Congenital tibial deficiencies: treatment using the Ilizarov's external fixator.

INTRODUCTION: Congenital longitudinal deficiency of the tibia is a rare and often syndromic anomaly. Amputation is usually the preferred treatment option in complete absence of the tibia; however, a conservative management might be implemented in partial forms or in case of amputation refusal. Our experience with the Ilizarov fixator, convinced us this device was the best suited for progressive correction of lower limbs length discrepancies and articular or bone angular limb deformities (ALD). The aim of this study is to highlight the interest of the Ilizarov fixator in the multistage conservative treatment of congenital tibial deficiencies. MATERIAL AND METHODS: A retrospective study was conducted in nine patients suffering from Type I or II congenital tibial deficiencies (Jones) and sequentially managed using the Ilizarov technique. The functional outcome after treatment completion was then clinically assessed. RESULTS: The different stages of correction were recorded for each individual patient. Patients were assessed at a mean follow-up of 18,3 years (4-32 years). The mean maximum knee flexion was 35 degrees (0 degrees -90 degrees ) in type I deficiencies and 118 degrees (90 degrees -140 degrees ) in type II deficiencies. One patient underwent amputation and a bilateral knee arthrodesis was performed in another case. DISCUSSION: Few series in the literature report a comparable length of follow-up period in the conservative management of severe congenital tibial deficiencies. In our study, the Ilizarov fixator provided satisfactory progressive corrections of severe congenital tibial deficiencies. LEVEL OF EVIDENCE: Level IV therapeutic retrospective study.

Popliteal pterygium knee contracture: treatment with the Ilizarov technique.

INTRODUCTION: Various treatment options are in use to address severe knee flexion contractures in children. Their success depends on an adequate selection of the proper one applying to each individual anatomical situation. HYPOTHESIS: Applied to limb deformity, the Ilizarov technique combines progressive correction, to joint structures flexibility restitution in case of severe knee flexion contracture. We review a continuous series of popliteal pterygium syndrome patients managed with this technique. PATIENTS AND METHODS: Medical records of eight children (11 knees), consecutively treated between 1986 and 2007, were reviewed. Knee flexion ranged from 40 to 120 degrees. Contracture (> 90 degrees) was extremely severe in 10 cases. Progressive correction was gained by Ilizarov external fixation. Complications during and following articular chain distraction-lengthening were noted. Follow-up ranged from 1 to 21 years. RESULTS: Surgical realignment was rendered particularly complex by the popliteal cutaneous band itself, partly responsible of the joint stiffness and sciatic nerve shortening. Deformities were corrected by the Ilizarov technique. Complete extension was obtained in all cases. In six cases, flexion contracture reccurrence required to repeat the correction, using the same technique, at a mean interval of 3-4 years. During follow-up, four evolutive partial posterior tibial dislocations and one complete dislocation were diagnosed, all associated with recurrence of the flexion contracture. LEVEL OF EVIDENCE: Level IV. Therapeutic Study.

Intra-articular recurrence of benign chondroblastoma with articular involvement at initial presentation--a case report.

We report a case of chondroblastoma involving the upper tibial epiphysis, which had an unusually aggressive course, with articular involvement at initial presentation. Intra-articular and soft tissue recurrence occurred after treatment consisting of curettage and bone grafting. Although the incidence of local intraosseous recurrence of chondroblastoma is relatively high, intra-articular and soft tissue implantation is rare and is usually due to intra-articular spillage during surgery. Spontaneous articular involvement is rarely seen.

[Femoral shaft fracture in children: treatment with early spica cast]. / Fracture de la diaphyse fémorale de l'enfant: traitement par plâtre pelvi-pédieux d'emblée.

PURPOSE OF THE STUDY: Fractures of the femoral shaft are frequent in children and prognosis is good. For children aged less than 7 years, most authors propose orthopedic treatment using prolonged traction then cast immobilization, or orthopedic reduction under general anesthesia and early cast immobilization. The purpose of the present work was to analyze results of orthopedic treatment using a spica early for children aged less than 7 years in comparison with other published series with similar age or management. MATERIAL AND METHOD: Forty-seven unselected children aged less than 7 years with a closed fracture of the femoral shaft were treated by orthopedic reduction under general anesthesia and immobilization with a spica cast after a short period (<48 hr) of traction in the plane of the bed. Hip immobilization was set in flexion and minimal abduction, knee in flexion. The children were discharged the day the cast was installed or the next day. Follow-up examinations (tolerance) with x-rays cast in place were scheduled for day 8 and day 15. Clinical and radiological assessment was also recorded at removal of the cast, at 3 months, at 1 year, and at maximum follow-up. RESULTS: Mean age was 37 months (range 2-83 months). Boys predominated (n=34, 73%). Mean duration of traction was 1.13 days (range 0-5 days). Mean duration of cast immobilization was 45.4 days (range 28-78 days). Mean hospital stay was 2.8 days (range 1-10 days). Four patients (8%) were rehospitalized for secondary displacement under the cast. Weight bearing was effective at day 49 (range 33-78). Mean follow-up was 12.1 months (range 3 months-6 years). Five children (10.5%) developed leg length discrepancy greater than 10 mm. The anatomic axis of the femur in the saggital and frontal planes exhibited deviation in 7 children measuring less than 8 degrees in all cases. DISCUSSION: The localization and type of fracture observed in this series of children was similar to that reported in epidemiological studies. There were no treatment-related complications. In respect to healing time, deviation, and residual leg length discrepancy, results in this series were equivalent to those reported by others, irrespective of the treatment method employed. The major advantage of early spica is a short hospital stay allowing cost containment and rapid return to the child's everyday environment.

Upper limb lengthening.

Congenital deficiencies and developmental deformities of the upper extremity often result in complex deformities that include, to variable degrees, shortening and angulation. Because of the nonweight-bearing status of the upper extremity, these deformities are better tolerated and often of less functional significance than their counterparts in the lower extremity. The need for lengthening therefore is less common in the upper extremity than in the lower extremity. When planning a lengthening procedure to the upper limb, the surgeon must be aware of some specific indications, goals, and complications. In the final analysis, one must weigh the risk of upper limb lengthening against the benefits. In fact, there are many pitfalls, and it is not a surgery to be undertaken lightly. In the hands of experienced specialists, it can achieve excellent results. The potential complication rate is high initially, but tends to diminish with increasing experience. The authors believe that the functional, cosmetic, and psychological benefits of upper limb lengthening outweigh the risk of permanent sequelae and functional impairment in selected patients.

Scoliosis and fused ribs. A case report.

STUDY DESIGN: This case report illustrates a scoliotic patient with congenital fusion of several ribs associated with a thoracic curvature. OBJECTIVES: To report the procedure used to correct scoliosis in association with congenitally fused ribs. SUMMARY OF BACKGROUND DATA: All cases of congenitally fused ribs reported in the literature are associated with malformed vertebrae. For this reason, the only proposed treatment for patients with progressive scoliosis is a spinal fusion. This report presents the first case of progressive scoliosis associated with fused ribs, but without vertebral malformation, that was managed by resection of these ribs. METHODS: When worsening of the thoracic scoliosis was observed from 30 degrees at 13 months to 44 degrees at 4 years, the three fused ribs were resected en bloc. No spinal fusion was performed. The spine was held in correction by a localizer cast for 3 months. RESULTS: Fourteen years after treatment, the spine is almost normal; thoracic and lumbar curves are 10 degrees. CONCLUSIONS: When malformed fused ribs are on the concave side of a progressive scoliosis with no vertebral malformation at the same level, resection of these ribs is probably an efficient and sufficient method of treatment.

Evolution of the angle of obliquity of the femoral diaphysis during growth--correlations.

Numerous studies of the bicondylar angle of the adult femur have been carried out in human anatomy, paleoanthropology and primatology. The aim of this paper is to study the evolution of this angle in relation to age and acquisition of walking in young children. Seventy-seven radiographs of children, ranging from 5 months to 17 years postnatally, and of four dead newborn were analysed. The measurements concern the bicondylar angle (A.O.F.), the collo-diaphyseal angle (A.C.D.), the length of the femoral neck (L.N.) and of the femur (L.F.) and the interacetabular distance (D.I.A.). Some children were x-rayed at different ages, which permits a longitudinal as well cross-sectional study. The results show that there is no sexual dimorphism and that the increase in the angle is closely related to the age of the child. The bicondylar angle starts at 0 degree at birth and then increases progressively with growth to reach adult values of at least 6 degrees-8 degrees between 4 and 8 years postnatally. In adults, the mean values are between 8 degrees and 11 degrees and the maximum range is between 6 degrees and 14 degrees. The obliquity angle corresponds to an angular remodeling of the femoral diaphysis, which is independent of the growth and shape of the distal femoral epiphysis. The tibio-femoral angle measures the evolution of a physiologic phenomenon, from the load "in varus" to the load "in valgus" of the lower limb. It is linked with the bicondylar angle but is different from it.

Ilizarov technique in the treatment of congenital pseudarthrosis of the tibia.

This study analyzes the risks and benefits of Ilizarov's technique in congenital pseudarthrosis of the tibia (CPT). This was a retrospective review of 14 patients treated between 1985 and 1993 for CPT, by using Ilizarov's technique. In 12 cases, this technique was used after failure of previous surgical treatment. Realignment, end-to-end compression, and leg lengthening were undertaken in all the cases, without excision of the pseudarthrosis site. The mean fixation duration was 7.8 months. Union was achieved with the initial treatment in seven cases. Bone grafting was used in six of the seven remaining cases and achieved bone healing in three of them. Refracture occurred in one case, and ended with nonunion. At 3.5-year average follow-up, the tibia was united in nine cases. We found that the best indications for Ilizarov's technique in CPT were the normotrophic and hypertrophic types of pseudarthrosis (Apoil II), after the age of 5 years. Secondary massive bone grafting is to be considered in some cases. The major disadvantage of this method is the lack of excision of the pseudarthrosis site. Even after healing is achieved, the bone remains dystrophic and fragile and necessitates a permanent protective orthosis, until the end of bone growth.

[Contralateral preventive screwing in proximal femoral epiphysiolysis]. / Vissage préventif controlatéral dans l'épiphysiolyse fémorale supérieure.

PURPOSE: The incidence of bilaterality in slipped capital femoral epiphysis (SCFE) ranges, in the literature, from 19 per cent to 80 per cent. The role of contralateral pinning is to prevent late slipping of the femoral epiphysis and its complications. The purpose of this study is to assess the usefulness of routine preventive contralateral pinning in SCFE, and to evaluate its complications. MATERIAL AND METHODS: We reviewed retrospectively 74 consecutive patients treated for unilateral SCFE by associated routine preventive contralateral pinning. The age at surgery ranged from 10 years and 6 months to 16 years and 10 months. The osteosynthesis was achieved by a single cannulated holothreaded screw with a cross grooved head, with or without the use of washers. RESULTS: An accidental pin penetration was noted in 4 cases. The epiphyseal position of the screw was satisfactory in 56 cases. The patient was allowed to walk the second or third day after surgery with the use of crutches. All our patients were reviewed after the end of squeletal growth. The follow-up ranged from 2 to 12 years and 8 months. The age at which the patient was last seen ranged from 15 years and 7 months to 27 years. Two major complications were noted: a femoral fracture at the level of the screw penetration in one case, and secondary slipping of the epiphysis after premature removal of the screw in two cases. The removal of the screw was considered to be very difficult in 10 cases. A relative overgrowth of the greater trochanter was noted in 8 cases, and was of no clinical significance. No infection was noted. At last follow-up, the shape of the femoral head and the function of the hip were normal in all cases except for one hip where severe coxa vara developed because of a secondary slip after premature removal of the screw. DISCUSSION: There is a lot of controversy about the real necessity of routine preventive contralateral osteosynthesis in SCFE. In our experience this surgery succeeded in reducing the incidence of secondary contralateral slipping. The two cases in our series could have been prevented by an accurate timing of screw removal. The complications of this procedure are rare, and it could be done during the same operative time as the SCFE side's. The only case of femoral fracture was secondary to a violent car accident. CONCLUSION: The routine prophylactic controlateral osteosyntheis in SCFE, using a single screw is a safe procedure and allows to reduce the incidence of bilaterality.

[Contribution of Ilizarov's equipment in the treatment of congenital pseudarthrosis of the lower limb]. / Apport du matériel d'Ilizarov dans le traitement de la pseudarthrose congénitale de jambe.

INTRODUCTION: Risks and benefits of using Ilizarov apparatus in the treatment of congenital tibial or fibular pseudarthrosis (CTFP) are presented in this retrospective study. MATERIALS AND METHODS: We reviewed with an average follow-up of 3 years and 4 months, the outcome of twenty consecutive patients treated between 1985 and 1993, for a CTFP using the Ilizarov apparatus. Sixteen patients were treated for non union of both tibia and fibula, 1 patient for an isolated non union of the fibula, and 3 patients for correction of a previously treated, malunited pseudarthrosis. The apparatus was used in four different ways: Realignement, end to end compression, and leg lengthening in 14 cases, Simple external fixation in association with another method of treatment in 2 cases, Progressive correction of malunion in 3 cases, Progressive diaphyseal reconstruction in 1 case (fibula). RESULTS: The mean fixation duration was 7.3 months. Union was achieved with the initial treatment in 11 out of 20 cases (including the 3 cases of malunion correction). Bone grafting was used in 7 out of the 9 remaining cases, and led to bone healing in 3 of them. Five complications were encountered: deep infection in 1 case, repeated stress fracture in 1 case, repeated fracture of the pins in 1 case, malunion in 6 cases, and less than 3 cm leg length discrepancy in 4 cases. DISCUSSION: Ilizarov external fixator is an efficient solution for many cases of CTFP, in which healing did not occur with other methods of treatment. The best indication for its use are the normotrophic and the hypertrophic types of non union (Apoil II), after the age of 4 or 5. Secondary massive bone grafting is to be considered in some cases, since it can either achieve bone union or strengthen it. The major disadvantage of this method is the lack of excision of the dystrophic tissue at the non union site. So, even after the non union is healed, the bone remains dystrophic and fragile, and necessitates a permanente protective orthosis, until the end of bone growth.

Treatment of severe flexion deformity of the knee in children and adolescents using the Ilizarov technique.

We have used the Ilizarov technique for severe flexion deformity of the knee in 11 patients (13 knees) between 1986 and 1994 and have followed them up for an average of 4.1 years. The age of the patients at operation ranged from 1.7 to 18.8 years. The femoral and tibial components were connected by two anterior hinges, medial and lateral, and two posterior distraction rods. The deformity was corrected to a femorotibial lateral shaft angle of less than 20 degrees. A permanent orthosis was applied after removal of the fixator. Fractures occurred in four patients and paralysis of the common peroneal nerve in another. There was a recurrence of the deformity in four patients. At the last review all patients were able to walk on their operated leg with or without an orthosis. We have found the Ilizarov method to be helpful in correcting severe fixed flexion deformity of the knee, with relatively few complications, but the basic principles of the method must be carefully followed.

Study of the arterial vascularisation of the medial tibial condyle in the fetus.

Varus deformity of the knee is common in young children who have suffered from fulminating purpura. This study was directed at the anatomic features of the vascularisation of the upper end of the tibia that might account for such deformation. It was based on the dissection of 28 anatomic specimens prepared by injection of Indian ink into the vascular trunk. 16 specimens were diaphanised for better analysis of the intracartilaginous distribution of the vessels. The study showed that the vascularisation of the medial condyle of the tibia is poor and of terminal nature, which may explain the occurrence of ischemic growth disorders following fulminating purpura.

[Treatment of limb deformities by the Ilizarov method]. / Traitement des déformations des membres par la méthode d'Ilizarov.

INTRODUCTION: The numerous possibilities for adapting the Ilizarov apparatus allows the progressive correction of complex angular deviations, for which flat apparatus are sometimes difficult to adapt and this report describes our experience using the Ilizarov apparatus to treat axial limb deformities. MATERIAL AND METHODS: A total of 48 patients (22 girls and 26 boys aged between 2 and 18 years-old) suffering from 58 angular deformities were treated with an Ilizarov device. 40 of the deformities involved bones: 22 tibias, 13 femurs and 6 radius. The remaining 18 deformities involved joints, (17 knees and 1 elbow), 12 were total ankylosis and 6 were flexion contractures. 31 of the cases involved an isolated deformity (16 bones and 16 joints) and 27 were associated with other orthopedic problems. The cause of the deformities were either malformation or infection in most cases. In 39 cases the angular deformities were deviations in a single plane: 13 in two planes and 6 déformities were complex, involving deviation in all three planes. Correction was progressive in 49 cases and immediate in 9 cases. Unequal limb length was treated in 21 cases: 19 of these were caused by bone deformity. The apparatus should cover the entire bone segment to be corrected, from metaphysis to metaphysis. When the deformity is close to a joint, the joint should be bridged so as to stabilize the brace. The fastening of the sides of the deformity involves a maximum of three pins in two different planes. The apparatus must be absolutely rigid so as to avoid any lateral slipping or any movement of the rings relative to the segments of the limbs. The two parts of the apparatus fixed on either side of the deformity should be linked by two groups of three threaded rods with articulations at the ends. When the correction is in a single plane, it is effected around the axis formed by two threaded rods at the point of the deformity. When the deformity is major, 90 degrees or more, the rings tend to shift under the strain, and this leads to a loss of correction and cutaneous problems on the concave face. This may be avoided by fixing threaded rods to the ring, perpendicular to the plane of the deformity. For knee flexion contractures, the rods should be connected to the ring where it crosses the frontal plane passing through the femoral diaphysis. RESULTS: 48 angular deviations were completely corrected. In 10 cases the deformity persisted, but was less than 20 degrees. The deformity reoccurred in 6 of the children: in 3 cases due to the persistence of muscular imbalance, in two cases by assymetric growth, in the other case by plastic deformation on the insufficiently mineralized regenerated bone tissue formed during lengthening. In one case, the common, motor and sensor peroneal nerve was paralyzed, complicating the correction of an anterior dislocation of the knee. The paralysis occurred at the end of the correction and recovery began after 6 months. One 10 year old child, suffering from nail patela syndrome, was left with a completely immobilized elbow after treatment of a webbed, 100 degrees flexion contracture. A total of 9 epiphyseal separations (Salter I type) occurred during the correction of severe deformities, with little or no displacement, all occurred around the knee. These epiphyseal separations did not interfere with the treatment of the angular deviations in three cases, however, advantage was taken of these events to effect the intended lengthening of the bone. DISCUSSION: The Ilizarov method for correcting joint ankylosis is difficult to perform, and depends on a detailed knowledge of the apparatus and braceing system, and requires rigourous installation of the pins, ring, joints and rods. Whatever the position of the two rings in relation to each other, it is always possible to link them by a system which can be adjusted. This is not possible with other external braces which have only a single plane.

Lumbosacral agenesis. Three cases of reconstruction using Cotrel-Dubousset or L-rod instrumentation.

Three patients with lumbosacral agenesis underwent surgery to lock the lumbopelvic instability. All three patients had an unstable sitting position and a kyphotic bearing that impaired intestinal transit or hampered further colostomy or ureterostomy. Luque instrumentation with iliac fixation performed according to Galveston was used in one patient. Cotrel-Dubousset instrumentation was used for the two other patients. Autografts plus allografts provided sufficient bone for fusion without requiring lower leg amputations. Increased hip flexion was obtained after pelvic stabilization, but knee flexion contracture remained the same. All patients showed improved intestinal transit or decreased urinary infections, and two patients attained a stable sitting position without aid.

[Spinal deformities in Marfan disease]. / Déformations rachidiennes de la maladie de Marfan.

Spinal deformities are frequent in Marfan's disease. 37 patients were examined at the average age of 11 years. 32 showed a spinal deformity. The treatment of the spinal deformity was a brace in 15 cases, surgery in 16 cases (including 5 cases after brace-failure). 7 cases were just followed-up without treatment. Bracing was efficient only for mild curves, this treatment was satisfying 4 times out of 11 with adequate follow-up. The surgical treatment in 16 cases was a posterior fusion twice associated with anterior fusion. We used the same technique as for idiopathic scoliosis with Harrington instrumentation 5 times, Harrington with sublaminar wires 4 times and Cotrel-Dubousset instrumentation 7 times. The correction of scoliosis was achieved in 48.2 per cent. The use of segmental instrumentation compared with the use of the Harrington instrumentation can explain the small number of non unions and the improvement of the lateral spinal balance. We did not note any cardiovascular complication during or early after the operation. Aortic lesions were responsible of one death in the long term, three patients went through a surgical replacement of aortic valves or the aorta.

Arterial supply of the acetabulum in the fetus: application to periacetabular surgery in childhood.

The need to perform triple osteotomies of the pelvis passing very close to the articular surface leads to the isolation of bony fragments whose vascularisation may be precarious. To assess the risks of such surgery, we undertook an anatomic study of the vascularisation of the acetabulum in the fetus. 53 specimens were injected to study the vessels to the acetabulum and their distribution within the osteocartilaginous specimen. The acetabular a., a branch of the obturator a., gives a central pedicle distributed to the acetabular fossa, the triradiate cartilage and ending in the three primary bony components. The superior gluteal, inferior gluteal, internal pudendal and obturator aa. form a periacetabular vascular circle. The abundant vascularity of the acetabulum makes massive necrosis improbable in the child. However, there is a zone of precarious anastomosis at the anterior portion of the acetabulum.

[Current surgical therapy of congenital clubfoot]. / Heutige operative Therapie des angeborenen Klumpfusses.

The operative treatment of congenital club foot deformity in childhood consists of a release of soft tissues which are retracted in fibrous "knots" at the hindfoot and midtarsus. This release is more or less extensive, depending on the importance of the contracture and allows reduction of the triple deformity i.e. varus and equinus of the hindfoot and adduction at the midtarsus. Surgery is performed preferably before walking commences in order to take advantage of the functional self-treatment by bearing weight after removal of the cast. During the last several years, the different operative procedures employed for release have gained in precision and efficiency by addressing the deformity at their precise location. The difficult aim of the surgical procedure is to avoid insufficient release causing pseudo-recurrencies or overcorrection.