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.

INTRODUCTION: 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. HYPOTHESIS: Comprehensive medical and surgical care of patients with sickle cell disease will reduce the complications and disability associated with this disease. POPULATIONS AND METHODS: Two populations were compared to evaluate the impact of comprehensive disease management on the occurrence of avascular necrosis (AVN) of the femoral head (femoral head AVN). The case-control series, [E-1994], included 115 patients (58 SS and 57 S) without orthopaedic monitoring and was evaluated retrospectively. The other patient series, [E-2008], included 215 patients (94 SS and 121 SC) with systematic orthopaedic care and was followed prospectively. Age, gender, duration of follow-up, haemoglobin levels, genotype, pain before treatment, associated humerus AVN and leg ulcers were analysed. RESULTS: Femoral head AVN occurred in young adult patients (35.3 ± 4 years for [E-1994] and 29 ± 3.4 years for [E-2008]). Only elevated haemoglobin levels were associated with the occurrence of femoral head AVN, which suggests that increased blood viscosity contributes to the condition ([E-1994], P<0.0001; [E-2008], P=0.001). Treatment in [E-2008] patients reduced the number of femoral head AVN cases from 36.5% in [E-1994] to 14.4% in [E-2008] (P<0.0001). DISCUSSION: The prevention and management of femoral head AVN must include medical treatment of the disease to reduce the occurrence of painful vaso-occlusive crises, which are known to trigger femoral head AVN. The effectiveness of this programme hinged on identifying risk factors and using simple approaches (hydration, pain medication, rest and crutches) to manage painful joint crises before femoral head AVN appeared. These approaches could be implemented in disadvantaged countries where sickle cell disease is prevalent. CONCLUSION: By knowing the risk factors, symptomatic patients who are at risk for femoral head AVN can be identified and additional evaluations can be performed early on in cases of hip pain.

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 degrees 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.

Elbow osteonecrosis in sickle cells anemia: a study of six cases.

A series of six elbows with aseptic osteonecrotic lesions is reported in a population of sickle cell anemic patients. Pain and range of motion limitation were the most commonly observed symptoms during the course of the disease. Three of these elbows had to undergo a surgical procedure (core decompression or loose body excision).

Prevalence, clinical features, and risk factors of osteonecrosis of the femoral head among adults with sickle cell disease.

This prospective study examined the prevalence, clinical features, and risk factors of osteonecrosis of the femoral head among adult sickle cell disease patients in Guadeloupe. Screening of osteonecrosis of the femoral head was performed using radiography, bone scintigraphy, and tomodensitometry. One hundred thirteen adults with sickle cell disease (67 SS and 46 SC patients) comprised the study population. Forty-two (37.2%) patients had osteonecrosis of one or both hips (67 [29.6%] hips) without association to a particular genotype, although bilateral involvement was more frequent among SS patients. While the prevalence of femoral head osteonecrosis increased with age, patients of all ages were affected, particularly young SC adults. Osteonecrosis of the femoral head was diagnosed at preradiographic stages (stage I) in 30% of hips and was frequently asymptomatic (60% of all cases; 95% and 90% of stages I and II, respectively). Osteonecrosis of the femoral head was significantly associated with a history of leg ulcer and osteonecrosis of the humeral head. SS patients with higher hemoglobin levels had an increased risk of osteonecrosis of the femoral head.

Osteonecrosis of the femoral head among adult patients with sickle-cell disease in Guadeloupe: prevalence, clinical features and risk factors

Osteonecrosis of the femoral head (ONFH) is a frequent complication of sickle-cell disease (SCD). We conducted systematic screening of ONFH by using the association of radiography, bone scintigraphy and tomodensitometry. This paper presents our first results concerning 113 adult patients (67 SS, 46 SC), prevalence, clinical features, risk factors, and association with other SCD complications. Forty-two patients (37.2 percent) were found to have osteonecrosis of one or both hips (67 hips, 29.6 percent) without association to a particular genotype, although bilateral involvement was considerably more frequent among SS patients. ONFH affected patients of all ages and in particular SC young adult patients were found to be affected frequently. ONFH was diagnosed at pre-radiological stages (Stage 1) for 31.3 percent of hips and was frequently aysmptomatic (60 percent of all cases, 95 and 90 percent of Stages I and II, respectively), indicating need for systematic screening when early diagnosis is desired. For SS patients, ONFH was more frequent when follow-up began later. ONFH was also associated with higher parity and history of leg ulcer for both genotypes. No correlation was found with biological data, in particular haematological variables, except for high glucose levels for SS patients. We have initiated a prospective study of ONFH among patients followed at the SCD Centre of Guadeloupe, with special concern in diagnosis at pre-radiological stages, and outcome of patients. Further data will be provided by longitudinal follow-up of adult patients as well as inclusion of children followed from birth after neonatal screening (AU)