MedPix Medical Image Database

COW - Case of the WeekCase Contributor: Victor Longo IIIAffiliation: Philadelphia College of Osteopathic Medicine

MedPix No: 13263 - HistoryPt Demographics: Age = 25 y.o. Gender = woman25 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.

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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

Sickle Cell Disease

May, one year ago, Coronal PDFS knee MRI shows patchy bone marrow edema (white) suggesting evolving marrow infarcts.

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Sickle Cell Disease

May, one year ago - Sag PDFS knee MRI shows patchy bone marrow edema (white) suggesting evolving marrow infarcts.

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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.

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Sickle Cell Disease

May, one year ago, MRI of the pelvis demonstrates no sign of AVN on this T2FS image.

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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.

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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.

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Sickle Cell Disease

Frog leg lateral of left hip January this year show subchondral collapse from avascular necrosis of the femoral head.

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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.

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Sickle Cell Disease

Coronal CT August, one year ago, with AVN of the right hip (sclerosis and subchondral collapse) and a left THA.

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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.

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Sickle Cell Disease

August last year, with mild endplate depressions (early H shape)and patchy sclerosis of L4 and L5 suggesting bone infarcts.

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Sickle Cell Disease

Bilateral total hip arthroplasties (THA) for treatment of AVN secondary to Sickle Cell Disease.

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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.

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

Diagnosis: Homozygous Sickle Cell Disease with Avascular NecrosisDx Confirmed by: Blood Smear and imaging

DISCUSSIONSickle 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=AVN&srch_type=all- - MRI of SSD - http://www.radsource.us/clinic/0511