1. MedPix Medical Image Database COW - Case of the Week Case Contributor: Victor Longo III Affiliation: Philadelphia College of Osteopathic Medicine

2. MedPix No: 13263 - History Pt Demographics: Age = 25 y.o. Gender = woman 25 y.o. woman c/o bilateral hip and right knee pain for 2 months. Increased pain with ambulation. Denies any injury, trauma, fever, or chills. Prior *CVA* at age 9. She gets blood transfusions every 6-8 weeks. Downloaded by (-1)

3. MedPix No: 13263 - EXAM & LABS Right Knee: Swelling, (-) effusion, pain on PROM Both hips: (-) swelling, decrease ROM/pain with passive internal/ external rotation, strength 5/5, neurovascular intact both LE

4. Sickle Cell Disease May, one year ago, Coronal PDFS knee MRI shows patchy bone marrow edema (white) suggesting evolving marrow infarcts. Downloaded by (-1)

5. Sickle Cell Disease May, one year ago - Sag PDFS knee MRI shows patchy bone marrow edema (white) suggesting evolving marrow infarcts. Downloaded by (-1)

6. Sickle Cell Disease May, one year ago, MRI of the pelvis demonstrates dark marrow signal on T1 - conversion from yellow to red marrow. However, there is no sign of AVN at this time. Downloaded by (-1)

7. Sickle Cell Disease May, one year ago, MRI of the pelvis demonstrates no sign of AVN on this T2FS image. Downloaded by (-1)

8. Sickle Cell Disease January this year, AP pelvis shows both abnormal increased density and contours of both femoral heads - most consistent with bilateral hip AVN (avascular necrosis) with subchondral collapse. Downloaded by (-1)

9. Sickle Cell Disease AP of left hip January this year shows both abnormal density and contours of the femoral head - most consistent with AVN (avascular necrosis) with subchondral collapse. Downloaded by (-1)

10. Sickle Cell Disease Frog leg lateral of left hip January this year show subchondral collapse from avascular necrosis of the femoral head. Downloaded by (-1)

11. Sickle Cell Disease July this year right hip with sclerosis and subchondral collapse. The patient has had a total hip arthroplasty replacement, on the left, for AVN. Downloaded by (-1)

12. Sickle Cell Disease Coronal CT August, one year ago, with AVN of the right hip (sclerosis and subchondral collapse) and a left THA. Downloaded by (-1)

13. Sickle Cell Disease Axial CT performed August this yearshows gallstones. Children with sickle cell disease have chronic hemolysis, and are prone to develop pigmented gallstones. Downloaded by (-1)

14. Sickle Cell Disease August last year, with mild endplate depressions (early H shape)and patchy sclerosis of L4 and L5 suggesting bone infarcts. Downloaded by (-1)

15. Sickle Cell Disease Bilateral total hip arthroplasties (THA) for treatment of AVN secondary to Sickle Cell Disease. Downloaded by (-1)

16. FINDINGS Knee MR - Patchy bone marrow edema, related to evolving infarcts. Yellow to red marrow conversion. Pelvis/Hips - Initial MR with no AVN, but yellow to red marrow conversion present. Increasing AVN and subchondral collapse on multiple plain films. Spine - Early endplate depressions (H shaped vertebra) and vague sclerosis (infarcts) in L4 and L5 on CT.

17. DIFFERENTIAL DIAGNOSIS What is your Differential Diagnosis? Knee - Evolving infarcts - Bone bruises - Hip Avascular Necrosis - Sickle cell disease - Lupus - Chronic steroids - Caisson*s disease -

18. Diagnosis: Homozygous Sickle Cell Disease with Avascular Necrosis Dx Confirmed by: Blood Smear and imaging

19. DISCUSSION Sickle cell disease can cause avascular necrosis (AVN) through thrombosis during Sickle Crisis and obstruction of the arteries supplying blood flow to and within the bones. - - Other cases of AVN (Avascular necrosis) - - http://rad.usuhs.edu/medpix/medpix.html?mode=image_finder&action=search&srchstr=AV N&srch_type=all - - MRI of SSD - http://www.radsource.us/clinic/0511