Contribution à l'étude de l'ostéonécrose drépanocytaire de la tête fémorale de l'adulte: épidémiologie, diagnostic et traitement

La drépanocytose est la maladie moléculaire et héréditaire (transmission mendélienne récessive et autosomique) la plus répandue au monde. Elle est un problème de santé publique par sa gravité et ses implications socio-économiques dans de nombreux pays. Seuls les sujets homozygotes (SS) ou hétérozygotes composites (SC) sont malades, les hétérozygotes (AS) ne sont que des transmetteurs du gène S. Elle est la première cause d’OstéoNécrose de la Tête Fémorale (ONTF), douloureuse évoluant vers l’arthrose, en l’absence de traitement chez un patient jeune.La Guadeloupe compte 450.000 habitants, dont 12% sont porteurs de l’hémoglobine S. Le nombre des drépanocytaires est estimé à 1.200 dont les 3/4 sont suivis au Centre Caribéen de la Drépanocytose (CCD), créé en 1990. Le centre assure la prise en charge médicale des enfants dès leur naissance et des adultes malades. Nos activités au CHU de Pointe-à-Pitre, au CCD et à l’Unité INSERM-UMR S458 depuis juillet 1992 nous ont permis d’étudier:- le diagnostic de l’ONTF;- l’évaluation de l’hyperpression osseuse dans l’ONTF et l’évaluation du traitement par forage simple;- l’étude de l’impact de la prise en charge orthopédique précoce sur la survenue et l’évolution de l’ONTF.Notre étude concerne les patients drépanocytaires adultes homozygotes (SS) et double hétérozygotes (SC):- une série rétrospective de 1993-1994 [E-1994] portant sur 115 patients (58 SS, 57 SC) identifiés en 1984,sans suivi médical ni orthopédique;- une série prospective de 1995 à 2008 [E-2008] portant sur 215 patients (94 SS, 121 SC) avec prise encharge médicale et orthopédique.L’IRM est l’examen de référence pour le diagnostic de l’ONTF comme dans la nécrose idiopathique. En absence d’imagerie moderne, la radiographie traditionnelle réalisée de façon complète (profil et, surtout, faux profil), permet le diagnostic avant toute déformation. Seules les lésions cliniquement symptomatiques et évolutives (examen clinique itératif, contrôle radiologique, tomographie, TDM ou IRM) ont une indication opératoire.L’hyperpression intra osseuse, dans l’ONTF drépanocytaire, est significativement liée à la douleur (que les patients soient homozygotes ou hétérozygotes). Sa diminution a un effet antalgique objectif, observée après forage. Elle permet de confirmer le diagnostic d’ostéonécrose au stade précoce, dans les régions où l’IRM est inexistante.Un forage réalisé aux stades précoces de l’ONTF permet un arrêt rapide de l’évolution des lésions vers une arthrose, avec une efficacité certaine pour les stades I et II. Il garde une efficacité limitée pour le stade III. En plus de l’indolence apportée par la décompression, le bénéfice du forage se manifeste par l’allongement du délai avant arthroplastie (de 7,4 ± 2,7 ans). La technique est réalisable dans les régions sous équipées, où la drépanocytose est fréquente.La description histologique aux différents stades radiologiques de l’ONTF montre toujours des lésions de nécrose médullaire et osseuse. A l’inverse des lésions idiopathiques, les lésions drépanocytaires sont caractérisées par la présence d’une inflammation, en dehors de tout processus infectieux.Dans la littérature, la fréquente de l’ONTF drépanocytaire chez l’adulte est voisine de 40%, proche de celle observée dans [E-1994], notre population non suivie (36,5%). En comparant les études [E-1994] et [E-2008], la fréquence de l’ONTF passe de 36,5% à 14,4%. L’officialisation en 1992 d’une prise en charge médicale et d’un suivi orthopédique régulier au CCD et au CHU de Pointe-à-Pitre, a permis la réduction de la fréquence de l’ONTF et d’autres morbidités.Le rappel sur la drépanocytose révèle la complexité de la maladie, la variabilité de son expression clinique et de ses complications. L’amélioration de vie des patients nécessite une prévention primaire, secondaire et tertiaire, en l’absence d’un traitement spécifique de la maladie.La prise en charge médicale, complétée par une prévention et un traitement précoce (orthopédique ou chirurgical) telle que réalisés au CCD en Guadeloupe, a permis une réduction significative de la survenue de la nécrose de hanche et de ses complications. Pour une prévention tertiaire des complications ostéo-articulaires, nous suggérons:- une prise en charge médicale régulière des enfants et des adultes afin de réduire les crises vaso-occlusives;- une éducation des patients à la recherche de signes d’appel de l'ONTF et, aussi, d’autres articulations;- un examen clinique ostéo-articulaire lors des bilans annuels et après toute crise vaso-occlusive;- une attention particulière à l’adolescence (passage enfant-adulte), après une grossesse;- une prise en charge précoce, orthopédique ou chirurgicale conservatrice (forage ou ostéotomie) face à unenécrose, afin de réduire les complications invalidantes de l’ONTF.Sickle-cell anemia is the most widespread hereditary (autosomal recessive Mendelian transmission) molecular pathology in the world. It is a public health issue in many countries, due to its severity and socio-economic impact. Only homozygous (SS) and double heterozygous (SC) subjects are affected, heterozygous (AS) subjects merely transmitting the gene S. Sickle-cell anemia is the most frequent cause of osteonecrosis of the femoral head (ONFH), a painful condition which evolves towards osteoarthritis if not treated at an early age.Guadeloupe has a population of 450,000, 12% of whom are carriers of hemoglobin S. There are estimated to be 1,200 sickle-cell anemia sufferers, three-quarters of whom are followed in the Caribbean Sickle-Cell Center (Centre Caribéen de la Drépanocytose: CCD), which was set up in 1990. The Center provides medical care for adult patients and for children as of birth. Work has been ongoing since July 1992, in the Pointe-à-Pitre University Hospital, the CCD and the INSERM-UMR S458 research unit, focusing on:- diagnosis of ONFH;- bone hyperpressure measurement in ONFH and assessment of simple drilling treatment;- the impact of early orthopedic treatment on the onset and evolution of ONFH.The present study involved homozygous (SS) and double heterozygous (SC) adult sickle-cell anemia patients:- a retrospective series, from 1993 to 1994 [S-1994], including 115 patients (58 SS, 57 SC) identified in 1984,who had no medical or orthopedic care;- a prospective series, from 1995 to 2008 [S-2008], including 215 patients (94 SS, 121 SC), with medical and orthopedic care.MRI is the diagnostic gold-standard in ONFH, as in idiopathic necrosis. Where such modern imaging is not available, complete standard X-ray (lateral and especially false lateral) enables diagnosis to be made before deformity sets in. Surgery is indicated only for clinically symptomatic evolutive lesions on iterative clinical check-up, X-ray control, tomography, CT or MRI.Intraosseous hyperpressure in sickle-cell ONFH shows a significant correlation with pain, in both homozygous and heterozygous patients. Pressure reduction is objectively pain-relieving, as seen after drilling, and can confirm diagnosis of ONFH at an early stage, in places where MRI is not available.Drilling performed in the early stages of ONFH quickly arrests evolution towards osteoarthritis, with proven efficacy in grades I and II, and a certain degree of effectiveness in grade III. Over and above the pain-relief provided by decompression, drilling also enables hip replacement to be postponed, by 7.4±2.7 years. Moreover, the technique is feasible in those under-equipped regions in which sickle-cell disease is widespread.Histologic description of radiologic ONFH stages consistently finds medullary and bone necrosis. In contrast to idiopathic lesions, sickle-cell related lesions show inflammation without any associated infection.In the literature, the frequency of adult sickle-cell ONFH is reported to be nearly 40%, close to the 36.5% found in the S-1994 study of a non-treated population. In the S-2008 study of a population with medical and orthopedic care, ONFH frequency fell to 14.4%. The official provision of medical care and regular orthopedic follow-up in the CCD and Pointe-à-Pitre Hospital has reduced the frequency of ONFH and other morbidities.A review of sickle-cell disease reveals its complexity: the variability of its clinical expression and associated complications. Improving patients’ quality of life requires primary, secondary and tertiary prevention, in the absence of specific treatment.Medical care, supplemented by early prevention and treatment (orthopedic or surgical), as practiced in the Guadeloupe CCD, has significantly reduced the rates of ONFH and associated complications. We recommend the following CCD protocol for tertiary prevention of osteoarticular complications:- regular medical care for children and adults, to reduce the incidence of vaso-occlusive crises;- patient education in alarm signs of osteonecrosis of the femoral head and of other joints;- systematic osteoarticular assessment at yearly check-up and after all vaso-occlusive crises;- special focus on adolescence (child-to-adult transition) and following pregnancy;- early care, both orthopedic and by conservative surgery (drilling or osteotomy), in case of necrosis, to reduce the rate of disabling complications of ONFHDoctorat en Sciences médicalesinfo:eu-repo/semantics/nonPublishe

Traitement de l'ostéonécrose drépanocytaire par forage de la tête fémorale

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Treatment of Sickle cell disease's hip necrosis by core decompression: A prospective case-control study

Introduction: The young age of patients, total arthroplasties complications risks, and implant costs justify evaluation of the results of core decompression in the treatment of sickle-cell disease avascular necrosis of the femoral head (ONFH). Hypothesis: In sickle-cell disease necrosis, core decompression offers good relief from pain and delays the use of total arthroplasty in comparison to a conservatively treated control group by a simple non-weight bearing protocol. Materials and Methods: From 1994 to 2008, among 215 drepanocytic adults, 42 patients (22 genotype SS, 20 genotype SC; 15 men, 27 women) presented symptomatic ONFH. We report the data from a prospective study of two patients' groups: a non-operated group (16 patients aged 36.5 ± 6.5 years, 23 hips) and an operated group (26 patients aged 30.3 ± 2.8 years, 42 hips). The results were considered on the basis of change in clinical status according to the numeric evaluation of pain scale, the functional score of Merle d'Aubigné-Postel (MAP), the radiological progression of lesions, and the time delay to total arthroplasty. Results: Twenty-three hips were conservatively treated by discharge (a pair of canes). After a follow-up period of 13.4 ± 0.5 years, no pain improvement was noted (p = 0.76), and MAP score was unchanged (p = 0.27). Out of 23 hips managed by discharge, 9 stage IV hips (degenerative arthritis, 39.1%) underwent arthroplasty after an average delay of 2.6 ± 2.4 years. Forty-two hips were treated by core decompression. The duration of follow-up was 11.3 ± 1.8 years. Postoperatively, pain reduction and MAP score improvement were significant in 39 out of 42 hips (93%, p < 0.0001). Twenty-nine out of these 42 hips had a favorable evolution. Ten hips (23.8%) progressed to total arthroplasty, after a period of 7.4 ± 2.7 years, longer than the one of the non-operated group (p = 0.0007). By comparing the two groups (operated and non-operated), the benefit of core decompression appeared very significant (p < 0.0001). In addition to allocating Patients osteonecrosis stages, the Koo and Kim Index estimated the severity and evolution of necrotic lesions in both groups. It indicated decline in the non-operated group (p = 0.002) and improvement for operated patients (p = 0.0002). Discussion: Core decompression had a favorable clinical and radiological outcome superior to surgical abstention. Stages I and II ONFH remained stable after drilling, necessitating no arthroplasty (considered as a failure of drilling). The Koo and Kim Index above 30° in the non-operated group was a significant indicator of lesions degradation (p = 0.002). In addition to the indolence obtained by core decompression, the benefit of drilling was manifested by the prolonging the adjournment before arthroplasty end-point. It was respectively 7.4 ± 2.7 years in the operated group versus 2.6 ± 2.4 years in the non-operated group (difference of 4.8 years, p < 0.01). Conclusion: The technique of core decompression remains a valid option place in the treatment sickle-cell disease avascular necrosis of the femoral head (ONFH). It may be especially recommended in under-equipped regions where drepanocytosis and its osteo-articular complications are frequent. Level of Evidence: Level III case-control therapeutic study. © 2009.SCOPUS: ar.jinfo:eu-repo/semantics/publishe

Histopathology of aseptic necrosis of the femoral head in sickle cell disease.

This study compares the histopathology of bone biopsies from patients suffering from sickle cell anaemia (homozygote SS) to heterozygote patients (SA) and homozygotes with aseptic osteonecrosis (AA). The sensitivity to bacterial infection of sickle cell patients raises the question of the aetiology of sepsis in the onset of the necrosis. To our knowledge this study is the first to analyse the histopathology of osteonecrosis of the femoral head, at its early stages, in sickle cell anaemia. At the University Hospital of Pointe-à-Pitre, from 1994 to 2007, 38 bone biopsies were obtained from adult patients with avascular necrosis of the femoral head at the time of a core decompression procedure (SS, SC: 27; AS: 5; AA: 6). The histology of the biopsies confirmed the necrosis; all bacteriological cultures were negative. Patients displaying one S gene (SS, SC, AS) compared to homozygote subjects (AA) showed a significant increase of a nonspecific inflammatory granulomatosis (p = 0.003). No relationship was observed between the radiological stages and the histology whatever the genotype (p = 0.1). Inflammatory histopathology without sepsis or advanced alteration characterises the early stages of sickle cell necrosis. This inflammatory process is absent in idiopathic avascular necrosis.Journal ArticleSCOPUS: cp.jinfo:eu-repo/semantics/publishe

Telemedicine in Diabetic Foot Care: A Systematic Literature Review of Interventions and Meta-analysis of Controlled Trials

International audienceThe care of individuals with diabetic foot ulcers is costly and requires multiple hospital visits. Inadequate care leads to serious complications and a high risk of lower extremity amputation. In this review, we aimed at evaluating whether telemedicine can be effective in diabetic foot patient care. We searched Medline through Embase and PubMed and Cochrane Central Register of Controlled Trials (CENTRAL) for relevant studies, published up to April 2017. The studies were summarized and discussed in a narrative method and a meta-analysis of 2 controlled trials was conducted using the fixed-effects model. The main outcomes, assessed in the retrieved studies were the healing rate and satisfaction of patients and health care personnel. Most of the studies showed that implementing telemonitoring programs increased the rate of complete ulcer healing, while the patients were highly satisfied. Two trials providing data on 213 patients on telemedicine and 301 patients on usual care were included for meta-analysis. Subjects in telemedicine, as well as control groups had statistically similar healing time (43 vs 45 days; P = .83), healing time ratio adjusted for age (1 vs 1.4; P = .1), unhealed ulcers or loss to follow-up (3 of 20 vs 7 of 120; P = .13), and amputations (12 of 193 vs 14 of 182; P = .59). Subjects in the telemedicine group experienced a significantly higher mortality rate (8 of 193 vs 1 of 181; P = .0001) due to unexplained factors. No adverse events were attributed to using the telemedicine technology. The odds of complete ulcer healing were statistically similar between the telemedicine group and controls (odds ratio = 0.86; 95% CI = 0.57-1.33; P = .53). Telemedicine care is promising for the management of diabetic foot patients as the results were comparable with usual care. However, further large-scale studies need to be undertaken before it can be implemented widely

Facteurs de risque et intérêt de la surveillance orthopédique sur le devenir de l'ostéonécrose aseptique de la tête fémorale chez les adultes porteurs d'anémie falciforme: Étude de 215 patients contre un groupe témoin

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Risk factors and impact of orthopaedic monitoring on the outcome of avascular necrosis of the femoral head in adults with sickle cell disease: 215 patients case study with control group.

Sickle cell disease is a public health problem. The WHO has recommended that global management be implemented to reduce mortality and morbidity. Since no comprehensive care programme for bone and joint complications exists, the Caribbean Sickle Cell Disease Center added orthopaedic consultation to screen for and monitor these complications in 1992.Comparative StudyJournal ArticleResearch Support, Non-U.S. Gov'tSCOPUS: ar.jinfo:eu-repo/semantics/publishe

Relationships between hemodynamic, hemorheological and metabolic responses during exercise

International audienceAerobic performance is dependent on both cardio-respiratory and peripheral factors with hemodynamic parameters playing a major role. However, whether blood rheology might affect aerobic performance through an effect on hemodynamic factors is not known. The aim of the present study was to assess the relationships between hemodynamic, hemorheological and metabolic parameters in response to a sub-maximal cycling exercise protocol consisting of three successive levels of nine min duration (50, 100 and 150 W). Ten young sportsmen participated in the present study. Mean arterial pressure (MAP) was measured manually, with thoracic impedance used to monitor cardiac output (Qc): systemic vascular resistance (SVR) was then calculated. Whole blood viscosity (etab) was measured and used to calculate systemic vascular hindrance. Hematocrit (Hct) was determined by micro-centrifugation and red blood cell (RBC) deformability (EI) was determined by ecktacytometry. A breath-by-breath gas analyzer was used to measure oxygen uptake (VO2); the Fick equation was used to calculate arterio-venous oxygen difference [(a-v)O(2)] from VO(2) and Qc. All measurements were performed at rest, during exercise and during recovery. Compared to baseline, Qc, MAP, Hct, EI, VO(2), and (a-v)O(2) increased during exercise. etab increased above baseline only at 150 W and remained elevated during recovery; the increase in etab during the last level of exercise was associated with a decrease of SVR and systemic vascular hindrance. There was a significant negative correlation between EI and SVR (r=-0.35, p<0.01) and a significant positive relationship between EI and (a-v)O(2) (r=0.35, p<0.01) and between EI and VO(2) (r=0.37, p<0.01) across all exercise workloads, thus suggesting a potential role for RBC deformability as a factor affecting aerobic performance via oxygen delivery to tissues. These data lend support to the concept that hemorheological parameters may contribute to hemodynamic and cardio-respiratory adaptations in response to exercise in moderately trained sportsmen