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. upper part of the shaft where bowing is often 5.1.4). Once the endomedullary canal has been considerable. This percutaneous femoral tele- prepared and the different osteotomies have been scopic rodding is only a variant of the traditional performed, the drill is pushed toward the greater technique. 222 V. Topouchian et al.

Fig. 5.1.4. a Incorrect position of the proximal end of the rod with persistent varus of the femoral neck in a 12-year-old girl. b±d Correct positioning after valgus osteotomy for the same patient

Tibia Telescopic Rodding lar ligaments are sutured. If multiple osteotomies are performed, prophylactic anterior compartment Tibia telescopic rodding is performed in the same fasciotomy may be appropriate to avoid compart- way and follows the same principles as femoral rod- mental syndrome. Special care must be taken not ding. The proximal tibia epiphysis is exposed with to induce rotational anomaly with cast immobiliza- a short arthrotomy allowing drill insertion at the tion. An above-the-knee cast is applied for 3±4 base of the anterior cruciate ligament. As for the fe- weeks postoperatively. A short immobilization is mur, the rod direction is perpendicular to the fe- essential to avoid secondary osteopenia. morotibial space. In patients with a genu recurva- tum, a flexion osteotomy is performed at the prox- imal tibia segment. Reaming is performed down- Humeral Telescopic Rodding ward with a long drill. Multiple shaft osteotomies are performed. No surgical approach is necessary Humeral rodding principles are the same as de- for the fibula; simple osteoclastic correction is suf- scribed above. The whole upper limb is prepared ficient. The ankle joint is exposed through a large and included in the operating field. An Esmarch anterolateral incision and the talus is dislocated band may be used during the distal humeral medially and posteriorly. The long drill is pushed approach. With a posterolateral incision, the elbow downward through the medullary canal and lower joint is exposed. The drill is introduced at the lateral distal epiphysis. A drill guide and a spatula allow margin of the trochlea perpendicular to the elbow for better positioning of the drill at the center of joint area. Diaphyseal osteotomies are performed the ankle joint cartilage. It would be a technical er- using a lateral humeral approach, taking care to ror to use an anteriorly positioned insertion point avoid the radial nerve. Osteotomies are carried of the rod at the distal tibia end. Since the long drill out through small incisions with limited subperios- is more rigid than the definitive sleeve part of the teal exposure [5, 15, 21]. The upper end of the tu- rod, reaming is performed with no distortion, bular sleeve is driven through the proximal epiphy- and the sliding between the two parts of the rod sis and pushed out of the skin anteriorly to the ac- is not impaired. The T-piece is screwed in and romion. The inner obturator rod may be equally in- the sleeve is tapped into the cartilage at the distal serted at the proximal or distal segment of the hu- end of the tibia in the subchondral region. The in- merus. T-pieces are tapped into the bone epiphysis. ner obturator is driven downward and engaged into As for the femur and the tibia, care should be taken the sleeve, its T-shaped end is tapped into the prox- not to induce torsion anomaly during cast or ban- imal tibia epiphysis. The arthrotomy and tibiofibu- dage immobilization, usually applied for 4 weeks. Chapter 5.1 Intramedullary Stabilization of the Long Bones in Children with Osteogenesis Imperfecta 223 Telescopic Intramedullary Pinning

This technique is more and more frequently used. Pinning is often carried out percutaneously and surgical procedure if necessary is limited. The im- plantable material is not expensive. Pins can be put in place with epiphyseal anchorage in a tele- scopic mode allowing for long-lasting protection against bowing and fractures during child growth.

Femoral Telescopic Pinning

As for telescopic rodding, the first pin is intro- duced at the intercondylar notch of the distal femoral epiphysis. This pin is pushed upward into the neck of the femur. The second pin is posi- tioned downward through the greater trochanter. Usually this pinning is carried out percutaneously. A small surgical procedure may be necessary for osteotomies, especially the valgus osteotomy of the proximal extremity of the femur (Fig. 5.1.5).

Fig. 5.1.5. a, b Anteroposterior and lateral radiographs of a femoral telescopic pinning. c Erroneous anchorage of pin extremities into the epiphysis, with protrusion into soft tis- sue limiting joint mobility

Fig. 5.1.6. a A 4-year-old girl who had type III osteogenesis telescopic intramedullary pinning. c Same patient 2 years imperfecta. Preoperative radiographs. b Anteroposterior later demonstrating growth of the tibia, no recurrence of and lateral views of the same patient 6 months following deformity and elongation of pins 224 V. Topouchian et al.

Tibia Telescopic Pinning effect. As previously described for telescopic hum- eral rodding, multiple osteotomies are performed Tibia telescopic pinning is a simple and attractive if necessary. Special care must be taken to prevent technique usually performed percutaneously. The varus deviation of the elbow and internal rotation first pin is inserted through the medial malleolus of the brachial segment. and directed upward into the proximal metaphy- sis. The descending pin is introduced in the pre- spinal space downward as far as the distal meta- Forearm Telescopic Pinning physis. Into each epiphysis a pin is bent and tapped to obtain a telescopic effect. Whenever the Multiple fractures and significant bowing of the femur and tibia need to be operated on it is tech- radius and the ulna require realignment and os- nically more appropriate to start osteosynthesis of teosynthesis. Telescopic rods have a wide diame- the tibia first, to protect the synthesis with a thick ter; they are not suitable for forearm osteosynthe- bandage and then to operate the femur (Fig. sis. Intramedullary pinning remains the most 5.1.6). suitable technique (Fig. 5.1.8).

Osteosynthesis of the Ulna: the pin is introduced Humeral Telescopic Pinning through the olecranon. Osteotomies are performed, depending on the degree of bowing and the pin is This technique is simple and effective (Fig. 5.1.7). driven in as far as the distal metaphysis. The prox- The first pin is introduced laterally through the imal part of the pin can be curved into a ªZº shape lateral condyle and driven up the intramedullary and stuck into the proximal epiphysis to have a se- canal until just beneath the proximal epiphysis. cure purchase and to prevent the pin from sliding The second pin is introduced through the proxi- (Fig. 5.1.9). For patients with an almost inexistent mal humeral epiphysis percutaneously anterior to medullary canal, the periosteum of the osteoto- the acromion and directed downward as far as mized segment is gently split longitudinally, the the distal metaphysis. The extremities of the pin pin is placed underneath, and then the periosteum are bent and stuck into the epiphyseal bone to is sewn up again. Gradually the pin is pushed for- prevent the pin sliding and impairment of joint ward as far as the distal end of the ulna. This meth- mobility. Epiphyseal anchorage allows a telescopic od ensures maintenance of the position without creating a solid osteosynthesis. The ossification of the periosteum will progressively incorporate the nail into the bone shaft. Pseudarthrosis of the ulna may produce disparity of bone length, with short- ening of the ulna, and as a result limit rotational movements. For pseudarthrosis of the ulna, it is best to advise against operation. Secondary pseu- darthrosis may follow radial head dislocation espe- cially with hypertrophic callus formation. In such situations there is no hope of putting the radial head back into position; its excision is the best pal- liative operation.

Osteosynthesis of the Radius: radius osteosynthe- sis is difficult. The pin is introduced upward until it reaches the radial head. The pin must be bent to follow the normal curve of the radius. A straight radius impinges on the movement of the radioulnar joint and limits prono-supination. Os- teosynthesis of the radius constitutes invasive sur- gery for the wrist joint. We prefer, whenever skel- etally mature or nearly mature patients need no more forearm telescopic pinning, to carry out a solid osteosynthesis of the ulna with an intramed- Fig. 5.1.7. a Preoperative and b postoperative radiographs showing humeral telescopic pinning. Note pin extremities ullary nail and multiple osteotomies of the radius bent and stuck into the epiphysis with no synthesis. Chapter 5.1 Intramedullary Stabilization of the Long Bones in Children with Osteogenesis Imperfecta 225

Fig. 5.1.8. Important deformity of both forearm bones. a Clinical preoperative and b, c final postoperative appearance. d Preoperative and e postoperative radiographs showing improved alignment

Technical Problems of Osteosynthesis

Limb surgery in patients with osteogenesis imper- fecta is very tricky and requires appropriate tech- niques, particularly in severe cases. Bleeding may be limited with the use of an electrocautery dur- ing the surgical procedure and an Esmarch band. Fig. 5.1.9. Postoperative radiograph of the forearm. Note proximal part of the pin curved into a ªZº shape and stuck Fasciotomy is recommended whenever multiple into the proximal epiphysis of the ulna osteotomies are practiced. For severe curvatures, 226 V. Topouchian et al.

is too thin, it will not provide sufficient protec- tion to prevent fractures and bowing. Using too thick material will induce a stress shielding phe- nomenon with bone resorption and cause second- ary fractures to the bone and intramedullary de- vice. The intramedullary telescopic rods most fre- quently used for young patients in our institution have diameters of 3.5, 4 and 4.5 mm. The pins usually used are of 2, 3 and 3.5 mm in diameter. It is rarely necessary to use larger material except for adult patients. Generally, the osteosynthesis materials are not removed, and if they cannot be left in place, they must be replaced by a more ap- propriate device depending on patients' age and their degree of bone fragility. It is preferable to remove too thick rods if they induce cortical re- sorption and replace them with thinner and more flexible pins ensuring bone protection. This pin replacement is generally carried out once skeletal growth is achieved. Lower limb length discrepancy is frequently encountered in osteogenesis imperfecta patients, particularly in severe forms of the disease. This may be avoided by limiting the shortening to the osteotomy site, especially in patients with signifi- cant bowing of long bones. Shortening osteo- tomies are not performed to correct length differ- ence in this particular pathology because further fractures may still modify lower limb length. We have not attempted limb lengthening even in pa- tients with moderate forms of osteogenesis imper- fecta. This is to be taken into consideration in the future, particularly with the use of elongating Fig. 5.1.10. Anteroposterior femoral radiograph showing rods. bone resorption at femoral shaft due to a too large rod Pelvis deformity is frequent in osteogenesis im- perfecta, especially in severe forms (type III of Sillence) [19]. Acetabular protrusion leads to hip osteotomies may be carried out first. Correction joint motion limitation and to an invalid posture of the distortion without limb length shortening (Fig. 5.1.11). Pelvis deformity with sacral distor- is obtained by progressive axial limb traction. tion and acetabular protrusion occur progres- Once the bone segments are aligned, osteosynthe- sively and are worsened once spinal fusion has sis is carried out. been achieved and both femurs are nailed. At the Reaming of the medullary canal and nail inser- present time we have no solution to prevent this tion are usually carried out manually. Motorized complication. drills may induce bone cortex burst and growth Femoral neck fractures are frequently seen in plate damage. During surgery, care must be taken patients with acetabular protrusion. Osteosynthe- to avoid fractures in the operated limb, especially sis of the femoral neck is impossible if the hip of the femoral neck. If some of the osteotomized has severe limitation of motion and it is prefer- segments are very compact with no medullary ca- able to let pseudarthrosis become established. nal, pins may be slid under the periosteum and This will cause better and painless hip mobility maintained in position with wires and periosteal even with patients who can walk. suture. Periosteal ossification will gradually incor- porate the pin into the medullary cavity. The choice of pin or rod diameter to be in- serted is made according to contradictory require- ments (Fig. 5.1.10). If the osteosynthesis material Chapter 5.1 Intramedullary Stabilization of the Long Bones in Children with Osteogenesis Imperfecta 227

Fig. 5.1.11. Right femoral neck fracture in a patient operated from bilateral fem- oral rodding and spinal fu- sion. Note acetabular pro- trusion and pelvis defor- mity

Treatment Indications more aggressive surgery is necessary, with short- ening osteotomies. Patients and their family must be informed about Most often, fortunately, telescopic rodding and how the treatment will proceed and they must osteotomies can be carried out percutaneously in support the treatment program. Multidisciplinary patients with satisfactory general treatment and management and coordination between physicians as long as surgery is performed before the onset and the associations dealing with osteogenesis of severe deformities. Choosing between tele- imperfecta are necessary for patients and their scopic rods and pins may depend on the surgeon's families to achieve the best medical and surgical experience and also on the availability of material. results. Pins are less expensive and widely available. Tele- The primary concern of the orthopedic surgeon scopic pinning is more appropriate than rodding is to reduce fracture recurrences and to limit for tibia osteosynthesis since it is easier to insert bone bowing. and is less aggressive and impairing to the knee Until walking capacities are acquired, surgery and ankle joints. Humeral and forearm distortions is rarely necessary, although in severe forms with are frequently seen with severe forms of osteogen- significant bowing in very young patients osteo- esis imperfecta and especially in patients with hy- synthesis may be essential for the well-being of pertrophic callus [13]. These cases are best man- the patient. Bone protection will make nursing aged with multiple osteotomies and telescopic easier and will reduce pain and fracture recur- pinning. Upper limb curvatures must be operated rences. Telescopic rodding whenever possible is on before significant deformities make surgery the preferred mode of osteosynthesis for the fe- more difficult. In young adult patients this sur- mur since it affords effective long-lasting protec- gery will lead to frequent complications, such as tion. Pins in young patients with severe forms are delayed bone healing and pseudarthrosis. less effective and may migrate through the corti- cal bone. When walking is acquired, protective osteo- Recurrent Surgery synthesis should be practiced particularly in the of Intramedullary Osteosynthesis femur. Delaying osteosynthesis is not a good idea since bone bowing will increase and secondary The occurrence of fractures despite intramedul- bone demineralization will be exacerbated due to lary osteosynthesis is a frequent event. Fractures repeated immobilizations. If that happens, surgery may be the result of persistent bone fragility and/ will be technically more difficult. In these condi- or insufficient osteosynthesis. Fractures may also tions percutaneous surgery cannot be considered: occur following a severe trauma in patients who 228 V. Topouchian et al. have achieved a fair surgical result. In many cases eal purchase and fractures. These complications osteosynthesis prevents fracture displacement and justify reoperation if they impair the possibility simple orthopedic immobilization can be enough of verticalization and walking capacities. to ensure bone healing. Occasionally bone frac- Delayed bone healing and pseudarthrosis are ture is associated with rod or pin bending; in exceptional in young patients with osteogenesis such situations external manual correction avoids imperfecta. They can result from an inappropriate open surgery. If bending is considerable or the in- osteosynthesis. Bone healing can be more problem- tramedullary device is fractured, open surgery atic in older patients. will be necessary. Replacement of osteosynthesis The onset of epiphysiodesis with premature in a fragile bone is often difficult. If fractures oc- growth plate closure is not frequent. In Bailey-Du- cur despite intramedullary osteosynthesis, this bow rods a lack of sliding of the telescopic device may be the result of technical errors or due to a generally does not lead to growth plate damage. real and major trauma. The same thing is observed for the pins. Although Inadequate lining of lower limbs, with small a limited amount of epiphysiodesis is observed in anteroposterior, lateral or rotational distortions patients who have been operated upon, it is not are well tolerated in children and they do not re- clear that this is due to the telescopic device. quire immediate reoperation, which can be post- Some nonoperated patients have spontaneous pre- poned till skeletal maturity is achieved. Surgery mature closure of the growth plate, probably be- can be necessary in growing children if residual cause severe bone fragility also concerns the deformities compromise walking possibilities or growth structure, which may also be impaired. they induce exacerbated risks of fracture. Surgery and Bisphosphonates Complications Bisphosphonate treatment was initially reported Complications are not frequently encountered; by F. Glorieux [11, 12]; since that time it has been they depend largely on the experience of the sur- widely used for children suffering from bone fra- geon. gility due to osteogenesis imperfecta. Bisphospho- Operating complications are mainly technical nates are synthetic analogs of pyrophosphate and difficulties due to bone fragility. There is a risk of are potent inhibitors of bone resorption. In co- additionally induced bone bursts. Bone manipula- hort studies, bisphosphonates have shown their tion must be carefully managed using nonaggres- effectiveness in improving bone mass in children sive instruments. Excessive bleeding can be with severe forms of osteogenesis imperfecta [1, avoided by using an Esmarch band as a tourni- 4]. This medical treatment seems to be effective quet; the surgical approach will be carried out since the pain is reduced, the bone is more dense using an electrocautery. After intramedullary dril- and the conditions for physiotherapy are im- ling of bone segments a guide is left in the med- proved. Although the bone mass is increased, ullary canal to limit bone marrow bleeding. In skeletal plastic bone distortion and fractures are case of multiple surgical procedures, while operat- still encountered. Surgery with multiple osteo- ing on one site, the other wounds are closed with tomies and intramedullary osteosynthesis is still a slightly compressive bandage. required with different technical problems. The Compartmental syndrome and nerve palsy can bone is more compact, drilling requires the fre- be prevented by a nontraumatic technique with quent use of a motorized drill, the shafts are less prophylactic fasciotomy and the correction of se- slender. In such situations it is often possible to vere deformities avoiding excessive traction with use osteosynthesis of a larger diameter. elongation of vasculo-nervous bonds. The osteo- genesis imperfecta fragile bone is well vascular- ized with very thin cortices and has a better resis- Conclusion tance to infection. Although this fact is not re- ported in the literature, the incidence of postoper- At the present time, better management of pa- ative infection seems particularly low in our ex- tients with osteogenesis imperfecta thanks to co- perience. ordination between physiotherapy programs, bis- Axial and rotational abnormalities are second- phosphonate treatment and surgery leads to an ary to intraoperative misalignment, and a lack of improved functional outcome. correct postoperative immobilization. Secondary In patients with moderate forms of osteogen- deviation can result from a noneffective epiphys- esis imperfecta (types I and IV of Sillence), if Chapter 5.1 Intramedullary Stabilization of the Long Bones in Children with Osteogenesis Imperfecta 229 treatment during infancy has been adequate with 6. Cole WG. Etiology and pathogenesis of heritable con- no major deformities of long bones in adulthood, nective tissue diseases. J Pediatr Orthop 1993; 13(3): 392±403. the functional prognosis will be good, and they 7. Eyre DR. Concepts in collagen biochemistry: evidence can have normal autonomy and a nearly normal that collagenopathies underlie osteogenesis imperfecta. existence. Clin Orthop 1981; 159:97±107. 8. Fassier F. Ostogen se imparfaite de l'enfant. Confr- In severe forms (type III of Sillence), the prog- ences d'enseignement 1999, pp. 235-252. In: Cahiers nosis is now improved with bisphosphonate treat- d'enseignement de la SOFCOT. Expansion Scientifique ment, intramedullary long bone stabilization and Franœaise, Paris, 1999. 9. Finidori G. Treatment of osteogenesis imperfecta in particularly with the treatment of severe kypho- children. Ann NY Acad Sci 1988; 543:167±169. scoliosis and prevention of respiratory restric- 10. Finidori G. Ostogen se imparfaite. Indications thra- tions. Major growth disturbances can cause severe peutiques chez l'enfant. Confrences d'enseignement 1988, pp. 327±345. 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Modern trends in internal fixation of femo- growth resulting in an extensible nail. Surg Forum ral shaft fractures in children. A critical review. J Pe- 1963; 14:455±458. diatr Orthop B 1997; 6(2):117±125. 3. Bailey RW, Dubow HI. Evolution of the concept of an 19. Sillence D. Osteogenesis imperfecta: an expanding pa- extensible nail accommodating to normal longitudinal norama of variants. Clin Orthop 1981; 159:11±25. bone growth: clinical considerations and implications. 20. Sofield HA, Miller EA. Fragmentation, realignment, and Clin Orthop 1981; 159:157±170. intramedullary rod fixation of the deformities of the 4. Bembi B, Parma A, Bottega M, Ceschel S, Zanatta M, long bone of children. A ten year approval. J Bone Joint Martini C, Ciana G. Intravenous pamidronate treatment Surg 1959; 42-A:1371. in osteogenesis imperfecta. J Pediatr 1997; 131(4):622± 21. Wilkinson JM, Scott BW, Clarke AM, Bell MJ. Surgical 625. stabilisation of the lower limb in osteogenesis imperfec- 5. Cole WG. Early surgical management of severe forms of ta using the Sheffield Telescopic Intramedullary Rod osteogenesis imperfecta. Am J Med Genet 1993; 45(2): System. J Bone Joint Surg Br 1998; 80(6):999±1004. 270±274. 22. Association de l'Ostogen se Imparfaite. BP 075. F 80082 Amiens Cedex 2, France. www.aoi.asso.fr