Analysis of Series of Cases of Fibrous Dysplasia of Proximal Femur in Pediatric Population.

Abstract

Introduction: Fibrous dysplasia (FD) accounts for 5-7% of all benign bone tumors. It presents in two main forms - monostotic, which is more common affecting a single bone and presenting usually in 3rd decade of life; polyostotic, affecting several bones, is less common, and is seen mainly in the 1st decade of life. These usually present as bone pain or pathological fracture. It may also be part of McCune-Albright syndrome. Since the femur is an important weight-bearing bone in the human body, most cases of FD affecting the femur present as pathological fracture more early than other sites. The mainstay of management includes treatment of pathological fracture and prevention of bony deformity until skeletal maturity. The aim of this study was to analyze a series of cases of FD of femur affecting the pediatric population.

Materials and methods: A retrospective study was conducted at two tertiary-level centers of urban India, wherein all case records of FD under follow-up between January 2015 and December 2023 were analyzed. In all, there were 13 cases. Definitive management was decided on a case-by-case basis. We grouped the cases into 2 categories based on recurrence.

Observations: Out of 13 cases, 11 were males while 2 patients were females. The mean age at presentation was 10.5 years (range 4-12 years). Two cases were of polyostotic FD, while 11 cases were of monostotic FD. In 11 cases, the intertrochanteric region of the femur was affected, while in 2 cases the affection extended into the proximal shaft of the femur as well. The mean follow-up period was 48 months (Range 15-84 months). In category one (no recurrence) there were 10 patients, while in category two (recurrence of FD) there were 3 patients. The recurrence rate in our series was 23%.

Results: Although FD is also seen in the adult population, its implications are more pronounced in a growing skeleton. The series of cases where surgical intervention has been done with a long follow-up in the pediatric population are limited. In literature, clinical classification of FD, anatomical location in the proximal femur, variation in neck-shaft angle and osteocalcin levels have been found to be significant in predicting causes of fractures and their recurrences.

Conclusion: Internal fixation is preferable to prevent deformities where there are high chances of a pathological fracture as is evident by the natural history of FD. Long-term follow-up is important as there are chances of recurrence in childhood until puberty.