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Mccune-Albright syndrome managed with multiple osteotomies, massive curettage, intramedullary nailing, and bone
Yuni Artha Prabowo Putro1,2, Ahmad Syaukat1,2, Rahadyan Magetsari1,2
1Department of Orthopedics and Traumatology, Dr. Sardjito General Hospital, D.I.Yogyakarta, Indonesia.
Introduction And Importance:
McCune-Albright syndrome (MAS) is a rare, benign mosaic syndrome. Limb length discrepancy and deformity are common, and the new bone formed following corrective osteotomy is dysplastic, indicating that recurrence of deformity should be anticipated.
Presentation Of Case:
A 15-year-old boy presented with left leg bowing following a previously healed femoral fracture with a persistent deformity. His parents reported early pubic and axillary hair development at the age of eight and a rapid growth spurt by the age of 12. Radiographs showed a 42.6° lateral bowing of the left femur, suggestive of fibrous dysplasia. Multiple osteotomies, intramedullary nail fixation, and synthetic bone grafting were performed, resulting in improved lower extremity function based on Lower Extremity Functional Scale scores.
Clinical Discussion:
The diagnosis of MAS requires fibrous dysplasia, café-au-lait macules, and precocious puberty. Surgical management of limb deformity and discrepancy in polyostotic fibrous dysplasia is challenging, and not all procedures provide optimal results. Intramedullary nailing of the femur or tibia is considered the preferred option.
Conclusion:
In cases of MAS, multiple osteotomies to correct deformity, followed by massive curettage to remove the fibrous lesion, intramedullary nailing, and bone grafting, resulted in favorable clinical and functional outcomes in this patient.
