Dr. David Folorunso: Surgical Treatment Of Monostotic Craino-Facial Fibrous Dysplasia: Changing The Narratives
I. O. Gbujie*, E. A. Dahillo, D. F. Fulorunso, B. E. Nwankwo, O. R. Quadri, B. I. Egbe, B. F. Bello, F. M. Damtong, T. S. Ibekwe
Introduction
Fibrous dysplasia is a non-neoplastic bone tumour. Fibro-osseous tumour is used interchangeability by pathologists and clinicians universally to depict the same pathology. It is a genetically anchored developmental aberration of bone-forming mesechymal tissues with defective osteoblastic differentiation and maturation, resulting to the replacement of normal bony tissue by fibrous tissue of variable cellularity and immature woven bone [1]-[6].
It is as a result of post-natal muta-tion of GNASI gene which triggers the activation and proliferation of undiffe-rentiated mesenchymal tissue. Generally, this lesion clinically presents in three forms namely Monostotic, polyostotic and polyostotic with endocrine dysfunc-tion and skin hyperpigmentation (café-au-lait spots).
This third form is also known as McCune Albright syndrome. It is worthy to note that a very small percentage of Cherubism which is a hereditary, autosomal dominant disorder, resulting from SH3BP2 gene mutation can present with a monostotic lesion [3] [6]. It is relatively uncommon in our region and worldwide has a prevalence of 1:4000 to 1:10,000 accounting for 7% of benign bone tumours. There is slight female preponderance.
The monostotic form accounts for 70%, polyostotic 27% and McCune Albright syndrome 3% of all fibrous dysplasia. Cranio-facial region is involved in 27% of monostotic lesion and 50% of polyostotic lesion [3] [7] [8]. Monostotic craniofacial Fibrous dysplasia starts in the first and second dec-ades of life, exhibits slow growth rate and generally believed to stop growing af-ter puberty in the third decade [1] [3] [6]. However, several reports documented lesions manifesting rapid growth in adult life [9] [10]. Clinically, there are no specific symptoms and signs of the tumour.
Its ma-nifestation is dependent on the particular structure and organ involved. Gener-ally, it presents a painless swelling of the involved cranio-facial bone, with its at-tendant aesthetic deformity. Extension into nasal fossa, alveolus, orbit and optic nerve, may result in nasal blockage, malocclusion and dental anarchy, as well as visual impairment. Grossly, the tumour appears variable in consistency, depending on the tissue composition [2] [4].
It may be, gritty, hard in a portion, firm in a largely fibrous component or soft when cystic degeneration has taken place. It is well circum-