SlideShare a Scribd company logo
1 of 42
Download to read offline
Discuss the Orthopaedic
Manifestations of SCD &
their Management
Dr. Arojuraye S.A
National Orthopaedic Hospital
Dala-Kano
Outline
 Introduction
 Pathophysiology
 Orthopaedic manifestations
 Management
 Conclusion
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
Introduction…
Incidence
 Black American
 AS = 8 – 10%
 SS = 0.2 - 0.5%
 West African
 AS = 20%
Introduction…
Diagnosis
 Peripheral blood smear = sickle rbc
 Hb electrophoresis = abnormal haemoglobins
 Diagnosis of the major genotypes = simple
 Differentiation of various subtypes = complex
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
Pathophysiology
 Hypoxia & dehydration
 Sickling
 Membrane distortion
 Rbc stickiness
Haemolysis &
Vaso-occlusion
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.
Pathophysiology…
Clinical manifestations
 CNS: CVA, meningitis
 Ocular: Retinopathy
 Pulmonary: Acute chest syndrome, pneumonia,
restrictive lung disease
Clinical manifestations…
 Renal: Haematuria, nephropathy
 Spleen: Massive splenomegaly, autosplenectomy
 Biliary: Cholelithiasis, cholecystitis
 CVS: HF, MI, cor pulmonale
Orthopaedic manifestations
Acute
 Vaso-occlusive crisis
 Osteomyelitis
 Septic arthritis
 Stress fracture
 Vertebral collapse
Chronic
 Osteonecrosis
 Chronic arthritis
 Chronic osteomyelitis
 Osteoporosis
 Impaired growth
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
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
Vaso-occlusive crisis…
Plain radiograph
 Diagnosis is clinical
 Not useful in acute phase
 Later: subchondral &
intramedullary lucency &
patchy sclerosis
Vaso-occlusive crisis…
MRI
Radioisotope scan
 Very sensitive
 Not specific (Infarction vs OM)
 Not routinely used
Vaso-occlusive crisis…
Dactylitis (hand-foot syndrome)
 Described by Danford
 Subsequently Xterized by Smith
 Under 5yrs (typically 6 – 18months)
 Small bones of hands & feet
Vaso-occlusive crisis…
Dactylitis
 Clinically; acute, painful swelling
 Erythema & warmth
 Most episodes resolve within 2 weeks
 Epiphyses = premature fusion & shortened fingers
Vaso-occlusive crisis…
Dactylitis
 Radiological evidence of
new bone (2weeks)
 ‘Moth-eaten’ appearance
bcs of cortical thinning &
irregular attenuation of
the medullary spaces
Vaso-occlusive crisis…
Dactylitis
 Histology:
 Infarction of the marrow, medullary trabeculae &
inner layer of the cortex
 Subperiosteal new bone formation
Vaso-occlusive crisis…
Treatment
 Infection, dehydration, acidosis, hypoxia, cold,
 IVF & analgesia
 Paracetamol
 NSAIDs
 Opioid derivatives
 Antibiotics
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
Osteomyelitis
Common organisms
 Staphylococcus aureus
 Salmonella (Hodges & Holt, 1951)
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
Osteomyelitis …
Plain radiograph
 Always first step
 Not diagnostic
 May exclude other dxs
 Bony changes in 2-3wks
Osteomyelitis …
USS
 Soft tissue disturbance
 Subperiosteal collection
 Useful for aspiration
Osteomyelitis …
 Bone scan
 MRI
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
Septic arthritis
 Not common
 Tends to occur with painful VOC
 Aetiology as in OM
 Salmonella arthropathy is very rare
Septic arthritis…
Rx
 Early aggressive drainage, debridement & splintage
 A two-week course of IV antibiotic
Osteonecrosis
AVN
 Most common complication
 More common in SC
 > 50% of SCD > 30yrs
 M:F = 1
Osteonecrosis…
Presentation
 There may be hx of trauma
 Pain, limited motion
 Occasionally with pain at rest
 May be asymptomatic (shoulder)
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
Osteonecrosis…
Plain radiographs
 Mottled attenuation of
the epiphysis
 Subchondral lucency
 Flattening/collapse of
the articular surfaces
 Narrow joint space
 Articular sclerosis &
osteophyte formation
Osteonecrosis…
MRI
 Best for early disease
 Double line sign
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
Osteopaenia & Osteoporosis
 Reduced bone mineral density (BM hyperplasia)
 Common in the vertebrae
 Vertebral collapse
Osteopaenia & Osteoporosis…
Radiograph
 Increased radiolucency
 Prominence of vertebral trabeculae
 Smooth, biconcave vertebrae
 ‘Fish-mouth’ vertebrae
Growth disturbance
BM hyperplasia
 Vertebrae
 Ischaemia of the
vertebral growth plate
 Disturbance of
vertebral growth
 Characteristic ‘H’
shaped vertebrae
 Some develop ‘tower’
vertebra
Growth disturbance…
 Long bones
 Premature closure of
epiphyses
 Impaired growth of the
long bones
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 .
References
 Dgere A, Ndjoko R, Docquier P, Mousny M, Rombouts J. Orthopaedic
complications associated with sickle-cell disease. Acta Orthop. Belg., 2006, 72,
741-747.
 Antonio A, Irene R. Bone involvement in sickle cell disease. British J Haemat,
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.
Principles and practice of surgery including pathology in the tropics, 4th ed;
51: 1015 – 25.
 Geraldo B, Elizabeth D, Francisco A. Osteoarticular involvement in sickle cell
disease. Review article. Rev Bras Hematol Hemoter. 2012;34(2):156-64

More Related Content

What's hot

Acute and sub-acute Osteomyelitis
Acute and sub-acute OsteomyelitisAcute and sub-acute Osteomyelitis
Acute and sub-acute OsteomyelitisAIIMS Bhopal
 
Tuberculosis of knee
Tuberculosis of kneeTuberculosis of knee
Tuberculosis of kneeArd Nepid
 
Lisfranc injury
Lisfranc injuryLisfranc injury
Lisfranc injuryMahak Jain
 
AVASCULAR NECROSIS OF HIP (AVN HIP)
AVASCULAR NECROSIS OF HIP (AVN HIP)AVASCULAR NECROSIS OF HIP (AVN HIP)
AVASCULAR NECROSIS OF HIP (AVN HIP)Dr.A.Mohan krishna
 
Avascular necrosis hip
Avascular necrosis hipAvascular necrosis hip
Avascular necrosis hipvinod naneria
 
Calcaneal fractures
Calcaneal fracturesCalcaneal fractures
Calcaneal fracturesRohit Vikas
 
Growth plate & Various disorders affecting growth plate by Dr.Vinay
Growth plate & Various disorders affecting growth plate by Dr.VinayGrowth plate & Various disorders affecting growth plate by Dr.Vinay
Growth plate & Various disorders affecting growth plate by Dr.VinayVenkat Vinay
 
Bone tumour , enchondroma , osteochondroma
Bone tumour , enchondroma , osteochondromaBone tumour , enchondroma , osteochondroma
Bone tumour , enchondroma , osteochondromaSagar Savsani
 
Pseudoarthrosis tibia
Pseudoarthrosis tibiaPseudoarthrosis tibia
Pseudoarthrosis tibiasaikat ghosh
 
Acetabulum fractures
Acetabulum fracturesAcetabulum fractures
Acetabulum fracturesmithilesh216
 
Principles of management of open fracture
Principles of management of open fracturePrinciples of management of open fracture
Principles of management of open fractureAbdullahi Sanusi
 
Compartment syndrome
Compartment syndromeCompartment syndrome
Compartment syndromeRohit Vikas
 
Tuberculosis of bones and joints
Tuberculosis of bones and jointsTuberculosis of bones and joints
Tuberculosis of bones and jointsVishal Sankpal
 
Tuberculosis of bones and joints
Tuberculosis of bones and jointsTuberculosis of bones and joints
Tuberculosis of bones and jointsairwave12
 

What's hot (20)

Acute and sub-acute Osteomyelitis
Acute and sub-acute OsteomyelitisAcute and sub-acute Osteomyelitis
Acute and sub-acute Osteomyelitis
 
Tuberculosis of knee
Tuberculosis of kneeTuberculosis of knee
Tuberculosis of knee
 
Lisfranc injury
Lisfranc injuryLisfranc injury
Lisfranc injury
 
Non Union
Non UnionNon Union
Non Union
 
AVASCULAR NECROSIS OF HIP (AVN HIP)
AVASCULAR NECROSIS OF HIP (AVN HIP)AVASCULAR NECROSIS OF HIP (AVN HIP)
AVASCULAR NECROSIS OF HIP (AVN HIP)
 
Avascular necrosis hip
Avascular necrosis hipAvascular necrosis hip
Avascular necrosis hip
 
Calcaneal fractures
Calcaneal fracturesCalcaneal fractures
Calcaneal fractures
 
Calcaneal fractures
Calcaneal fracturesCalcaneal fractures
Calcaneal fractures
 
Growth plate & Various disorders affecting growth plate by Dr.Vinay
Growth plate & Various disorders affecting growth plate by Dr.VinayGrowth plate & Various disorders affecting growth plate by Dr.Vinay
Growth plate & Various disorders affecting growth plate by Dr.Vinay
 
Bone tumour , enchondroma , osteochondroma
Bone tumour , enchondroma , osteochondromaBone tumour , enchondroma , osteochondroma
Bone tumour , enchondroma , osteochondroma
 
Pseudoarthrosis tibia
Pseudoarthrosis tibiaPseudoarthrosis tibia
Pseudoarthrosis tibia
 
Blounts disease
Blounts diseaseBlounts disease
Blounts disease
 
Acetabulum fractures
Acetabulum fracturesAcetabulum fractures
Acetabulum fractures
 
Suppurative Arthritis
Suppurative ArthritisSuppurative Arthritis
Suppurative Arthritis
 
perthes disease
perthes disease perthes disease
perthes disease
 
Osteogenesis imperfecta (dr. mahesh)
Osteogenesis imperfecta (dr. mahesh)Osteogenesis imperfecta (dr. mahesh)
Osteogenesis imperfecta (dr. mahesh)
 
Principles of management of open fracture
Principles of management of open fracturePrinciples of management of open fracture
Principles of management of open fracture
 
Compartment syndrome
Compartment syndromeCompartment syndrome
Compartment syndrome
 
Tuberculosis of bones and joints
Tuberculosis of bones and jointsTuberculosis of bones and joints
Tuberculosis of bones and joints
 
Tuberculosis of bones and joints
Tuberculosis of bones and jointsTuberculosis of bones and joints
Tuberculosis of bones and joints
 

Viewers also liked

Sickle cell disease
Sickle cell diseaseSickle cell disease
Sickle cell diseaseYara Mostafa
 
Discusstheclinicalmanifestationsmanagementofacute 150423161917-conversion-gate02
Discusstheclinicalmanifestationsmanagementofacute 150423161917-conversion-gate02Discusstheclinicalmanifestationsmanagementofacute 150423161917-conversion-gate02
Discusstheclinicalmanifestationsmanagementofacute 150423161917-conversion-gate02Soliudeen Arojuraye
 
Sickle cell anemia
Sickle cell anemiaSickle cell anemia
Sickle cell anemiashamsheerpt
 
Hematology Rivas2009lecture2
Hematology Rivas2009lecture2Hematology Rivas2009lecture2
Hematology Rivas2009lecture2Miami Dade
 
Diagnositc Imaging of Bone Marrow Disease
Diagnositc Imaging of Bone Marrow DiseaseDiagnositc Imaging of Bone Marrow Disease
Diagnositc Imaging of Bone Marrow DiseaseMohamed M.A. Zaitoun
 
Peripheral smear..RBC disorders.. Dr.Padmesh
Peripheral smear..RBC disorders.. Dr.PadmeshPeripheral smear..RBC disorders.. Dr.Padmesh
Peripheral smear..RBC disorders.. Dr.PadmeshDr Padmesh Vadakepat
 
Structure and function of hemoglobin
Structure and function of hemoglobinStructure and function of hemoglobin
Structure and function of hemoglobinAsif Zeb
 
Acute and Chronic Osteomyelitis - Infection of Bone
Acute and Chronic Osteomyelitis - Infection of BoneAcute and Chronic Osteomyelitis - Infection of Bone
Acute and Chronic Osteomyelitis - Infection of BoneRahul Singh
 
Sickle Cell Anemia. Student Presentation
Sickle Cell Anemia. Student PresentationSickle Cell Anemia. Student Presentation
Sickle Cell Anemia. Student PresentationSEPA_genomics
 

Viewers also liked (15)

Sickle cell disease
Sickle cell diseaseSickle cell disease
Sickle cell disease
 
Sickle cell disease
Sickle cell diseaseSickle cell disease
Sickle cell disease
 
Discusstheclinicalmanifestationsmanagementofacute 150423161917-conversion-gate02
Discusstheclinicalmanifestationsmanagementofacute 150423161917-conversion-gate02Discusstheclinicalmanifestationsmanagementofacute 150423161917-conversion-gate02
Discusstheclinicalmanifestationsmanagementofacute 150423161917-conversion-gate02
 
Sickle cell anemia
Sickle cell anemiaSickle cell anemia
Sickle cell anemia
 
The Treatment of Sickle Cell Disease
The Treatment of Sickle Cell DiseaseThe Treatment of Sickle Cell Disease
The Treatment of Sickle Cell Disease
 
Sickle cell disease
Sickle cell diseaseSickle cell disease
Sickle cell disease
 
Adult Stem cells in Orthopaedics
Adult Stem cells in OrthopaedicsAdult Stem cells in Orthopaedics
Adult Stem cells in Orthopaedics
 
Hematology Rivas2009lecture2
Hematology Rivas2009lecture2Hematology Rivas2009lecture2
Hematology Rivas2009lecture2
 
Sickle Cell Disease
Sickle Cell DiseaseSickle Cell Disease
Sickle Cell Disease
 
Diagnositc Imaging of Bone Marrow Disease
Diagnositc Imaging of Bone Marrow DiseaseDiagnositc Imaging of Bone Marrow Disease
Diagnositc Imaging of Bone Marrow Disease
 
Peripheral smear..RBC disorders.. Dr.Padmesh
Peripheral smear..RBC disorders.. Dr.PadmeshPeripheral smear..RBC disorders.. Dr.Padmesh
Peripheral smear..RBC disorders.. Dr.Padmesh
 
Structure and function of hemoglobin
Structure and function of hemoglobinStructure and function of hemoglobin
Structure and function of hemoglobin
 
Osteomyelitis
OsteomyelitisOsteomyelitis
Osteomyelitis
 
Acute and Chronic Osteomyelitis - Infection of Bone
Acute and Chronic Osteomyelitis - Infection of BoneAcute and Chronic Osteomyelitis - Infection of Bone
Acute and Chronic Osteomyelitis - Infection of Bone
 
Sickle Cell Anemia. Student Presentation
Sickle Cell Anemia. Student PresentationSickle Cell Anemia. Student Presentation
Sickle Cell Anemia. Student Presentation
 

Similar to Discuss the orthopaedic manifestations of sickle cell disease

Avascular necross
Avascular necrossAvascular necross
Avascular necrossramarawand
 
Pagests disease,eosinophilic granuloma,heterotopic ossification
Pagests disease,eosinophilic granuloma,heterotopic ossificationPagests disease,eosinophilic granuloma,heterotopic ossification
Pagests disease,eosinophilic granuloma,heterotopic ossificationluay hassan
 
Arthritis and arthroplasty- dr. Mahmoud Abdel Kareem
Arthritis and arthroplasty- dr. Mahmoud Abdel KareemArthritis and arthroplasty- dr. Mahmoud Abdel Kareem
Arthritis and arthroplasty- dr. Mahmoud Abdel KareemAhmed-shedeed
 
Tuberculosis of the skeletal system - surgical needs
Tuberculosis of the skeletal system - surgical needsTuberculosis of the skeletal system - surgical needs
Tuberculosis of the skeletal system - surgical needsPraveen Yadav
 
Transient Osteoporosis of Hip
Transient Osteoporosis of HipTransient Osteoporosis of Hip
Transient Osteoporosis of Hipvinod naneria
 
Presentation1.pptx, radiological imaging of paget disease.
Presentation1.pptx, radiological imaging of paget disease.Presentation1.pptx, radiological imaging of paget disease.
Presentation1.pptx, radiological imaging of paget disease.Abdellah Nazeer
 
Orthopedic Aspects Of Metabolic Bone Disease By Xiu
Orthopedic Aspects Of Metabolic Bone Disease By XiuOrthopedic Aspects Of Metabolic Bone Disease By Xiu
Orthopedic Aspects Of Metabolic Bone Disease By XiuXiu Srithammasit
 
Avascular Necrosis
Avascular NecrosisAvascular Necrosis
Avascular NecrosisJavaid Meo
 
Neuropathic (Charcots) joints
Neuropathic (Charcots) joints Neuropathic (Charcots) joints
Neuropathic (Charcots) joints Subodh Pathak
 
Charcot joint / Neuropathic Joint
Charcot joint / Neuropathic JointCharcot joint / Neuropathic Joint
Charcot joint / Neuropathic Jointmaulik patel
 
Sickle cell disease (bone changes)_Torfs
Sickle cell disease (bone changes)_TorfsSickle cell disease (bone changes)_Torfs
Sickle cell disease (bone changes)_TorfsMichaël Torfs
 
Presentation1, radiological imaging of ostepopikilosis
Presentation1, radiological imaging of ostepopikilosisPresentation1, radiological imaging of ostepopikilosis
Presentation1, radiological imaging of ostepopikilosisAbdellah Nazeer
 

Similar to Discuss the orthopaedic manifestations of sickle cell disease (20)

Avascular necross
Avascular necrossAvascular necross
Avascular necross
 
Avascular necross
Avascular necrossAvascular necross
Avascular necross
 
Pagests disease,eosinophilic granuloma,heterotopic ossification
Pagests disease,eosinophilic granuloma,heterotopic ossificationPagests disease,eosinophilic granuloma,heterotopic ossification
Pagests disease,eosinophilic granuloma,heterotopic ossification
 
Avn
AvnAvn
Avn
 
Arthritis and arthroplasty- dr. Mahmoud Abdel Kareem
Arthritis and arthroplasty- dr. Mahmoud Abdel KareemArthritis and arthroplasty- dr. Mahmoud Abdel Kareem
Arthritis and arthroplasty- dr. Mahmoud Abdel Kareem
 
Ghega
GhegaGhega
Ghega
 
Tuberculosis of the skeletal system - surgical needs
Tuberculosis of the skeletal system - surgical needsTuberculosis of the skeletal system - surgical needs
Tuberculosis of the skeletal system - surgical needs
 
Transient Osteoporosis of Hip
Transient Osteoporosis of HipTransient Osteoporosis of Hip
Transient Osteoporosis of Hip
 
Presentation1.pptx, radiological imaging of paget disease.
Presentation1.pptx, radiological imaging of paget disease.Presentation1.pptx, radiological imaging of paget disease.
Presentation1.pptx, radiological imaging of paget disease.
 
AVN
AVNAVN
AVN
 
Avn
AvnAvn
Avn
 
Radiology 5th year, 3rd lecture (Dr. Salah Mohammad Fatih)
Radiology 5th year, 3rd lecture (Dr. Salah Mohammad Fatih)Radiology 5th year, 3rd lecture (Dr. Salah Mohammad Fatih)
Radiology 5th year, 3rd lecture (Dr. Salah Mohammad Fatih)
 
Orthopedic Aspects Of Metabolic Bone Disease By Xiu
Orthopedic Aspects Of Metabolic Bone Disease By XiuOrthopedic Aspects Of Metabolic Bone Disease By Xiu
Orthopedic Aspects Of Metabolic Bone Disease By Xiu
 
bone diseases.pptx
bone diseases.pptxbone diseases.pptx
bone diseases.pptx
 
Radiology 5th year, 4th lecture (Dr. Salah Mohammad Fatih)
Radiology 5th year, 4th lecture (Dr. Salah Mohammad Fatih)Radiology 5th year, 4th lecture (Dr. Salah Mohammad Fatih)
Radiology 5th year, 4th lecture (Dr. Salah Mohammad Fatih)
 
Avascular Necrosis
Avascular NecrosisAvascular Necrosis
Avascular Necrosis
 
Neuropathic (Charcots) joints
Neuropathic (Charcots) joints Neuropathic (Charcots) joints
Neuropathic (Charcots) joints
 
Charcot joint / Neuropathic Joint
Charcot joint / Neuropathic JointCharcot joint / Neuropathic Joint
Charcot joint / Neuropathic Joint
 
Sickle cell disease (bone changes)_Torfs
Sickle cell disease (bone changes)_TorfsSickle cell disease (bone changes)_Torfs
Sickle cell disease (bone changes)_Torfs
 
Presentation1, radiological imaging of ostepopikilosis
Presentation1, radiological imaging of ostepopikilosisPresentation1, radiological imaging of ostepopikilosis
Presentation1, radiological imaging of ostepopikilosis
 

More from Soliudeen Arojuraye

Principles of Shoulder Arthroscopy.pptx
Principles of Shoulder Arthroscopy.pptxPrinciples of Shoulder Arthroscopy.pptx
Principles of Shoulder Arthroscopy.pptxSoliudeen Arojuraye
 
Management of peri prosthetic fractures
Management of peri prosthetic fracturesManagement of peri prosthetic fractures
Management of peri prosthetic fracturesSoliudeen Arojuraye
 
Poliomyelitis and its management
Poliomyelitis and its managementPoliomyelitis and its management
Poliomyelitis and its managementSoliudeen Arojuraye
 
Principles of management of volkmann’s contracture
Principles of management of volkmann’s contracturePrinciples of management of volkmann’s contracture
Principles of management of volkmann’s contractureSoliudeen Arojuraye
 
Discuss the differential diagnosis and management of a
Discuss the differential diagnosis and management of aDiscuss the differential diagnosis and management of a
Discuss the differential diagnosis and management of aSoliudeen Arojuraye
 
Discuss approaches to the knee and Describe in detail TKR
Discuss approaches to the knee and Describe in detail TKRDiscuss approaches to the knee and Describe in detail TKR
Discuss approaches to the knee and Describe in detail TKRSoliudeen Arojuraye
 
Principles of management of malignant bone tumours
Principles of management of malignant bone tumoursPrinciples of management of malignant bone tumours
Principles of management of malignant bone tumoursSoliudeen Arojuraye
 
Management of bladder injuries dr aroju
Management of bladder injuries dr arojuManagement of bladder injuries dr aroju
Management of bladder injuries dr arojuSoliudeen Arojuraye
 
Discuss the value of psa & gleason score
Discuss the value of psa & gleason scoreDiscuss the value of psa & gleason score
Discuss the value of psa & gleason scoreSoliudeen Arojuraye
 

More from Soliudeen Arojuraye (13)

Principles of Shoulder Arthroscopy.pptx
Principles of Shoulder Arthroscopy.pptxPrinciples of Shoulder Arthroscopy.pptx
Principles of Shoulder Arthroscopy.pptx
 
Management of knee dislocation
Management of knee dislocationManagement of knee dislocation
Management of knee dislocation
 
Management of peri prosthetic fractures
Management of peri prosthetic fracturesManagement of peri prosthetic fractures
Management of peri prosthetic fractures
 
Poliomyelitis and its management
Poliomyelitis and its managementPoliomyelitis and its management
Poliomyelitis and its management
 
Principles of management of volkmann’s contracture
Principles of management of volkmann’s contracturePrinciples of management of volkmann’s contracture
Principles of management of volkmann’s contracture
 
Discuss the differential diagnosis and management of a
Discuss the differential diagnosis and management of aDiscuss the differential diagnosis and management of a
Discuss the differential diagnosis and management of a
 
Discuss approaches to the knee and Describe in detail TKR
Discuss approaches to the knee and Describe in detail TKRDiscuss approaches to the knee and Describe in detail TKR
Discuss approaches to the knee and Describe in detail TKR
 
Principles of management of malignant bone tumours
Principles of management of malignant bone tumoursPrinciples of management of malignant bone tumours
Principles of management of malignant bone tumours
 
Principles of amputation
Principles of amputationPrinciples of amputation
Principles of amputation
 
Skin grafting
Skin graftingSkin grafting
Skin grafting
 
Management of bladder injuries dr aroju
Management of bladder injuries dr arojuManagement of bladder injuries dr aroju
Management of bladder injuries dr aroju
 
Discuss intestinal obstruction
Discuss intestinal obstructionDiscuss intestinal obstruction
Discuss intestinal obstruction
 
Discuss the value of psa & gleason score
Discuss the value of psa & gleason scoreDiscuss the value of psa & gleason score
Discuss the value of psa & gleason score
 

Recently uploaded

power point presentation of Clinical evaluation of strabismus
power point presentation of Clinical evaluation  of strabismuspower point presentation of Clinical evaluation  of strabismus
power point presentation of Clinical evaluation of strabismusChandrasekar Reddy
 
Bulimia nervosa ( Eating Disorders) Mental Health Nursing.
Bulimia nervosa ( Eating Disorders) Mental Health Nursing.Bulimia nervosa ( Eating Disorders) Mental Health Nursing.
Bulimia nervosa ( Eating Disorders) Mental Health Nursing.aarjukhadka22
 
SGK NGẠT NƯỚC ĐHYHN RẤT LÀ HAY NHA .pdf
SGK NGẠT NƯỚC ĐHYHN RẤT LÀ HAY NHA    .pdfSGK NGẠT NƯỚC ĐHYHN RẤT LÀ HAY NHA    .pdf
SGK NGẠT NƯỚC ĐHYHN RẤT LÀ HAY NHA .pdfHongBiThi1
 
pA2 value, Schild plot and pD2 values- applications in pharmacology
pA2 value, Schild plot and pD2 values- applications in pharmacologypA2 value, Schild plot and pD2 values- applications in pharmacology
pA2 value, Schild plot and pD2 values- applications in pharmacologyDeepakDaniel9
 
SGK RỐI LOẠN KALI MÁU CỰC KỲ QUAN TRỌNG.pdf
SGK RỐI LOẠN KALI MÁU CỰC KỲ QUAN TRỌNG.pdfSGK RỐI LOẠN KALI MÁU CỰC KỲ QUAN TRỌNG.pdf
SGK RỐI LOẠN KALI MÁU CỰC KỲ QUAN TRỌNG.pdfHongBiThi1
 
How to cure cirrhosis and chronic hepatitis naturally
How to cure cirrhosis and chronic hepatitis naturallyHow to cure cirrhosis and chronic hepatitis naturally
How to cure cirrhosis and chronic hepatitis naturallyZurück zum Ursprung
 
blood bank management system project report
blood bank management system project reportblood bank management system project report
blood bank management system project reportNARMADAPETROLEUMGAS
 
MedMatch: Your Health, Our Mission. Pitch deck.
MedMatch: Your Health, Our Mission. Pitch deck.MedMatch: Your Health, Our Mission. Pitch deck.
MedMatch: Your Health, Our Mission. Pitch deck.whalesdesign
 
"Radical excision of DIE in subferile women with deep infiltrating endometrio...
"Radical excision of DIE in subferile women with deep infiltrating endometrio..."Radical excision of DIE in subferile women with deep infiltrating endometrio...
"Radical excision of DIE in subferile women with deep infiltrating endometrio...Sujoy Dasgupta
 
AORTIC DISSECTION and management of aortic dissection
AORTIC DISSECTION and management of aortic dissectionAORTIC DISSECTION and management of aortic dissection
AORTIC DISSECTION and management of aortic dissectiondrhanifmohdali
 
Different drug regularity bodies in different countries.
Different drug regularity bodies in different countries.Different drug regularity bodies in different countries.
Different drug regularity bodies in different countries.kishan singh tomar
 
Generative AI in Health Care a scoping review and a persoanl experience.
Generative AI in Health Care a scoping review and a persoanl experience.Generative AI in Health Care a scoping review and a persoanl experience.
Generative AI in Health Care a scoping review and a persoanl experience.Vaikunthan Rajaratnam
 
historyofpsychiatryinindia. Senthil Thirusangu
historyofpsychiatryinindia. Senthil Thirusanguhistoryofpsychiatryinindia. Senthil Thirusangu
historyofpsychiatryinindia. Senthil Thirusangu Medical University
 
Female Reproductive Physiology Before Pregnancy
Female Reproductive Physiology Before PregnancyFemale Reproductive Physiology Before Pregnancy
Female Reproductive Physiology Before PregnancyMedicoseAcademics
 
ANATOMICAL FAETURES OF BONES FOR NURSING STUDENTS .pptx
ANATOMICAL FAETURES OF BONES  FOR NURSING STUDENTS .pptxANATOMICAL FAETURES OF BONES  FOR NURSING STUDENTS .pptx
ANATOMICAL FAETURES OF BONES FOR NURSING STUDENTS .pptxWINCY THIRUMURUGAN
 
Adenomyosis or Fibroid- making right diagnosis
Adenomyosis or Fibroid- making right diagnosisAdenomyosis or Fibroid- making right diagnosis
Adenomyosis or Fibroid- making right diagnosisSujoy Dasgupta
 
Neurological history taking (2024) .
Neurological  history  taking  (2024)  .Neurological  history  taking  (2024)  .
Neurological history taking (2024) .Mohamed Rizk Khodair
 
Breast cancer -ONCO IN MEDICAL AND SURGICAL NURSING.pptx
Breast cancer -ONCO IN MEDICAL AND SURGICAL NURSING.pptxBreast cancer -ONCO IN MEDICAL AND SURGICAL NURSING.pptx
Breast cancer -ONCO IN MEDICAL AND SURGICAL NURSING.pptxNaveenkumar267201
 

Recently uploaded (20)

power point presentation of Clinical evaluation of strabismus
power point presentation of Clinical evaluation  of strabismuspower point presentation of Clinical evaluation  of strabismus
power point presentation of Clinical evaluation of strabismus
 
How to master Steroid (glucocorticoids) prescription, different scenarios, ca...
How to master Steroid (glucocorticoids) prescription, different scenarios, ca...How to master Steroid (glucocorticoids) prescription, different scenarios, ca...
How to master Steroid (glucocorticoids) prescription, different scenarios, ca...
 
Bulimia nervosa ( Eating Disorders) Mental Health Nursing.
Bulimia nervosa ( Eating Disorders) Mental Health Nursing.Bulimia nervosa ( Eating Disorders) Mental Health Nursing.
Bulimia nervosa ( Eating Disorders) Mental Health Nursing.
 
SGK NGẠT NƯỚC ĐHYHN RẤT LÀ HAY NHA .pdf
SGK NGẠT NƯỚC ĐHYHN RẤT LÀ HAY NHA    .pdfSGK NGẠT NƯỚC ĐHYHN RẤT LÀ HAY NHA    .pdf
SGK NGẠT NƯỚC ĐHYHN RẤT LÀ HAY NHA .pdf
 
pA2 value, Schild plot and pD2 values- applications in pharmacology
pA2 value, Schild plot and pD2 values- applications in pharmacologypA2 value, Schild plot and pD2 values- applications in pharmacology
pA2 value, Schild plot and pD2 values- applications in pharmacology
 
Rheumatoid arthritis Part 1, case based approach with application of the late...
Rheumatoid arthritis Part 1, case based approach with application of the late...Rheumatoid arthritis Part 1, case based approach with application of the late...
Rheumatoid arthritis Part 1, case based approach with application of the late...
 
SGK RỐI LOẠN KALI MÁU CỰC KỲ QUAN TRỌNG.pdf
SGK RỐI LOẠN KALI MÁU CỰC KỲ QUAN TRỌNG.pdfSGK RỐI LOẠN KALI MÁU CỰC KỲ QUAN TRỌNG.pdf
SGK RỐI LOẠN KALI MÁU CỰC KỲ QUAN TRỌNG.pdf
 
How to cure cirrhosis and chronic hepatitis naturally
How to cure cirrhosis and chronic hepatitis naturallyHow to cure cirrhosis and chronic hepatitis naturally
How to cure cirrhosis and chronic hepatitis naturally
 
blood bank management system project report
blood bank management system project reportblood bank management system project report
blood bank management system project report
 
MedMatch: Your Health, Our Mission. Pitch deck.
MedMatch: Your Health, Our Mission. Pitch deck.MedMatch: Your Health, Our Mission. Pitch deck.
MedMatch: Your Health, Our Mission. Pitch deck.
 
"Radical excision of DIE in subferile women with deep infiltrating endometrio...
"Radical excision of DIE in subferile women with deep infiltrating endometrio..."Radical excision of DIE in subferile women with deep infiltrating endometrio...
"Radical excision of DIE in subferile women with deep infiltrating endometrio...
 
AORTIC DISSECTION and management of aortic dissection
AORTIC DISSECTION and management of aortic dissectionAORTIC DISSECTION and management of aortic dissection
AORTIC DISSECTION and management of aortic dissection
 
Different drug regularity bodies in different countries.
Different drug regularity bodies in different countries.Different drug regularity bodies in different countries.
Different drug regularity bodies in different countries.
 
Generative AI in Health Care a scoping review and a persoanl experience.
Generative AI in Health Care a scoping review and a persoanl experience.Generative AI in Health Care a scoping review and a persoanl experience.
Generative AI in Health Care a scoping review and a persoanl experience.
 
historyofpsychiatryinindia. Senthil Thirusangu
historyofpsychiatryinindia. Senthil Thirusanguhistoryofpsychiatryinindia. Senthil Thirusangu
historyofpsychiatryinindia. Senthil Thirusangu
 
Female Reproductive Physiology Before Pregnancy
Female Reproductive Physiology Before PregnancyFemale Reproductive Physiology Before Pregnancy
Female Reproductive Physiology Before Pregnancy
 
ANATOMICAL FAETURES OF BONES FOR NURSING STUDENTS .pptx
ANATOMICAL FAETURES OF BONES  FOR NURSING STUDENTS .pptxANATOMICAL FAETURES OF BONES  FOR NURSING STUDENTS .pptx
ANATOMICAL FAETURES OF BONES FOR NURSING STUDENTS .pptx
 
Adenomyosis or Fibroid- making right diagnosis
Adenomyosis or Fibroid- making right diagnosisAdenomyosis or Fibroid- making right diagnosis
Adenomyosis or Fibroid- making right diagnosis
 
Neurological history taking (2024) .
Neurological  history  taking  (2024)  .Neurological  history  taking  (2024)  .
Neurological history taking (2024) .
 
Breast cancer -ONCO IN MEDICAL AND SURGICAL NURSING.pptx
Breast cancer -ONCO IN MEDICAL AND SURGICAL NURSING.pptxBreast cancer -ONCO IN MEDICAL AND SURGICAL NURSING.pptx
Breast cancer -ONCO IN MEDICAL AND SURGICAL NURSING.pptx
 

Discuss the orthopaedic manifestations of sickle cell disease

  • 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.
  • 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
  • 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  Dgere A, Ndjoko R, Docquier P, Mousny M, Rombouts J. Orthopaedic complications associated with sickle-cell disease. Acta Orthop. Belg., 2006, 72, 741-747.  Antonio A, Irene R. Bone involvement in sickle cell disease. British J Haemat, 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. Principles and practice of surgery including pathology in the tropics, 4th ed; 51: 1015 – 25.  Geraldo B, Elizabeth D, Francisco A. Osteoarticular involvement in sickle cell disease. Review article. Rev Bras Hematol Hemoter. 2012;34(2):156-64