordered during the

workup for invasive fungal sinusitis?

A

If a patient is symptomatic but the CT and/or examination
are equivocal or to evaluate the extent of intracranial or
intraorbital invasion as suggested by CT or examination

25
Q

What two fungal organisms are most commonly

involved in acute invasive fungal rhinosinusitis?

A

● Mucorales

● Aspergillus

26
Q

Which fungal organisms have nonseptate
(aseptate) twisted hyphae that branch at 90-degree
angles, are seen in a necrotic background, and often demonstrate angioinvasion,
which most commonly occurs in diabetic ketoacidosis?

A

Mucormycoses (order: Mucorales)

27
Q

What are the most common Mucorales species
associated with acute invasive fungal
rhinosinusitis, and where are they found in the
environment?

A
● Mucor
● Rhizopus
● Absidia
● Cunninghamella
● Rhizomucor
● Mortierella
● Saksenaea
● Apophysomycoses
● Zygomycoses
Found in the soil or associated with decaying organic
matter such as leaves, wood, or compost
28
Q

What fungal organisms are known to have septate
hyphae that branch at 45-degree angles and are
best seen by using methenamine silver stain?

A

Aspergillus spp.

29
Q

What are the keys to effective management of
acute invasive fungal rhinosinusitis, which carries
a very poor overall prognosis?

A

● Rapid diagnosis and intervention!
● IV antifungal medications
● Aggressive surgical resection
● Reverse underlying immune dysfunction
If a patient is cured and lifelong immunosuppression is
required, then lifelong oral suppressive antifungal therapy
can be used.

30
Q

What IV antifungal medications should be
considered empirically in the early management
of acute invasive fungal rhinosinusitis?

A

IV amphotericin B (drug of choice for mucormycosis) + /-

voriconazole (drug of choice for Aspergillus)

31
Q

What type of rhinosinusitis results from invasion
by fungal elements and tissue destruction over a
period of > 12 weeks?

A

Chronic invasive fungal rhinosinusitis

32
Q

What is the most common initial manifestation
in patients with chronic invasive fungal
rhinosinusitis?

A

Symptoms suggestive of CRS with few, if any, systemic

complaints

33
Q

What patient populations are most at risk for
development of chronic invasive fungal
rhinosinusitis?

A
● Mildly immunocompromised
● Elderly
● Diabetes mellitus
● Glucocorticoid use
● AIDS
34
Q

True or False. Patients with chronic invasive
fungal rhinosinusitis do not develop the severe
complications associated with acute infection.

A

False. They can develop orbital and intracranial invasion.

Prognosis is poor.

35
Q

What are the most common CT findings
associated with chronic invasive fungal
rhinosinusitis?

A

Thickened mucosa associated with bony erosions and a
mass lesion in a single paranasal sinus (most common in the
sphenoid or ethmoid)

36
Q

What are the findings on histopathology that

suggest chronic invasive fungal rhinosinusitis?

A

● Dense accumulation of hyphae
● + /- Vascular invasion
● Little to no inflammatory reaction to invasion and
destruction of local structures

37
Q

What is the most common fungal species
associated with chronic invasive fungal
rhinosinusitis?

A

Aspergillus fumigatus

38
Q

What is the recommended treatment for chronic

invasive fungal rhinosinusitis?

A

Rapid diagnosis and intervention:
● IV antifungal medications
● Aggressive surgical resection
● Reverse underlying immune dysfunction

39
Q

What form of invasive fungal rhinosinusitis is
diagnosed after 12 weeks’ duration, most
commonly in immunocompetent patients from
Sudan, India, Pakistan, or Saudi Arabia?

A

Chronic granulomatous invasive fungal rhinosinusitis

40
Q

What is the most common manifestation seen
in patients with chronic granulomatous invasive
fungal rhinosinusitis?

A

● Enlarging mass (cheek, orbit, nose, paranasal sinus)

● Gradual onset of symptoms

41
Q

What are the histopathologic findings most
commonly seen in chronic granulomatous invasive
fungal rhinosinusitis?

A

● Significant fibrosis
● Noncaseating granulomas
● Foreign body–type reaction
● Langherhans type multinucleated giant cells
● Possible vasculitis, vascular proliferation, perivascular
fibrosis

42
Q

What fungal species is most often implicated in
chronic granulomatous invasive fungal
rhinosinusitis?

Aspergillus flavus

340 What is the recommended treatment for chronic
granulomatous invasive fungal rhinosinusitis?

A

● Surgical biopsy and debridement

● Systemic antifungals