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

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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.

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References

  1. Chong VFH, Khoo JBK, Fan Y-F (2002) Fibrous dysplasia involving the base of the skull. AJR Am J Roentgenol 178(3):717–720

    Article  PubMed  Google Scholar 

  2. 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

    Article  Google Scholar 

  3. 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

    CAS  PubMed  PubMed Central  Google Scholar 

  4. Eversole R, Su L, ElMofty S (2008) Benign fibro-osseous lesions of the craniofacial complex. A review. Head Neck Pathol 2(3):177–202

    Article  PubMed  PubMed Central  Google Scholar 

  5. 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

    Article  CAS  Google Scholar 

  6. 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

    Article  Google Scholar 

  7. 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

    Article  CAS  PubMed  Google Scholar 

  8. 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

    PubMed  Google Scholar 

  9. 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

    Article  CAS  PubMed  Google Scholar 

  10. Lambert PR, Brackmann DE (1984) Fibrous dysplasia of the temporal bone: the use of computerized tomography. Otolaryngol Neck Surg 92(4):461–467

    Article  CAS  Google Scholar 

  11. 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

    Article  PubMed  PubMed Central  Google Scholar 

  12. Sadeghi SM, Hosseini SN (2011) Spontaneous conversion of fibrous dysplasia into osteosarcoma. J Craniofac Surg 22(3):959–961

    Article  PubMed  Google Scholar 

  13. 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

    Article  PubMed  Google Scholar 

  14. 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

  15. 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

    Article  CAS  PubMed  Google Scholar 

  16. Lei P, Bai H, Wang Y, Liu Q (2009) Surgical treatment of skull fibrous dysplasia. Surg Neurol 72(Suppl 1):S17-20

    Article  PubMed  Google Scholar 

  17. 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

    Article  CAS  PubMed  Google Scholar 

  18. 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

  19. 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

    Article  PubMed  Google Scholar 

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This research did not receive any specific grant from funding agencies in the public, commercial, or not for profit sectors.

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Correspondence to Shruti Bhargava.

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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

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