Craniofacial fibrous dysplasia: Surgery and literature review

Abstract

Objective:To highlight the clinical and radiologic features and management of craniofacial fibrous dysplasia with review of literature.Materials and Methods:A retrospective review of 6 patients who underwent surgical treatment in a tertiary healthcare centre was done using the parameters of patients' details, clinical features, radiological findings, management and postoperative review.Results:Of the six patients, 3 females and 2 males were in the 2nd decade of life and 1 male in the 1st decade of life. The disease was restricted to maxilla in 3 patients, involved the temporal and frontal bones in addition to maxilla in one, involved the frontal bone in one patient and involved frontal and parietal bones in one patient. The primary reason for seeking treatment in all the 6 cases was facial deformity. There was absence of pain in all 6 cases. For surgical treatment in all three cases involving the maxilla, the approach was intraoral while bicoronal approach was used for the other three cases. Treatment consisted of surgical contouring and reshaping the area. All cases were followed up over a period of 2 years with no signs of recurrence.Conclusion:Treatment of craniofacial fibro-osseous lesions is highly individualized. Most cases of craniofacial fibrous dysplasia manifest as swellings that cause facial deformity and surgical recontouring after cessation of growth seems to provide the best results.

Fi̇bröz Di̇splazi̇: Bi̇r Olgu Sunumu

“…5,11,12 Radyolojik olarak mineralizasyon derecesiyle ilişkili radyolusent (basit kemik kistinden ayırımı zor), buzlu cam (fibröz ve kemik yapılar eşit oranda) ve sklerotik (kemik dokuları belirgin) görünümlerde olabilir. 13 Hastamızda kemik dokuların yoğun olduğu sklerotik görünüm baskındı. MRI yumuşak doku komponentlerinin değerlendirilmesinde ve ayırıcı tanısında faydalıdır.…”

Fibrous dysplasia of the craniofacial region - A brief understanding of the disease

“…Teeth are displaced in approximately 35% cases. [8] Three distinct patterns were described by Panda et al [3,10] The pagetoid type (on CT imaging) characterized by bone expansion and scattered islands of bone formation in a low-attenuation field. The sclerotic type with homogeneous ground-glass appearance.…”

“…[8] If there is obliteration of bony cavities or constriction of foramina: Proptosis, diplopia, orbital dystopia, blindness, epiphora, strabismus, tinnitus, hearing loss, nasal obstruction, facial paralysis, etc., may be evident. [3] Pain is not an initial presenting symptom but might occur later due to the disease obstructing the sinus or compressing the nerves passing through various foramina. [4] Resorption of roots is rarely observed.…”

“…Perhaps the best terminology to describe FD is "fibro-osseous dysplasia" or "fibrous osteodysplasia." [3] Incidence and Distribution It is a rare condition known to have an incidence of 1:4000-1:10,000 and represent 7% of all benign bone tumors and has a slight female predilection. It usually presents in the first three decades of life and usually stabilizes after reaching skeletal maturity.…”

“…Usually, unilateral side is involved and more commonly in the posterior region. [3] Types of FD Monostotic …”