Sickle Cell Anemia: Musculoskeletal Aspects

(see also: 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.