Anterior and Central Skull Base Fibrous Dysplasia: A 12 Years’ Experience

Fibrous dysplasia of paranasal sinuses with anterior skull-base extension presenting with pneumocephalus; a case report

“…Fibrous dysplasia is a rare and potentially benign bone tumor with a prevalence of about 7 % of all non-malignant bone tumors [ 2 , 4 , 19 ], especially in the craniofacial region. It is more frequent in women than men, and the ethmoid bone is the most commonly involved [ 3 , 9 ]. Reports of Fibrous Dysplasia involving both paranasal sinuses and the anterior skull-based region are rare, especially if this condition is accompanied by pneumocephalus.…”

“…Reports of Fibrous Dysplasia involving both paranasal sinuses and the anterior skull-based region are rare, especially if this condition is accompanied by pneumocephalus. Symptomatic patients complain of facial asymmetry, progressive headache, dystopia, and visual impairment [ 2 , 3 ]. While there is no consensus on management approaches, most clinicians state that surgery (mostly endoscopic approach) is indicated in symptomatic patients [ 3 ].…”

“…Symptomatic patients complain of facial asymmetry, progressive headache, dystopia, and visual impairment [ 2 , 3 ]. While there is no consensus on management approaches, most clinicians state that surgery (mostly endoscopic approach) is indicated in symptomatic patients [ 3 ]. If the patient is asymptomatic or due to the patient's condition, the potential benefit of surgery is limited and medical treatment or surveillance could be considered [ 6 ].…”

“…Craniofacial FD is a lesion of unknown etiology [ 1 ]. However, it has been reported that post-zygotic missense mutations of the GNAS1 gene (protein bound to the guanine nucleotide α-stimulating activity of polypeptide1) initiate the formation of the disease particularly in syndromic states [ [2] , [3] , [4] , [5] ]. Most FD patients have no apparent symptoms [ 3 , 4 ] and are diagnosed incidentally for other reasons by CT scan [ 3 , 6 , 7 ].…”

“…However, it has been reported that post-zygotic missense mutations of the GNAS1 gene (protein bound to the guanine nucleotide α-stimulating activity of polypeptide1) initiate the formation of the disease particularly in syndromic states [ [2] , [3] , [4] , [5] ]. Most FD patients have no apparent symptoms [ 3 , 4 ] and are diagnosed incidentally for other reasons by CT scan [ 3 , 6 , 7 ]. Some studies categorize FD into three main groups: monostotic, which is the most common [ 2 , 4 ], polyostotic which has an earlier onset and almost affects a craniofacial bone [ 8 , 9 ]; and syndrome-related FD, such as McCune-Albright [ 10 , 11 ], Mazabraud [ 12 , 13 ] and Jaffe-Lichtenstein Syndrome [ 2 ].…”