Fractures and Low Bone Mass in Cerebral Palsy

*David A. Spiegel, M.D.

*John P. Dormans, M.D.
#Michael Levine, M.D.

Division of *Orthopaedic Surgery and #Endocrinology

Childrens Hospital of Philadelphia
The University of Pennsylvania School of Medicine

Summary

Low bone mass is a common problem in patients with cerebral palsy,

and the rate of fractures in this population is greater than age matched
controls. Risk factors for decreased bone mineral density include the degree
of functional involvement (greatest if severely involved, GMFCS IV/V,
nonambulatory), anticonvulsant use, feeding problems, and previous
fractures. Risk factors for fractures include previous fracture, gastrostomy
tube, higher body fat, mixed tone, usage of standing equipment,
anticonvulsants, and seizures. The prevention and treatment of low bone
mass involves enhancing or maintaining adequate nutritional status,
maximizing weight bearing activities, and by medical management in
selected patients, although the indications for newer therapies such as
bisphosphanates are evolving.
Most fractures in cerebral palsy involve the lower extremities, and can be
managed nonoperatively. A soft, bulky dressing with or without a light splint
may be the best option for the majority of fractures, certainly for those stable
injuries such as a buckle fracture of the distal femur. Special care should be
taken if casts are applied, given a high risk of decubitus ulcers (and rarely
conversion to an open fracture). While malunion is common following closed
treatment, untoward outcomes from these malunions have not been
described in the nonambulatory population.
In ambulatory patients, recommendations for surgical intervention are
similar to those for the same injuries in patients without a diagnosis of
cerebral palsy. While nonoperative treatment is successful in the majority of
nonambulatory patients, surgical care does play a role in a small subset of
patients. The indications must be individualized, depending upon the
patient’s overall level of function, the fracture (pattern, location and
stability), and the degree of spasticity. Surgery should be strongly considered
for proximal femur fractures, and many upper extremity fractures. Open
reduction and internal fixation is the best choice for proximal femur fractures,
especially when the patient has fractured around an implant. Flexible
intramedullary nails may be considered for unstable femoral shaft fractures.
Eliminating or minimizing postoperative cast immobilization following elective
orthopaedic surgical procedures may also reduce the risk of fractures.
 There is greater need for orthopaedic surgeons to work in collaboration
with medical colleagues, to address both the fracture and the low bone mass.
While the indications for medical treatment will continue to evolve, we
typically refer patients who have sustained one or more fractures, when the
risk factors noted above are present.

1. Decreased Bone Mineral Density in Cerebral Palsy

a. Bone mineral density is decreased by 22-77% (z < -2) in
Cerebral Palsy (Henderson 1995, 2002, King, Hartman, Ali)
i. Henderson JBJS 1995
1. Average 1 SD below mean (Lumbar spine and
proximal femur)
2. Ambulatory status most important
3. Nutritional status second most important (skin
folds, caloric intake, Body Mass Index)
4. Recommend maximizing ambulation, optimizing
nutrition, supplement calcium
5. Laboratory studies (calcium, phosphorus, alkaline
phosphatase) do not correlate with bone mineral
density
b. Determinants of a decrease in Bone Mineral Density (King,
Hartman, Baer, Chad)
i. GMFCS level (nonambulators)
ii. Feeding difficulties
iii. Previous fracture
iv. Anticonvulsants
v. Higher fat mass (triceps skin folds)
c. Recommendations for decreased BMD based on systematic
review of the literature (Mergler)
i. Monitor BMD if on anticonvulsants and have feeding
difficulties
ii. Optimize calcium intake and Vitamin D intake
iii. Interventions that increase muscle mass are desirable
d. Evidenced based review of bone health in cerebral palsy (Cohen)
i. Is bone strength impaired in CP?
1. Yes, bone mass is reduced
ii. Risk factors for decreased bone mineral density in CP
1. Increased risk with low body mass index, feeding
difficulties, low motor functional ability, delayed
puberty and growth, anticonvulsant drugs
iii. Are children with CP at increased risk for fractures?
1. Suggest monitoring for children with previous
fracture, gastrostomy tube, higher body fat, mixed
tone, usage of standing equipment,
anticonvulsants, seizures
iv. Is there a relationship between BMD and fractures
1. No conclusive evidence, causal relationship
possible
v. What methods are available to assess bone health?
 1. Markers of biomechanical turnover are not helpful
2. Non invasive measures of bone density
a. Dual-energy xray absorptiometry
i. Site is important (lumbar spine, femur,
etc.)
ii. Whole body may be the best for CP
1. Includes all skeletal regions
(trabecular and cortical)
2. Low radiation
3. Longer scan time
b. Quantitative Computed Tomography
i. Lack of normative data
ii. High radiation
iii. Cost issues
iv. Interference with metal implants and
movement
c. Quantitative ultrasound
i. Technical challenge if thick tissues or
agitated patient
ii. Limited experience in children
iii. Reproducibility?
d. Magnetic resonance Imaging
i. Age limitation
ii. Long scan times, motion artifact
iii. Not if metallic implants
3. Conclusion: Dual-energy xray absorptiometry is
method of choice, whole body scan is preferred
vi. Can bone strength be improved in children with cerebral
palsy?
1. Reviewed evidence for physical activity/load
bearing, Vit D and calcium supplementation,
Bisphosphanates, Growth hormaone, Vitamin K.
2. Physical activity is only suggested modality
3. Consider bisphosphanates if history of fracture and
low BMD.
2. Fractures in Cerebral Palsy
a. Bone strength depends upon bone mass and density and on the
structural properties of bone
b. Decreased Bone Mineral Density or “low bone mass”
c. Epidemiology and Risk Factors:
i. Incidence 2.7-6.7% (Stevenson, Henderson 1997)
ii. Prevalence: 12-23% (Leet, Bischof)
iii. Probable Risks (Henderson 2004, Stevenson, Ko, Sturm,
Stasikelis,Leet)
1. History of a fracture
2. Immobilization Feeding tube
3. Anticonvulsants
4. Increased body fat (9.7% if in upper 25% for body
weight)
5. Mixed tone? (Leet)
 6. Older age at first fracture (Leet)
d. Fracture patterns
i. Femur and tibia most common
ii. Metaphyseal and diaphyseal
iii. Vary from buckle to complete displacement
iv. Rarely transphyseal separation (seizures, scurvy (Ballal,
Aroojis)
v. Think of scurvy (Vitamin C deficiency) especially in
developing countries (Aroojis)
e. Selected Literature Review
i. Presedo JPO 2007
1. 156 patients, high risk if nonambulatory on
anticonvulsants, Prevalence 6%
a. First fracture at 10 years
b. 66% nonambulators
c. 66% spastic quadriplegia (83% were
nonambulatory)
d. 23% multiple fractures
2. 82% in lower extremities, only 4% in the spine
a. 36% around the knee
b. Femur (48%)
i. 26% proximal metaphysis, 24% shaft,
50% distal metaphysis
c. Tibia 27% of total
i. 46% proximal metaphysis, 36% shaft,
18% distal metaphysis
d. Contractures common near fracture
3. 48% are delayed in diagnosis
4. Etiology:
a. unknown (55%)
b. Trauma (23%)
i. 7% during transfers, 4% physical
therapy, 2% seizures
5. Treatment
a. 80% treated with a soft bulky dressing
b. 7% cast or splint
c. 13% internal fixation
i. Open reduction and internal fixation
(16)
1. Most in proximal femoral region,
often to treat a fracture at an
implant
ii. Intramedullary nails (5)
6. Treatment recommendations
a. Around or below the knee (Jones dressing,
long leg cast, knee immobilizer)
b. Femoral shaft (Soft dressing and splint, IM
nailing)
c. Proximal femur (open reduction and internal
fixation)
 d. Spine (Soft thoracolumbosacral orthosis)
e. Upper limb (treat as would healthy children)
ii. Leet, JPO 2006
1. 418 patients, 10.6 years of age
2. 58% spastic quadriplegia, 5% mixed tone, 63%
ambulatory
3. 70% lower extremity (50% femur), 25% upper
extremity
4. Presence of contractures only correlated with
fractures at the ankle
5. Those with fracture were significantly older (12.1
vs. 10.4 years) and were on Valproic Acid
6. Recommend considering treatment (nutritional
supplementation, weightbearing programs,
bisphosphonates) if older than 12 years and/or on
valproic acid
iii. Leet, J Child Orthop 2009
1. Femur fractures in CP
a. Nonambulators
i. 11/32 treated nonoperatively result in
malunion, 5 have pressure sores
ii. 1/6 malunion if operative
b. Ambulators
i. 1/5 nonoperative loses reduction,
treated operatively
c. Conclusion: Can manage nonoperatively
most of the time, but follow closely and
careful casting should help prevent pressure
sores
3. Prevention of Fractures?
a. Mechanical loading
i.
ii. Selected Literature Review
1. Ward 2004
a. Prospective, randomized, double-blind
placebo controlled
b. Ambulant disabled children randomized to
standing on active vs. placebo, 10 mins/day,
5 days/week, for 6 months
c. 3-D quantitative computed tomography
d. Compliance was 44%
e. Conclusions
i. Spinal BMD was improved
ii. Diaphyseal bone does not show a
response
2. Caulton (2004)
a. Increased upright time by 60% (15-675
minutes per week)
 b. Improved lumbar BMD by 3D Ct study, no
improvement in Tibial BMD
4. Medical Treatment?
a. Indications for Referral?
b. Indications for Treatment?
c. Medical Treatment
i. Calcium, Vitamin D
ii. Bisphosphanates
iii. Other
d. Literature
i. Bisphosphanates
1. Plotkin 2006
a. Low dose Pamidronate on BMD in CP
(Plotkin)
i. 23 nonambulatory Children
ii. 0.75 mg/kg/d for 2 days every 4
months (intravenous)(4.12
mg/kg/year)
iii. Significant increase in BMD at one
year
1. Z scores increase 1.6 points for
femoral neck and 1.9 points for
lumbar spine
iv. No side effects
v. Suggest the need for larger controlled
studies
2. Grissom 2005
a. 18 pt. with spastic quadriplegia
b. IV Pamidronate at 2-8 month intervals.
1mg/kg x 3 consecutive days
c. 13 months followup
d. Spine BMD increased 47.4 +/- 39%
e. Lateral distal femur BMD increased 65.7 +/-
55.2%.
f. No additional fractures
g. Conclude that IV Pamidronate at 3-4 month
intervals can increase BMD in cerebral palsy
3. Iwasaki (2008)
a. 20 pt. treated for 6 months
b. Randomized, double-blind
c. Spine and Hip (radius if can’t image hip) BMD
d. Vitamin D vs. Vitamin D with Risendronate
e. BDM increases with both treatments, more in
the group with Vit D and risendronate

References
1. Ali O, Shim M, Fowler E, et al. Spinal bone mineral density, IGF-1 and IGFBP-3
n children with cerebral palsy. Horm Res 2007;68:316-320.

2. Allington N, Vivegnis D, Gerard P. Cyclic administration of pamidronate to

treat osteoporosis in children with cerebral palsy or a neuromuscular disorder:
a clinical study. Acta Orthop Belg. 2005;71:91-97.

3. Aroojis AJ, Gajjar SM, Johari AN. Epiphyseal separations in spastic cerebral
palsy. J Pediatr Orthop B. 2007;16:170-174.

4. Baer MT, Kozlowski BW, Blyler EM, et al. Vitamin D, calcium, and bone status
in children with developmental delay in relation to anticonvulsant use and
ambulatory status. Am J Clin Nutr 1997;65:1042-1051.

5. Ballal MS, Dawoodi A, Sampath J, Bass A. Traumatic transepiphyseal

separation of the upper femoral epiphysis following seizures in two children
with cerebral palsy. J Bone Joint Surg Br. 2008;90:382-384.

6. Bischof F, Basu D, Pettifor JM. Pathological long-bone fractures in residents

with cerebral palsy in a long-term care facility in South Africa. Dev Med Child
Neurol. 2002;44:119-122.

7. Brunner R, Doderlein L. Pathological fractures in patients with cerebral palsy. J

Pediatr Orthop B. 1996;5:232-238.

8. Castillo H, Samson-Fang L; American Academy for Cerebral Palsy and

Developmental Medicine Treatment Outcomes Committee Review Panel.
Effects of bisphosphonates in children with osteogenesis imperfecta: an
AACPDM systematic review. Dev Med Child Neurol. 2009;51:17-29.

9. Caulton JM, Ward KA, Alsop CW, Dunn G, Adams JE, Mughal MZ. A randomized
controlled trial of standing programme on bone mineral density in non-
ambulant children with cerebral palsy. Arch Dis Child. 2004;89:131-135.

10. Chad KE, McKay HA, Zello GA, et al. Body composition in nutritionally
adequate ambulatory and non-ambulatory children with cerebral palsy and a
healthy reference group. Dev Med Child Neuro 2000;42:334-339.

11. Cohen M, Lahat E, Bistritzer T, Livne A, Heyman E, Rachmiel M. Evidence-

based review of bone strength in children and youth with cerebral palsy. J
Child Neurol. 2009;24:959-967.

12. Grissom LE, Kecskemethy HH, Bachrach SJ, et al. Bone densitometry in
pediatric patients treated with pamidronate. Pediatr Radiol 2005;35:511-517.

13. Hartman C, Brik R, Tamir A, et al. Bone quantitative ultrasound and nutritional
status in severely handicapped, institutionalized children and adolescents.
Clin Nutr 2004;23:89-98.

14. Heinrich SD, Drvaric DM, Darr K, et al. The operative stabilization of
pediatric diaphyseal femur fractures with flexible intramedullary nails: A
prospective study. J Pediatr Orthop 1994;14:501-507.

15. Henderson R. Low doses of pamidronate for the treatment of osteopenia in

non-ambulatory children. Dev Med Child Neurol. 2006;48:708.
16. Henderson RC, Berglund LM, May R, Zemel BS, Grossberg RI, Johnson J, Plotkin
H, Stevenson RD, Szalay E, Wong B, Kecskemethy HH, Harcke HT. The
Relationship Between Fractures and DXA Measures of BMD in the Distal Femur
of Children and Adolescents With Cerebral Palsy or Muscular Dystrophy. J Bone
Miner Res. 2009 Oct 12.

17. Henderson RC, Kairalla J, Abbas A, Stevenson RD. Predicting low bone density
in children and young adults with quadriplegic cerebral palsy. Dev Med Child
Neurol. 2004;46:416-419.

18. Henderson RC, Lark RK, Gurka MJ, Worley G, Fung EB, Conaway M, Stallings
VA, Stevenson RD. Bone density and metabolism in children and adolescents
with moderate to severe cerebral palsy. Pediatrics. 2002;110:e5.

19. Henderson RC, Lin PP, Greene WB. Bone-mineral density in children and
adolescents who have spastic cerebral palsy. J Bone Joint Surg Am.
1995;77:1671-81.

20. Henderson RC. Bone density and other possible predictors of fracture risk in
children and adolescents with spastic quadriplegia. Dev Med Child Neuro
1997;39:224-227.

21. Henderson RC, Lark RK, Kecskemethy HH, et al. Bisphosphanates to treat
oeteopenia in children with quadriplegic cerebral palsy: A randomized,
placebo-controlled clinical trial. J Pediatr 2002;141:644-651.

22. Houlihan CM, Stevenson RD. Bone density in cerebral palsy. Phys Med Rehabil
Clin N Am. 2009;20:493-508.

23. Iwasaki T, Takei K, Nakamura S, et al. Secondary osteoporosis in long-term

bedridden patients with cerebral palsy. Pediatr Int 2008;50:269-275.

24. Jekovec-Vrhovsek M, Kocijancic A, Prezelj J. Effect of vitamin D and calcium on

bone mineral density in children with CP and epilepsy in full-time care. Dev
Med Child Neurol. 2000 ;42:403-405.

25. King W, Levin R, Schmidt R, Oestreich A, Heubi JE. Prevalence of reduced bone
mass in children and adults with spastic quadriplegia. Dev Med Child Neurol.
2003;45:12-16.

26. Lee JJ, Lyne ED. Pathologic fractures in severely handicapped children and
young adults. J Pediatr Orthop. 1990;10:497-500.

27. Leet AI, Shirley ED, Barker C, Launay F, Sponseller PD. Treatment of femur
fractures in children with cerebral palsy. J Child Orthop. 2009;3:253-258.

28. Leet AI, Mesfin A, Pichard C, Launay F, Brintzenhofeszoc K, Levey EB, D

Sponseller P. Fractures in children with cerebral palsy. J Pediatr Orthop.
2006;26:624-627.

29. Lingam S, Joester J. Spontaneous fractures in children and adolescents with

cerebral palsy. BMJ.1994;309:265.

30. Maruyama K, Nakamura K, Nashimoto M, Kitamoto F, Oyama M, Tsuchiya Y,

Yamamoto M. Bone fracture in physically disabled children attending schools
for handicapped children in Japan. Environ Health Prev Med. 2009 Nov 26.
31. McIvor WC, Samilson RL. Fractures in patients with cerebral palsy. J Bone Joint
Surg [Am] 1966;48:858-866.

32. Mergler S, Evenhuis HM, Boot AM, De Man SA, Bindels-De Heus KG, Huijbers
WA, Penning C. Epidemiology of low bone mineral density and fractures in
children with severe cerebral palsy: a systematic review. Dev Med Child
Neurol. 2009;51:773-778.

33. Miller PR, Glazer DA. Spontaneous fractures in thebrain-crippled, bedridden

patient. Clin Orthop Rel Res 1976;120:134-137.

34. Plotkin H, Coughlin S, Kreikemeier R, Heldt K, Bruzoni M, Lerner G. Low doses

of pamidronate to treat osteopenia in children with severe cerebral palsy: a
pilot study. Dev Med Child Neurol. 2006 Sep;48(9):709-12. Erratum in: Dev
Med Child Neurol. 2006;48:1024.

35. Presedo A, Dabney KW, Miller F. Fractures in patients with cerebral palsy. J
Pediatr Orthop. 2007;27:147-53.

36. Samaniego EA, Sheth RD. Bone consequences of epilepsy and antiepileptic
medications. Semin Pediatr Neurol. 2007;14:196-200.

37. Stasikelis PJ, Lee DD, Sullivan CM. Complications of osteotomies in severe
cerebral palsy. J Pediatr Ortho 1999;19:207-210.

38. Stevenson RD, Conaway M, Barrington JW, Cuthill SL, Worley G, Henderson
RC. Fracture rate in children with cerebral palsy. Pediatr Rehabil. 2006;9:396-
403.

39. Sturm PF, Alman BA, Christie BL. Femur fractures in institutionalized patients
after hip spica immobilization. J Pediatr Orthop 1993;13:246-248.

40. Sugiyama T, Taguchi T, Kawai S. Spontaneous fractures and quality of life in

cerebral palsy. Lancet. 2004 Jul 3-9;364(9428):28.

41. Vogtle LK. Pain in adults with cerebral palsy: impact and solutions. Dev Med
Child Neurol. 2009;51 Suppl 4:113-121.

42. Ward K, Alsop C, Caulton J, et al. Low magnitude mechanical loading is

osteogenic in children with disabling conditions. J Bone Miner res
2004;19:360-369.

43. Ward KA, Caulton JM, Adams JE, Mughal MZ. Perspective: cerebral palsy as a
model of bone development in the absence of postnatal mechanical factors. J
Musculoskelet Neuronal Interact. 2006;6:154-159.