Esperience in surgical treatment in OI, mistakes and complicatios and early phisical activity

1. 20 Years of Surgical Treatment in
Osteogenesis Imperfecta
Dr. Parra García JI
Dr. Bueno Sánchez AM
Hospital Universitario de Getafe
MADRID

2. Osteogenesis Imperfecta
• No clear definition
• Genetic disorder, bones fragility (30)
• Skeletal manifestations
• Extraskeletal manifestations
• 1/10.000
Plotkin H. Two Cuestions About Osteogenesis Imperfecta. J Pediatr Orthop
2006;26:148-149

3. Classification of Osteogenesis Imperfecta revisited
F.S. Van Dijk a,*, G. Pals a, R.R. Van Rijn b, P.G.J. Nikkels c, J.M. Cobben
European Journal of Medical Genetics xxx (2009)
“a heterogeneous group of diseases characterized by
susceptibility to bone fractures with variable severity
and presumed or proven defects in collagen type I
biosynthesis”

4. Sillence slide OI International Congres
2011

5. Osteogénesis Imperfecta
Bifosfonates treatment
Phisiotherapy
Surgery

6.  Generally, we feel that the treatment applied to OI patients
has change their lives:
 There is a tremendous difference between patients who are
born today and have received proper treatment, and the ones
who were born 20 or 30 years ago.
 Type III children, who would normally use a wheelchair and
suffer from serious bone deformities in long bones, have now
an almost normal social, physical and psychologically balanced
life, and are able to walk unaided.

7. ARE WE IMPROVING THE PHYSICAL
SITUATION OF OUR PATIENTS?
IS IT WORTH IT, AFTER UNDERGOING
MULTIPLE SURGERIES?

8. 1972

9. 1984

10. 19 years after

11. 1992

12. May 1993
"Displasias óseas; Enfoque diagnóstico y tratamiento
quirúrgico". Profesor M. Tachdjian, Profesor Michael Golberg

13. Surgical treatment
Autor Año Tipoclavo P Telesc. Maciz. OperTOTAL
Tiley 1973 Cl Macizo 13 129 129
Cole 1991 Cl Macizo 3
King-Bobensko 1971 Macizos 52 127 127
Root 1980 Macizos 36 24
Lecuire 1987 Macizos 18 72
Rioppy 1987 Macizos 14 55 55
Li,Chow,Leong 2000 Macizos 10 58 58
Middletton 1984 Macizos 3 13 13
Middletton 1987 Macizos 12
McKale 1994 Macizos percutaneos 7 25 25
Gargan 1996 Rush y Kirsnner 7 7
Finidori 1979 Telescopico 11
Rodriguez 1980 Telescopico 20 33 33
Bailey 1981 Telescopico 12 31 31
Lang-Stevenson 1984 Telescopico 10 28 28
Stockley 1989 Telescopico 24 117 117
Nicholas 1990 Telescopico 16 56 56
Brunelli 1993 Telescopico 16 40 40
Engelbert 1995 Telescopico 10
Zionts 1998 Telescopico 15 40 40
Wilkinson 1998 Telescopico 24 60 60
Janus 1999 Telescopico 34 110 110
Cassis-Dubow 1975 Telescopico/Cl. Macizo 34 16 18 34
Niemann 1981 Telescopico/Cl. Macizo
Gamble 1988 Telescopico/Cl. Macizo 29 42 66 108
Porat 1991 Telescopico/Cl. Macizo 20 22 24 56
Kishore Mulpuri 2000 Telescopico/Cl. Macizo 16 42 24 66
Luhmann 1998 Telescopico/Rush cruzados 12 30 6 36

14. Data base 65 p.
RubenGG 7
JavierTS 10
LauraR 6
EstebanVM 7
JuanNS 4 11 Patients
PedroP 3 45 Surgeries
RubenM 2 35 Multiple Osteotomies
CritinaTF 2
JoseA.FB 1
DanielMP 2
Miranda LP 1 2000

15. BS 1
LPM 1
Data base 130 p.
RGG 7
JTS 11
LR 6
EVM 7
DMP 2
JNS 7
PP 3
RMA 5
CTF 2
25 Patients
JFB 1
DMP 2
MLP 1
MC 2
80 Surgeries
VB 3
SPQ 4
RGF 3
MJG 1
ABM 1
MLP 2
AAS 1
AAS 2
AGM 1
2006
PHG 2
ACD 1

16. Khalid 2001 SofieldAnálisi 34
s de marcha
Zionts 2002 17 Fracturas 10
Olecranon
Boutaud, 2004 Macizos/Elast 14 36
Laville icos cruzados
J Pediatr Orthop B. 2005 Sep;14(5):311-9 The choice of intramedullary devices for the femur and
the tibia in osteogenesis imperfecta.
Joseph B, Rebello G, B CK.
El Sobky M, Zaky H, Atef A, et al. Surgery versus surgery plus pamidronate in the management
of osteogenesis imperfecta patients: a comparative study. J Pediatr Orthop B 2006; 15:222-
228.
Interlocking Telescopic Rod for Patients with Osteogenesis Imperfecta -- Cho et al_ The Journal
of Bone and Joint Surgery (American). 2007;89:1028-1035.
Surgical treatment of osteogenesis imperfecta: current concepts Esposito, Paul;
Plotkin, Horacio Current Opinion in Pediatrics: February 2008 - Volume 20 - Issue 1 - p 52-57
El-Adl G, Khalil MA, Enan A, Mostafa MF, El-Lakkany MR. Telescoping versus non-telescoping
rods in the treatment of osteogenesis imperfecta. Acta Orthop Belg. 2009 Apr;75(2):200-8. 10
patients

17. Abulsaad M, Abdelrahman A. Modified Sofield-Millar operation: less invasive surgery of
lower limbs in osteogenesis imperfecta. Int Orthop. 2009 Apr;33(2):527-32.
Birke O, Davies N, Latimer M, Little DG, Bellemore M. J Pediatr Orthop. 2011 Jun;31(4):458-
64. Experience with the Fassier-Duval telescopic rod: first 24 consecutive cases with a minimum
of 1-year follow-up.
Surgical index in OI
Number o patients operated / surgical procedures
Around 3

19. Osteogénesis Imperfecta
JIP

22. 9.01

25. Fassier-Duval
telescopic nails

27. Pediatric endocrinologist
Physiotherapists
Child Orthopaedic Surgeons
Geneticists
Otolaryngologists
Odontology
Neurosurgeons
Gynecologists
Psychologist

28. MATERIAL and METHODS
• We analyzed the results of surgeries with Rush
rods, Fassier-Duval (FD), Bailey-Dubow (BD) and
solid Telescopic nails
• October 1991 to December 2010 ( 19 years)
• We evaluated the functional situation and the
type of multi-disciplinary treatment which
enabled the patients to improve their activity.

29. Of a total of 199 patients with OI, 52 patients had to be operated
totaling 172 surgical procedures
PATIENTS: 199
TYPES OF TREATMENT
26%
74% Conservative 147
Surgery 52
Surgycal Index 3.3

30. The average patient age was 8.00 years (standard deviation of
4.64 and a range from 1 to 44).
Of the 172 surgeries, 159 were carried out
in 45 patients under the age of 18
SURGERIES 172
CHILDREN 159 ADULTS 13
8%
92%

31. Of the 172 surgeries, 144 were carried out
in 162 long bones (in some cases we operated on more
than one bone)
TYPES OF SURGERIES
Long bones Other surgeries
11%
89%

32. 162 long bones
• 152 femurs and tibias and 10 hips
• 152 long bones were treated with intramedullary
nailing.

33. Nails employed:
35 Rush
17 Bailey-Dubow telescopic nails
88 Fassier-Duval telescopic nails
Other nails: elastic or Interlocking nail
Others 3
2%
RUSH 35
24%
B-D 17
F-D 88 12%
62%

34. Reasons for primary surgery:
43 due to deformity of femur or tibia
9 for a deformity associated with a fracture
39 cases due to fractures
22 in other surgeries in feet, knees or coxa vara
Deformity Deformity and fracture
FRACTURE OTHERS
43
39
22
9

35. Requisites
85% of the patients in the first surgery
were being treated with bisphosphonates
and pre and postop. with physical therapy
Surgery used always tried to minimize
bleeding and muscle injury

36. Fassier F, Glorieux F. Osteogenesis imperfecta. In: Surgical techniques in
orthopaedics and traumatology. Paris: Elsevier; 2003. pp. 1–8.

37. 4-5 years
Engelbert, Raoul H. 'Intramedullary rodding in type III
osteogenesis imperfecta: Effects on neuromotor
development in 10 children', Acta Orthopaedica, 66:
2 y 3.5 years
4, 361 — 364 1995
Surgical treatment of osteogenesis imperfecta: current
concepts
Iniciating
Esposito, Paul; Plotkin, Horacio walking
Current Opinion in Pediatrics:
February 2008 - Volume 20 - Issue 1 - p 52-57
172 Surgeries 144 long bones
11%
35 Under 3 years
89%

38. Of the 172 surgeries, 159 were carried out
in 45 patients under the age of 18
• Average 7.61
• Range from 1 to 17.13
• 24.5% under 3 years

45. Older patiens that walk

50. Bone quality
Jessica M. Fritz a,∗, Yabo Guana,d, MeiWanga, Peter A. Smithb, Gerald F. Harrisa,b,c
A fracture risk assessment model of the femur in children with osteogenesis
imperfecta (OI) during gait Medical Engineering & Physics 31 (2009) 1043–1048

51. 9 year

55. Postop
1. Passive movement of the affected articulations after 24
hours
2. Pressure exercises in axis with resistence after 24 hours
3. Progressive weight in the pool as soon as scarring is
completed
4. Anti rotation dressing on femur surgery 3 weeks
5. Children are sent home 1 or 2 days after surgery.
6. We encouraged the patients to bear weight on the bone
very early depending on the age of the patient and the
evolution of bone consolidation.

56. COMPLICATIONS
• We have 28.8% (45 cases) repeated surgeries
due to complications in the nail (42%)
• In 23% (35 cases) we had to remove the nail
(25%)

57. Birke O, Davies N, Latimer M, Little DG, Bellemore M.
J Pediatr Orthop. 2011 Jun;31(4):458-64.
Experience with the Fassier-Duval telescopic rod: first
24 consecutive cases with a minimum of 1-year follow-
up.
We found the OI patient group associated with a 13%
reoperation rate (2 of 15 cases) for proximal rod migration and a
40% complication rate (6 of 15 cases): rod migration and limited
telescoping (5) and intraoperative joint intrusion (1). There were
no infections.
24 cases
40% de complications

58. Fassier-Duval telescopic rod
Complications
BD 39%
No telescopic 50%
FD 15%

59. JBJS 2011 Nov 2;93(21):1994-2000.
Use of the Sheffield telescopic intramedullary rod system for the management of osteogenesis
imperfecta: clinical outcomes at an average follow-up of nineteen years.
Nicolaou N, Bowe JD, Wilkinson JM, Fernandes JA, Bell MJ.
Sheffield Children’s Hospital, Sheffield, UK.
BACKGROUND:
Elongating intramedullary rods have been used in the management of osteogenesis imperfecta for the past
fifty years. The complication rates reported in many reviews of the available techniques have been high.
This study reviews the long-term functional outcomes and complications following the use of the Sheffield
system of telescopic intramedullary rods.
METHODS:
We conducted a retrospective analysis of patients with osteogenesis imperfecta who were at least eighteen
years of age and who had at least thirteen years of follow-up. Complications, reoperations, and data from a
disease-specific questionnaire and the Short Form-36 questionnaire were recorded.
RESULTS:
Data for twenty-two patients with osteogenesis imperfecta who had been treated with Sheffield telescopic
intramedullary rods were available at an average of nineteen years after the initial surgery. Reoperations
involving thirty-three (50%) of the sixty-six rods were performed: ten rods (15%) were exchanged because
of rod disengagement due to growth, thirteen rods (20%) were exchanged because of complications, and
ten rods (15%) required further surgery other than exchange because of complications. Mobility was
significantly improved at the initial postoperative visit (p = 0.0015), and this improvement was maintained
into adulthood (p = 0.0077). Back pain was the most frequent symptom. Symptoms related to rod insertion
across the knee and ankle were rare, but symptoms related to proximal femoral trochanteric entry were
common. Physeal damage was not seen following surgery, and all rods elongated with growth. All patients
were satisfied with the outcome of the surgical procedures. Short Form-36 scores for all physical domains
and for social function and vitality were significantly worse than those in a normal population.
CONCLUSIONS:
The outcomes of this technique are satisfactory in adulthood; reoperation rates are high but are most
commonly related to the patient outgrowing the rods. Concerns regarding insertion of this fixed device at
the knee and ankle were unfounded, although proximal femoral fixation remains a problem.

60. Nail changes
• In the 35 cases when it was necessary to
remove the nails, the percentages were as
follows:
 41.6% of the B-D
 25.5% of the Rush
 32.9% of the F-D

71. Causes of seconds surgeries
We have carried out 45 second surgeries due to
complications with the nail (28.8%):
– 16 cases due to fracture with a bent nail,
– 18 for displaced or non-telescoping of nails
– 3 due to inadequate insertion
– 3 due to hip surgery
– 1 due to an infected nail
– 1 due to end of telescopy.

72. Once a nail is inserted filling the canal and
walking is stimulated, the nail is usually too
small in relation to the cortical thickness and
usually bends along with the bone.

75. She has an 8-10 minute walk to school!!!!!!!!

76. • All the complications were resolved satisfactorily.
• This percentage of complications has been
decreasing year after year and we feel this is due to
the fact that we have overcome the learning curve
• No coxa valga
• No necrosis avascular
• No physeal arrest

77. No coxa valga

78. No physeal closing

80. 11 years
20 fractures

86. Coxa vara
Wagner technique, used by Finidori and Fassier
Bone bank block reinforcement

88. Bone bank block

89. Bone bank block

92. Hands in pocket sign!!!

93. Nail can prevent some fractures, but not all
On occasions, the nail will endure the
fractures, so the child will be able to return to
an active life style.

94. More activity = more fractures

105. GAP Nail

106. RESULTS
The patients have skipped a step or two in the
El Sobky classification
bedridden wheel chair crutches walking
El Sobky and alts. Egyptian Orthopedic J. 1999

107. RESULTS 83% walking (preop 14.2%),
11.9% crutches (preop 4.7%)
5.1% wheelchairs (preop 79%)
none bedridden (preop 2.3%)
Walking Crutches Bedridden Wheel chairs
79% 83%
14%
4.70%
2.30% 11.90%
5.10%
0.00%
PRETREATMENT
POSTREATMENT

108. Conclusions
• The most important objective was to improve
the independence in daily living.
• The bones continue to deform resulting in
fractures, despite the successful surgeries and
the use of biphosphonates.
• Until we have etiologic treatments, our
multiple disciplinarian treatment should be
aimed at obtaining a maximum of functional
activity despite the complications.

109. The sooner treatment is begun, the better
the results
Although waiting until the child is 4 years old
might extend the life of the telescopic
nail, generally the deformity or the fractures
force us to perform surgery when they are 2
years old.
In 35 bones, operation under 4 years

110. He doesn’t walk, he runs!