20 Years of Surgical Treatment in    Osteogenesis Imperfecta             Dr. Parra García JI          Dr. Bueno Sánchez AM...
Osteogenesis Imperfecta•    No clear definition•    Genetic disorder, bones fragility (30)•    Skeletal manifestations•   ...
Classification of Osteogenesis Imperfecta revisitedF.S. Van Dijk a,*, G. Pals a, R.R. Van Rijn b, P.G.J. Nikkels c, J.M. C...
Sillence slide OI International Congres2011
Osteogénesis Imperfecta                Bifosfonates treatment                Phisiotherapy                Surgery
 Generally, we feel that the treatment applied to OI patients  has change their lives: There is a tremendous difference ...
ARE WE IMPROVING THE PHYSICAL  SITUATION OF OUR PATIENTS? IS IT WORTH IT, AFTER UNDERGOING MULTIPLE SURGERIES?
1972
1984
19 years after
1992
May 1993   "Displasias óseas; Enfoque diagnóstico y tratamiento   quirúrgico". Profesor M. Tachdjian, Profesor Michael Gol...
Surgical treatment     Autor           Año      Tipoclavo                   P        Telesc.   Maciz.   OperTOTAL     Tile...
Data base 65 p.RubenGG 7JavierTS 10LauraR 6EstebanVM 7JuanNS 4              11 PatientsPedroP 3              45 SurgeriesR...
BS 1LPM 1         Data base 130 p.RGG 7JTS 11LR 6EVM 7DMP 2JNS 7PP 3RMA 5CTF 2                  25 PatientsJFB 1DMP 2MLP 1...
Khalid               2001   SofieldAnálisi 34                               s de marcha   Zionts               2002   17 F...
Abulsaad M, Abdelrahman A. Modified Sofield-Millar operation: less invasive surgery oflower limbs in osteogenesis imperfec...
Osteogénesis ImperfectaJIP
9.01
Fassier-Duvaltelescopic nails
Pediatric endocrinologistPhysiotherapistsChild Orthopaedic SurgeonsGeneticistsOtolaryngologistsOdontologyNeurosurgeonsGyne...
MATERIAL and METHODS• We analyzed the results of surgeries with Rush  rods, Fassier-Duval (FD), Bailey-Dubow (BD) and  sol...
Of a total of 199 patients with OI, 52 patients had to be operated                 totaling 172 surgical procedures       ...
The average patient age was 8.00 years (standard deviation of               4.64 and a range from 1 to 44).         Of the...
Of the 172 surgeries, 144 were carried outin 162 long bones (in some cases we operated on more                    than one...
162 long bones• 152 femurs and tibias and 10 hips• 152 long bones were treated with intramedullary  nailing.
Nails employed:35 Rush17 Bailey-Dubow telescopic nails88 Fassier-Duval telescopic nailsOther nails: elastic or Interlockin...
Reasons for primary surgery:43 due to deformity of femur or tibia9 for a deformity associated with a fracture39 cases due ...
Requisites85% of the patients in the first surgerywere being treated with bisphosphonatesand pre and postop. with physical...
Fassier F, Glorieux F. Osteogenesis imperfecta. In: Surgical techniques inorthopaedics and traumatology. Paris: Elsevier; ...
4-5 yearsEngelbert, Raoul H. Intramedullary rodding in type IIIosteogenesis imperfecta: Effects on neuromotordevelopment i...
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...
Older patiens that walk
Bone qualityJessica M. Fritz a,∗, Yabo Guana,d, MeiWanga, Peter A. Smithb, Gerald F. Harrisa,b,cA fracture risk assessment...
9 year
Postop1. Passive movement of the affected articulations after 24   hours2. Pressure exercises in axis with resistence afte...
COMPLICATIONS• We have 28.8% (45 cases) repeated surgeries  due to complications in the nail (42%)• In 23% (35 cases) we h...
Birke O, Davies N, Latimer M, Little DG, Bellemore M.J Pediatr Orthop. 2011 Jun;31(4):458-64.Experience with the Fassier-D...
Fassier-Duval telescopic rod   ComplicationsBD                     39%No telescopic          50%FD                      15%
JBJS 2011 Nov 2;93(21):1994-2000.Use of the Sheffield telescopic intramedullary rod system for the management of osteogene...
Nail changes• In the 35 cases when it was necessary to  remove the nails, the percentages were as  follows:     41.6% of ...
Causes of seconds surgeriesWe have carried out 45 second surgeries due to complications with the nail (28.8%):  –   16 cas...
Once a nail is inserted filling the canal andwalking is stimulated, the nail is usually toosmall in relation to the cortic...
She has an 8-10 minute walk to school!!!!!!!!
• All the complications were resolved satisfactorily.• This percentage of complications has been  decreasing year after ye...
No coxa valga
No physeal closing
11 years20 fractures
Coxa varaWagner technique, used by Finidori and FassierBone bank block reinforcement
Bone bank block
Bone bank block
Hands in pocket sign!!!
Nail can prevent some fractures, but not allOn occasions, the nail will endure thefractures, so the child will be able to ...
More activity = more fractures
GAP Nail
RESULTSThe patients have skipped a step or two in theEl Sobky classificationbedridden   wheel chair          crutches     ...
RESULTS   83% walking                            (preop 14.2%),          11.9% crutches                         (preop 4.7...
Conclusions• The most important objective was to improve  the independence in daily living.• The bones continue to deform ...
The sooner treatment is begun, the better                    the resultsAlthough waiting until the child is 4 years oldmig...
He doesn’t walk, he runs!
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
Surgical treatment in OI  2012 Polonia
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Surgical treatment in OI 2012 Polonia

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

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Surgical treatment in OI 2012 Polonia

  1. 1. 20 Years of Surgical Treatment in Osteogenesis Imperfecta Dr. Parra García JI Dr. Bueno Sánchez AM Hospital Universitario de Getafe MADRID
  2. 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. 3. Classification of Osteogenesis Imperfecta revisitedF.S. Van Dijk a,*, G. Pals a, R.R. Van Rijn b, P.G.J. Nikkels c, J.M. CobbenEuropean Journal of Medical Genetics xxx (2009)“a heterogeneous group of diseases characterized bysusceptibility to bone fractures with variable severityand presumed or proven defects in collagen type Ibiosynthesis”
  4. 4. Sillence slide OI International Congres2011
  5. 5. Osteogénesis Imperfecta Bifosfonates treatment Phisiotherapy Surgery
  6. 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. 7. ARE WE IMPROVING THE PHYSICAL SITUATION OF OUR PATIENTS? IS IT WORTH IT, AFTER UNDERGOING MULTIPLE SURGERIES?
  8. 8. 1972
  9. 9. 1984
  10. 10. 19 years after
  11. 11. 1992
  12. 12. May 1993 "Displasias óseas; Enfoque diagnóstico y tratamiento quirúrgico". Profesor M. Tachdjian, Profesor Michael Golberg
  13. 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. 14. Data base 65 p.RubenGG 7JavierTS 10LauraR 6EstebanVM 7JuanNS 4 11 PatientsPedroP 3 45 SurgeriesRubenM 2 35 Multiple OsteotomiesCritinaTF 2JoseA.FB 1DanielMP 2Miranda LP 1 2000
  15. 15. BS 1LPM 1 Data base 130 p.RGG 7JTS 11LR 6EVM 7DMP 2JNS 7PP 3RMA 5CTF 2 25 PatientsJFB 1DMP 2MLP 1MC 2 80 SurgeriesVB 3SPQ 4RGF 3MJG 1ABM 1MLP 2AAS 1AAS 2AGM 1 2006PHG 2ACD 1
  16. 16. Khalid 2001 SofieldAnálisi 34 s de marcha Zionts 2002 17 Fracturas 10 Olecranon Boutaud, 2004 Macizos/Elast 14 36 Laville icos cruzadosJ Pediatr Orthop B. 2005 Sep;14(5):311-9 The choice of intramedullary devices for the femur andthe 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. 17. Abulsaad M, Abdelrahman A. Modified Sofield-Millar operation: less invasive surgery oflower 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 minimumof 1-year follow-up. Surgical index in OI Number o patients operated / surgical procedures Around 3
  18. 18. Osteogénesis ImperfectaJIP
  19. 19. 9.01
  20. 20. Fassier-Duvaltelescopic nails
  21. 21. Pediatric endocrinologistPhysiotherapistsChild Orthopaedic SurgeonsGeneticistsOtolaryngologistsOdontologyNeurosurgeonsGynecologistsPsychologist
  22. 22. 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.
  23. 23. 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
  24. 24. 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%
  25. 25. Of the 172 surgeries, 144 were carried outin 162 long bones (in some cases we operated on more than one bone) TYPES OF SURGERIES Long bones Other surgeries 11% 89%
  26. 26. 162 long bones• 152 femurs and tibias and 10 hips• 152 long bones were treated with intramedullary nailing.
  27. 27. Nails employed:35 Rush17 Bailey-Dubow telescopic nails88 Fassier-Duval telescopic nailsOther nails: elastic or Interlocking nail Others 3 2% RUSH 35 24% B-D 17 F-D 88 12% 62%
  28. 28. Reasons for primary surgery:43 due to deformity of femur or tibia9 for a deformity associated with a fracture39 cases due to fractures22 in other surgeries in feet, knees or coxa vara Deformity Deformity and fracture FRACTURE OTHERS 43 39 22 9
  29. 29. Requisites85% of the patients in the first surgerywere being treated with bisphosphonatesand pre and postop. with physical therapySurgery used always tried to minimizebleeding and muscle injury
  30. 30. Fassier F, Glorieux F. Osteogenesis imperfecta. In: Surgical techniques inorthopaedics and traumatology. Paris: Elsevier; 2003. pp. 1–8.
  31. 31. 4-5 yearsEngelbert, Raoul H. Intramedullary rodding in type IIIosteogenesis imperfecta: Effects on neuromotordevelopment in 10 children, Acta Orthopaedica, 66: 2 y 3.5 years4, 361 — 364 1995Surgical treatment of osteogenesis imperfecta: currentconcepts IniciatingEsposito, Paul; Plotkin, Horacio walkingCurrent Opinion in Pediatrics:February 2008 - Volume 20 - Issue 1 - p 52-57172 Surgeries 144 long bones 11% 35 Under 3 years 89%
  32. 32. 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
  33. 33. Older patiens that walk
  34. 34. Bone qualityJessica M. Fritz a,∗, Yabo Guana,d, MeiWanga, Peter A. Smithb, Gerald F. Harrisa,b,cA fracture risk assessment model of the femur in children with osteogenesisimperfecta (OI) during gait Medical Engineering & Physics 31 (2009) 1043–1048
  35. 35. 9 year
  36. 36. Postop1. Passive movement of the affected articulations after 24 hours2. Pressure exercises in axis with resistence after 24 hours3. Progressive weight in the pool as soon as scarring is completed4. Anti rotation dressing on femur surgery 3 weeks5. 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.
  37. 37. 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%)
  38. 38. 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: first24 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 a40% complication rate (6 of 15 cases): rod migration and limitedtelescoping (5) and intraoperative joint intrusion (1). There wereno infections. 24 cases 40% de complications
  39. 39. Fassier-Duval telescopic rod ComplicationsBD 39%No telescopic 50%FD 15%
  40. 40. JBJS 2011 Nov 2;93(21):1994-2000.Use of the Sheffield telescopic intramedullary rod system for the management of osteogenesisimperfecta: 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 pastfifty 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 Sheffieldsystem of telescopic intramedullary rods.METHODS:We conducted a retrospective analysis of patients with osteogenesis imperfecta who were at least eighteenyears of age and who had at least thirteen years of follow-up. Complications, reoperations, and data from adisease-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 telescopicintramedullary rods were available at an average of nineteen years after the initial surgery. Reoperationsinvolving thirty-three (50%) of the sixty-six rods were performed: ten rods (15%) were exchanged becauseof rod disengagement due to growth, thirteen rods (20%) were exchanged because of complications, andten rods (15%) required further surgery other than exchange because of complications. Mobility wassignificantly improved at the initial postoperative visit (p = 0.0015), and this improvement was maintainedinto adulthood (p = 0.0077). Back pain was the most frequent symptom. Symptoms related to rod insertionacross the knee and ankle were rare, but symptoms related to proximal femoral trochanteric entry werecommon. Physeal damage was not seen following surgery, and all rods elongated with growth. All patientswere satisfied with the outcome of the surgical procedures. Short Form-36 scores for all physical domainsand 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 mostcommonly related to the patient outgrowing the rods. Concerns regarding insertion of this fixed device atthe knee and ankle were unfounded, although proximal femoral fixation remains a problem.
  41. 41. 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
  42. 42. Causes of seconds surgeriesWe 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.
  43. 43. Once a nail is inserted filling the canal andwalking is stimulated, the nail is usually toosmall in relation to the cortical thickness andusually bends along with the bone.
  44. 44. She has an 8-10 minute walk to school!!!!!!!!
  45. 45. • 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
  46. 46. No coxa valga
  47. 47. No physeal closing
  48. 48. 11 years20 fractures
  49. 49. Coxa varaWagner technique, used by Finidori and FassierBone bank block reinforcement
  50. 50. Bone bank block
  51. 51. Bone bank block
  52. 52. Hands in pocket sign!!!
  53. 53. Nail can prevent some fractures, but not allOn occasions, the nail will endure thefractures, so the child will be able to return toan active life style.
  54. 54. More activity = more fractures
  55. 55. GAP Nail
  56. 56. RESULTSThe patients have skipped a step or two in theEl Sobky classificationbedridden wheel chair crutches walking El Sobky and alts. Egyptian Orthopedic J. 1999
  57. 57. 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
  58. 58. 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.
  59. 59. The sooner treatment is begun, the better the resultsAlthough waiting until the child is 4 years oldmight extend the life of the telescopicnail, generally the deformity or the fracturesforce us to perform surgery when they are 2years old. In 35 bones, operation under 4 years
  60. 60. He doesn’t walk, he runs!

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