Corresponding author:
Serdar Baylançiçek, M.D.
Department of Otorhinolaryngology, School of Medicine,
Marmara University Hospital, Altunizade, Istanbul, Türkiye e-mail: serdarbay@isnet.net.tr
Marmara Medical Journal 2005;18(3);151-152 PHOTO QUIZ
PHOTO QUIZ
Serdar Baylançiçek, Murat Sarı, Alper Tutkun
Kulak Burun Boğaz Anabilim Dalı, Tıp Fakültesi, Marmara Universitesi, İstanbul, Türkiye
A 17-year-old female patient presented with headache and swelling of the left cheek over a one-year period. She also complained of recent onset facial tingling, and rare attacks of seeing lightning flashes. She denied any nasal obstruction, and nasal or postnasal discharge. There was no family history of bone disease, genetic abnormalities, or endocrinopathies. Otorhinolaryngologic examination revealed retraction of the left tympanic membrane and minimal left infraorbital swelling. Her neurologic and ophthalmologic examinations were unremarkable. The routine laboratory tests including serum calcium, phosphorus,
and alkaline phosphatase were within normal limits. The patient was evaluated with Water’s graphy and computerized tomography of the paranasal sinuses, revealing an undefined “mass” within the left maxillary sinus, with extention into the adjacent ethmoid cells and sphenoid sinus. The patient underwent radical antrostomy, with debulking of the fibroosseos mass with a hard mud-like consistency filling the left maxillary, ethmoid, and sphenoid sinuses. Water’s graphy, and axial computed tomography section at the level of the inferior turbinates are shown below. What is the most probable diagnosis?
Marmara Medical Journal 2005;18(3);151-152 Serdar Baylançiçek, et al
photo quiz
ANSWER TO PHOTO QUIZ
The diagnosis is “fibrous dysplasia” (FD). FD is a benign, localized bone disorder of unknown etiology. Pathologically, the disease is characterized by a slowly progressive replacement of normal medullary bone by fibrotic tissue and disorganized bony trabeculae1. The bony cortex may be thinned, but remains intact, maintaining a smooth contour2.
Because of the slow progression of FD, it may take years to become symptomatic1. The presenting findings are ptosis, exophthalmos, diminution of visual acuity, and cosmetic deformity1-3. The disorder occurs during childhood and becomes inactive when skeletal growth is completed1. The craniofacial skeleton is a common site of occurrence.
The disease has three different variants1-5: type 1, characterized by single or multiple lesions in the same bone (monostotic); type 2, characterized by multiple lesions involving two or more bones (polyostotic); and type 3, characterized by dissemination of pathological bone changes connected to Albright’s syndrome (precocious puberty, cutaneous pigmented patches over the involved bones). Monostotic FD accounts for 70% of cases and tends to become evident in late childhood. Polyostotic FD accounts for about 30% of cases and usually becomes evident earlier as a result of multiple bone involvement.
Craniofacial bones are affected 10-30% in monostotic form, and 50-100% in polyostotic form depending on the severity. Craniofacial FD is most frequently located in the mandible and maxilla. The paranasal sinuses are rarely involved. With craniofacial involvement, symptoms relate to bony overgrowth1.
Diagnosis mainly depends on the radiographic findings. Craniofacial FD has been classified into 3 radiographic patterns: pagetoid, sclerotic, and cystlike1,5,6. The pagetoid form (56%) is with remarkable bone expansion and
alternating zones of radiodensity and radiolucency. This type more freqently appears in patients over 30 years of age. The sclerotic type (23%) demonstrates a homogeneous density, again accompanied by bone expansion, whereas the cyst like pattern (21%) is usually a single, well-defined radiolucent lesion, oval in shape, edged by a thin, sclerotic margin clinically resembling an eggshell. Both are frequently seen in children or younger adults who have had symptoms for less than 3 years.
CT provides greater detail of the extent of FD than does conventional radiography. CT findings are pathognomonic, and also aid in monitoring the progress of the disease. On CT, FD has a characteristic homogeneous ground-glass appearance1.
Treatment is recommended in symptomatic cases, and the only treatment modality is surgical debulking. As FD is likely to become quiescent, surgical intervention should be as conservative as possible, and should be guided by the radiographic findings. The patient should be informed of the unpredictability of FD and the tendecy for recurrence. Prognosis depends on the progression of the disease and is good in nearly all cases. Use of periodic CT can be helpful to follow the progression of the disease.
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4. Morrissey DD, Talbot JM, Schleuning AJ 2nd. Fibrous
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