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Sickle Cell Disease &
Total Hip Arthroplasty
The three principle
      orthopaedic
     manifestations
• pyogenic infections
• marrow hyperplasia
• osteonecrosis
       J Am Acad Orthop Surg 2005;13:208-217
Organisms

• Staphylococcus aureus
• Streptococcus pneumoniae
• Salmonella typhi,
• Klebsiella pneumoniae
        N Engl J Med 1999;340: 1021-1030
        Clin Orthop 1993;294:140-148
Marrow Hyperplasia


• Increased erythropoiesis
• widening of the medullary canal and
  thinning of the trabeculae and cortices,



        J Bone Joint Surg Br 1989;71: 465-470
Osteonecrosis

• hypoxia-induced erythrocyte sickling, along
    with extravascular compression of the
    intraosseous blood supply caused by
    medullary hyperplasia
•    bone or marrow infarction, focal and
    patchy areas
Patient Evaluation and
   Natural History

• Groin pain
• limited ROM
• Often bilateral
Nonsurgical
         Management

• pain management
• assistive devices
• typically progresses

        J Bone Joint Surg Am 2003;85:500-504
Surgical Management

• disabling hip pain
• severely damaged hip joint
• Medically fit
Surgical Management
• THA
• Core decompression
• Femoral osteotomy
• Arthrodesis
• Hemiarthroplasty
• Resection arthroplasty
• No Level 1 or 2 evidence
• Steinberg stages I and II

       J Am Acad Orthop Surg 2005;13:208-217
• it does not address the primary underlying
  pathophysiology of vaso-occlusion
• femoral head involvement usually is diffuse
  and too large to decompress
• chronic nature of the disease makes future
  infarcts inevitable despite decompression

            Am J Orthop 1995;24:18-24
Preoperative
       Considerations
• preventing sickle cell crises
• paying careful attention to cardiac status
  and fluid balance
• assessing infection
• using preoperative transfusion or
  plasmapheresis
• considering anesthetic modalities and pain
  management technique
Preoperative
      Considerations


• hemoglobin S level < 30%


  Am J Hematol. 1999 Nov;62(3):129-38
Intraoperative
       Considerations

• wide medullary canal an thin cortices
• sclerosis, and canal obliteration
Accetabular
  Preparation &
Component Insertion
• Acetabular bone quality may be poor
• patches of densely sclerotic bone
  (eccentric reaming
• acetabular protrusio ( medial reaming, Bone
  Graft, acetabular support and In-situ
  Osteotomy)
       J Am Acad Orthop Surg 2005;13:208-217
Femoral Canal
         Preparation
• Areas of sclerosis may be so diffuse that
  the canal becomes completely obliterated
• hyperplastic medullary canals, thin cortices
• introducing a drill bit under image
  intensifier until it is possible to insert a
  guidewire for flexible reamers

            Int Orthop 2002;26:157-161
Medical Complications

• excessive bleeding (18%)
• pulmonary complications
• sickle cell crises
• cardiac complications (4%)
• mental status changes
          Am J Hematol 1999;62:129-138
Surgical Complications
• Infection (25%)
 • There are no reported cases of
    Salmonella osteomyelitis complicating a
    THA; therefore, prophylactic
    administration of antibiotics that provide
    coverage for this gram-negative
    microorganism is not recommended
          Am J Orthop 1996;25: 353-356
          Clin Orthop 1993;294:140-148
Surgical Complications


• Hip Dislocation (26%)


          J Arthroplasty 1997;12:420-425

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Hip

  • 1. Sickle Cell Disease & Total Hip Arthroplasty
  • 2. The three principle orthopaedic manifestations • pyogenic infections • marrow hyperplasia • osteonecrosis J Am Acad Orthop Surg 2005;13:208-217
  • 3. Organisms • Staphylococcus aureus • Streptococcus pneumoniae • Salmonella typhi, • Klebsiella pneumoniae N Engl J Med 1999;340: 1021-1030 Clin Orthop 1993;294:140-148
  • 4. Marrow Hyperplasia • Increased erythropoiesis • widening of the medullary canal and thinning of the trabeculae and cortices, J Bone Joint Surg Br 1989;71: 465-470
  • 5. Osteonecrosis • hypoxia-induced erythrocyte sickling, along with extravascular compression of the intraosseous blood supply caused by medullary hyperplasia • bone or marrow infarction, focal and patchy areas
  • 6. Patient Evaluation and Natural History • Groin pain • limited ROM • Often bilateral
  • 7. Nonsurgical Management • pain management • assistive devices • typically progresses J Bone Joint Surg Am 2003;85:500-504
  • 8. Surgical Management • disabling hip pain • severely damaged hip joint • Medically fit
  • 9. Surgical Management • THA • Core decompression • Femoral osteotomy • Arthrodesis • Hemiarthroplasty • Resection arthroplasty
  • 10. • No Level 1 or 2 evidence • Steinberg stages I and II J Am Acad Orthop Surg 2005;13:208-217
  • 11. • it does not address the primary underlying pathophysiology of vaso-occlusion • femoral head involvement usually is diffuse and too large to decompress • chronic nature of the disease makes future infarcts inevitable despite decompression Am J Orthop 1995;24:18-24
  • 12. Preoperative Considerations • preventing sickle cell crises • paying careful attention to cardiac status and fluid balance • assessing infection • using preoperative transfusion or plasmapheresis • considering anesthetic modalities and pain management technique
  • 13. Preoperative Considerations • hemoglobin S level < 30% Am J Hematol. 1999 Nov;62(3):129-38
  • 14. Intraoperative Considerations • wide medullary canal an thin cortices • sclerosis, and canal obliteration
  • 15. Accetabular Preparation & Component Insertion • Acetabular bone quality may be poor • patches of densely sclerotic bone (eccentric reaming • acetabular protrusio ( medial reaming, Bone Graft, acetabular support and In-situ Osteotomy) J Am Acad Orthop Surg 2005;13:208-217
  • 16. Femoral Canal Preparation • Areas of sclerosis may be so diffuse that the canal becomes completely obliterated • hyperplastic medullary canals, thin cortices • introducing a drill bit under image intensifier until it is possible to insert a guidewire for flexible reamers Int Orthop 2002;26:157-161
  • 17. Medical Complications • excessive bleeding (18%) • pulmonary complications • sickle cell crises • cardiac complications (4%) • mental status changes Am J Hematol 1999;62:129-138
  • 18. Surgical Complications • Infection (25%) • There are no reported cases of Salmonella osteomyelitis complicating a THA; therefore, prophylactic administration of antibiotics that provide coverage for this gram-negative microorganism is not recommended Am J Orthop 1996;25: 353-356 Clin Orthop 1993;294:140-148
  • 19. Surgical Complications • Hip Dislocation (26%) J Arthroplasty 1997;12:420-425