Module 53, General Orthopaedist Level

1,2,3

This 9 year old girl was diagnosed as having sickle cell anemia during early childhood. She experienced two major crises each year, treated with hydration and pain medication. Her first bone symptoms occured at age 9 following a crises with concomitant left hip pain(image1 & 2). Bone scan was also obtained which demonstrated a cold area. This girl was followed until age 14 when she presented with a 2 month history of left hip pain, aggravated with activity. Clinical findings were again not suggestive of infection (image 3).

Question 53A

This is a late onset Perthes disease of the femoral head and is best treated with an abduction orthosis to maintain containment.

Question 53B

The time period of the first bone episode at age 9 until her symptomatic disease at age 14 is a common occurrence since the avascular bone does not become symptomatic for many years.

Question 53C

A good treatment option at this stage at age 14 is to do a core decompression.

Question 53D

You reassure the family that nothing could have done differently at age 9 when she presented and further you reassure the family that 1/4 of all individuals with sickle cell disease will get femoral head avascular necrosis..

Question 53E

Intravascular sickling of RBC's in the bones causes an inflammatory response which in part leads to avascular necrosis.

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