• Peculiarities of clinical-radiological course of different forms of fibrous dysplasia
To content Full text of article

Peculiarities of clinical-radiological course of different forms of fibrous dysplasia

Paediatric surgery.Ukraine.2019.4(65):10-18; DOI 10.15574/PS.2019.65.10

Y.M. Guk, A.M. Zima, N.O. Naumenko, Т.А. Kinchaya-Polishchuk, А.І. Cheverda, О.Y. Skuratov, M.F. Sivak
SI «The Institute of Traumatology and Orthopedics by NAMS of Ukraine», Kyiv

For citation: Guk YM, Zima AM, Naumenko NO, Kinchaya-Polishchuk ТА at al. (2019). Peculiarities of clinical-radiological course of different forms of fibrous dysplasia. Paediatric Surgery.Ukraine. 4(65): 10-18. doi 10.15574/PS.2019.65.10
Article received: Aug 27, 2019. Accepted for publication: Dec 16, 2019.

The aim of the study: to improve the diagnosis of forms of fibrous dysplasia by investigating clinical and radiological features, depending on the disease form, age and sex of the patient.
Materials and methods. The research was conducted by analyzing results of examination and treatment of 80 patients with different forms of fibrous dysplasia at Kyiv Institute of Traumatology and Orthopedics by NAMS of Ukraine from 2000 to 2019. The diagnosis was made on the basis of studying features of the medical history of the disease, its clinical course and radiological examination method. There were 56 patients with monoosal disorder, 14 with polyosal, and 10 with Albright syndrome. Clinical-radiological method of the research was used to clarify the diagnosis of the disease and to study features of the disease. The radiological examination was performed on the Multix UP in the department of functional diagnostics «ITO NAMSU» according to the standard technique in direct posterior and lateral projections with capture of adjacent joints. Localization, volume of lesion and condition of cortical layer at pathological dysplastic process of long bones of lower extremities were evaluated.
Results. The article specifies clinical-radiological features revealed in various forms of fibrous dysplasia and shows that pathological fractures of the long bones and their deformation depend on the form of the disease, age and sex of patients. The incidence of pathological fractures of long bones of lower extremities was determined depending on the localization, form of the disease and sex of patients with different forms of fibrous dysplasia: femur – 72.3% (proximal part – 76.7%); tibia bones – 27.3% (diaphyseal part – 78.3%); it was found that the number of fractures was significantly greater (49.4%) with the monoosal form of fibrous dysplasia than with the polyosal form (30.1%, p<0.05) and Albright syndrome (20.5%, p<0,5); in males with monoosal form and Albright syndrome, the number of fractures was significantly greater (p<0.05) than in females.
Conclusions. Features of the course of clinical and radiological manifestations depending on the form of the disease, age and sex of the patient have been covered in the article, which will allow reducing and eliminating diagnostic errors related to this pathology and create a basis for improvement of the system of orthopedic treatment of this nosology.
The research was conducted in accordance with principles of the Declaration of Helsinki. The research protocol was approved by institution’s Local Ethics Committee. Informed consent was obtained from parents of children (or their guardians) for the research.
Authors declare that there was no conflict of interest.
Key words: fibrous dysplasia, pathological bone fractures, bone deformity, diagnosis, clinical manifestations, radiological symptoms.

REFERENCES

1. Dolnitsky OB. (2009). Congenital malformations. Fundamentals of diagnosis and treatment. Kyiv: Business Polygraph: 516–523.

2. Zubairov TF. (2009). Diagnosis and surgical treatment of fibrous osteodysplasia: dissertation. St. Petersburg: 161.

3. Kosinska NA. (1966). Disorders of the development of the bone and joint apparatus. Leningrad: Medicine: 357.

4. Sadovenko EG. (1992). Fibrous dysplasia (diagnostics, therapeutic tactics): dissertation. Kyiv: 145.

5. Breck LW. (1972). Treatment of fibrous dysplasia of bone by total femoral plating and hip nailing. Clin Orthop. 82: 82-91. https://doi.org/10.2106/JBJS.O.00547; PMid:26842411 PMCid:PMC4732545

6. DiCaprio MR, Enneking WF. (2005). Fibrous dysplasia. Pathophysiology, evaluation, and treatment. J Bone Joint Surg.87-A(8): 1848-1864. https://doi.org/10.2106/00004623-200508000-00028

7. Jung ST, Chung JY, Seo HY, Bae BH, Lim KY. (2006). Multiple osteotomies and intramedullary nailing with neck cross-pinning for shepherd’s crook deformity in polyostotic fibrous dysplasia: 7 femurs with a minimum of 2 years follow-up. Acta Orthop. 77(3): 469-73. https://doi.org/10.1080/17453670610046415; PMid:16819687

8. Kushare V, Colo D, Bakhshi H, Dormans JP. (2014). Fibrous dysplasia of the proximal femur: surgical management options and outcome. J Child Orthop.8: 505-511. https://doi.org/10.1007/s11832-014-0625-9; PMid:25409925 PMCid:PMC4252268

9. Lichtenstein L. (1938). Polyostotic fibrous dysplasia. Arch Surg.36: 874-98. https://doi.org/10.1001/archsurg.1938.01190230153012

10. Lichtenstein L, Jaffe H. (1942). Fibrous dysplasia of bone. Arch Path.33: 777-816.

11. Shah ZK, Peh WC, Koh WL, Shek TW. (2005). Magnetic resonance imaging appearances of fibrous dysplasia. Br. J Radiol.78(936): 1104. https://doi.org/10.1259/bjr/73852511; PMid:16352586

12. Stephenson RB, London MD, Hankin FM, Kaufer H. (1987). Fibrous dysplasia. An analysis of options for treatment. J Bone Joint Surg Am. 69: 400-409. https://doi.org/10.2106/00004623-198769030-00012

13. Thomsen MD, Rejnmark L. (2014). Clinical and radiological observations in a case series of 26 patients with fibrous dysplasia. Calcif Tissue Int.94: 384-395https://doi.org/10.1007/s00223-013-9829-0; PMid:24390518

14. Yao L, Eckardt JJ, Seeger LL. (1994). Fibrous dysplasia associated with cortical bony destruction: CT and MR findings. J Comput Assist Tomogr.18: 91-94. https://doi.org/10.1097/00004728-199401000-00019; PMid:8282892