eMedicine Specialties > Orthopedic Surgery > Neoplasms

Osteogenesis Imperfecta: Follow-up

Author: Manoj Ramachandran, MBBS, MRCS, FRCS, Consultant Trauma and Orthopaedic Surgeon, Barts and the London NHS Trust; Honorary Senior Lecturer, William Harvey Research Institute, Barts and The London School of Medicine and Dentistry, Queen Mary's, University of London, UK
Coauthor(s): David H A Jones, MB, ChB, FRCS, FRCS Ed(Orth), Consultant Orthopedic Surgeon, Great Ormond Street Hospital for Children; Senior Clinical Lecturer, University College London Hospitals, UK; Pramod Achan, MBBS, FRCS(Orth), Senior Registrar, Royal National Orthopaedic Hospital, UK; Peter R Calder, MBBS, FRCS(Eng), FRCS (Tr&Orth), Consulting Surgeon, Department of Pediatric Orthopedic Surgery, The Royal National Orthopaedic Hospital, UK
Contributor Information and Disclosures

Updated: Nov 20, 2008

Follow-up

Further Outpatient Care

  • Home visits and regular clinic assessments are necessary, particularly in the first few years of life. Postoperatively, close follow up is vital to ensure fracture healing and restoration of function.

Inpatient & Outpatient Medications

Complications

Prognosis

  • See the table with the adapted Sillence classification in the Clinical, History section, above.

Patient Education

  • Patients with osteogenesis imperfecta are generally well motivated and keen to achieve as much as possible despite their physical limitations.
  • Education is extremely important, particularly for those who may respond to their condition in a more negative way and therefore be prone to low self-esteem and depression.
  • Education is also important for parents and families to help them deal with the day-to-day implications and management of the patient's condition.

Miscellaneous

Medicolegal Pitfalls

  • Anesthetic-related problems may arise from in patients with relatively large heads and tongues and in those with short necks.
  • Chest deformities may cause respiratory complications.
  • On the operating table, fractures may arise as a result of the application of a blood pressure cuff or tourniquet, or they may occur during transfers.
  • Watch for hyperthermia and increased sweating.
 


More on Osteogenesis Imperfecta

Overview: Osteogenesis Imperfecta
Differential Diagnoses & Workup: Osteogenesis Imperfecta
Treatment & Medication: Osteogenesis Imperfecta
Follow-up: Osteogenesis Imperfecta
References
Further Reading
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References

  1. Smith R, Francis MJ, Houghton GR. The brittle bone syndrome. In: Osteogenesis Imperfecta. London: Butterworth. 1983.

  2. Brusin JH. Osteogenesis imperfecta. Radiol Technol. Jul-Aug 2008;79(6):535-48. [Medline].

  3. Cole WG. The Nicholas Andry Award-1996. The molecular pathology of osteogenesis imperfecta. Clin Orthop. Oct 1997;235-48. [Medline].

  4. Cole WG. Advances in osteogenesis imperfecta. Clin Orthop. Aug 2002;6-16. [Medline].

  5. Cole WG. Bone, cartilage and fibrous tissue disorders. In: Benson MKD, Fixsen JA, MacNicol MF, Parch K, eds. Children's Orthopaedics. 2002: 67-92.

  6. Baujat G, Lebre AS, Cormier-Daire V, Le Merrer M. [Osteogenesis imperfecta, diagnosis information (clinical and genetic classification)]. Arch Pediatr. Jun 2008;15(5):789-91. [Medline].

  7. Sillence D. Osteogenesis imperfecta: an expanding panorama of variants. Clin Orthop. Sep 1981;11-25. [Medline].

  8. Sillence DO, Senn A, Danks DM. Genetic heterogeneity in osteogenesis imperfecta. J Med Genet. Apr 1979;16(2):101-16. [Medline].

  9. Labuda M, Morissette J, Ward LM. Osteogenesis imperfecta type VII maps to the short arm of chromosome 3. Bone. Jul 2002;31(1):19-25. [Medline].

  10. Ward LM, Rauch F, Travers R. Osteogenesis imperfecta type VII: an autosomal recessive form of brittle bone disease. Bone. Jul 2002;31(1):12-8. [Medline].

  11. Duro Friedl EA, Ferrari Mayans L, Desalvo Portal LN, Ferrari Ruiz P, Bidondo Horno MP, Astraldi Tellechea MM. [Bruck syndrome: Osteogenesis imperfecta with congenital joint contractures.]. An Pediatr (Barc). Jul 2008;69(1):90-1. [Medline].

  12. Francis MJ, Smith R, Bauze RJ. Instability of polymeric skin collagen in osteogenesis imperfecta. Br Med J. Mar 9 1974;1(905):421-4. [Medline].

  13. Jones D, Hosalkar H, Jones S. The orthopaedic management of osteogenesis imperfecta. Clin Orthop. 2002;16:374-88.

  14. Zeitlin L, Fassier F, Glorieux FH. Modern approach to children with osteogenesis imperfecta. J Pediatr Orthop B. Mar 2003;12(2):77-87. [Medline].

  15. Forin V. [Paediatric osteogenesis imperfecta: medical and physical treatment]. Arch Pediatr. Jun 2008;15(5):792-3. [Medline].

  16. Sofield HA, Page MA, Mead NC. Multiple osteotomies and metal-rod fixation for osteogenesis imperfecta. J Bone Joint Surg. 1952;34A:500-2.

  17. Glorieux FH, Bishop NJ, Plotkin H, et al. Cyclic administration of pamidronate in children with severe osteogenesis imperfecta. N Engl J Med. Oct 1 1998;339(14):947-52. [Medline].

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Keywords

osteogenesis imperfecta, brittle bones, brittle bone disease, brittle-bone disease, blue sclera syndrome, blue-sclera syndrome, fragile bone disease, fragile-bone disease, Lobstein disease, Lobstein's disease, dentinogenesis imperfecta, Sillence classification, COL1A gene, COL2A gene, popcorn bones, osteoporosis-pseudoglioma, Bruck syndrome, Cole-Carpenter syndrome, OI, bone fragility, osteogenesis imperfecta congenita, osteogenesis imperfecta tarda, platyspondylia, platyspondylisis, broken bones

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Contributor Information and Disclosures

Author

Manoj Ramachandran, MBBS, MRCS, FRCS, Consultant Trauma and Orthopaedic Surgeon, Barts and the London NHS Trust; Honorary Senior Lecturer, William Harvey Research Institute, Barts and The London School of Medicine and Dentistry, Queen Mary's, University of London, UK
Manoj Ramachandran, MBBS, MRCS, FRCS is a member of the following medical societies: British Orthopaedic Association
Disclosure: Nothing to disclose.

Coauthor(s)

David H A Jones, MB, ChB, FRCS, FRCS Ed(Orth), Consultant Orthopedic Surgeon, Great Ormond Street Hospital for Children; Senior Clinical Lecturer, University College London Hospitals, UK
David H A Jones, MB, ChB, FRCS, FRCS Ed(Orth) is a member of the following medical societies: British Orthopaedic Association
Disclosure: Nothing to disclose.

Pramod Achan, MBBS, FRCS(Orth), Senior Registrar, Royal National Orthopaedic Hospital, UK
Disclosure: Nothing to disclose.

Peter R Calder, MBBS, FRCS(Eng), FRCS (Tr&Orth), Consulting Surgeon, Department of Pediatric Orthopedic Surgery, The Royal National Orthopaedic Hospital, UK
Peter R Calder, MBBS, FRCS(Eng), FRCS (Tr&Orth) is a member of the following medical societies: British Medical Association
Disclosure: Nothing to disclose.

Medical Editor

Miguel A Schmitz, MD, Consulting Surgeon, Department of Orthopedics, Klamath Orthopedic and Sports Medicine Clinic
Miguel A Schmitz, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American Orthopaedic Society for Sports Medicine, and Arthroscopy Association of North America
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Ian D Dickey, MD, FRCSC, Adjunct Professor, Department of Chemical and Biological Engineering, University of Maine; Consulting Staff, Adult Reconstruction, Orthopedic Oncology, Department of Orthopedics, Eastern Maine Medical Center
Ian D Dickey, MD, FRCSC is a member of the following medical societies: American Academy of Orthopaedic Surgeons, British Columbia Medical Association, Canadian Medical Association, and Royal College of Physicians and Surgeons of Canada
Disclosure: Stryker Orthopaedics Consulting fee Consulting

CME Editor

Dinesh Patel, MD, FACS, Associate Clinical Professor of Orthopedic Surgery, Harvard Medical School; Chief of Arthroscopic Surgery, Department of Orthopedic Surgery, Massachusetts General Hospital
Dinesh Patel, MD, FACS is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American Association of Physicians of Indian Origin, American College of International Physicians, and American College of Surgeons
Disclosure: Nothing to disclose.

Chief Editor

Harris Gellman, MD, Consulting Surgeon, Broward Hand Center; Voluntary Clinical Professor of Orthopedic Surgery and Plastic Surgery, Departments of Orthopedic Surgery and Surgery, University of Miami School of Medicine
Harris Gellman, MD is a member of the following medical societies: American Academy of Medical Acupuncture, American Academy of Orthopaedic Surgeons, American Orthopaedic Association, American Society for Surgery of the Hand, and Arkansas Medical Society
Disclosure: Nothing to disclose.

 
 
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