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Marfan Syndrome Updated 4/2011

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BASICS

  • Description
  • Epidemiology
  • Risk Factors
  • General Prevention
  • Etiology
  • Associated Conditions

DIAGNOSIS

  • Signs and Symptoms
  • Tests
  • Differential Diagnosis

TREATMENT

  • Medication (Drugs)
  • Additional Treatment
  • Surgery

Ongoing Care

  • Follow-Up Recommendations
  • Patient Education
  • Prognosis
  • Complications
The following is an excerpt....
BASICS
Description
  • Marfan syndrome (MFS) is an inherited disorder of connective tissue.
  • System(s) affected: Musculoskeletal; Cardiovascular; Ocular; Pulmonary; Skin/Integument; Connective Tissue (Dura)
  • Because many features of MFS appear in the general population, specific diagnostic criteria (Ghent nosology) were established, recognizing a constellation of features, with major and minor criteria for establishing the diagnosis. (1)
  • The nosology was revised (23,) because the previous criteria were not sufficiently validated, were not consistently applicable in children, or necessitated expensive and specialized tests.
ALERT: Pediatric Considerations
  • Early surgical intervention may reduce the degree of scoliosis.
ALERT: Pregnancy Considerations
  • Manage pregnancy in MFS as high-risk, preferably with a cardiologist. Pre-pregnancy evaluation should include a screening transthoracic echocardiogram for aortic root ...

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FIG. 96.1. Marfan syndrome in a 14-year-old boy. Note arachnodactyly, relatively long limbs (dolichostenomelia), pectus carinatum, sparse subcutaneous fat, unilateral genu valgum, and pes planus. Ectopia lentis and scoliosis also were present. This patient died of aortic rupture at age 15 years.Credit: William J. Koopman, Larry W. Moreland, Arthritis and Allied Conditions A Textbook of Rheumatology, 15e. Philadelphia: Lippincott Williams & Wilkins, 2005.
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