Avascular Necrosis in

Patients with Sickle Cell

Disease
Megan Gaugler

Vasoocclusive Pain Episodes Pathophysiology

Deoxygenation of RBCs causes the Hb
to polymerize into a long triple-helical
chain
Disrupts cell integrity
Ion fluxes leading to cell dehydration
Alteration of cell membrane composition

Increased RBC fragility which leads to

sickling
Chronic hemolytic anemia
Sticky cells leading to adhesions to
other blood cells and vascular
endothelium which causes occlusion

Vasoocclusion of Musculoskeletal
System
Vasoocclusion can occur almost anywhere
Most common places in musculoskeletal system include:
Bone marrow
Periosteum
Deep muscles

Slow movement of RBC through sinusoids and

capillaries + relative hypoxia increased likelihood of
sickling

Vasoocclusion Pain Episode

Clinical Presentation
Acute deep-seated pain
Mild erythema and warmth
Local tenderness to palpation
Possible low-grade fever
Acutely, plain radiographs do not show abnormalities
Necrosis and remodeling can be seen 1-2 weeks after an
event

Vasoocclusion Pain Episodes Treatment

Hydration 1.5-2L/m2/day
Warm packs
Pain and anti-inflammatory medications
Continued pain monitoring

Oxygenation monitoring

Avascular Necrosis
Common complication of SCD
Most common in head of humerus and head of femur

AVN of Humeral Head

5% of people with SCD have
radiographic evidence of osteonecrosis
of one or both shoulders
High likelihood of progressing to
collapse
As high as 86% of patients have
humeral head collapse 20 years after
AVN diagnosis with the median interval
between onset of pain and collapse
being 8 years.

AVN of Femoral Head

Occurs at any age
Prevalence increases with age
First symptoms of pain occur around 12 years old
Progresses from childhood into adulthood
80% of femoral heads with AVN showed to have both pain and
some sort of permanent damage (decreased mobility,
abnormal gait, limb-length discrepancy) 19 years after the
diagnosis

AVN of Femoral Head

Some have no pain at
diagnosis
Look at the other hip,
even with no
symptoms

AVN Imaging
An immature epiphysis can
undergo remodeling despite
initial necrosis and result in
flattening of the femoral
head
AVN of mature femoral
heads leads to collapse,
degenerative changes, and
pain
MRI will show increased
signal within the femoral
head

AVN Management
No general consensus
Start with conservative management

Pain management (especially in acute phase)

Reduced or non-weight bearing
Bed rest
Mobilization with crutches or wheelchair
Sustained PT to maintain range of motion, strengthen
muscles, gait training

Try for 4-6 months before surgical intervention

AVN Treatment
Core decompression

Removes necrotic tissue +/- filling cored area with bone graft
Unknown if this slows progression of joint damage
PT alone shown to be as effective as core decompression + PT
No evidence that adding core decompression to PT resulted in
clinical improvement
When looked at for all etiologies, surgery showed benefits in
early cases of AVN
Pathophysiology of ANV in SCD vs non-SCD could differ,
therefore use conservative management first

AVN Treatment
Arthroplasty
Reserved for those not helped by conservative and core
decompression management
Many cases do not provide better or prolonged mobility
Can have high post-operative morbidity, but improving
Failure rate around 19% at 15 years, but improving
If using cementless prosthetic components, outcomes are
comparable to those outcomes in patients without SCD

Although treatments are improving, it is still

recommended to start with conservative therapy

References
George, A., & DeBaun, M. R. (2014, 11 13). Bone and Joint Complications in Sickle Cell
Disease. Retrieved
from UpToDate:
http://www.uptodate.com/contents/bone-and-joint-complications-in-sickle-celldisease?
source=search_result&search=sickle+cell+bone+disease&selectedTitle=1%7E150#H99
3585
Lee E. Rubin, Scott A. Ritterman and Timothy McTighe (2013). Neck-Sparing Total Hip
Arthroplasty,
Arthroplasty - Update, Prof. Plamen Kinov (Ed.), ISBN: 978-953-51-0995-2, InTech, DOI:
10.5772/54830. Available from: http://www.intechopen.com/books/arthroplastyupdate/-necksparing-total-hip-arthroplasty
Luijkx, T., & Gaillard, F. (2014, 11 13). Avascular Necrosis. Retrieved from Radiopaedia:
http://radiopaedia.org/
articles/avascular-necrosis
Shetty, A., & Gaillard, F. (2014, 11 13). Avascular Necrosis of the Hip Classification.
Retrieved from Radiopaedia:
http://radiopaedia.org/articles/avascular-necrosis-of-the-hip-classification