- 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.
DM Dines et al.
JBJS. Vol 58-A. 1976. p 662-666.
-
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.
-
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);
-
naturual 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.
Orthopedics. Apr 2000. p 357
-
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.
P Hernigou MD et al.
JBJS Vol 79-A.
No 11. Nov 1997. p 1726.
- The shoulder in sickle cell disease.
HG David et al.
JBJS. Vol 75-B. 1993. p 538-545.
-
The shoulder in sickle-cell disease.
-
Osteonecrosis of the humeral head in 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;
- references:
-
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 DL Skaggs et al, JBJS Vol 83-A, No 12, Dec 2001, the authors used radionucleotide scanning inorder 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;
- 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 intraopeative hypothermia;
- postop O2 (35%) and hydration;
- tourniquet use:
- Stein and Urbaniak, CORR 1980
- no contraindication to use of tourniquets, no incr complications;
- references:
-
Use of the tourniquet during surgery in patients with sickle cell hemoglobinopathies.
Acute surgical illness in patients with sickle cell anemia.
