Jawbone fibrous dysplasia: retrospective evaluation in a cases series surgically treated and short review of the literature Jawbone fibrous dysplasia: retrospective data analysis

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Gino Amleto Pacino
Salvatore Cocuzza
Giovanni Tonoli
Paolo Boscolo Rizzo
Giancarlo Tirelli
Margherita Tofanelli
Giorgio Ciprandi
Ignazio La Mantia
Antonino Maniaci
Maria Cristina Da Mosto
Giacomo Spinato

Keywords

Fibrous dysplasia - jawbone - bone remodelling – craniofacial dysmorphisms

Abstract

Background and aim of the work. Fibrous dysplasia is a fibro-osseous osteopathy in which the normal bone architecture is replaced by fibrous tissue and non-functional trabeculae-like osseous structures. In head and neck area monostotic or polyostotic lesions cause a progressively expanding destructive bone swelling producing cosmetic deformities and functional impairments. The aim of this article is to present a retrospective review of a clinical case series with pathologically confirmed jawbone fibrous dysplasia for over an 8-year-period.


Material and Methods. Clinical presentation and radiographic features of fibrous dysplasia affecting the jawbone skeletal area, surgical procedures performed including the reconstructive methods employed and clinical outcomes were analysed for each patient.


Results. Seven cases were classified as having monostotic fibrous dysplasia while the others four cases were classified as having polyostotic form. The mandible was most commonly involved. The most common presenting features included marked facial deformity, intraoral bulging, malocclusion and dental alterations. Aesthetic and/or functional impairments were the major indications for surgical treatment in all the patients of this series. Six patients underwent bone remodelling while in the remaining cases subtotal or total resection was performed. Bone reconstruction by means of autologous free bone grafts or revascularized free bone flaps was made in three cases.


Conclusion. The choice of the tailored therapeutic approach should be evaluated according the patient’s age, rate of growth, anatomic location, type of involvement and the presence or not of functional disturbances and cosmetic alterations. Surgery remains the best therapeutic option.

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References

1. Neville BD, Damm DD, Allen CM, Bouquot JE. Bone pathology. In Oral and maxillofacial pathology. II ed. Philadelphia, WB Saunders Company 2002; pag. 553-557.
2. Eversole LR, Sabes WB, Povin S. Fibrous dysplasia, a nosologic problem in the diagnosis of fibro-osseous lesions of the jaws. J Oral Pathol 1972; 1: 189-220.
3. Waldron CA. Fibro-osseous lesions of the jaws. J Oral Maxillofac Surg 1993; 51: 828-835.
4. Gorlin RJ, Cohen MM, Levin LS (eds). Syndromes of the head and neck, 3rd ed., New York: Oxford University Press, 1990.
5. Lichteinstein L. Polyostotic fibrous dysplasia. Arch Surg 1938; 36: 874-898.
6. Lichtestein L, Jaffe HL. Fibrous dysplasia of bone. A condition affecting one, several or many bones, the graver cases of wich may represent abnormal pigmentation of skin, premature sexsual development, hyperthyroidism or still other extraskeletal abnormalities. Arch Pathol 1942; 33: 777-816.
7. Albraight F, Butler AM, Hampton AO, Smith P. Syndrome characterized by osteitis fibrosa disseminata, area of pigmentation and endocrine dysfunction, with precocious puberty in females: report of five cases. N Engl J Med 1937; 216: 727- 746.
8. Mazabraund A, Semat P, Roze R. Association of soft tissue fibromyxoma with fibrous bone dysplasia. Presse Med 1967; 75: 2223-2228.
9. Pollandt K, Lohmann CH, Werner M, Delling G. Clinical pathological aspects in Mazabraund’s syndrome. Pathologe 2002; 23: 357-360.
10. Burke AB, Collins MT, Boyce AM. Fibrous dysplasia of bone: craniofacial and dental implications. Oral Dis 2017; 23(6):697–708.
11. Ruggieri P, Sim FH, Bond JR, Unni KK. Malignancies in fibrous dysplasia. Cancer 1994; 73: 1411-1424.
12. Yabut SM, Kenan S, Sissons HA et al. Malignant trasformation of fibrous dysplasia. Clin Orthop 1988; 228:281-289.
13. Ebata K, Usami T, Tonhai J, Kaneda T. Chondrosarcoma and osteosarcoma arising in polyostotic fibrous dysplasia. J Oral Maxillofac Surg 1992; 50: 761-764.
14. Smith JF. Fibrous dysplasia of the jaws. Arch Otolaryngol 1965; 81: 592-601.
15. Shenker A, Weinstein LS, Sweet DE, Speigel AM. An activating Gs alpha mutation is present in fibrous dysplasia of bone in McCune-Albright syndrome. J Clin Endocrinol Metab 1994; 79: 750-755.
16. Caltabiano R, Serra A, Bonfiglio M et al. A rare location of benign osteoblastoma: case study and a review of the literature. Eur Rev Med Pharmacol Sci 2012; 16(13): 1891-4.
17. Caltabiano R, Cocuzza S, Maira S, et al. Respiratory epithelial adenomatoid hamartoma: case report and literature review. Pathologica 2008; 100(3): 185-8.
18. Mandrioli S, Carinci F, Dallera V, Calura G. Fibrous displasia. The clinico-therapeutic picture and new data on etiology. A review of the literature. N Engl J Med 1995; 332: 1546-1551.
19. Marie PJ, De Pollak C, Chanson P, Lomri A. Increased proliferation of osteoblastic cells expressing the activating Gs alpha mutation in monostotic and polyostotic fibrous dysplasia. Am J Pathol 1997; 150: 1059-1069.
20. Cohen MM Jr, Howell RE. Etiology of fibrous dysplasia and McCune-Albright syndrome. Int J Oral Maxillofac Surg 1999; 28: 366-371.
21. Pierce AM, Sampson WJ, Wilson DF, Goss AN. Fifteen-year follow-up of a family with inherited craniofacial fibrous dysplasia. J Oral MaxilloFac Surg 1996; 54: 780-788.
22. El Deeb M, Waite DE, Gorlin RJ. Congenital monostotic fibrous dysplasia : a new possibly autosomal recessive disorder. J Oral Surg 1979 ; 37 : 520-524.
23. Dal Cin P, Sciot R, Speleman F, Samson I et al. Chromosome aberration in fibrous dysplasia. Cancer Genet Cytogenet 1994; 77: 114-117.
24. Weereprandist W, Cheerat S, Punyashing J, Parichantikannond P, Wannakraitot P. Fibrous dysplasia in Thais. J Dent Assoc Thai 1989;39: 80-86.
25. Ogunsalu CO, Lewis A, Doonquah L. Benign fibro-osseous lesions of the jaw bones in Jamaica: analysis of 32 cases. Oral Disease 2001; 7: 155-162.
26. Langdon JD, Rapidis AD, Patel MF. Ossifying fibroma: one disease or six? An analysis of 39 fibro-osseous lesions of the jaws. Br I Oral Surg 1976; 14:1-11.
27. MacDonald Jankowski D. Fibrous dysplasia in the jaws of a Hong Kong population: radiographic presentation and systematic review. Dentomaxillofac Radiol 1999;28: 195-202.
28. Mohammadi Araghi H, Haery C. Fibro-osseous lesions of cranio-facial bones: the role of imaging. Radiol Clin North Am 1993; 31:121-134.
29. Khairallah E, Antonyshyn O, Farb R et al. Progressive unilateral mandibular swelling in adolescent: a diagnostic dilemma. J CranioFac Surg 1997; 8: 32-37.
30. Bessho K, Tagawa T, Murata M, Komaki M. Monostotic fibrous dysplasia with involvement of the mandibular canal. Oral Surg Oral Med Oral Pathol 1991; 72: 284-288.
31. Shapeero LG, Vanel D, Ackerman LV et al. Aggressive fibrous dysplasia of the maxillary sinus. Skeletal Radiol 1993; 22: 563-568.
32. Daffner RH, Kirks DR, Gehweiler JA Jr: Computed tomography of fibrous dysplasia. Am J Roentgenol 1982; 139: 943-948.
33. Conrad GR, Dean BL, Baumann RJ, Seabold JE. Scintigraphy, MRI and CT of a vascular fibrous dysplasia of the craniofacial bones. Clin Nucl Med 1991; 16: 743-746.
34. Vojtek TM, Ro JY, Edeiken J, Ayala AG. Fibrous dysplasia and cement-ossifying fibroma. A histological spectrum. Am J Surg Pathol 1995; 19: 775-781.
35. Edgerton MT, Persing JA, Jane JA. The surgical treatment of fibrous dysplasia with emphasis on recent contribution from cranio-maxillo-facial surgery. Ann Surg 1985; 202: 459-479.
36. Camilleri AE. Craniofacial fibrous dysplasia. J Laryngol Otol 1991; 105: 662- 666.
37. Ozek C, Gundogan H, Bilkay U, Tokat C, Gurler T, Songur E. Craniomaxillofacial fibrous dysplasia. J Craniofac Surg 2002; 13: 382-389.
38. Chen YR, Nordohoff MS. Treatment of craniomaxillofacial fibrous dysplasia: how early and how extensive? Plast Reconstr Surg 1990; 86: 835.
39. Posnick JC. Fibrous dysplasia of the craniomaxillofacial region: current surgical perspective. Br J Oral Maxillofac Surg 1998; 36: 264-273.
40. Frodel JL, Funk G, Boyle J, Richardson M. Management of aggressive midface and orbital fibrous dysplasia. Arch Facial Plast Surg 2000; 2: 187-195.
41. Zenn MR, Zuniga J. Treatment of fibrous dysplasia of the mandible with radical excision and immediate reconstruction: case report. J Craniofac Surg 2001; 12:259-263.
42. Feingold RS, Argamaso RV, Strauch B. Free fibula flap reconstruction for oral obstruction secondary to giant fibrous dysplasia. Plast Reconstr Surg 1996; 97:196-201.
43. Lazio MS, Luparello P, Mannelli G, Santoro GP, Bresci S, Braggion C, Gallo O, Maggiore G (2019) Quality of Life and Impact of Endoscopic Sinus Surgery in Adult Patients with Cystic Fibrosis. American journal of rhinology & allergy 33 (4):413-419.
44. Kaplan H, Kessler A. One-stage flap reconstruction of mandibular defects. Harefuah 1990; 118:627-32.
45. Yeow VKL, Chen YR. Orthognathic surgery in craniomaxillofacial fibrous dysplasia. J Craniofac Surg 1999; 10: 155-159.
46. Liens D, Delmas PD, Meunier PJ. Long-term effects of intravenous pamidronate in fibrous dysplasia of bone. Lancet 1994; 343: 953-954.
47. Andaloro C, Sati M, Grillo C, Grillo CM, LA Mantia I. Relationship between sleeping difficulties and airway symptoms severity with the health-related quality of life in patients with GERD. Minerva Gastroenterol Dietol 2017; 63(4): 307-312.
48. La Mantia I, Andaloro C. Effectiveness of intranasal sodium hyaluronate in mitigating adverse effects of nasal continuous positive airway pressure therapy. Am J Rhinol Allergy. 2017; 31(6): 364-369.
49. Lane JM, Khan SN, O'Connor WJ, Nydick M, Hommen JP, Schneider R, Tomin E, Brand J, Curtin J. Bisphosphonate therapy in fibrous dysplasia. Clin Orthop 2001; (382):6-12.
50. Long A, Loughlin T, Towers RP, McKenna TJ. Polyostotic fibrous dysplasia with contrasting responses to calcitonin and mithramycin: aetiological and therapeutic implications. Ir J Med Sci 1988; 157: 229-234.
51. Yasuoka T, Takagi N, Hatakeyama D, Yokoyama K. Fibrous dysplasia in the maxilla: possible mechanism of bone remodelling by calcitonin treatment. Oral Oncol 2003; 39: 301-395.
52. Spinato G, Cazzato G, Ferlito S et al. Therapeutical innovations and medical responsibility: What's new in otolaryngology. Acta Medica Mediterranea 2018; 34(2): 307-312.