Purely medical

1. Discuss the Orthopaedic
Manifestations of SCD &
their Management
Dr. Arojuraye S.A
National Orthopaedic Hospital
Dala-Kano

2. Outline
 Introduction
 Pathophysiology
 Orthopaedic manifestations
 Management
 Conclusion

3. Introduction
 SCD = Inherited disorder due to HbS
 Substitution of valine for glutamic acid at 6th a.a of the
ß globin chain
 Inheritance = autosomal recessive
 Major genotypes = SS, SC & sickle-beta thalassemia
 HbS is poorly soluble
 It assumes sickle shape when deoxygenated

4. Introduction…
Incidence
 Black American
 AS = 8 – 10%
 SS = 0.2 - 0.5%
 West African
 AS = 20%

5. Introduction…
Diagnosis
 Peripheral blood smear = sickle rbc
 Hb electrophoresis = abnormal haemoglobins
 Diagnosis of the major genotypes = simple
 Differentiation of various subtypes = complex

6. Introduction…
 1910: First description of SCD was made in Chicago
by Herrick.
 1927: Hahn & Gillespie showed that sickling of the
rbc was induced by deoxygenation & reversed with
reoxygenation.
 1949: Electrophoretic abnormalities of Hb were
demonstrated by Pauling et al

7. Pathophysiology
 Hypoxia & dehydration
 Sickling
 Membrane distortion
 Rbc stickiness
Haemolysis &
Vaso-occlusion

8. Pathophysiology…
 Haemolysis results from
destruction of the sickled
rbc by monocytes &
macrophages.
 Vaso-occlusion is due to
entrapment of sickled cells
in the microcirculation =
ischaemia.

9. Pathophysiology…

10. Clinical manifestations
 CNS: CVA, meningitis
 Ocular: Retinopathy
 Pulmonary: Acute chest syndrome, pneumonia,
restrictive lung disease

11. Clinical manifestations…
 Renal: Haematuria, nephropathy
 Spleen: Massive splenomegaly, autosplenectomy
 Biliary: Cholelithiasis, cholecystitis
 CVS: HF, MI, cor pulmonale

12. Orthopaedic manifestations
Acute
 Vaso-occlusive crisis
 Osteomyelitis
 Septic arthritis
 Stress fracture
 Vertebral collapse
Chronic
 Osteonecrosis
 Chronic arthritis
 Chronic osteomyelitis
 Osteoporosis
 Impaired growth

13. Vaso-occlusive crisis
VOC
 Affects virtually all patients
 Begins in late infancy & recurs throughout life
 Can occur in any organ
 Particularly common in bone marrow

14. Vaso-occlusive crisis…
Clinical presentation
 Intense pain
 Localized tenderness
 Swelling & erythema
 Febrile episodes
 Juxta-articlar area = joint effusion
 Leucocytosis are also common
 Most patients recover with no complications

15. Vaso-occlusive crisis…
Plain radiograph
 Diagnosis is clinical
 Not useful in acute phase
 Later: subchondral &
intramedullary lucency &
patchy sclerosis

16. Vaso-occlusive crisis…
MRI
Radioisotope scan
 Very sensitive
 Not specific (Infarction vs OM)
 Not routinely used

17. Vaso-occlusive crisis…
Dactylitis (hand-foot syndrome)
 Described by Danford
 Subsequently Xterized by Smith
 Under 5yrs (typically 6 – 18months)
 Small bones of hands & feet

18. Vaso-occlusive crisis…
Dactylitis
 Clinically; acute, painful swelling
 Erythema & warmth
 Most episodes resolve within 2 weeks
 Epiphyses = premature fusion & shortened fingers

19. Vaso-occlusive crisis…
Dactylitis
 Radiological evidence of
new bone (2weeks)
 ‘Moth-eaten’ appearance
bcs of cortical thinning &
irregular attenuation of
the medullary spaces

20. Vaso-occlusive crisis…
Dactylitis
 Histology:
 Infarction of the marrow, medullary trabeculae &
inner layer of the cortex
 Subperiosteal new bone formation

21. Vaso-occlusive crisis…
Treatment
 Infection, dehydration, acidosis, hypoxia, cold,
 IVF & analgesia
 Paracetamol
 NSAIDs
 Opioid derivatives
 Antibiotics

22. Osteomyelitis
 90% of SCD develop OM before 10yrs
 No bone is exempted
 Multifocal
 Precipitated by VOC
Risk factors in SCD
 Hyposplenism
 Impaired complement activity
 Infarcted or necrotic bone

23. Osteomyelitis
Common organisms
 Staphylococcus aureus
 Salmonella (Hodges & Holt, 1951)

24. Osteomyelitis …
Diagnosis
 Common mgt dilemma
 Failure = life threatening infection
 Erroneous diagnosis = unnecessary 6wks of antibiotics
 Pain, swelling, tenderness & pseudoparalysis
 Most common sites: Femur, tibia & humerus
 Often affect diaphysis
 Features are similar to those of VOC

25. Osteomyelitis …
Plain radiograph
 Always first step
 Not diagnostic
 May exclude other dxs
 Bony changes in 2-3wks

26. Osteomyelitis …
USS
 Soft tissue disturbance
 Subperiosteal collection
 Useful for aspiration

27. Osteomyelitis …
 Bone scan
 MRI

28. Osteomyelitis …
Treatment
 Based on organism
 3rd generation cephalosporin
 Ciprofloxacin in older children
 Drainage
 Bone drilling
 Once AO COM
 Conservative Rx, until involucrum is formed
 Sequestrectomy

29. Septic arthritis
 Not common
 Tends to occur with painful VOC
 Aetiology as in OM
 Salmonella arthropathy is very rare

30. Septic arthritis…
Rx
 Early aggressive drainage, debridement & splintage
 A two-week course of IV antibiotic

31. Osteonecrosis
AVN
 Most common complication
 More common in SC
 > 50% of SCD > 30yrs
 M:F = 1

32. Osteonecrosis…
Presentation
 There may be hx of trauma
 Pain, limited motion
 Occasionally with pain at rest
 May be asymptomatic (shoulder)

33. Osteonecrosis…
Common sites
 Femoral heads
 Head of the humerus
 Knee joint
 Small joints of the hands & feet
 Common to have multiple joints affected
 Hip: bilateral in >50%
 Shoulder : 74% have AVN of HOF

34. Osteonecrosis…
Plain radiographs
 Mottled attenuation of
the epiphysis
 Subchondral lucency
 Flattening/collapse of
the articular surfaces
 Narrow joint space
 Articular sclerosis &
osteophyte formation

35. Osteonecrosis…
MRI
 Best for early disease
 Double line sign

36. Osteonecrosis…
 Untreated, 87% of HOF will collapse within 5 years
 Bed rest & symptomatic Rx: unacceptable
 Early dx: Core decompression & osteotomy
 Late dx: Arthroplasty
SCD patients must be cared for in specialized centres
with expertise in SCD as they have a very high
incidence of perioperative complications

37. Osteopaenia & Osteoporosis
 Reduced bone mineral density (BM hyperplasia)
 Common in the vertebrae
 Vertebral collapse

38. Osteopaenia & Osteoporosis…
Radiograph
 Increased radiolucency
 Prominence of vertebral trabeculae
 Smooth, biconcave vertebrae
 ‘Fish-mouth’ vertebrae

39. Growth disturbance
BM hyperplasia
 Vertebrae
 Ischaemia of the
vertebral growth plate
 Disturbance of
vertebral growth
 Characteristic ‘H’
shaped vertebrae
 Some develop ‘tower’
vertebra

40. Growth disturbance…
 Long bones
 Premature closure of
epiphyses
 Impaired growth of the
long bones

41. Conclusion
 SCD prognosis is still poor
 Orthopaedic manifestations: major cause of morbidity
 Mgt of its orthopaedic complications is challenging
 Necrosis & infections are responsible for major
functional impairment
 Surgery is the mainstay of Rx of these complications
While waiting for new genetic therapy for SCD, the
surgeon will treat the complications but should keep
in mind that anaesthesia is more risky for these
patients .

42. References
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2005; 129:482–490
 Michael HH, Gary EF, James SM. Orthopeedic Manifestations of Sickle-Cell
Disease. J Bio Med, 1990; 63:195-207
 Louis S. Osteonecrosis and related disorders. Apley’s system of orthopaedics
and fractures, 9th ed; Hodder Arnold, 2010; 6: 103 – 15.
 Archampong EQ, Addo AO. Surgical aspects of the hemoglobinopathies.
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disease. Review article. Rev Bras Hematol Hemoter. 2012;34(2):156-64