Todisco Marco, Viotto Marianna, Campanacci Laura, Di Gennaro Giovanni Luigi, Depaoli Alessandro, Rocca Gino, Trisolino Giovanni
Pediatric Orthopedics and Traumatology, IRCCS Istituto Ortopedico Rizzoli, 40136 Bologna, Italy.
Department of Orthopedic Oncology, IRCCS Istituto Ortopedico Rizzoli, 40136 Bologna, Italy.
Life (Basel). 2024 Nov 25;14(12):1543. doi: 10.3390/life14121543.
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.
骨纤维异常增殖症（FD）是一种罕见疾病，正常的松质骨和皮质骨被非肿瘤性纤维组织替代，导致骨基质减弱，病理性骨折和畸形风险增加。治疗这些畸形对外科医生构成重大挑战。虽然已有使用髓内钉进行手术稳定和肢体延长的各种病例报道，但关于动力化髓内肢体延长钉（MILLNs）在FD患者中的应用证据有限。本病例报告介绍了一名FD患者的临床病史，该患者接受了多次手术干预，以解决由多处骨折引起的严重下肢长度差异（LLD）和角畸形。
一名患有多骨型FD的16岁白种女孩右侧出现严重创伤后LLD达10厘米，伴有髋内翻、膝外翻和髌骨不稳定。股骨近端畸形通过外翻和旋转截骨术进行处理。然而，该手术加剧了膝关节的外翻畸形，仅部分纠正了LLD，因此决定进行股骨延长。为此使用了逆行磁性髓内钉（PRECICE，NuVasive）。术后约三个月，X线片显示近端锚固螺钉松动，而钉子已达到最大牵伸。然后我们提议重新激活先前植入的钉子。最后一次手术后九个月，站立位长腿X线片显示残留缩短1厘米，骨折部位愈合良好，钉子和螺钉仍牢固在位。对患者进行定期监测，最后一次延长手术后四年零五个月进行了最新随访。
本病例报告描述了一名多骨型骨纤维异常增殖症患者MILLN的重新激活。虽然文献中先前已描述过钉子重新激活，但据我们所知，尚未有用于治疗FD引起的并发症的报道。在出现机械并发症的情况下，这种方法可以平衡腿长差异并纠正畸形，避免额外的侵入性手术并降低医疗成本。由于这是一种超说明书用药治疗，需要患者及其父母的术前同意。
