Abstract
Fibrous dysplasia, specially of anterior and central skull base region, is a rare disorder. This article discusses about our experience in this pathology. A tertiary care institute based retrospective type study was conducted over a period of 12 years. Demographics, radiology, intraoperative details, pathology and follow up were taken into consideration and the data was analysed. Sixteen patients with complaints of proptosis, diplopia, nasal obstruction and/or facial deformity, underwent endoscopic sinus surgery. Subtotal resection was done in 5 patients. Ethmoid bone involvement was seen in 12 patients. Post operatively, diplopia persisted in one patient and one patient had epistaxis. All patients were followed up for 2–10 years with no other complications reported. Anterior and central skull base involvement is rare in fibrous dysplasia. However, it can be removed effectively by endoscopic approach. Overall safety of patient has more concern rather than complete removal of disease.
Similar content being viewed by others
References
Chong VFH, Khoo JBK, Fan Y-F (2002) Fibrous dysplasia involving the base of the skull. AJR Am J Roentgenol 178(3):717–720
McCune DJ, Bruch H (1937) Osteodystrophia Fibrosa: report of a case in which the condition was combined with precocious puberty, pathologic pigmentation of the skin and hyperthyroidism, with a review of the literature. Am J Dis Child 54(4):806–848
Riminucci M, Fisher LW, Shenker A, Spiegel AM, Bianco P, Gehron RP (1997) Fibrous dysplasia of bone in the McCune-Albright syndrome: abnormalities in bone formation. Am J Pathol 151(6):1587–1600
Eversole R, Su L, ElMofty S (2008) Benign fibro-osseous lesions of the craniofacial complex. A review. Head Neck Pathol 2(3):177–202
Lustig LR, Holliday MJ, McCarthy EF, Nager GT (2001) Fibrous dysplasia involving the skull base and temporal bone. Arch Otolaryngol Neck Surg 127(10):1239–1247
Khattab DM, Mohamed S, Barakat MS, Shama SA (2014) Role of multidetector computed tomography in assessment of fibro-osseous lesions of the craniofacial complex. Egypt J Radiol Nucl Med 45:723–734
Faivre L, Nivelon-Chevallier A, Kottler ML et al (2001) Mazabraud syndrome in two patients: clinical overlap with McCune-Albright syndrome. Am J Med Genet 99(2):132–136
Chen Y-R, Wong F-H, Hsueh C, Lo L-J (2002) Computed tomography characteristics of non-syndromic craniofacial fibrous dysplasia. Chang Gung Med J 25(1):1–8
Lee JS, FitzGibbon EJ, Chen YR et al (2012) Clinical guidelines for the management of craniofacial fibrous dysplasia. Orphanet J Rare Dis 7 Suppl 1(Suppl 1):S2–S2
Lambert PR, Brackmann DE (1984) Fibrous dysplasia of the temporal bone: the use of computerized tomography. Otolaryngol Neck Surg 92(4):461–467
Jeyaraj P (2019) Histological diversity, diagnostic challenges, and surgical treatment strategies of fibrous dysplasia of upper and mid-thirds of the craniomaxillofacial complex. Ann Maxillofac Surg 9(2):289–314
Sadeghi SM, Hosseini SN (2011) Spontaneous conversion of fibrous dysplasia into osteosarcoma. J Craniofac Surg 22(3):959–961
Lee JS, FitzGibbon E, Butman JA et al (2002) Normal vision despite narrowing of the optic canal in fibrous dysplasia. N Engl J Med 347(21):1670–1676
Devogelaer J-P (2000) Treatment of bone diseases with bisphosphonates, excluding osteoporosis. Curr Opin Rheumatol 12(4). https://journals.lww.com/co-rheumatology/Fulltext/2000/07000/Treatment_of_bone_diseases_with_bisphosphonates,.17.aspx
Eugster EA, Shankar R, Feezle LK, Pescovitz OH (1999) Tamoxifen treatment of progressive precocious puberty in a patient with McCune-Albright syndrome. J Pediatr Endocrinol Metab 12(5):681–686
Lei P, Bai H, Wang Y, Liu Q (2009) Surgical treatment of skull fibrous dysplasia. Surg Neurol 72(Suppl 1):S17-20
Shkarubo AN, Lubnin AY, Bukharin EY et al (2017) Endoscopic transnasal surgery for giant fibrous dysplasia of the skull base, spreading to the right orbital cavity and nasopharynx (a case report and literature review). Zh Vopr Neirokhir Im N N Burdenko 81(1):81–87
AlMomen AA, Molani FM, AlFaleh MA, AlMohisin AK (2020) Endoscopic endonasal removal of a large fibrous dysplasia of the paranasal sinuses and skull base. J Surg Case Rep 2020(1):rjz404
DeKlotz TR, Stefko ST, Fernandez-Miranda JC, Gardner PA, Snyderman CH, Wang EW (2017) Endoscopic endonasal optic nerve decompression for fibrous dysplasia. J Neurol Surg B Skull Base 78(1):24–29
Funding
This research did not receive any specific grant from funding agencies in the public, commercial, or not for profit sectors.
Author information
Authors and Affiliations
Contributions
All the authors have contributed equally in all aspects of the research.
Corresponding author
Ethics declarations
Conflict of Interest
All the authors declare that they have no conflict of interest.
Ethical approval
All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.
Informed Consent
Informed consent was obtained from all individual participants included in the study.
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
About this article
Cite this article
Grover, M., Gupta, A., Samdhani, S. et al. Anterior and Central Skull Base Fibrous Dysplasia: A 12 Years’ Experience. Indian J Otolaryngol Head Neck Surg 74 (Suppl 2), 1462–1467 (2022). https://doi.org/10.1007/s12070-021-02542-8
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12070-021-02542-8