Fibrous dysplasia of middle turbinate associated with Widal syndrome: endoscopic treatment of a rare case.
Fibrous dysplasia, a rare bony disease, is characterised by substitution of normal bone with immature tissue embedded in a fibrous stroma. It can be either monostotic or involve several bones. Fibrous dysplasia is usually asymptomatic but, in the advanced stage, pain due to neural compression or pathological fractures may occur. In the case of cranio-facial involvement, ocular, masticatory, respiratory or auditory functional alterations are possible. A case of fibrous dysplasia, limited to the middle turbinate and associated with Widal triad (sinus-nasal polyposis, asthma, acetyl salicylic acid intolerance), is described. Craniofacial computed tomography revealed enlargement of left middle turbinate with characteristic "ground-glass" appearance. The patient underwent anterior bilateral functional endoscopic sinus surgery with near-total resection of left middle turbinate. Histopathological examination confirmed the diagnosis of fibrous dysplasia. After 2 years the patient is still asymptomatic. Videorhinoscopy shows good sinus-nasal patency without disease recurrence. Even though exceptional, localization of fibrous dysplasia at middle turbinate has been described, therefore, it must be considered in the differential diagnosis of the craniofacial ossifying disorder. For localized and symptomatic lesions, endoscopic surgery is an effective option. Scrupulous life-long follow-up is necessary due to the high percentage of recurrence and possible malignant degeneration.
Saetti
R
R
Airway Endoscopic Surgery Unit, Padua University Hospital, Padua, Italy. saettirob@tiscali.it
Silvestrini
M
M
Marino
F
F
Narne
S
S
D000328:Adult; D001241:Aspirin / Q000009:adverse effects*; D001249:Asthma / Q000150:complications*; D003937:Diagnosis, Differential; D004342:Drug Hypersensitivity / Q000150:complications; D004724:Endoscopy; D005357:Fibrous Dysplasia of Bone / Q000150:complications / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000601:surgery; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D009298:Nasal Polyps / Q000150:complications*; D013577:Syndrome; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D014420:Turbinates / Q000000981:diagnostic imaging / Q000601:surgery
D001241:Aspirin