Dental management of Osteogenesis imperfecta.slideshare

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A presentation I gave in Chicago to the OI foundation. Hope to raise interest in the cranoifa cial management of this orphan disease

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Dental management of Osteogenesis imperfecta.slideshare

  1. 1. Dental Defects and Craniofacial Implications Dr. Jean-Marc Retrouvey, D.M.D, M,Sc, FRCD(C) and Dr. Stephane Schwartz D.M.D, M.ScD, FRCD(C) Montreal Children Hospital
  2. 2. Part 1• Dental and Orthodontic Manifestations of OI Depending on Age and Type/SeverityPart 2• Occlusal Indices in OI as a Quantifiable Phenotype of the Condition
  3. 3.  large influence on the severity of dentoalveolar and craniofacial involvments.* *Schwartz, S. and P. Tsipouras, Oral findings in osteogenesis imperfecta* 1. Oral Surgery, Oral Medicine, Oral Pathology, 1984. 57(2): p. 161-167.
  4. 4. Type I• Fairly short, Multiple fractures , Dentinogenesis imperfecta .Type III• Most severe. Very short in stature, Severe bone fragility, Most DI• Triangular face, Dentoalveolar abnormalities, impacted teeth, MalocclusionType IV• Same as type III but less severe manifestations. Less DIType V , VI and VII• Autosomal recessive transmission, similar clinical findings as OI type I, but more severe (less common than typeI). *Zeitlin, L., F. Fassier, and F.H. Glorieux, Modern approach to children with osteogenesis imperfecta. Journal of Pediatric Orthopaedics B, 2003. 12(2): p. 77.
  5. 5. • Collagen Type I represents 86 to 90% of the organic matrix of the dentin. If collagen type I is involved, the dentition may be severely affected.• Dentinogenesis Imperfecta (DI). Twenty-four to seventy three percent percent of OI patients have DI*,**. * Malmgren, B. and S. Norgren, Dental aberrations in children and adolescents with osteogenesis imperfecta. Acta Odontologica, 2002. 60(2): p. 65-71. ** Lund, A.M., et al., Dental manifestations of osteogenesis imperfecta and abnormalities of collagen I metabolism. Journal of craniofacial genetics and developmental biology, 1998. 18(1): p. 30.
  6. 6. Normal tooth OI-DI tooth • Yellow-dark brown color • Short crown with friable enamel • Weak dentin that may collapse easily • Discoloration from grey to dark brown • Short thin root with a tendency to dilacerate
  7. 7. OI Type I OI Type III OI Type IV• 30% of the pts • 82% of the • 60% of the had DI pts had DI pts had DI (150 patients reviewed by Dr. Blotnick-Shriners hospital)
  8. 8. Color: Varies from grayish- grey to yellow-brown. The color tone intensifies with time. It is intrinsic• DI is one of the most significant aspect of OI• Not all teeth are affected in the same manner• If one OI member of the family has DI, all the other OI member will have DI as well
  9. 9. 1. Blue or brown opalescence,2. Early obliteration of the pulpal tissues,3. Severe microscopic disturbances in the dentin,4. Increased constriction of the cementoenamel junction,5. Short and narrow roots,6. Excessive wear of the teeth. Oral findings in osteogenesis imperfecta Stephane Schwartz, D.D.S., MS.,* and Petros Tsipouras. M.D.,** Montreal, Quebec, Canada
  10. 10. • The shape: The crowns are bulbous and the roots are slender X rays taken 1 year apart• The pulpal spaces undergo (normal 3 years) a accelerated obliteration
  11. 11. • The dental fractures are severe, due to the defective dentin • Some authors think that the enamel- dentin junction is defective**Lindau, B., et al., Morphology of dental enamel and dentine–enamel junction in osteogenesis imperfecta. International Journal of Paediatric Dentistry, 1999. 9(1): p. 13-21.
  12. 12.  Dilacerated Roots
  13. 13.  Ectopic Eruptions
  14. 14.  Impacted Molars
  15. 15. Normal Patient OI-(DI) type III patient
  16. 16. Class II Class I Class III 25% 70% 5%
  17. 17. • OI, specially type III and IV have severe effect on the dental occlusion• Usually, an edge to edge occlusion in resent in the primary dentition
  18. 18. • Normal distribution of Class III malocclusions: 3% of the population. Normally, highly hereditary pattern• Class III with open bite malocclusions: much rarer in normal population• Class III in OI patients: at least 66%. Almost the norm, if we exclude the OI type I
  19. 19.  Anterior  Posterior
  20. 20. Not a dental problemJaw size discrepancy in the transverse plane.
  21. 21. • Lower midline deviation. • Asymmetrical condylar growth pattern • Potential TMJ dysfunction OI type I OI type III37% of type III and 47% of type IV patients present a posterior crossbite *Up to 67% present crossbites according to Schwartz and Tsipouras ** * OConnell, A. and J. Marini, Evaluation of oral problems in an osteogenesis imperfecta population. Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontology, 1999. 87(2): p. 189-196. ** Schwartz, S. and P. Tsipouras, Oral findings in osteogenesis imperfecta* 1. Oral Surgery, Oral Medicine, Oral Pathology, 1984. 57(2): p. 161-167.
  22. 22. Anterior openbite are present in 31.8% of Type III and 17.7%in type IV OI*.Worsens due to potential mandibular overgrowth but mainlyto stunted maxillary growth ** (Maxillary length: 52mm vs 56mm for controls). *OConnell, A. and J. Marini, Evaluation of oral problems in an osteogenesis imperfecta population. Oral Surgery, Oral medicine,Oral Pathology, Oral Radiology, and Endodontology, 1999. 87(2): p. 189-196. **Kindelan, J., et al., Orthodontic and orthognathic management of a patient with osteogenesis imperfecta and dentinogenesis imperfecta: a case report. Journal of Orthodontics, 2003. 30(4): p. 291-296.
  23. 23. Lack of dental eruption? Lack of alveolar bone growth?
  24. 24. • Occlusion usually progresses into an anterior crossbite
  25. 25. T.Q. Apr. 2005 T.Q. Apr. 2008
  26. 26. T.Q. Apr. 2008
  27. 27.  Avez vous des photos faciales do OI ex: Jalbert Deshaies, ect
  28. 28. • During craniofacial growth, sutural membranous and growth sites are affected to various degrees
  29. 29. • Sutural level• Apposition-resorption level  Maxillary development  Dental eruption process• Mandibular length largely unaffected• Postural alterations due to the weight of the head (potential)
  30. 30.  Triangular Face Bossing of the forehead. Ears set low**Rauch, F. and F.H. Glorieux, Osteogenesis imperfecta. The Lancet, 2004. 363(9418): p. 1377-1385.
  31. 31. • Type I: o Slight reduction of size of jaws and skull (WNL)• Type III and IV: o Flattened cranial base o Maxilla posteriorly inclined and retrusive o Mandibular overjet *Jensen, B.L. and A.M. Lund, Osteogenesis imperfecta: clinical, cephalometric, and biochemical investigations of OI types I, III, and IV. Journal of craniofacial genetics and developmental biology, 1997. 17(3): p. 121.
  32. 32. Part 1• Dental and Orthodontic Manifestations of OI Depending on Age and Type/SeverityPart 2: Research (clinical)• Occlusal Indices in OI as a Quantifiable Phenotype of the Condition
  33. 33. Very difficult to conductas standard referenceswere unreliable…OIaffects also cranialbase*. *Cheung, M.S., et al., Cranial base abnormalities in osteogenesis imperfecta: Phenotypic and genotypic determinants. Journal of Bone and Mineral Research, 2011. 26(2): p. 405-413.
  34. 34.  Dr Stéphane Schwartz, DMD, M.Sc Dr Francis Glorieux MD, PhD Dr Frank Rauch, MD, PhD
  35. 35. 1. To quantify the degree of malocclusion present in Osteogenesis Imperfecta (OI) patients using the PAR (Peer Assessment Review) and the DI (Discrepancy Index) indexes of malocclusion
  36. 36. Dental Crowding Overbite
  37. 37. Table 3. Means and standard deviations (SD) for DI parameters for OI and Normal groups Mean (SD) for Mean (SD) for OI control PTotal 29.8 (20.2) 12.4 (6.8) 0.000Overjet 5.7 ( 6.9) 2.4 (1.5) 0.002Overbite 0.2 (0.8) 1.3 (1.6) 0.000Anterior open bite 3.7 (6.0) 0.8 (3.4) 0.004Lateral open bite 7.1 (11.8) 0.3 (0.9) 0.000Crowding 3.6 (2.9) 3.1 (2.5) 0.418Occlusion 5.3 (5.0) 2.9 (2.9) 0.005Lingual post X-bite 2.0 (1.7) 0.5 (1.1) 0.000Buccal post X-bite 0.0 (0.0) 0.4 (1.7) 0.102Other 2.0 (2.7) 0.5 (1.3) 0.001
  38. 38. Posterior open bites were present in 27% of the patients with type III OI and 33% of the patients with type IV OI. They were also very severe.
  39. 39. Posterior open bites developed and worsen with age and were often bilateral; their incidence was 46% in patients greater than 9 years of age for both groups (O’Connell, 1999).
  40. 40.  Correlation appears to be present between the manifestation of the condition at the occlusal level and the severity of the general condition of the patient. * Type III and IV show consistently more severe malocclusions. Arponen, H., et al., Prevalence and natural course of craniocervical junction anomalies during growth in patients with osteogenesis imperfecta. Journal of Bone and Mineral Research, 2012
  41. 41. 1. Improve patient’s masticatory function 2. Better understand bone remodeling processes for better therapeutic outcomes3. Identify pharmacology involvement (effectsof bisphosphonate therapy)
  42. 42.  13 year old with significant masticatory disability Referred to our clinic by the Shriners Hospital Severely compromised masticatory function Digestive blockages requiring complex surgeries (poor mastication is not the only contributing factor)
  43. 43.  Anterior cross-bite with traumatic occlusion Severe posterior bilateral open-bite No chewing capability. Irreversible facial asymmetry No DI (17% of unaffected OI type III patients)
  44. 44. 1- Correct anterior crossbite 2 - Improve midline alignment 3 - Reduce bilateral posterior openbite4 - Improve mastication
  45. 45.  Goal is to better define the OI condition than types I, III and IV and use phenotypes as a more precise way to classify:  Facial patterns and occlusal condition may become viewed as a particular phenotype of the condition
  46. 46. • Dentinogenesis imperfecta, spontaneous dental fractures and difficult repairs• Lack of dental eruptions and alveolar bone vertical growth• Development of severe dental malocclusions, Class III with discrepancies between the upper and lower jaws increasing with severity of condition

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