Osteogenesis Imperfecta Treatment
Samantha A. Spencer
Operative Indications
Osteogenesis Imperfecta With Significant Bowing and/or Displaced Fracture
Equipment
Fassier-Duval instrument set and assorted rods, T-handle universal chuck, Midas Rex with diamond wheel burr, box of assorted solid drill bits, osteotomes, Kirschner wires (K-wires), (18-gauge Luque cerclage wires if correcting coxa vara), open reduction and internal fixation (ORIF) set with usually small lobster claw clamps. C-arm with appropriate drapes (C-armour or half sheets for laterals).
Positioning
Bilateral femurs/tibias—supine on blanket table on flat radiolucent table. Unilateral femur or humerus—sloppy lateral with bumps, prep and drape entire extremity free with U/bar drapes for femur/humerus or extremity for tibias (Figure 29.1A and B).
Surgical Approach
Access the apex of the worst bow or fracture. Always anterolateral. For femur, OK to go through vastus if midshaft rather than more extensile lateral subvastus approach. For coxa vara/proximal third bow, standard lateral approach. For humerus anterolateral, find and dissect out radial nerve up into spiral groove and mobilize first.
Techniques in Steps
Access bow or fracture. Place lobster claw clamps proximal and distal (Figures 29.2 and 29.3)
Strip as little periosteum as necessary (usually 3-5 cm) and ensure sub periosteal dissection with baby Bennetts or big blunt Hohmanns before placing lobster claw clamps
Drill bit/osteotome osteotomy taking appropriate closing wedge and saving bone. Trim ends as needed with rongeur (Figure 29.4)
Drill proximally and distally with drill bit usually size 2.5 or 3.2 (Figure 29.5)
Retrograde a 1.6-mm guidewire out the appropriate location
a. Middle to medial aspect of trochanter for femur: leg in hip flexion and adduction
b. Anterior proximal tibial epiphysis anterior to intermeniscal ligament with the knee hyperflexed
c. Bare area: anterolateral humeral head with the shoulder extended or flexed to avoid acromion (depends on deformity)
Retrieve guidewire through 1-inch incision: medial parapatellar for tibial rods (Figure 29.6)Related posts:
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