1. Allergic Fungal Rhinosinusitis: A Case Report
Maha Yousif, MS-3, Melissa Smith-Phillips, MD, PhD
University of Texas Medical Branch, Galveston, TX
Abstract
Allergic fungal rhinosinusitis (ARFS) is a unique noninvasive fungal sinusitis, that is defined by the presence of allergic mucin. This diagnosis is easily missed by many clinicians due to its vague presentation, and similarity to allergic rhinitis or sinusitis. Prompt diagnosis and early treatment helps prevent long term sequelae of the disease including: visual changes, cavernous venous thrombosis, and expansion of allergic mucin into the brain3. Despite being around since the late 1970’s, this condition continues to go under-diagnosed. This case describes a classic presentation of this condition in a 17-year-old male.
Case Presentation cont.
MRI was obtained and demonstrated: Extensive fungal allergic sinusitis and sinonasal polyposis with focal breakthrough into the anterior left cranial fossa without significant dural or neuro axis involvement.
Allergic fungal rhinosinusitis is a relatively new diagnosis, being first recognized in It is a unique noninvasive fungal sinusitis, defined by the presence of allergic mucin. Although difficult to recognize, the diagnosis is made primarily with a thorough clinical history. The current accepted criteria for diagnosis is the Bent and Kuhn Diagnostic Criteria. Patients must meet all the major criteria which include: history of Type I hypersensitivity, nasal polyposis, characteristic CT findings (unilateral or asymmetric involvement of the sinuses, heterogeneous signal intensity, bony erosion and expansion of the fungal mucin), eosinophilic mucin without invasion, and positive fungal stain1. The minor criteria are: asthma, unilateral disease, bone erosion, fungal cultures, Charcot-Leyden crystals, and serum eosiniophilia1. Also the clinical history demonstrates sinus disease intractable to traditional medical and even surgical treatment, and treatment with several courses of antibiotics and topical nasal preparations without success. Some unique characteristics include: young (mean age is 22 years), immunocompetent patients with unilateral or asymmetric involvement of the paranasal sinuses, a history of atopy, nasal casts, and polyposis, and a lack of significant pain1. The current treatment of AFRS includes: surgery, oral corticosteroids, and immunotherapy1. Due to the obstructive nature of this disease, potential complications include compromise of the sinus walls leading to: destruction of middle ear, cerebrovascular accidents, intracranial invasion, loss of vision, diplopia, and ophthalmoplegia3.
Introduction
Based on MRI and CT findings, ENT performed a Functional Endoscopic Sinus Surgery with bilateral maxillary antrostomies, sphenoidotomy, and fornal sinusotomy, and total Ethmoidectomy, with tissue removal.
The findings of the procedure were: Complete nasal obstruction secondary to nasal polyps, middle turbinates were medially displaced. Severe allergic mucin involving all sinuses requiring extensive sinus debridement.
Surgical pathology showed that the mass excised consisted of an allergic polyp, and was positive for the fungal species Aspergillus.
Outcome
At time of discharge, patient denied any headaches, and eye discharge. Periorbital swelling had decreased, but residual proptosis remains.
The patient was discharged home on Montelukast, Oxymetazoline, and voriconazole with follow up with ENT, Infectious disease, and Allergy
Patient continued on voriconazole for 6 weeks due to the positive fungal stains
Allergy testing determined patient was allergic to drechslera/curvularia and fusarium.
Patient has had to have a debridement once since being discharged due to polypoid recurrence
Initial presentation
17 year old African American, previously healthy, presented to urgent care for a 6 month history of progressive bilateral eye swelling, lateral eye deviation, and 3 day history of red eyes and clear eye discharge. He was also experiencing brief frontal headaches that were sharp and stabbing in quality that radiated periorbitally and were relieved with Advil and were associated with , with intermittent blindness.
At the onset of symptoms, he had seen his PCP who prescribed Allegra, which patient took for a month, but stopped due to drowsiness side effect. Mother states that over time, he started to “look like a chameleon”
On admission, his pertinent physical exam findings were:
TTP to medial epicanthi bilaterally, conjunctiva injected, eyelids swollen, white purulent drainage/exudate present, Left eye orbit vertically deviated up. Increased interpupillary distance. Periorbtal edema bilaterally,
Green mucous and exudate obstructing nasal passage, nasal turbinates unable to be visualized, patient unable to clear nasal passageway upon asking to 'blow nose’, 1 lymph node <1cm right submandibular
Workup
CT Head and sinuses were obtained and demonstrated: Heterogeneously dense expansile content throughout the paranasal sinuses causing bilateral proptosis and intracranial extension along the left anterior cranial cavity. (arrow)
Case Presentation
Discussion
ARFS is a relatively rare condition, however, with an estimate of 5-10% of  those with chronic sinusitis carrying this diagnosis2, it is important to keep this diagnosis in mind, particularly with patients who do not respond as expected to treatment. Clinicians should be aware of the difference in presentation between children and adults, with children tending to have more facial dysmorphism, typically in the form of proptosis, and increased prevalence of bony erosion into adjacent cavities and intra cranial expansion. Early diagnosis and treatment may minimize the risk of: visual changes, cavernous venous thrombosis, and expansion of allergic mucin into the brain.
References
Glass, Daniel, MD, and Ronald G. Amedee, MD. "Allergic Fungal Rhinosinusitis: A Review." The Ochsner Journal 11.3 (2011): Web.
Mcclay, John E., Brad Marple, Lav Kapadia, Michael J. Biavati, Brian Nussenbaum, Mark Newcomer, Scott Manning, Tim Booth, and Nathan Schwade. "Clinical Presentation of Allergic Fungal Sinusitis in Children." The Laryngoscope (2002):
Bozeman, Sarah, Richard Deshazo, Scott Stringer, and Leigh Wright. "Complications of Allergic Fungal Sinusitis." The American Journal of Medicine (2011):
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