Orthopaedic Manifestations of Sickle Cell Disease :
- Infections are common in Diaphysis
- Common organisms – Salmonella and Staph.aureus
- Patients present with Acute Bony Crisis due to Bony Infarcts , which is also referred to as Hand Foot Syndrome when extremities are involved , most frequent between the ages of 6 months – 2 years
- In a painful crisis , the symptoms should subside within 24 – 48 hours with hydration
- Acute bony infarcts should be differentiated from Osteomyelitis
- Septic Arthritis is less common in sickle cell disease compared to Osteomyelitis and Staph.aureus is the most common organism
- Painful Crisis : gradual or rapid worsening of anemia due to RBC destruction and are associated with fever , nausea , icterus , vomiting , abdominal pain due to deformation of RBC’s which produces vaso-occlusion and tissue death.
- Other Clinical Features : Hepatosplenomegaly , cardiomegaly , chronic leg ulcers specially over malleoli
- Pulmonary abnormalities like pneumonia and infarction , abdominal pain , cholelithiasis , jaundice , peptic ulcer disease , haematuria , priapism
- Ostenecrosis of femoral and humeral heads is quite common as shown
- X rays show patchy radiolucency of the shaft with surrounding periostitis in the metacarpals , metatarsals and phalanges
- Femoral epiphyseal osteonecrosis may mimic Perthes’ disease but it is usually bilateral and seen in a much elder age group
- H vetrtebra : Central depression of the vertebral bodies with squared off endplate depressions
- For osteomyelitic changes – Newer beta lactam antibiotics and drainage if necessary
- For Osteonecrosis : Decompression in early stages and osteotomies later on
- Bone within Bone appearance
- The use of a tourniquet is allowed as it does not induce Sickling
Very informative….