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Abstract
RÉSUMÉ
Introduction. La drépanocytose est l'une des hémoglobinopathies les plus fréquentes en Afrique subsaharienne et s'accompagne souvent de complications ostéoarticulaires, en particulier infectieuses, qui augmentent la morbidité et le handicap fonctionnel. Au Gabon, où la prévalence du trait drépanocytaire est élevée, ces complications restent mal décrites en milieu chirurgical. L'objectif de ce travail était de décrire les caractéristiques épidémiologiques, cliniques, microbiologiques et thérapeutiques des manifestations ostéoarticulaires chez les patients drépanocytaires hospitalisés en chirurgie. Méthodologie. Nous avons mené une étude rétrospective descriptive multicentrique dans trois services de chirurgie et d'orthopédie du Gabon, du 1er mai 2023 au 1er mai 2025. Nous avons inclus tous les patients drépanocytaires homozygotes SS hospitalisés pour une complication ostéoarticulaire avec dossier complet. Résultats. Vingt-quatre dossiers ont été retenus, dont 15 patients de sexe masculin (sex-ratio 1,5). L'âge moyen était de 12 ans (4–25 ans) et 12 patients étaient régulièrement suivis en pédiatrie. Les complications étaient dominées par les infections ostéoarticulaires (20/24, 83 %), principalement les ostéomyélites chroniques (11 cas, soit 46 %), les ostéomyélites aiguës (3 cas) et les arthrites septiques (genou, hanche, épaule). Les localisations les plus fréquentes étaient le fémur distal, le tibia proximal et l'humérus proximal. Une ostéonécrose de la tête fémorale a été observée chez 2 patients (8 %). Un prélèvement bactériologique était positif chez 12 patients, avec prédominance de Staphylococcus aureus. Douze patients ont bénéficié d'un traitement médico-chirurgical (curetage, séquestrectomie) associé à une antibiothérapie ciblée, avec évolution globalement favorable. Conclusion. Dans notre contexte, les complications ostéoarticulaires de la drépanocytose prises en charge en chirurgie sont largement dominées par les infections osseuses. Un diagnostic précoce et une prise en charge médico-chirurgicale adaptée semblent permettre de limiter les séquelles fonctionnelles.
ABSTRACT
Introduction. Sickle cell disease is one of the most common genetic hemoglobinopathies in sub-Saharan Africa and is frequently associated with osteoarticular complications, particularly infectious ones, which increase morbidity and functional disability. Methodology. In Gabon, where the prevalence of the sickle cell trait is high, these complications remain poorly described in surgical settings; therefore, this multicenter retrospective descriptive study was conducted in three surgical and orthopedic departments from May 1, 2023, to May 1, 2025, including all homozygous SS patients hospitalized for osteoarticular complications with complete records. Results. Twenty-four cases were reviewed, involving 15 male patients (sex ratio 1.5) with a mean age of 12 years (range 4–25 years), half of whom received regular pediatric follow-up. Complications were dominated by osteoarticular infections (20/24, 83%), primarily chronic osteomyelitis (11 cases, 46%), acute osteomyelitis (3 cases), and septic arthritis of the knee, hip, and shoulder, with the most frequent locations being the distal femur, proximal tibia, and proximal humerus. Additionally, femoral head osteonecrosis was observed in 2 patients (8%), and bacteriological cultures were positive in 12 cases—predominantly Staphylococcus aureus—leading to combined medico-surgical treatment (curettage, sequestrectomy) and targeted antibiotic therapy for 12 patients, resulting in generally favorable outcomes. Conclusion. In our context, osteoarticular complications of sickle cell disease managed surgically are largely dominated by bone infections, suggesting that early diagnosis and adapted medico-surgical management can help limit functional sequelae.
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References
- 1. Piel FB, Steinberg MH, Rees DC. Sickle cell disease. N Engl J Med. 2017;376(16):1561-73.
- 2. Almeida A, Roberts I. Bone involvement in sickle cell disease. Br J Haematol. 2005;129(4):482-90.
- 3. Ejindu VC, Hine AL, Mashayekhi M, Shorvon PJ, Misra RR. Musculoskeletal manifestations of sickle cell disease. Radiographics. 2007;27(4):1005-21.
- 4. Ngolet LO, Kocko I, Elira-Dokekias A, Ossou-Nguiet PM. La drépanocytose au Congo-Brazzaville : épidémiologie et aspects cliniques. Rev Med Brazzaville. 2016;2(1):12-8.
- 5. Ware RE, de Montalembert M, Tshilolo L, Abboud MR. Sickle cell disease. Lancet. 2017;390(10091):311-23.
- 6. Wastnedge E, Waters D, Patel S, Morrison K, Bhatt DL, Bhattacharya S, et al. The global burden of sickle cell disease in children under five years of age: a systematic review and meta-analysis. J Glob Health. 2018;8(2):021103.
- 7. Hebbel RP. Adhesive interactions of sickle erythrocytes with endothelium. J Clin Invest. 1997;100(11 Suppl):S83-6.
- 8. Vichinsky EP, Neumayr LD, Haberkern C, Earles AN, Eckman J, Koshy M, et al. The perioperative complication rate of orthopedic surgery in sickle cell disease. Am J Hematol. 1999;62(3):129-38.
- 9. Bello-Manga H, DeBaun MR, Kassim AA. Epidemiology and changing landscape of sickle cell disease in sub-Saharan Africa with implications for healthcare systems. Adv Exp Med Biol. 2019;1096:1-14.
- 10. Aken'Ova YA, Bakare RA, Okunade MA. Septic arthritis and osteomyelitis in sickle cell disease: a report from Ibadan, Nigeria. West Afr J Med. 1993;12(2):81-4.
- 11. Gbadoé AD, Agbétiafa K, Koffi S, Lawson-Evi K, Tatagan-Agbi K, Assimadi JK. Complications ostéo-articulaires de la drépanocytose chez l'enfant au Togo. Arch Pédiatr. 2007;14(3):273-8.
- 12. Burnett MW, Bass JW, Cook BA. Etiology of osteomyelitis complicating sickle cell disease. Pediatrics. 1998;101(2):296-7.
- 13. Milner PF, Kraus AP, Sebes JI, Sleeper LA, Hampel KA, Marchetti AL, et al. Sickle cell disease as a cause of osteonecrosis of the femoral head. N Engl J Med. 1991;325(21):1476-81.
- 14. Hernigou P, Habibi A, Bachir D, Galacteros F. The natural history of asymptomatic osteonecrosis of the femoral head in adults with sickle cell disease. J Bone Joint Surg Am. 2006;88(12):2565-72.
- 15. Grosse SD, Odame I, Atrash HK, Amendah DD, Piel FB, Williams TN. Sickle cell disease in Africa: a neglected cause of early childhood mortality. Am J Prev Med. 2011;41(6 Suppl 4):S398-405.
- 16. Bansah EA, Aidoo EK, Kyere KA, Acheampong E. Osteomyelitis in sickle cell disease: a difficult differential diagnosis with vaso-occlusive pain crisis. Int J Surg Case Rep. 2020;68:1-5.
- 17. Diallo DA, Baby M, Diallo A, Sangaré A, Dembélé M, Touré BA, et al. Complications ostéo-articulaires de la drépanocytose en milieu pédiatrique à Bamako. Mali Med. 2006;21(1):26-30.
- 18. Booth C, Inusa B, Obaro SK. Infection in sickle cell disease: a review. Int J Infect Dis. 2010;14(1):e2-e12.
- 19. Mwita JC, Amusa YB, Olubunmi A, Ogunbiyi S. Pattern and management of osteomyelitis in sickle cell disease. Niger Postgrad Med J. 2009;16(3):225-8.
- 20. Umans H, Haramati N, Flusser G. The diagnostic role of gadolinium enhanced MRI in distinguishing between acute medullary bone infarct and osteomyelitis in sickle cell disease. Skeletal Radiol. 2000;29(6):316-21.
- 21. Adeyokunnu AA, Hendrickse RG. Salmonella osteomyelitis in childhood. A report of 63 cases seen in Nigerian children of whom 57 had sickle cell anaemia. Arch Dis Child. 1980;55(3):175-84.
- 22. Lew DP, Waldvogel FA. Osteomyelitis. Lancet. 2004;364(9431):369-79.
- 23. Chambers JB, Forsythe DA, Bertrand SL, Iwinski HJ, Steflik DE. Retrospective review of osteoarticular infections in a pediatric sickle cell age group. J Pediatr Orthop. 2000;20(5):682-5.
- 24. Pearson HA, Spencer RP, Cornelius EA. Functional asplenia in sickle cell anemia. N Engl J Med. 1969;281(17):923-6.
- 25. Mukisi-Mukaza M, Manicom O, Alexis C, Bashoun K, Donkerwolcke M, Burny F. Treatment of sickle cell disease's hip osteonecrosis by core decompression: a prospective case-control study. Orthop Traumatol Surg Res. 2009;95(7):498-504.
- 26. Grimes DA, Schulz KF. Bias and causal associations in observational research. Lancet. 2002;359(9302):248-52.
- 27. Anim-Sampong S, Amponsah-Manu F, Asante DB, Abaka-Yawson A, Quaye L. Differentiating vaso-occlusive crisis from osteomyelitis in sickle cell disease: a review. Med Sci (Basel). 2022;10(3):46.
- 28. National Heart, Lung, and Blood Institute. Evidence-Based Management of Sickle Cell Disease: Expert Panel Report, 2014. Bethesda: NIH; 2014.
- 29. Kaplan J, Afifi RO, Bhatt DL. Management of bone and joint complications in sickle cell disease. J Am Acad Orthop Surg. 2021;29(12):e580-91.
References
1. Piel FB, Steinberg MH, Rees DC. Sickle cell disease. N Engl J Med. 2017;376(16):1561-73.
2. Almeida A, Roberts I. Bone involvement in sickle cell disease. Br J Haematol. 2005;129(4):482-90.
3. Ejindu VC, Hine AL, Mashayekhi M, Shorvon PJ, Misra RR. Musculoskeletal manifestations of sickle cell disease. Radiographics. 2007;27(4):1005-21.
4. Ngolet LO, Kocko I, Elira-Dokekias A, Ossou-Nguiet PM. La drépanocytose au Congo-Brazzaville : épidémiologie et aspects cliniques. Rev Med Brazzaville. 2016;2(1):12-8.
5. Ware RE, de Montalembert M, Tshilolo L, Abboud MR. Sickle cell disease. Lancet. 2017;390(10091):311-23.
6. Wastnedge E, Waters D, Patel S, Morrison K, Bhatt DL, Bhattacharya S, et al. The global burden of sickle cell disease in children under five years of age: a systematic review and meta-analysis. J Glob Health. 2018;8(2):021103.
7. Hebbel RP. Adhesive interactions of sickle erythrocytes with endothelium. J Clin Invest. 1997;100(11 Suppl):S83-6.
8. Vichinsky EP, Neumayr LD, Haberkern C, Earles AN, Eckman J, Koshy M, et al. The perioperative complication rate of orthopedic surgery in sickle cell disease. Am J Hematol. 1999;62(3):129-38.
9. Bello-Manga H, DeBaun MR, Kassim AA. Epidemiology and changing landscape of sickle cell disease in sub-Saharan Africa with implications for healthcare systems. Adv Exp Med Biol. 2019;1096:1-14.
10. Aken'Ova YA, Bakare RA, Okunade MA. Septic arthritis and osteomyelitis in sickle cell disease: a report from Ibadan, Nigeria. West Afr J Med. 1993;12(2):81-4.
11. Gbadoé AD, Agbétiafa K, Koffi S, Lawson-Evi K, Tatagan-Agbi K, Assimadi JK. Complications ostéo-articulaires de la drépanocytose chez l'enfant au Togo. Arch Pédiatr. 2007;14(3):273-8.
12. Burnett MW, Bass JW, Cook BA. Etiology of osteomyelitis complicating sickle cell disease. Pediatrics. 1998;101(2):296-7.
13. Milner PF, Kraus AP, Sebes JI, Sleeper LA, Hampel KA, Marchetti AL, et al. Sickle cell disease as a cause of osteonecrosis of the femoral head. N Engl J Med. 1991;325(21):1476-81.
14. Hernigou P, Habibi A, Bachir D, Galacteros F. The natural history of asymptomatic osteonecrosis of the femoral head in adults with sickle cell disease. J Bone Joint Surg Am. 2006;88(12):2565-72.
15. Grosse SD, Odame I, Atrash HK, Amendah DD, Piel FB, Williams TN. Sickle cell disease in Africa: a neglected cause of early childhood mortality. Am J Prev Med. 2011;41(6 Suppl 4):S398-405.
16. Bansah EA, Aidoo EK, Kyere KA, Acheampong E. Osteomyelitis in sickle cell disease: a difficult differential diagnosis with vaso-occlusive pain crisis. Int J Surg Case Rep. 2020;68:1-5.
17. Diallo DA, Baby M, Diallo A, Sangaré A, Dembélé M, Touré BA, et al. Complications ostéo-articulaires de la drépanocytose en milieu pédiatrique à Bamako. Mali Med. 2006;21(1):26-30.
18. Booth C, Inusa B, Obaro SK. Infection in sickle cell disease: a review. Int J Infect Dis. 2010;14(1):e2-e12.
19. Mwita JC, Amusa YB, Olubunmi A, Ogunbiyi S. Pattern and management of osteomyelitis in sickle cell disease. Niger Postgrad Med J. 2009;16(3):225-8.
20. Umans H, Haramati N, Flusser G. The diagnostic role of gadolinium enhanced MRI in distinguishing between acute medullary bone infarct and osteomyelitis in sickle cell disease. Skeletal Radiol. 2000;29(6):316-21.
21. Adeyokunnu AA, Hendrickse RG. Salmonella osteomyelitis in childhood. A report of 63 cases seen in Nigerian children of whom 57 had sickle cell anaemia. Arch Dis Child. 1980;55(3):175-84.
22. Lew DP, Waldvogel FA. Osteomyelitis. Lancet. 2004;364(9431):369-79.
23. Chambers JB, Forsythe DA, Bertrand SL, Iwinski HJ, Steflik DE. Retrospective review of osteoarticular infections in a pediatric sickle cell age group. J Pediatr Orthop. 2000;20(5):682-5.
24. Pearson HA, Spencer RP, Cornelius EA. Functional asplenia in sickle cell anemia. N Engl J Med. 1969;281(17):923-6.
25. Mukisi-Mukaza M, Manicom O, Alexis C, Bashoun K, Donkerwolcke M, Burny F. Treatment of sickle cell disease's hip osteonecrosis by core decompression: a prospective case-control study. Orthop Traumatol Surg Res. 2009;95(7):498-504.
26. Grimes DA, Schulz KF. Bias and causal associations in observational research. Lancet. 2002;359(9302):248-52.
27. Anim-Sampong S, Amponsah-Manu F, Asante DB, Abaka-Yawson A, Quaye L. Differentiating vaso-occlusive crisis from osteomyelitis in sickle cell disease: a review. Med Sci (Basel). 2022;10(3):46.
28. National Heart, Lung, and Blood Institute. Evidence-Based Management of Sickle Cell Disease: Expert Panel Report, 2014. Bethesda: NIH; 2014.
29. Kaplan J, Afifi RO, Bhatt DL. Management of bone and joint complications in sickle cell disease. J Am Acad Orthop Surg. 2021;29(12):e580-91.
