Sickle Cell Disease – What an Orthopaedician should know !

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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

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