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Approach to Diagnosing Pathologic Fractures in Children
1. Approach to Pathologic Fractures
in Children
Bahaa Ali Kornah
Prof. of Orthopedic Surgery
Al-Azhar University. Cairo Egypt
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
4. A pathological fracture occurs
without adequate trauma and is
caused by pre-existent
pathological bone lesion
• Evaluation of the child with Patholgical
fractures is challenging, as no clear guidelines
exist to distinguish traumatic from
pathological fractures.
• Weakness in the bone can be attributable to
multiple etiologies.
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
5. Pathologic Fractures
• Bone is abnormal due to
A collagen problem
• or
A mineral problem
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
6. Pathologic Fractures
• Abnormal bone lacks normal
biomechanic and viscoelastic
properties
– Intrinsic processes
• Localized - Bone cyst, neoplasm, etc.
• Systemic - OI, osteopenia, osteopetrosis,
rickets, etc.
– Extrinsic processes
• Radiation, biopsy, defects after plate removal,
etc.
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
7. Pathologic Fractures
• Tumors
– Primary (begin –malignant)
– secondary
• Disorders associated with fragility fractures in
children
• -Primary conditions Genetic disorders
• -Secondary conditions
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
12. • Fractures through benign and malignant bone
tumours should be recognised and managed
appropriately by the treating orthopaedic
surgeon.
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
The Right Care,
the Right Place,
the Right Time.
14. •The orthopaedic
surgeon may be the first
to have opportunity to
make the diagnosis.
(malignancy, metabolic
disease, etc.)
Often Need to Do More than
Treat the Fracture
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
15. • Patholgical Fr. differ from fractures in
normal bone
Etiology
Natural history
Treatment of underlying abnormality
• Must treat both fracture and underlying
cause!
Often Need to Do More than
Treat the Fracture
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
16. Pathologic Fractures
Benign Tumors
• Fractures more common in benign tumors (vs
malignant tumors)
– Most asymptomatic prior to fracture
– Antecedent nocturnal/rest symptoms rare
– most common in children
• humerus
• femur
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
17. The most common benign bone tumours that
cause pathological fractures in children are
•
Unicameral bone cysts,
Aneurysmal bone cysts,
non-ossifying fibromas
Fibrous dysplasia.
Eosinophilic granuloma
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
18. • to determine the cause and guide treatment
by
• History,
• Physical examination and
• investigations = Lab +Radiologic assessment.
• Biopsy
• To identifying the underlying cause and to planning the right
treatment of a pathological fracture. Treatment must be
tailored to both the fracture and the underlying cause.
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
21. Clinical evaluation: History
• Different age (ability to communicate)
• History from the parents (observe ‘abnormal
• changes’ in their children’s physical or
• attitude)
• Accidental findings of x-ray
• Obvious episode of trauma
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
22. Clinical evaluation: History
• History should include information about:
• The patient's age: certain musculoskeletal
tumors are associated with specific age groups.
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
23. Clinical evaluation: History
Pain – most common, preceding fracture, night, constant,
dull, aggravated by activity
Pain before or after injury, including characterization of
pain and aggravating or alleviating factors.
• Trauma – Minor or no trauma?
– Less than anticipated for fracture pattern
Constitutional – anorexia, night sweats, weight loss, fatigue
Previous cancer
Carcinogen – smoking, radiation, occupational toxins
24. • Any antecedent pain?
–Only with activity vs. night pain
• Recent illness?
• Weight loss?
• Fevers?
Clinical evaluation: History
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
25. Clinical evaluation: History
• Ask about growth and development
• Dietary habits
• Kidney disease
– May suggest rickets or renal osteodystrophy, etc.
• Thyroid disease
• Family history
– Dysplasias, metabolic disorders, osteoporosis,
neuromuscular disorders, etc.
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
26. • Ask about prior malignancies, even in the
child!
• Families will not always volunteer this
information
Clinical evaluation: History
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
27. Clinical evaluation: History
• Medical history: it is important to note any
oncologic history or if there is any family
history remarkable for musculoskeletal
neoplasms, or related syndromes.
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
28. Physical examination
• Ability to cooperate
• Look for obvious bruises, swelling
• Feel for tenderness
• Strict limitation of movements
• Must compare with the uninvolved side
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
30. Physical examination
should cover:
• Examination of the skin, which can direct
diagnosis to an underlying systemic illness
such as neurofibromatosis or McCune-Albright
disease and rule out infection.
• Neurovascular examination.
• Soft-tissue involvement to include the size of
the lesion and characteristics such as mobility,
adherence to skin, overlying skin changes.
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
31. • Look for soft tissue mass vs. fracture
hematoma
• Other systems- skin, lymphatics, solid
organs
• Height - weight percentiles
Physical examination
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
32. 11 yo - Osteosarcoma
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
33. Physical examination
• Full extremity examination.
• Systemic examination, including the lymph
nodes as well as abdominal and pelvic
palpation.
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
34. • CBC with differential
• ESR
• Calcium (ionized), Phosphorus, Alkaline
phosphatase
• Bun/Cr
• Plasma protein elctropheresis and
immunoelectropheresis
• PTH
• Alpha-Fetoprotein (AFP) Test
Lab Tests
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
35. Radiological Examination
• 2 views + 2 joints
• Opposite limb if suspected growth plate
involvement
• 2 occasions
• Plain radiographs can offer a significant amount
of information and can determine if further
imaging studies, like MRI, are needed.
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
36. • Osteopenia
• Physeal width (rickets)
• Soft tissue calcifications
• Presence of mass
• Any periosteal reaction
Radiological Examination
Be suspicious!
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
37. Radiological Examination
• Effect on the bone:
• lytic,
• blastic, or
• mixed.
• Pathologic fracture is common in a lytic lesion
• Bone's response:
• if the bone has time to respond to the destructiveness of the
lesion and cortical thickening results, this may offer some
containment of the lesion and "protect" from a pathologic
fracture.
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
38. • Is pathology…
–Localized and isolated?
–Polyostotic?
–Generalized to entire skeletal
system?
–A generalized condition with skeletal
manifestations?
Radiological Examination
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
39. • Where is lesion located?
• What is lesion doing to
bone?
• What is bone doing to
lesion?
• Are there clues to type of
lesion?
Enneking’s 4 Questions
Enneking, et al. The surgical staging of MSK sarcoma. JBJS 62-A:1027-1030, 1980.
Enneking. A System of Staging MSK Neoplasms. CORR 204:9-24, 1986.
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
40. Radiological Examination
• Location of the lesion: specific tumors
occur more often in certain bones and
within specific areas of that bone.
• Size and extent of the lesion:
aggressive lesions often are large and
fast growing. A more aggressive or
malignant lesion will have a less-
defined zone of transition within the
bone.
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
41. •Size
•Margination
•Cortex
•Soft tissue mass
Benign vs. Malignant
Mankin’s Criteria
Gebhardt, Ready & Mankin. Tumors about the knee in children.
Clin Orthop 255:86-110, 1980.
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
42. • Benign bone lesion
• Malignant bone
lesion
• Infection
• Metabolic bone
disease
Categorize/Make
Diagnosis
• Skeletal dysplasia
• Neuropathic
• Osteopenia-disuse
• Overuse
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
52. What You Should Know Before
Surgery
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
53. "Don't touch lesions"
Tumor and tumor-like lesions
• Fibrous cortical defect
Nonossifying fibroma (healing phase)
Periosteal desmoid
Small, solitary focus of fibrous dysplasia
Intraosseous ganglion
Enchondroma in a short, tubular bone
Stress fracture
Avulsion fracture (healing stage)
Bone infarct
Bone island (enostosis) (References only)
Myositis ossificans
Degenerative and post traumatic cysts (References only)
Brown tumor of hyperparathyroidism
•
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
54. Treatment
• Union best achieved
by correcting
biomechanical and
biological
environment
• While chemo &
radiation slow
healing, they provide
a beneficial response
in presence of
rapidly dividing
malignant cells
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
55. Need for Help
Management problems
• discharge planning
• rehab planning
Medication
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
59. • Treatment by etiology (-)Unicameral bone cyst
• Aneurysmal bone cyst
• Non-ossifying fibroma
• Fibrous dysplasia
• Bone infection
• Sarcomas
• Bone metastasis
• Hematological malignancies
• Irradiated bone
• Osteogenesis imperfecta
• Sclerosing bone disorders
• Enchondromatosis
• Neuromuscular diseases
• Fracture after hardware removal or extended immobilization
• Fracture following limb lengthening
• Idiopathic juvenile osteoporosis
• Congenital pseudarthrosis of the tibiaBahaa Kornah- Al Azhar Un. Cairo - EGYPT
60. Non operative treatment
• Non pharmacological ttt - Splinting –
traction brace etc
• Pharmacological ttt pain control druges
• Radiotherapy
• Chemotherapy
• Ambulatory aid
• rehabilitation
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
61. General Measures:
• Control pain.
• Reduce forces with walking aid or by
placing patient at bed rest.
Activity:
• Reduce activity.
– Recommend ambulatory aid.
– If patient is unable to walk, recommend a wheelchair.
– If unable to control pain, recommend bed rest.
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
62. Nursing:
• Assess patient and recommend general
measures:
– Walking aid
– Activity reduction
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
63. NON OPERATIVE TREATMENT
Bracing:
• Bracing of an impending or actual pathologic fracture should
be performed if the patient is not a surgical candidate.
Nonsurgical candidates are those with limited life
expectancies, severe comorbidities, small lesions, or
radiosensitive tumors. Lesions most amenable to bracing are
those in the humeral diaphysis, forearm, and occasionally
the tibia
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
64. Prophylactic management
often is recommended if the weakened bone state is detected
before the fracture occurs.
• Impending fracture:
The goals of surgical treatment in a patient with an impending
pathologic fracture are to alleviate pain, reduce narcotic use,
restore skeletal stability, and regain functional independence.
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
65. •The Mirels system classifies the risk of pathologic fracture based on
scoring four variables on a scale of 1-3: location of lesion, radiographic
appearance, size, and pain. An overall score is calculated, and a
recommendation for or against prophylactic fixation is made.
•a Size is determined as a fraction of the diameter of the bone.
b Functional pain is defined as severe pain or pain aggravated by limb function.
321
Intertrochan
teric
Lower
extremity
Upper
extremity
Location
LyticMixedBlasticRadiographic
appearance
>2/31/3 - 2/3< 1/3Sizea
FunctionalbModerateMildPain
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
66. Recommendation%Fracture Risk
Prophylactic fixation is
recommended
33-100≥9
Clinical judgment should be
used
15=8
Observation and radiation
therapy can be used
<4≤7
Commonly, a lesion is considered to be at risk for fracture if it is painful,
larger than 2.5 cm, and involves more than 50% of the cortex
Advantages of prophylactic management :
Decreased morbidity
Decreased hospital stay
Easier rehabilitation
More immediate pain relief
Faster surgery and less complications
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
69. Operative ttt
• Curates and bone graft
• Curates and bone cement
• Bone blast
• Fixation ** internal medullary or surface
• ** external fixation
• Resection and Replacement
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
71. MANAGEMENT
Initial Stabilization
• Many patients have bone pain with activity, and it may occur weeks to
months before pathologic fracture.
– When activity-related pain exists with a radiographically
documented destructive lesion, an ambulatory support to reduce
loading should be recommended.
• Walker
• 2 crutches
• Single cane
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
72. OPERATIVE TREATMENT
• Goals of surgical intervention are:
– Prevention of disuse osteopenia.
– Mechanical support for weakened or fractured bone to permit the
patient to perform daily activities.
– Pain relief.
– Decreased length and cost of hospitalization.
• Internal fixation, with or without cement augmentation, is
the standard of care for most pathologic fractures,
particularly long bones. Internal fixation will eventually fail if
the bone does not unite.
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
73. • in the upper limb have traditionally been managed
with bridge plate stabilization
• intramedullary nailing may be indicated in humerus
shaft fracture.
• External fixation or cast immobilization usually is
preferred in case of osteomyelitis
• If bone loss is significant, the defect can be filled
with autogenous bone graft, a vascularized osseous
graft, or bone transport using the Ilizarov
technique.
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
74. • Subtrochnteric and intertrochanteric fracture
treated with reconstruction nail or interlocking
nail.
• femoral head and neck usually are best treated by
removal of the head and neck and replacement
with a femoral head prosthesis, If the acetabulum is
not involved, a hemiarthroplasty may be indicated;
however, with acetabular involvement, total hip
replacement is required.
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
75. • Single vertebral metastasis with cord compression: Surgery
• Multiple spinal metastasis : Radiotherapy
• Diffuse skeletal metastasis with severe pain : Radionuclide
therapy
Simple bone cysts tend to disappear once decompressed by
the trauma. Treated as any other fracture. In other words
the majority will need simple reduction and a plaster cast.
The exception is a fracture about the proximal femur. Here
open reduction and internal fixation is preferred.
• Recurrence of a cyst is an indication to do curretage and
bone graft
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
76. Amputation may needed in
• Pathological fracture through a high-grade sarcoma,especially if there is
a poor response to induction chemotherapy → Forequarter amputation
entails surgical removal of the entire upper extremity, scapula, and
clavicle).
• contamination of soft tissue during biopsy.
• non healing pathological fracture.
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
78. • Not true neoplasms, etiology unknown
• Often loculated and not truly “unicameral”
• Most frequently contain serous fluid
• Usually metaphyseal
• Proximal humeral & femoral lesions account for
94% of all lesions
• Most in patients 3-14 years old, average age 9
• Males > females (2:1)
Simple Bone Cyst
(Unicameral Bone Cyst)
Baig & Eady. Unicameral (Simple) Bone Cysts. South Med J. 99(9):966-76, 2006.
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
79. • Fallen leaf sign (or fragment) is virtually
pathognomonic
• Treatment
– Fracture heals; cyst persist in 50-90%
– Humerus - treat fracture, address lesion after fracture
is healed, if felt to be necessary
– Displaced proximal femur #s - Open reduction,
grafting and rigid fixation, unless very young
– Posterior facet #s of the calcaneus - Open reduction,
if necessary with grafting and fixation
SBC Pathologic Fracture
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
80. • Controversial!
– Open Management
• Curettage/graft
• Bone graft substitutes
– Minimally invasive techniques (injections)
• Steroid injections
• Bone marrow injections
– All seem to work with similar frequency (~90%)
• But can be recurrence with any of them!
• Disrupt hydraulics- puncture, screw, wires, rods,
etc.
SBC Treatment
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
81. • 18 ga spinal needle
• C-arm
• Serous fluid, straw colored
• 2nd needle- vent
• Depo-Medrol 160 mg
• Watch for immediate drainage from large outflow
veins
• May need multiple injections
SBC Injection
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
82. LJ, 8 yo with arm pain when throwing, injected once with methylprednisolone
(multiple sites), healing at 3 months
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
83. SBC - Risk Factors for
Recurrence
• Only reliable predictor of treatment
success is age of the patient
– > 10 yrs heal ~ 90% of time
– < 10 yrs heal ~ 60% of time
• Most cysts tend to heal after skeletal
maturity
Baig & Eady. Unicameral (Simple) Bone Cysts. South Med J. 99(9):966-76, 2006.
Spence et al. Solitary unicameral bone cyst: treatment with freeze-dried crushed
cortical-bone allograft. JBJS-A 58:636-41, 1976
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
85. • Expansile
• Often wider than physis
• Eccentric
• Aggressive at margins
Aneurysmal Bone Cyst
(ABC)
Cottalorda & Bourelle, Current treatments of primary ABCs. J Pediatr Orthop B 15:155-67, 2006.
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
86. • Symptoms usually present for < 6 months
• Lesion may attain considerable size before
recognized
• Can exist as…
– primary bone lesion (70%)
– secondary lesion in other osseous conditions (30%)
• Pelvic lesions account for 50% of all flat bone
lesions (~10% total)
– Treatment is difficult due to inaccessibility and
integrity of acetabulum
Aneurysmal Bone Cyst
(ABC)
Cottalorda et al. Aneurysmal Bone Cysts of the Pelvis in Children. J Pediatr Orthop. 25:471-5, 2005.
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
89. ABC
Bur, et al. Fluid-fluid levels in a unicameral bone cyst: CT and MR findings. J Comput Assist Tomogr 17:134-6, 1993.
Papagelopoulos, et al. Treatment of aneurysmal bone cysts of the pelvis and sacrum. JBJS-A 83:1674-81, 2001.
Look for fluid-fluid levels on MRI
(however, not especially specific)
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
90. 5 yo female with 1 year of hip pain and 4 prior steroid injections,
progressive coxa vara. Biopsy = ABC
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
92. 1 month after curettage, bone grafting, valgus/internal fixation,
spica immobilization
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
93. • Curettage and bone graft
• +/- internal fixation
• ? Injection of fibrosing agent (Ethibloc,
Ethicon, etc.) is controversial
• High recurrence
ABC
Cottalorda & Bourelle, Current treatments of primary ABCs. J Pediatr Orthop B 15:155-67, 2006.
Adamsbaum et al. Intralesional Ethibloc injection in primary ABCs. Skeltal Radiol. 32:559-66, 2003.
Varshney et al. Is Sclerotherapy Better than Intralesional Excision for treating ABCs. CORR epib 2009.
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
94. Nonossifying Fibroma
(NOF)
• Benign, nonosteoid-producing lesion
• Usually found in metaphyses of long bones
• Prediliction for lower extremities
• Usually asymptomatic
• Often incidental radiographic finding
– It is speculated that up to a 1/3 of children may have at
least a minor NOF/fibrous cortical defect
• Almost always regress by early 20’s
Betsy et al. Metphyseal fibrous defects. J Am Acad Orthop Surg. 12:89-95, 2004.
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
95. Nonossifying Fibroma
(NOF)
• Most treated non-op!
• Let fracture heal, excellent healing potential
• Most NOF’s persist after #, but heal by skeletal
maturity
• If fractures once with minimal trauma, potential
risk to fracture again unless bone changes with
healing
• If necessary, treat with curettage/bone graft
Betsy et al. Metphyseal fibrous defects. J Am Acad Orthop Surg. 12:89-95, 2004.
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
96. 10 yo male - running during soccer.
NOF fracture - at 4 weeks underwent
allograft DBM / cancellous bone graft.
Healed at 9 mos.
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
97. NOF - Prophylactic Bone Graft?
• Are size parameters predictable?
– Arata and Peterson, JBJS 1981
• Review of 23 fractures over 50 years
• Suggest tx if greater than 50% diameter, >33 mm length
– Easley and Kneisl, JPO 1997
• Review of 22 lesions, many without #s, over 25 years
• Only included large lesions (above criteria)
• Only 41% had fractures, no refractures
• Suggest prophylactic surgery not necessary in many
• Criteria for surgery still not well defined
Arata et al. Pathological fxs through NOFs. JBJS-A. 63:980-8, 1981.
Easley & Kneisl. Pathologic fxs through NOFs: is prophylactic treatment
warranted? J Pediatr Orthop 17:808-13, 1997.
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
98. Fibrous Dysplasia
• Developmental disorder of bone, etiology unclear
– May be mutation leading to activation of c-fos oncogene
• Can be associated with endocrine disorders (McCune-
Albright syndrome)
• Can be mono- or polyostotic
• Usually affects adolescents and young adults
• Many solitary asymp lesions found incidentally
– Most do not require intervention
– If increased fracture risk, treat with curettage, bone grafting and
sometimes internal fixation
Parekh et al. Fibrous Dysplasia. J Am Acad Orthop Surg. 12:303-13, 2004.
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
99. Fibrous Dysplasia
• Surgical treatment for:
– Progressive lesions
– Large lesions with pain
• Pain & deformity suggest microfractures
– Failure of conservative treatment
– Less successful in:
• Younger patients
• Larger and proximal femoral lesions
• Polyostotic disease, esp McCune-Albright syndrome
Enneking & Gearen. Fibrous dysplasia of the femoral neck: Treatment by cortical
bone-grafting. JBJS-A 68:1415-22, 1986
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
100. Fibrous Dysplasia
• Lesions are never eradicated, even with grafting
• All grafts are eventually absorbed into dysplastic bone
• Cortical grafts at a much slower rate and therefore
recommended for weight-bearing bones
• Enneking suggested cortical struts alone for femoral
neck
• Fixation in WB bones seems to improve outcome in
children
Enneking & Gearen. Fibrous dysplasia of the femoral neck: Treatment by cortical
bone-grafting. JBJS-A 68:1415-22, 1986.
Stephenson et al. Fibrous dysplasia: An analysis of options for treatment. JBJS-A
69:400-9, 1987.Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
101. 14 yo female - fell walking across front yard
3 months of left hip pain - Motrin
Referred for “path fx through Ewing’s sarcoma”
Dx -polyostotic fibrous dysplasia
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
102. 3 Years Postop
Notice resorption of graft and recurrence of cystic changes in femoral
neck. Can have secondary ABC develop within fibrous dysplasia.
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
103. Fibrous Dysplasia
• Consider other sites (polyostotic disease)
• Bone scan to help identify other lesions
– Although lesions usually revealed on scan, a ‘cold’
bone scan does not rule out lesions
• For extensive involvement (McCune-Albright)
consider intramedullary fixation/splinting
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
104. 11 yo male – fem neck path fx,
nondisplaced. Fibular allograft (neck) and
titanium elastic nails (subtroch and shaft)
105. 13 yrs old – 2 years postop. lesions in Rt. femur
and tibia. No pain in hip, in karate.
Fibular graft gone.
Treat painful tibia? Nail? Pamidronate?
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
106. 5 yo - Albright’s polyostotic
fibrous dysplasia
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
107. Prophylactic Treatment of
Fibrous Lesions (NOF /FD)
• Any mechanical pain?
• Location and size - relative issues
• Supracondylar femur, proximal femur more
worrisome
• Pharmacologic approach (bisphosphonates) for
painful fibrous dysplasia – some reported
successes
Parekh et al. Fibrous Dysplasia. J Am Acad Orthop Surg. 12:303-13, 2004.
DiCaprio & Enneking. Fibrous dysplasia. Pathophysiology, evaluation and
treatment. JBJS-A87:1848-64, 2005.
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
108. • Abnormal type I collagen
– COL1A and COL2A defects
– Location and type of mutation in collagen molecule
determine phenotype (Sillence)
• Severe types (II-IV)- multiple fractures prior to
skeletal maturity
– Also find joint laxity, gray-blue sclera, dentogenesis
imperfecta, premature deafness, kyphoscoliosis &
basilar invagination
• Lower extremity > upper extremity
Osteogenesis Imperfecta
(OI)
Sillence et al. Genetic heterogeneity in osteogenesis imperfecta. J Med Gen. 16:101-16, 1979.
Van Dijk et atl. Classification of Osteogenesis Imperfecta revisited. Eur J Med Genet 53:1-5, 2010.
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
109. • Early onset (fxs prior to walking)- more fractures
(2x)
– Direct relationship between increased bone turnover
and severity of disease
• Closed tx- limit immobilization time to reduce
further osteopenia
• IM fixation often needed
– Also used for realignment surgery and to decrease
fracture risk
OI
D’Astous & Carroll: Connective Tissue Diseases, in Vaccaro (ed):
OKU 8. Rosemont, IL, AAOS, 2005.
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
110. 3 yr old OI - multiple fxs Lt femur
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
112. OI – Olecranon Fx
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
113. OI- New Methods
• Minimize disuse
osteoporosis
• Early IM fixation
• New design
– Expandable nails
• Bisphosphonates
– Increase bone density
• Osteoclast inhibitors
– Decrease fracture rate
– Oral and IV effective
Phillipi et al. Bisphosphonate therapy for OI. Cochrane Database Syst Rev 8(4), 2008
Panigrahi et al. Response to zolendronic acid in children with type 3 OI.
J Bone Biner Metab, Feb 4, 2010. [Epub ahead of print]
114. Ollier’s Disease
(Multiple Enchondromas)
• Linear masses of cartilage in
metaphyseal and diaphyseal
regions of long bones
• Asymmetric, often unilateral
• Usually sporadic occurrence
• Pathologic fx may occur
Lewis et al. Benign and malignant cartilage tumors.
Instr Course Lect 36:87-114, 1987.
115. 7 yo male - femur fracture jumping on bed
Enchondromatosis Rt. femur/tibia/pelvis
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
116. • Infection should always be in differential of
pathologic fractures
• However, pathologic fracture uncommon in
osteomyelitis
• Often delayed diagnosis
• More common in weight-bearing bones (i.e.
femur, tibia)
• Involucrum may be supportive of diagnosis
Osteomyelitis
Taylor et al. Childhood osteomyelitis presenting as a pathologic fracture.
Clin Rad 63:348-51, 2008.
Gelfand, et al. Path fx in acute osteo of long bones secondary to community
Acquired MRSA? Am J Med Sci 332:357-60, 2006.
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
118. 10 yo female ALL - chemotherapy/steroids
Fx after fall from chair. Tx = immobilization
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
119. • Myelomeningocele, paraplegics, sensory
neuropathies, etc.
• Exam frequently reveals warm,
erythematous, swollen joint
• Often mistaken for infection, DVT, tumor
Neuropathic Fractures
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
120. 3 yo with spina bifida, swollen leg
Consult = DVT vs. infection?
X-ray - healing fx may look like malignancy
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
121. 11 yo male - Duchene Muscular Dystrophy
Hip pain for 2 months. Disuse fracture/nonunion
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
122. • Usually through normal bone subjected to
abnormal stresses
• May be mistaken for more serious pathology
(esp. longitudinal stress fxs)
• History of recent increased activity
• Proximal tibia, distal fibula, metatarsals most
common
• Treat with rest, which can be challenging to
achieve
Stress Fractures
Heyworth & Green. Lower extremity stress fractures in pediatric and
adolescent athletes. Curr Opin Pediatr 20:58-61, 2008.
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
123. 12 yr old male, activity related pain, training for baseball, running 6
miles per day, referred for Ewing’s sarcoma.
Dx: Longitudinal femoral stress fracture
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
124. Stress Fractures
• Can occur through pathologic bone
• Congenital abnormalities, metabolic
disorders (osteoporosis, osteomalacia)
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
125. Congenital Tibial Dysplasia
Presented at age 10 after fracture from minor trauma
Had “bowed leg”her entire life
No other msk abnormalities
Sakamoto et al. Congenital pseudarthrosis of the tibia: analysis of the
histology and the NF1 gene. J Orthop Sci 12:361-5, 2007.
126. • Referral to musculoskeletal oncologist
• Requires complete staging
• Biopsy needed - follow proper “rules” for biopsy
• Notify pathologist of fracture
– Avoid fracture callus (histology may look malignant)
– Biopsy soft tissue mass
Malignant Appearing
Pathologic Fracture
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
128. Malignant Pathologic Fractures
• May need immediate amputation
• Osteosarcoma
– some fx may heal during neoadjuvant chemotherapy
• Ewing’s
– closed immobilization
– chemotherapy
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
129. Path fx lesser trochanter
Stage IIB
MRI - soft tissue mass
posterior
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
131. Pediatric Pathologic Fxs
• Be suspicious - scrutinize every fracture film!
• Most frequently a benign process
• Make the diagnosis to guide treatment
• Appropriate referral / workup for suspected
malignancy
• Prophylactic treatment for benign lesions on an
individual basis
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
132. Bibliography
•Enneking, et al. The surgical staging of MSK sarcoma. JBJS 62-A:1027-1030, 1980.
•Enneking. A System of Staging MSK Neoplasms. CORR 204:9-24, 1986.
•Gebhardt, Ready & Mankin. Tumors about the knee in children. Clin Orthop 255:86-110, 1980.
•Baig & Eady. Unicameral (Simple) Bone Cysts. South Med J. 99(9):966-76, 2006.
•Spence et al. Solitary unicameral bone cyst: treatment with freeze-dried crushed cortical-bone allograft. JBJS-A
58:636-41, 1976
•Cottalorda & Bourelle, Current treatments of primary ABCs. J Pediatr Orthop B 15:155-67, 2006.
•Cottalorda et al. Aneurysmal Bone Cysts of the Pelvis in Children. J Pediatr Orthop. 25:471-5, 2005.
•Bur, et al. Fluid-fluid levels in a unicameral bone cyst: CT and MR findings. J Comput Assist Tomogr 17:134-6,
1993.
•Papagelopoulos, et al. Treatment of aneurysmal bone cysts of the pelvis and sacrum. JBJS-A 83:1674-81,
2001.
•Adamsbaum et al. Intralesional Ethibloc injection in primary ABCs. Skeltal Radiol. 32:559-66, 2003.
•Varshney et al. Is Sclerotherapy Better than Intralesional Excision for treating ABCs. CORR epib 2009.
•Betsy et al. Metphyseal fibrous defects. J Am Acad Orthop Surg. 12:89-95, 2004.
•Arata et al. Pathological fxs through NOFs. JBJS-A. 63:980-8, 1981.
•Easley & Kneisl. Pathologic fxs through NOFs: is prophylactic treatement warranted? J Pediatr Orthop 17:808-
13, 1997.
•Parekh et al. Fibrous Dysplasia. J Am Acad Orthop Surg. 12:303-13, 2004.
•Enneking & Gearen. Fibrous dysplasia of the femoral neck: Treatment by cortical bone-grafting. JBJS-A
68:1415-22, 1986
•Stephenson et al. Fibrous dysplasia: An analysis of options for treatment. JBJS-A 69:400-9, 1987.
•DiCaprio & Enneking. Fibrous dysplasia. Pathophysiology, evaluation and treatment. JBJS-A87:1848-64, 2005.
•Sillence et al. Genetic heterogeneity in osteogenesis imperfecta. J Med Gen. 16:101-16, 1979.
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
133. Bibliography
•Van Dijk et atl. Classification of Osteogenesis Imperfecta revisited. Eur J Med Genet 53:1-5, 2010.
•D’Astous & Carroll: Connective Tissue Diseases, in Vaccaro (ed): OKU 8. Rosemont, IL, AAOS, 2005.
•Phillipi et al. Bisphosphonate therapy for OI. Cochrane Database Syst Rev 8(4), 2008
•Panigrahi et al. Response to zolendronic acid in children with type 3 OI. J Bone Biner Metab, Feb 4, 2010.
[Epub ahead of print]
•Lewis et al. Benign and malignant cartilage tumors. Instr Course Lect 36:87-114, 1987.
•Taylor et al. Childhood osteomyelitis presenting as a pathologic fracture. Clin Rad 63:348-51, 2008.
•Gelfand, et al. Path fx in acute osteo of long bones secondary to communit acquired MRSA? Am J Med Sci
332:357-60, 2006.
•Heyworth & Green. Lower extremity stress fractures in pediatric and adolescent athletes. Curr Opin Pediatr
20:58-61, 2008.
•Sakamoto et al. Congenital pseudarthrosis of the tibia: analysis of the histology and the NF1 gene. J Orthop
Sci 12:361-5, 2007.
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT
135. Ewing sarcoma.
After neoadjuvant chemotherapy, the fracture healed (e) and patient
underwent a wide resection of a proximal femoral mass with endoprosthetic
Bahaa Kornah- Al Azhar Un. Cairo - EGYPT