Reactivating Sleeping Intramedullary Nail in a 16-Year-Old Female with Polyostotic Fibrous Dysplasia: A Case Report...

Fibrous dysplasia (FD) is a rare condition in which normal spongy and cortical bone is replaced by non-neoplastic fibrous tissue, leading to weakened bone matrix and increased risk of pathological fractures and deformities. Treating these deformities poses a significant challenge for surgeons. While various cases of surgical stabilization and limb lengthening using intramedullary nails have been reported, there is limited evidence on the use of Motorized Intramedullary Limb-Lengthening Nails (MILLNs) in FD patients. This case report presents the clinical history of a patient with FD who underwent multiple surgical interventions to address severe lower limb length discrepancy (LLD) and angular deformity caused by multiple fractures.

A sixteen-year-old Caucasian girl with polyostotic FD developed a severe post-traumatic LLD of 10 cm on the right side, associated with coxa vara, valgus knee, and patellar instability. The deformity of the proximal femur was addressed with a valgus and derotational femoral osteotomy. However, this procedure exacerbated the knee's valgus deformity and only partially corrected the LLD, leading to the decision to proceed with femoral lengthening. A retrograde magnetic intramedullary nail (PRECICE, NuVasive) was utilized for this purpose. Approximately three months postoperatively, radiographs revealed the loosening of the proximal anchoring screw, while the nail had reached maximum distraction. We then proposed reactivating the previously implanted nail. Nine months after the final surgery, standing long-leg radiographs showed a residual shortening of 1 cm, with excellent healing at the fracture sites and the nail and screws remaining securely in place. The patient was monitored regularly, with the latest follow-up occurring four years and five months after the conclusion of the last lengthening procedure.

This case report describes the reactivation of a MILLN in a patient with polyostotic fibrous dysplasia. While nail reactivation has been previously described in the literature, to our knowledge, it has not been reported for treating complications arising from FD. In cases of mechanical complications, this approach can equalize leg length discrepancies and correct deformities, avoiding additional invasive surgeries and reducing healthcare costs. As this is an off-label treatment, preoperative consent from both the patient and the parents is required.