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Intramedullary Stabilization of the Long Bones in Children with Osteogenesis Imperfecta

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Intramedullary Stabilization of the Long Bones in Children with Osteogenesis Imperfecta CHAPTER 5.1 Intramedullary Stabilization of the Long Bones in Children with Osteogenesis Imperfecta V. Topouchian, G. Finidori, C. Glorion Since the initial description by Sofield [20] of in- has been adequate with no significant deformities tramedullary nailing of long bones for patients of long bones at adulthood, functional prognosis with osteogenesis imperfecta, palliative stabiliza- will be reasonable and these patients will have a tion has not been contested. The concept of frag- nearly normal existence. mentation, realignment and intramedullary rod fixation is an essential contribution in the man- agement of long bone deformities. Segmental os- Aims of Osteosynthesis teosynthesis in a delicate bone structure remains questionable. Fragmentation by multiple osteo- Surgery must provide effective protection against tomies, realignment and intramedullary rodding bone fragility; it should prevent long bone bowing has been subject to multiple technical improve- and reduce the total number of fractures. ments, especially in children. The most significant Osteogenesis imperfecta can be considered as advances were: two separate ailments, constitutional and ac- · The development of Bailey-Dubow extensible quired. Genetic anomalies induced by the muta- rods [2, 3]. tion of genes coding for collagen synthesis [6, 7, · The expansion of intramedullary bipolar nail- 14] are the basic reason for initial osseous fragili- ing with two bowed Kirschner wires described ty. Multiple fractures, bone distortion and fre- by Metaizeau [16]. quent and long immobilizations contribute to sec- · Recent advances are due to the development of ondary osteopenia and a tragic worsening of the closed intramedullary pinning techniques in disease. It is essentially to improve this secondary children [17, 18]. pathology that treatment and particularly surgery may be of use. Surgical progress accomplished in the last three decades has occurred within the framework of a better pluridisciplinary management of the patho- Surgical Methods and Techniques logy. Nowadays, improvements in rehabilitation techniques, casting, anesthesiology and bisphos- Intramedullary rodding was first described by So- phonate therapy have profoundly modified the field with a large subperiosteal exposure of bone prognosis of patients with osteogenesis imperfecta. shaft, and numerous osteotomies, and realign- Kypho-scoliosis and respiratory restriction at ment has been much improved. Less aggressive adulthood is being managed by early surgical techniques, with limited surgical approaches and treatment for kypho-scoliosis and respiratory subperiosteal exposures are preferred. physiotherapy, especially in severe cases. Bailey and Dubow introduced extensible intra- Living within the community with the support medullary fixation devices to diminish the need of associations [22] has contributed to better for reoperation due to bone growth. Their tele- management of these patients and their family scoping rod can be elongated by being anchored members. These associations are absolutely neces- to the epiphysis without damage to bone growth sary to provide better information for patients plate [2, 3]. Since their initial description, Bailey- and the medical professionals. Dubow extensible rods have been considered to Prenatal screening tests have considerably re- be the best way to treat growing children suffer- duced the number of severe forms of osteogenesis ing from deformity or multiple fractures. Tele- imperfecta frequently encountered in the past. Pa- scopic rods comprise a sleeve portion with a de- tients with moderate forms have less severe bone tachable T-piece that can be screwed on at its ex- fragility at maturity. If treatment during infancy tremity; the second component is the inner ob- 220 V. Topouchian et al. turator with an incorporated T-piece at its ex- planes (Fig. 5.1.1). Classically the medullary canal tremity. This inner rod can telescope totally into is drilled toward the greater trochanter using a the sleeve. The T-shaped ends are anchored into detachable drill bit screwed into the tubular sleeve the epiphyses, and with longitudinal bone growth of the rod until it impinges on the cortex at one the telescoping rods elongate. Special instrument site of the curve. Osteotomy is carried out at this sets comprising a detachable drill and two nail site. It is better to use long and stiff drills rather guides are necessary. than the sleeve part of the rod. This procedure is Imaging in the anteroposterior, lateral and repeated until all shaft incurvation is corrected. maximal deformity planes is used to plan the sites The outer tubular sleeve of the device (with the of osteotomies and determine the length and T-piece detached) is driven into the upper femoral width of the bone in which the device is to be in- segment using a retrograde approach, through the serted. It is not advisable to cut the sleeve at the medullary canal, exiting on the upper-lateral as- time of operation since this could produce distor- pect of the femoral neck medial to the greater tro- tion of the tube and loss of the sliding mecha- chanteric notch. Through a separate small inci- nism. Rods are prepared and cut by the manufac- sion the upper end of the tubular sleeve is pushed turer as previewed by the surgeon using the pre- out through the skin, the T-piece is screwed in operative views. and tapped into the superior aspect of the femoral neck. The inner obturator rod is driven upward through the intercondylar notch of the femur and Femoral Intramedullary Telescopic Rodding fitted into the outer sleeve. The T-shaped end is tapped into the osseous part of the lower femoral Osteotomy and rod insertion can be carried out epiphysis. The surgical approach to the bowed as has been initially described [2, 3, 9, 10]. The bone uses subperiosteal exposure. The deformed entire lower limb should be prepared and in- bone is osteotomized into the appropriate number cluded in the operating field. Blood loss can be of fragments so that they can be aligned for inser- minimized with the use of an Esmarch band, tion of the rod. If femoral bowing is considerable, especially during the distal femoral approach. In- bone shortening may be necessary to ensure tramedullary reaming is performed starting at the proper lining of the femoral shaft. To avoid this intercondylar notch of the femur using an antero- drawback it is best to rod earlier before severe in- lateral approach and a parapatellar arthrotomy. curvations occur. Infratrochanteric valgus osteoto- The rod direction is perpendicular to the femoro- my is performed to avoid secondary varus bend- tibial space in the frontal and anteroposterior ing of the upper femoral extremity (Fig. 5.1.2). Fig. 5.1.1. Femoral telescopic rodding with Bailey-Dubow rods. a Preoperative radio- graph; b 3 months and c 4 years postoperatively Chapter 5.1 Intramedullary Stabilization of the Long Bones in Children with Osteogenesis Imperfecta 221 Fig. 5.1.2. Femoral bowing in a 4-year-old boy despite bisphosphonate treatment (distal sclerotic growth lines are produced by intravenous bisphosphonate treatment). a Initial radiograph. b Im- mediate postoperative radiograph. c At 2.5 years postoperatively with satis- factory extension of the rods After the wounds are closed, the patient is im- mobilized, usually for 4 weeks, in a hip spica cast. Later on a rehabilitation program for upright pos- ture and walking is established. Percutaneous Femoral Telescopic Rodding With these positive developments in technical and medical treatments, severe bone deformities with considerable bowing are less frequently encoun- tered. It seemed possible to consider less invasive modalities of surgical treatment. F. Fassier [8] de- veloped a new intramedullary rod and instrument Fig. 5.1.3. Percutaneous femoral telescopic rodding with a set to provide percutaneous femoral telescopic midline incision through the patellar ligament rodding. In our institution we have developed an original technique of percutaneous femoral tele- scopic rodding. We use the original Bailey-Dubow trochanter, and its upper end is driven out of the implant because of its proven mechanical charac- skin through a separate small incision. The tubu- teristics and low cost advantages. A special instru- lar sleeve of the device is attached to the distal ment set has been developed for percutaneous part of the drill and pushed upward with a metal rodding. A long stiff drill with a diameter two- guide till its proximal extremity comes out of the tenths greater than the rod to be inserted is intro- skin. The inner obturator rod is driven upward duced percutaneously with a midline short skin through the intercondylar notch and into the tu- incision through the patellar ligament with the bular sleeve. The T-piece is screwed in and tapped knee flexed to 908 (Fig. 5.1.3). Shaft reaming is into the upper femoral epiphysis. The T-shaped performed upward starting at the intercondylar end of the obturator rod is tapped into the distal notch with fluoroscopic control. Multiple osteo- epiphyseal bone under fluoroscopic control. Post- tomies can be performed percutaneously using operative care and immobilization are the same the stamping technique and osteoclastic correc- as for the initial classical technique. Depending tion. As for classical rodding, infratrochanteric on difficulties with the osteotomy, short surgical valgus osteotomy is performed to achieve a valgus approaches may be necessary, especially at the position of the upper femoral extremity (Fig.
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