Pathologic Fractures in Children

1
Pathologic Fracturesin Children

• Joshua Klatt, MD
• Original Author Steven Frick, MD March 2004
• 1st Revision Steven Frick, MD August 2006
• 2nd Revision Joshua Klatt, MD January 2010

2
Pathologic Fracture Fracture through abnormal
bone
3
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.

4
Osteopetrosis - failed fixation of femoral neck
fracture. No osteoclasts No remodeling.
5
With every fracture Ask the question - Is this
fracture through NORMAL bone?
6
Often Need to Do More than Treat the Fracture

• The orthopaedic surgeon may be the first to have
  opportunity to make the diagnosis. (malignancy,
  metabolic disease, etc.)

7
Often Need to Do More than Treat the Fracture

• Differ from fractures in normal bone in that one
  must take into account
• Etiology
• Natural history
• Treatment of underlying abnormality
• Must treat both fracture and underlying cause!

8
History

• Minor or no trauma?
• Less than anticipated for fracture pattern
• Any antecedent pain?
• Only with activity vs. night pain
• Recent illness?
• Weight loss?
• Fevers?

9
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.

10
History

• Ask about prior malignancies, even in the child!
• Families will not always volunteer this
  information

11
Physical Exam

• Look for soft tissue mass vs. fracture hematoma
• Other systems- skin, lymphatics, solid organs
• Height - weight percentiles

12
11 yo - Osteosarcoma
13
Lab Tests

• CBC with differential
• ESR
• Calcium (ionized), Phosphorus, Alkaline
  phosphatase
• Bun/Cr

14
RadiographsBe suspicious!

• Osteopenia
• Physeal width (rickets)
• Soft tissue calcifications
• Presence of mass
• Any periosteal reaction

15
Radiographs

• Is pathology
• Localized and isolated?
• Polyostotic?
• Generalized to entire skeletal system?
• A generalized condition with skeletal
  manifestations?

16
Ennekings 4 Questions

• Where is lesion located?
• What is lesion doing to bone?
• What is bone doing to lesion?
• Are there clues to type of lesion?

Enneking, et al. The surgical staging of MSK
sarcoma. JBJS 62-A1027-1030, 1980. Enneking. A
System of Staging MSK Neoplasms. CORR 2049-24,
1986.
17
Benign vs. MalignantMankins Criteria

• Size
• Margination
• Cortex
• Soft tissue mass

Gebhardt, Ready Mankin. Tumors about the knee
in children. Clin Orthop 25586-110, 1980.
18
Categorize/Make Diagnosis

• Benign bone lesion
• Malignant bone lesion
• Infection
• Metabolic bone disease

• Skeletal dysplasia
• Neuropathic
• Osteopenia-disuse
• Overuse

19
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

20
Simple Bone Cyst(Unicameral Bone Cyst)

• 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 gt females (21)

Baig Eady. Unicameral (Simple) Bone Cysts.
South Med J. 99(9)966-76, 2006.
21
SBC Pathologic Fracture

• 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

22
SBC Treatment

• 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.

23
SBC Injection

• 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

24
LJ, 8 yo with arm pain when throwing, injected
once with methylprednisolone (multiple sites),
healing at 3 months
25
SBC - Risk Factors for Recurrence

• Only reliable predictor of treatment success is
  age of the patient
• gt 10 yrs heal 90 of time
• lt 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 58636-41, 1976
26
SBC
27
Aneurysmal Bone Cyst(ABC)

• Expansile
• Often wider than physis
• Eccentric
• Aggressive at margins

Cottalorda Bourelle, Current treatments of
primary ABCs. J Pediatr Orthop B 15155-67, 2006.
28
Aneurysmal Bone Cyst(ABC)

• Symptoms usually present for lt 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

Cottalorda et al. Aneurysmal Bone Cysts of the
Pelvis in Children. J Pediatr Orthop. 25471-5,
2005.
29
ABC
30
ABC
31
ABC
Look for fluid-fluid levels on MRI
(however, not especially specific)
Bur, et al. Fluid-fluid levels in a unicameral
bone cyst CT and MR findings. J Comput Assist
Tomogr 17134-6, 1993. Papagelopoulos, et al.
Treatment of aneurysmal bone cysts of the pelvis
and sacrum. JBJS-A 831674-81, 2001.
32
5 yo female with 1 year of hip pain and 4 prior
steroid injections, progressive coxa vara. Biopsy
ABC
33
Curettage, biopsy consistent with aneurysmal bone
cyst
34
1 month after curettage, bone grafting,
valgus/internal fixation, spica immobilization
35
ABC

• Curettage and bone graft
• /- internal fixation
• ? Injection of fibrosing agent (Ethibloc,
  Ethicon, etc.) is controversial
• High recurrence

Cottalorda Bourelle, Current treatments of
primary ABCs. J Pediatr Orthop B 15155-67,
2006. Adamsbaum et al. Intralesional Ethibloc
injection in primary ABCs. Skeltal Radiol.
32559-66, 2003. Varshney et al. Is Sclerotherapy
Better than Intralesional Excision for treating
ABCs. CORR epib 2009.
36
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 20s

Betsy et al. Metphyseal fibrous defects. J Am
Acad Orthop Surg. 1289-95, 2004.
37
Nonossifying Fibroma(NOF)

• Most treated non-op!
• Let fracture heal, excellent healing potential
• Most NOFs 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. 1289-95, 2004.
38
10 yo male - running during soccer. NOF fracture
- at 4 weeks underwent allograft DBM / cancellous
bone graft. Healed at 9 mos.
39
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, gt33 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. 63980-8, 1981. Easley Kneisl.
Pathologic fxs through NOFs is prophylactic
treatment warranted? J Pediatr Orthop
17808-13, 1997.
40
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. 12303-13, 2004.
41
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 681415-22, 1986
42
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 681415-22,
1986. Stephenson et al. Fibrous dysplasia An
analysis of options for treatment. JBJS-A
69400-9, 1987.
43
14 yo female - fell walking across front yard 3
months of left hip pain - Motrin Referred for
path fx through Ewings sarcoma Dx -polyostotic
fibrous dysplasia
44
3 Years Postop
Notice resorption of graft and recurrence of
cystic changes in femoral neck. Can have
secondary ABC develop within fibrous dysplasia.
45
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

46
11 yo male fem neck path fx, nondisplaced.
Fibular allograft (neck) and titanium elastic
nails (subtroch and shaft)
47
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?
48
5 yo - Albrights polyostotic fibrous dysplasia
49
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. 12303-13, 2004. DiCaprio Enneking.
Fibrous dysplasia. Pathophysiology, evaluation
and treatment. JBJS-A871848-64, 2005.
50
Osteogenesis Imperfecta(OI)

• 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 gt upper extremity

Sillence et al. Genetic heterogeneity in
osteogenesis imperfecta. J Med Gen. 16101-16,
1979. Van Dijk et atl. Classification of
Osteogenesis Imperfecta revisited. Eur J Med
Genet 531-5, 2010.
51
OI

• 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

DAstous Carroll Connective Tissue Diseases,
in Vaccaro (ed) OKU 8. Rosemont, IL, AAOS,
2005.
52
3 yr old OI - multiple fxs Lt femur
53
OI
54
OI Olecranon Fx
55
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
56
Olliers 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 3687-114, 1987.
57
7 yo male - femur fracture jumping on
bed Enchondromatosis Rt. femur/tibia/pelvis
58
Osteomyelitis

• 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

Taylor et al. Childhood osteomyelitis presenting
as a pathologic fracture. Clin Rad 63348-51,
2008. Gelfand, et al. Path fx in acute osteo of
long bones secondary to community Acquired
MRSA? Am J Med Sci 332357-60, 2006.
59
Iatrogenic Osteoporosis

• Post-Irradiation
• Steroids
• Chemotherapy (MTX)

60
10 yo female ALL - chemotherapy/steroids Fx after
fall from chair. Tx immobilization
61
Neuropathic Fractures

• Myelomeningocele, paraplegics, sensory
  neuropathies, etc.
• Exam frequently reveals warm, erythematous,
  swollen joint
• Often mistaken for infection, DVT, tumor

62
3 yo with spina bifida, swollen leg Consult DVT
vs. infection? X-ray - healing fx may look like
malignancy
63
11 yo male - Duchene Muscular Dystrophy Hip pain
for 2 months. Disuse fracture/nonunion
64
Stress Fractures

• 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

Heyworth Green. Lower extremity stress
fractures in pediatric and adolescent
athletes. Curr Opin Pediatr 2058-61, 2008.
65
12 yr old male, activity related pain, training
for baseball, running 6 miles per day, referred
for Ewings sarcoma. Dx Longitudinal femoral
stress fracture
66
Stress Fractures

• Can occur through pathologic bone
• Congenital abnormalities, metabolic disorders
  (osteoporosis, osteomalacia)

67
Congenital Tibial Dysplasia
Presented at age 10 after fracture from minor
trauma Had bowed legher 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 12361-5, 2007.
68
Malignant Appearing Pathologic Fracture

• 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

69
Osteogenic Sarcoma
70
Malignant Pathologic Fractures

• May need immediate amputation
• Osteosarcoma
• some fx may heal during neoadjuvant chemotherapy
• Ewings
• closed immobilization
• chemotherapy

71
Path fx lesser trochanter Stage IIB MRI - soft
tissue mass posterior
72
Ewings sarcoma - allograft-prosthesis composite
73
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

74
Bibliography

• Enneking, et al. The surgical staging of MSK
  sarcoma. JBJS 62-A1027-1030, 1980.
• Enneking. A System of Staging MSK Neoplasms. CORR
  2049-24, 1986.
• Gebhardt, Ready Mankin. Tumors about the knee
  in children. Clin Orthop 25586-110, 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 58636-41, 1976
• Cottalorda Bourelle, Current treatments of
  primary ABCs. J Pediatr Orthop B 15155-67, 2006.
• Cottalorda et al. Aneurysmal Bone Cysts of the
  Pelvis in Children. J Pediatr Orthop. 25471-5,
  2005.
• Bur, et al. Fluid-fluid levels in a unicameral
  bone cyst CT and MR findings. J Comput Assist
  Tomogr 17134-6, 1993.
• Papagelopoulos, et al. Treatment of aneurysmal
  bone cysts of the pelvis and sacrum. JBJS-A
  831674-81, 2001.
• Adamsbaum et al. Intralesional Ethibloc injection
  in primary ABCs. Skeltal Radiol. 32559-66, 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. 1289-95, 2004.
• Arata et al. Pathological fxs through NOFs.
  JBJS-A. 63980-8, 1981.
• Easley Kneisl. Pathologic fxs through NOFs is
  prophylactic treatement warranted? J Pediatr
  Orthop 17808-13, 1997.
• Parekh et al. Fibrous Dysplasia. J Am Acad Orthop
  Surg. 12303-13, 2004.
• Enneking Gearen. Fibrous dysplasia of the
  femoral neck Treatment by cortical
  bone-grafting. JBJS-A 681415-22, 1986
• Stephenson et al. Fibrous dysplasia An analysis
  of options for treatment. JBJS-A 69400-9, 1987.
• DiCaprio Enneking. Fibrous dysplasia.
  Pathophysiology, evaluation and treatment.
  JBJS-A871848-64, 2005.
• Sillence et al. Genetic heterogeneity in
  osteogenesis imperfecta. J Med Gen. 16101-16,
  1979.

75
Bibliography

• Van Dijk et atl. Classification of Osteogenesis
  Imperfecta revisited. Eur J Med Genet 531-5,
  2010.
• DAstous 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 3687-114, 1987.
• Taylor et al. Childhood osteomyelitis presenting
  as a pathologic fracture. Clin Rad 63348-51,
  2008.
• Gelfand, et al. Path fx in acute osteo of long
  bones secondary to communit acquired MRSA? Am J
  Med Sci 332357-60, 2006.
• Heyworth Green. Lower extremity stress
  fractures in pediatric and adolescent athletes.
  Curr Opin Pediatr 2058-61, 2008.
• Sakamoto et al. Congenital pseudarthrosis of the
  tibia analysis of the histology and the NF1
  gene. J Orthop Sci 12361-5, 2007.

76
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