What is the role of surgery in the treatment of osteogenesis imperfecta (OI)?

Updated: Feb 24, 2020
  • Author: Manoj Ramachandran, MBBS, MRCS, FRCS; Chief Editor: Harris Gellman, MD  more...
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Answer

Painful bony deformities and recurrent fractures are typically treated with intramedullary stabilization with or without corrective osteotomies. In children with severe forms of OI (eg, type III), rodding of lower extremities is performed to correct deformities and provide preventive protection around the time of first attempts at standing. Osteotomies should be simple, preferably single, and performed under direct vision with maximum care and gentle handling of tissues.

Because the bone is soft in OI, rods (eg, extendable Sheffield rods or Bailey-Dubow rods), pins (eg, Rush pins), and wires (eg, Kirschner wires [K-wires]) are used rather than solid nails, plates, and screws; the latter are associated with increased fracture risk above and below the device and with poor fixation.

Rod placement is of particular use in the femur and is less commonly used in the tibia, humerus, and forearm. An experienced team can perform as many as four rod procedures in the long bones of the lower extremities in a single surgical session. Fractures heal normally in about 85% of patients with OI.

In the prebisphosphonate era, extendable rods were preferred to nonextendable ones in order to prevent bone bowing and bone growth beyond the end of the rod. Bailey-Dubow rods were complicated by a high incidence of mechanical failures (eg, migration and disconnection of T-parts); accordingly, Sheffield rods and the Fassier-Duval modification are now more commonly used. The latter also has the advantage of being inserted through the greater trochanter (as in adult fixations), thus avoiding the need for a knee arthrotomy in femoral surgery.

With the decreased fragility of bone exposed to bisphosphonate, the future role of extendable rods is unclear. In long bones (eg, tibiae and radii), nonextendable rods such as Rush pins and K-wires have most often been used. Complications of rod placement include breakage, rotational deformities, and migration. Extendable and nonextendable rods are associated with similar complications; however, the rate of repeat surgical intervention is lower with extendable rods than with nonextendable rods.

The use of a dual interlocking telescopic rod, in which both the sleeve and the obturator are anchored with interlocking pins, has been described for tibial stabilization in children with OI, [41]  with results comparable to or better than those seen with a single interlocking telescopic rod. Anchoring the sleeve at the proximal epiphysis appear to provide better anchorage and allow easier removal.


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