F. Chotel

Induced-membrane femur reconstruction after resection of bone malignancies: Three cases of massive graft resorption in children

Bone reconstruction after surgical resection of bone malignancies in children remains a difficult challenge. Induced-membrane reconstruction as described by Masquelet et al. was originally reported in traumatic or septic bone defects and is now adapted to this field. We report here three cases of massive femoral graft resorption requiring surgical revision in two boys aged 3 and 6 years and a 9-year-old girl. Hypotheses include the long delay between the two stages, nature of the bone graft, high varus loads specific to this location, and lack of stability of the fixation. This technique has recently provided promising preliminary results when applied to the field of bone tumours. However, reconstruction of the femur seems to be specifically associated with a risk of graft resorption. Identification of the origin of this major complication is needed to amend the technique or its indications.

Induced membrane technique for reconstruction after bone tumor resection in children: a preliminary study.

AIM Segmental long-bone defect due to tumor resection remains a challenge to treat. The induced membrane technique is a new alternative for biological reconstruction. During the first stage, a cement spacer is inserted after bone resection and stabilisation. The cement spacer is removed during a second stage procedure performed after chemotherapy, and cortico-cancellous bone autograft was placed in the biological induced chamber. The aim of this study was to assess preliminary results in eight children. PATIENTS AND METHODS This prospective study included six girls and two boys, with a mean age of 12.1 years (range 9.5 to 18) and treated for a mean 15 cm defect (range 10 to 22 cms) due to resection of osteosarcoma (n=4), Ewing sarcoma (n=3) and low grade sarcoma. All patients except one, were given pre- and postoperative chemotherapy. Surgery was performed for three patients with a distal femur tumor, two patients with a proximal tibial tumor and three patients who had proximal humerus, shaft of humerus and fibular tumors. Fixation was mainly performed with locking compression plate (n=4) and locked nail (n=2). The mean operating times for first and second step procedures were 4.8 and 4h respectively. The healing process was radiologically assessed. RESULTS After a mean follow-up of 21.6 months (15 to 30), all patients were free of disease and seven had bony union. For the lower limb reconstructions, full weight bearing was possible after a mean of 116 days (range 90 to 150) following the second stage. Mean time to bone union was 4.8 months (1.5 to 10). The early Musculoskeletal Tumor Society (MSTS) score was 25.2/30 (range 20-30). Complications were: non-union (n=1), paradoxical graft resorption (n=1) requiring graft revision. CONCLUSION This two stage procedure reduces the operating time during the first stage and it also reduces early complications. Rapid bone union is objectively obtained despite major bone resection and the patients receiving chemotherapy. SIGNIFICANCE The induced membrane technique could be an excellent alternative for biological reconstruction after tumor resection in children.

Aneurysmal bone cysts of the pelvis in children: a multicenter study and literature review.

The authors analyzed a series of 15 pelvic aneurysmal bone cysts (9 boys and 6 girls) in children and adolescents who were reviewed with an average follow-up of 50.3 months. Pain and limp were the main symptoms. Four patients had no treatment after the open biopsy. Eleven patients were treated with curettage. Preoperative selective arterial embolization was performed in three cases before curettage. Two recurrences were noted after curettage; recurrences were treated successfully with further curettage. As a result, the authors recommend curettage; more aggressive operative intervention does not appear to be indicated. No major intraoperative vascular complications occurred. Spontaneous healing in a few cases (even in active or aggressive lesions) argues for clinical and radiologic observation after biopsy when possible. In case of a propitious evolution, observation must be continued and surgery might be avoided, but if the lesion increases, treatment must be proposed.

Décollement épiphysaire du grand trochanter de l’enfant et proposition d’une classification: À propos de deux cas et revue de la littérature

Avulsion fractures of the greater trochanter are very rare in children. We report two such cases which led to femoral head necrosis. Based on these two cases and an extensive review of the literature, we discuss the pathophysiology of this complication and propose a new classification system. Three types of lesions can be identified as a function of the mechanism causing fracture. Type 1 lesions are avulsion fractures of the greater trochanter secondary to acute contraction of the gluteus muscles. This contraction produces a vertical displacement of the greater trochanter. Femoral head necrosis has never been reported as a complication after this type of fracture mechanism. Type 2 avulsion fractures are associated with fracture of the femoral neck with a subsequent risk of femoral head necrosis. Type 3 associates hip dislocation with apophyseal avulsion with, according to the literature, an inevitable progression to head necrosis. The two cases reported look identical with those described by Linhart and Kawenblum illustrate type 3 avulsion fractures of the greater trochanter.Resume La fracture separation de l’apophyse du grand trochanter chez l’enfant est une entite rare. Les auteurs rapportent deux cas ayant conduit a une necrose de la tete femorale. La physiopathologie de cette complication est discutee au travers d’une revue exhaustive de la litterature sur le sujet. Une nouvelle classification basee sur le mecanisme traumatique de ces lesions est etablie. Le type I represente les fractures avulsion du grand trochanter par contraction brutale des muscles gluteaux. Cette contraction est a l’origine d’un deplacement vertical du grand trochanter. Aucune necrose de la tete femorale n’a ete rapportee apres un tel mecanisme. Les fractures avulsion de type II sont les lesions associees a une fracture du col femoral. Dans cette situation, le risque de necrose de la tete femorale semble lie a la fracture du col elle-meme. Les lesions de type III associent luxation de hanche et decollement apophysaire du grand trochanter ; l’analyse de la litterature suggere dans ces cas une necrose quasi ineluctable de la tete femorale. Les deux cas que nous rapportons sont superposables a celui decrit par Linhart ainsi que celui de Kawenblum car, bien qu’isoles, ils repondent tous a un mecanisme traumatique de type III.

Vascularised fibula or induced membrane to treat congenital pseudarthrosis of the Tibia: A multicentre study of 18 patients with a mean 9.5-year follow-up

BACKGROUND The objective of this study was to compare outcomes of two surgical techniques used to treat congenital pseudarthrosis of the tibia (CPT), the induced membrane technique (IM) and the transfer of the contralateral vascularised fibula (VF). HYPOTHESIS The IM technique produces similar outcomes to those of VF grafting in terms of healing and function, while being simpler and having a lower complication rate. MATERIAL AND METHOD This retrospective multicentre study included 18 patients with a mean age of 2.8 years at surgery. Among them, 11 had neurofibromatosis type 1 (NF1). The IM technique was used in 10 patients and VF grafting in 8 patients. Mean follow-up was 9.5 years (range: 5-15 years). RESULTS The two groups showed no significant differences for healing or the occurrence of complications such as limb length discrepancy and residual malalignment. Two patients required amputation, one in each group. The mean number of surgical procedures per patient was 4.7 in the IM group and 5 in the VF group. DISCUSSION Outcomes are similar with the two techniques. Although VF grafting theoretically involves a single stage, the mean number of surgical procedures was not lower than after the IM technique. The IM technique was associated with lower risks of complications and residual donor site abnormalities. Regardless of the reconstruction technique, the quality of the initial bone resection and internal fixation, particularly regarding alignment, is of the utmost importance. LEVEL OF EVIDENCE IV, comparative retrospective study.

Early surgical anterior release for congenital and isolated elbow contracture in flexion: A case report of a 16-month-old child

Isolated congenital elbow contracture is a rare upper-extremity disorder and there are few data about management of this condition. Authors report their experience after aggressive management of children with isolated congenital elbow contracture in flexion. Because of total absence of range of motion (ROM) improvement despites physical therapy (ROM 90-120°) and bone deformity, an anterior surgical release of the elbow was performed through an extensive lateral approach, at sixteen months of age. After surgery, this child was treated by three casts at maximal gained extension followed by sequential Turnbuckles splints. After five years of follow-up, the result was excellent with ROM 5-135°, normal function and absence of growth disturbance. The limiting factor of this protocol was excessive traction in elbow extension on the neurovascular structures, especially the radial nerve. This treatment represents an aggressive management with multiple general anaesthesia, but was found to be a valid option.

93 Prise en charge et devenir des sarcomes des tissus mous chez l’enfant (rabdomyosarcomes exclus) : à propos d’une série de 85 cas

Introduction Les sarcomes des tissus mous de l’enfant representent un groupe heterogene constitue de differentes entites histologiques. Les principes de prise en charge chez l’enfant sont derives de l’experience des adultes. Le but de cette etude etait de rechercher les facteurs pronostiques de controle local et de survie des patients. Materiel et Methode Les donnees concernant 85 enfants et adolescents pris en charge entre 1984 et 2004 pour sarcome des tissus mous (rabdomyosarcomes et PNET exclus) ont ete extraites de la base de donnee prospective du service. Les patients avaient moins de 20 ans lors de la prise en charge et plus de 2 ans de recul lors de la derniere evaluation. La chimiotherapie a ete associee pour les tumeurs larges et chez les patients metastatiques tandis que la radiotherapie postoperatoire etait reservee aux cas dont les marges d’exereses etaient limites ou incertaines. Resultats Les diagnostics histologiques les plus courants etaient le synovialosarcome (n = 30), les tumeurs des gaines peripheriques des nerfs (n = 8) et le leiomyosarcome (n = 6). L’âge moyen des patients etait de 14,5 ans tandis que l’âge le plus souvent observe etait 19 ans. Les tumeurs etaient de haut grade pour 37 %, de grade intermediaire pour 33 % et de bas grade pour 30 %. Le delai diagnostique moyen etait de 21 mois pour les stades intermediaires et bas grade et de 10 mois pour les hauts grades. Sept patients presentaient des metastases au moment du diagnostic. La taille moyenne des tumeurs etait de 7 cm. Cinquante neuf tumeurs etaient profondes et 26 superficielles. La chimiotherapie a ete utilisee pour 23 patients, et la radiotherapie pour 15 patients. La survie globale etait de 75 % a 5 ans et de 65 % a 10 ans. Le taux de recidive local etait de 19 %. Le seul facteur pronostique de recidive local etait l’existence de marges de resection inappropriees, tandis que les facteurs pronostiques de survie globale etaient la taille et le grade histologique de la tumeur. Ni l’âge au diagnostic, ni la profondeur de la tumeur n’etaient des facteurs pronostiques de survie. Du fait de l’utilisation de la chimiotherapie pour les patients supposes avoir mauvais pronostic, il n’a pas ete possible de demontrer que la chimiotherapie ameliorait la survie des patients. Conclusion Les enfants atteints de sarcomes des tissus mous (rabdo exclus) ont de bonne chance de guerison si la tumeur est traitee precocement par une exerese chirurgicale avec des marges saines. Chimiotherapie et radiotherapie sont reserves a des cas selectionnes et si possible dans le cadre de protocole d’essai international.

91 Variabilité dans la présentation du synovialosarcome de l’enfant

Introduction Bien que rare, le synovialosarcome de l’enfant est le sarcome des tissus mous le plus frequent apres le rabdomyosarcome. Plusieurs etudes ont demontre que la taille au diagnostic etait un facteur pronostique de survie important. Un diagnostic precoce et une prise en charge adaptee devraient permettre de reduire la taille de la tumeur. Le but de cette etude etait d’identifier le cheminement diagnostique, les erreurs de prise en charge et les facteurs susceptibles de conduire a un long delai diagnostique. Materiel et Methode Les donnees concernant 35 enfants pris en charge entre 1985 et 2006 pour synovialosarcome ont ete extraites de la base de donnee prospective du service. Les premiers symptomes, signes cliniques et radiographiques initiaux ont ete recherches. Le diagnostic presume a ete note. La duree totale des symptomes a ete decomposee en « delai patient » et « delai medecin ». Resultats Le sexe ratio garcon-fille etait de 1,69 : 1. Les tumeurs etaient localisees principalement aux extremites (a l’exception de 4 sarcomes du tronc) et aux membres inferieurs (n = 26). La taille moyenne de la tumeur au diagnostic etait de 6 cm (de 1 a 18 cm). La presentation la plus courante etait la decouverte d’une tumefaction indolore (n = 14). Pour 10 enfants aucune masse n’a ete mise en evidence initialement. Sept patients se sont presentes avec un tableau de raideur articulaire chronique inexpliquee malgre de multiples explorations pour certains. Le nombre moyen de medecins consultes avant que le patient ne soit reference etait de 3 (de 1 a 6) ; il s’agissait surtout de medecins generalistes (n = 22) ou de chirurgiens orthopedistes (n = 28). Quatre rhumatologues ont ete consultes pour une raideur articulaire chronique. Les diagnostics initialement evoques etaient kyste arthrosynovial, kyste de Baker, lipome, schwanome, myosite ossifiante, hematome, fibromatose, synovite villonodulaire…. Le diagnostic etait obtenu pour 15 patients a l’issu d’une chirurgie d’exerese pour tumeur presumee benigne ; pour ces patients une re-excision du champ operatoire a ete realisee dans le but d’obtenir une marge d’exerese satisfaisante. La duree moyenne des symptomes etait de 102 semaines ; les « delai patient » et « delai medecin » etaient respectivement de 43 et 50 semaines. Les principaux facteurs de retard du diagnostic etaient : localisation au genou, tableau de raideur articulaire, biopsie exerese d’une tumeur presumee benigne, existence de calcifications sur la radiographie, et sous-type histologique monophasique. Quoiqu’il en soit, l’existence d’une longue duree des symptomes n’a pas ete assortie d’un moins bon pronostic(tumeurs potentiellement moins agressives ?). Conclusion Une meilleure connaissance des symptomes initiaux et des erreurs de prise en charge du synovialosarcome de l’enfant devrait permettre de reduire a l’avenir le « delai medecin » particulierement long dans cette serie pediatrique.