Sickle Cell Anemia: Musculoskeletal Aspects


- See: Osteomyelitis in Sickle Cell Patient

- Discussion:
    - affects 1% of blacks - is more severe but less common than sickle cell trait (8% prevalence);
    - two main genotypes include Hb S and Hb C forms of disease;
    - crises usually begin at age 2-3 years and may lead to bone infarctions;

- Sequelae of Sickle Cell:
    - growth retardation & skeletal immaturity;
    - pathologic fracture:
          - references:
                - Fractures in Thalassemia.   
                - Prevalence and pathologic features of sickle cell nephropathy and response to inhibition of angiotensin-converting enzyme.
                - Incidence and treatment of fractures in thalassemia.
    - osteomyelitis (often in diaphysis);
          - septic arthritis (probably best treated w/ 3rd generation cephalsporin)
          - staph is probably the most common infectious organism, but some studies have shown salmonella to be common as well;
          - aspiration may be necessary to differentiate infarction from swelling;
          - references:
                - Original Articles: Pain In Sickle Cell Disease: Rates and Risk Factors.
                - Osteomyelitis in Patients Who Have Sickle-Cell Disease. Diagnosis and Management
                - Systemic Salmonella infections in sickle cell anaemia.
    - pneumococcal infection:
          - pneumococcal septicemia & meningitis are leading causes of death;
          - ensure pneumococcal vaccine;
          - consider penicillin prophylaxsis
    - AVN of femoral head:
          - more common in the older child (prevalence peaks in adolescence)
          - develops by the age of 35 years in nearly half of all patients with homozygous sickle cell disease;
          - femoral AVN occurs in about 30-37% of patients and humeral head involvement occurs in about 50% of patients;
                 - among patients with AVN of hip, opposite side will be involved in over 50% of cases;
          - rate of AVN is higher when patients are aggresively screened with bone scan (bone scan may also reveal AVN of knees and lumbar vertebrae);
          - natural history:
                 - in children may produce the typical Perthes appearance in the younger child, with complete collapse of the femoral head;
                 - without intervention, the rate of femoral head collapse may be 87% or higher within 5 years after the initial diagnosis of the osteonecrosis;
          - surgical treatment: varus osteotomy, Chiari osteotomy, THR;
          - references:
                 - Osteonecrosis of the hip in the sickle-cell diseases. Treatment and complications.
                 - Deformities of the hip in adults who have sickle-cell disease and had avascular necrosis in childhood. A natural history of fifty-two patients.
                 - Sickle cell disease and silent avascular necrosis of the hip.
                 - Original Articles: Sickle Cell Disease as a Cause of Osteonecrosis of the Femoral Head.
                 - Hip arthroplasty in patients with sickle-cell haemoglobinopathy.
                 - Total hip arthroplasty in patients who have sickle-cell hemoglobinopathy.
                 - Prevalence, clinical features, and risk factors of osteonecrosis of the femoral head among adults with sickle cell disease
                 - The Natural History of Symptomatic Osteonecrosis in Adults with Sickle-Cell Disease. 

    - AVN of humeral head:
          - femoral AVN occurs in about 30% of patients and humeral head involvement occurs in about 50% of patients;
          - in the report by Hernigou, et al. (1997), treatment of bone marrow allograft led to normalization of the proximal humerus in a skeletally immature patient (w/ humeral AVN);
          - references:
                 - Bone Marrow Transplantation in Sickle Cell Disease.  Effect on Osteonecrosis. A case report with a four year follow up
                 - The shoulder in sickle-cell disease.
                 - Osteonecrosis of the humeral head in sickle cell disease
                 - The Natural Progression of Symptomatic Humeral Head Osteonecrosis in Adults with Sickle Cell Disease 
    - dactylitis (acute hand/foot swelling):
          - affects 20%-50% of children with sickle cell disease;
          - usually occurs between ages of 6 months and 2 years;
          - presents w/ swelling of hands and feet, limitation of motion of extremities, & elevated temperature, thought to be result of vascular occlusion;
          - radiologic findings:
                - small osteolytic lesions in metacarpals, metatarsals;
                - phalanges show periostitis;
          - dactylitis is self limiting after a few days or weeks;
          - reference:
                - Sickle-cell dactylitis.


- Bone Scan:
    - infarcts cause a persistent decrease in tracer uptake which may complicate matters if osteomyelitis sets in;
    - in the report by Skaggs et al., 2001, the authors used radionucleotide scanning in order to distinguish between bone infarction and acute osteomyelitis;
          - a radionucleotide bone marrow and bone scan was performed sequentially within a 24-hr period in 79 episodes of acute bone pain;
          - 70 cases of bone infarction were diagnosed on the basis of decreased uptake on bone marrow scans, and abnormal uptake on the bone scan;
          - Differentiation between bone infarction and acute osteomyelitis in children with sickle-cell disease with use of sequential radionuclide bone-marrow and bone scans


- PreOp Planning:
    - transfusion of pRBC to lower the percentage of hemoglobin S to less than 45% is recommended preoperatively;
          - consider the need for preop exchange transfusion;
    - transfusion may be combined with adequate preoperative hydration, avoidance of intraopeative hypothermia, maintenance of blood volume, and postop O2 (35%) and hydration;
          - Augmentation by erythropoietin of the fetal-hemoglobin response to hydroxyurea in sickle cell disease.
          - Clinical trial of a hemoglobin based blood substitute in patients with sickle cell anemia.
    - fluids: once patient is NPO: rate of 3 L/msq/24 hr until PO fluid OK;
    - avoidance of intraoperative hypothermia;
    - postop O2 (35%) and hydration;
    - tourniquet use:
         - Stein and Urbaniak, 1980
                - no contraindication to use of tourniquets, no incr complications;
         - reference:
                - Use of the tourniquet during surgery in patients with sickle cell hemoglobinopathies.


Reference

Acute surgical illness in patients with sickle cell anemia.



Original Text by Clifford R. Wheeless, III, MD.

Last updated by Data Trace Staff on Wednesday, August 22, 2012 12:44 pm