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Orthopaedic overview of sickle cell anemia
- 1. ORTHOPAEDIC OVERVIEW OF SICKLE CELL ANEMIA DR. SAURABH CHAUDHARY MBBS (JIPMER), MS, DNB
- 2. SICKLE CELL ANEMIA Anemia caused by defective hemoglobin Genetic disorder - Autosomal recessive
- 4. TYPES • Double copy of HbSS Sickle cell anemia – the severe form • less symptomatic, but… • Risk of sudden death on exertion Sickle cell trait – single copy
- 5. PATHOPHYSIOLOGY Misshapen RBCs Don’t deform Tend to aggregate Occlusion of small vessels - bones, abdominal organs, skin, gall bladder, intestine Multiple such episodes in spleen – hyposplenism / autosplenectomy – impaired immunity Anemia – marrow expansion
- 6. CLINICAL PRESENTATION Painful episodes – vaso-occlusive crises Anemia Infection
- 7. NON – ORTHOPAEDIC PICTURE Retinopathy Chronic renal disease Cerebrovascular accident Pulmonary disease Congestive cardiac failure Deep vein thrombosis Splenic infarct
- 8. SICKLE CELL CRISIS Sudden severe bone pain usually begins near age 2-3 years Occluded microvessels may lead to bone infarcts Treatment – analgesia and rest Hydration
- 9. TREATMENT UNDER PEDIATRICIAN / HEMATOLOGIST Blood transfusions Hydration plus oxygenation PAIN MANAGEMENT STEM CELL TRANSPLANTATION
- 10. ORTHOPAEDIC MANIFESTATIONS Sickle cell crisis Osteomyelitis Septic arthritis Osteonecrosis – femoral and humeral heads Bone infarcts Growth retardation Dactylitis
- 11. ORTHOPAEDIC MANIFESTATIONS Vaso-occlusive episodes leading to avascular necrosis of physis, metadiaphysis, • Pain, Joint degeneration, limb length discrepancy, angular deformities.
- 12. ORTHOPAEDIC MANIFESTATIONS Vaso-occlusive episodes to be aggressively managed with pain control – multimodal, opioids most effective • Warmth to the area, rest, splintage • Hydrate and oxygenate
- 13. SICKLE CELL DACTYLITIS – SUBPERIOSTEAL BONE, NECROSIS OF METATARSAL
- 14. ORTHOPAEDIC MANIFESTATIONS Infection – septic arthritis / osteomyelitis • Functional aplenia • Abnormal immune system • Abnormal intra-osseous perfusion • Chronic skin ulcers – staph • Intestinal mucosal sloughing – salmonella • Sequelae – joint degeneration / growth arrest
- 15. OSTEOMYELITIS Often in diaphysis / metaphyseo-diaphysis increased incidence of salmonella (?may spread from gallbladder infection) Staph. aureus and Salmonella are the most common organisms Salmonella osteomyelitis occurs most commonly in children with sickle cell disease
- 16. ACUTE INFECTION VS VASO- OCCLUSIVE EPISODE Differentiation is very important Elevated TLC, CRP in both PRO-CALCITONIN can be be useful, significantly elevated over baseline only in infection Contrast MRI /Bone scan can also help
- 17. OSTEOMYELITIS VS OCCLUSIVE CRISIS Imaging - radionuclide bone scan and radionuclide bone marrow scan can differentiate bone infarct from osteomyelitis • osteomyelitis: normal marrow uptake, abnormal bone scan • infarct: decreased marrow uptake, abnormal bone scan aspirate and culture to differentiate from a bone infarct • Contrast MRI – strong rim enhancement with irregular loculations in infection
- 18. OSTEOMYELITIS / SEPTIC ARTHRITIS Treatment irrigation & debridement consider preoperative oxygenation and exchange transfusion prior to surgery Prolonged antibiotics may be required DVT prophylaxis if required
- 19. AVASCULAR NECROSIS OF FEMORAL HEAD Osteonecrosis of the femoral head – 9% can be bilateral in sickle cell disease – 50% Presentation • hip pain with weight bearing Treatment • Nonoperative - partial weight bearing and range of motion
- 20. PATCHY DENSITY CHANGES, GROSS TRABECULATIONS
- 21. AVASCULAR NECROSIS OF FEMORAL HEAD Operative • total hip arthroplasty indications • failed non operative • intractable pain outcomes • results of total joint arthroplasty are poor due to ongoing remodeling of bone, increased chances of infection
- 22. ADULT HIP
- 23. THR IN SICKLE CELL DISEASE Very high complication rate – 7 to 100% Excessive bleeding Hematoma Tight canal Fracture risk Infection upto 20%
- 24. THR IN SICKLE CELL DISEASE High rate of loosening Aseptic wear and osteolysis Septic loosening much more common Uncemented THRs better
- 25. CAN THE AVN BE PREVENTED? Aggressive management of crises episodes from the beginning Hydration Rest crutches Pain management Transfusions
- 26. SPINE
- 27. H vertebrae
- 28. PEDIATRIC KNEE Deformity Growth arrest Effusion Osteopenia
- 29. ADULT KNEE Secondary osteoarthritis Osteopenia
- 30. SPECIAL ATTENTION IN PERI-OPERATIVE PERIOD Hb atleast 10gm/dl Decrease HbS to less than 30% during entire perioperative period Avoid GA Hydration Good oxygenation Good pain management – autonomic distress - dehydration Avoid hypothermia Fluids at room temperature
- 31. AVASCULAR NECROSIS OF HUMERAL HEAD
- 32. WHAT DOES THE PATIENT WANT Pain relief Empathy Mobility of joints
- 33. TAKE HOME MESSAGE Manage and prevent crises from early age Avoid dehydration / excessive exertion Avoid hypothermia Avoid hypotension Avoid surgery for minor disabilities Aggressive analgesia – opioids / NSAIDs/ Paracetamol / physical methods