Use of flexible intramedullary nailing in combination with an external fixator for a postoperative defect and pseudarthrosis of femur in a girl with osteogenesis imperfecta type VIII: a case report

Abstract

Telescopic rodding has been developed in order to obtain long-lasting osteosynthesis in the growing long bones of children with osteogenesis imperfecta (OI). The major and still unsolved drawback of any telescopic rod or nail design is a lack of rotational stability and, currently, no telescopic system allows immediate weight-bearing. When these problems are associated with insufficient longitudinal bone stability and diminished healing capacity, the result can be unfavourable causing secondary bone fragment displacement, delayed or non-union. This article presents a case report of operative treatment in a 9-year-old girl affected with OI type VIII complicated with postoperative defect and pseudarthrosis of the femur causing functional impairment with loss of walking ability. A combination of intramedullary flexible nailing and minimal external fixation was applied for treatment of femoral defect-pseudarthrosis in a girl of 9 years with OI type VIII. Intramedullary and extramedullary nails with wrapping of titanium nickel mesh subperiosteally provided osteosynthesis and deformity correction of the tibia of a small intramedullary canal diameter. Upright standing and walking with progressive weight-bearing was started 4 days after surgery. There were no septic or vascular complications nor complications related to Ilizarov fixator. Radiographs demonstrated bone union in the femur 46 days after surgery. At the follow-up visit 9 months after fixator removal, clinical alignment remained excellent without any relapse of deformity. Bone remodelling with restitution of medullary canal was noted on lower limb radiographs. The patient was able to stand and walk without pain with an aide or walker. At the follow-up visit 17 months after fixator removal, there was no decrease in achieved functional abilities and the treatment outcome remained satisfactory. Use of an external fixator with intramedullary nailing for treatment of postoperative pseudarthrosis in patient with severe OI (recessive form of OI, type VIII) provides longitudinal, rotational and angular stability. Furthermore, this approach ensured early functional activity and walking with full weight-bearing, both favourable conditions for bone tissue regeneration. The external fixator was applied for a short period and only for additional stability and not for progressive deformity correction or other manipulation. In addition, the combination of intramedullary and extramedullary nailing and subperiosteal titanium nickel mesh seems to be promising for osteosynthesis in the deformity correction of bones with small diameter in children with OI.

Combination of flexible intramedullary nailing and Ilizarov frame for salvage of femur and humerus nonunion in a girl with osteogenesis imperfecta

“…Whatever the exact implant design telescopic rodding carries a steep learning curve, and even in the most experienced hands has a high complication rate, partly related to the lack of rotational and longitudinal stability of the implant [6,11,14,15]. The technique is known to be even more difficult and prone to these complications in the humerus, including secondary displacement, malunion, nonunion, hardware failure and migration over time and bone growth [16][17][18][19]. This means that revision surgery in these cases is not at all unusual, and is even more challenging than primary cases requiring individual solutions as well as classic techniques [9,18,[20][21][22].…”

“…The technique is known to be even more difficult and prone to these complications in the humerus, including secondary displacement, malunion, nonunion, hardware failure and migration over time and bone growth [16][17][18][19]. This means that revision surgery in these cases is not at all unusual, and is even more challenging than primary cases requiring individual solutions as well as classic techniques [9,18,[20][21][22].…”

“…The initial treatment of this child with regards to the right femur was described in a published report [18]. Two years later the patient represented to us with a symptomatic nonunion in the other (left) femur, having been treated with primary FD rod elsewhere in 2013, and then attempted revision with exchange FD rod followed by additional titanium plate 4 months before presentation.…”

Combined technique of titanium telescopic rods and external fixation in osteogenesis imperfecta patients: First 12 consecutive cases

“…12 However, the major limitation of any telescopic system is related to its inherent rotational and longitudinal instability. [13][14][15][16] Furthermore, all telescopic systems do not allow an immediate weight-bearing in early postoperative period. 12,17,18 This inconvenience of the system does not prevent secondary bone mass reduction, disuse osteoporosis and further fractures.…”

“…In literature, the combination of telescopic flexible intramedullary nailing with an external fixator in patients with osteogenesis imperfecta or other severe underlying bone pathology demonstrated advantages of stability, early weight-bearing and rehabilitation. 9,13,16,24 The objective of the current study was to examine the outcomes of deformity correction by combined technique in children with severe and moderate-to-severe types of OI classification with a minimum 1year follow-up. The accuracy of alignment, walking ability in early postoperative period by computed gait analysis, maintain of deformity correction in one-year follow-up, telescoping of rods, complications were assessed in the study.…”