12. cobey marfan syndrome


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12. cobey marfan syndrome

  1. 1. Marfan Syndrome Cobey, Marjorie P. DMD2D
  2. 2. Definition• Also called Marfans syndrome• It is a genetic disorder of the connective tissue• People with Marfans tend to be unusually tall, with long limbs and long, thin fingers.• The syndrome is inherited as a dominant trait, carried by the gene FBN1, which encodes the connective protein fibrillin-1
  3. 3. • Marfan syndrome is caused by a single gene for fibrillin on chromosome 15, which is inherited in most cases from an affected parent.• Fibrillin-1 plays an important role as thebuilding block for connective tissue in thebody.
  4. 4. • The gene defect alsocauses too much growth ofthe long bones of the body.This causes the tall heightand long arms and legs seenin people with thissyndrome
  5. 5. • The aorta, the main blood vessel that takes blood from the heart to the body may stretch or become weak (called aortic dilation or aortic aneurysm)
  6. 6. Signs and Symptoms• Affects the eyes & vision. Nearsightedness and astigmatism are common, but farsightedness can also result • Lens dislocation in Marfans syndrome; the lens was kidney-shaped and was resting against the ciliary body
  7. 7. • People with Marfan syndrome are usually tall with long, thin arms and legs and spider-like fingers -- called arachnodactyly. When they stretch out their arms, the length of their arms is greater than their height.
  8. 8. • A chest that sinks in or sticks out -- funnel chest or pigeon breast• Flat feet• One of the hidden signs of Marfans syndrome is the high arched palate crowded teeth
  9. 9. Dental Correlation• Patients typically have a high-arched palate produced by a narrow maxilla and skeletal Class II malocclusion due to mandibular retrognathia• Individuals affected by the syndrome routinely seek orthodontic treatment to correct the orofacial manifestations
  10. 10. Generalized gingival inflammation was seen consistently at each visit during treatment. (A) Frontal. (B) Right buccal. (C) Left buccal.• Orthognathic surgery sometimes is required tocorrect severe orofacial discrepancies in patientswith Marfan syndrome
  11. 11. • The treating orthodontist may be apprehensive of including a surgical procedure in the treatment plan because of potential complications related to surgery• Surgeons should work closely with the patients’ cardiologists when planning treatment
  12. 12. Classification• It is a single gene disorder• Autosomal Dominant Inheritance• Autosomal dominant inheritance means that the gene is located on one of the autosomes (chromosome pairs 1 through 22)• The gene for Marfan syndrome, called fibrillin- 1, is localized to chromosome #15 on the long arm (q) at 15q21.1