Rosh Pediatrics Practice Exam

Which skeletal deformity is most commonly associated with Marfan syndrome in adolescents?

1. Arachnodactyly

2. Dolichostenomelia

3. Pectus excavatum

4. Scoliosis

The correct answer is: Scoliosis

In adolescents with Marfan syndrome, scoliosis is a common skeletal deformity that can develop due to the underlying connective tissue abnormalities associated with the condition. Marfan syndrome is characterized by a defect in fibrillin-1, which affects the integrity and strength of connective tissues throughout the body. This can lead to abnormal growth patterns in the spine, causing it to curve laterally in a condition known as scoliosis. Adolescents are particularly susceptible to developing scoliosis as they experience rapid growth during puberty, and the abnormal connective tissue can result in uneven growth of the vertebrae. Scoliosis in Marfan syndrome can become noticeable in school-age children or during adolescence, often requiring monitoring or intervention such as bracing or surgery if the curvature is significant. While arachnodactyly, dolichostenomelia, and pectus excavatum are also associated with Marfan syndrome, scoliosis tends to be the most frequently noted skeletal deformity during the adolescent years. Arachnodactyly refers to long fingers and toes, dolichostenomelia involves long limbs, and pectus excavatum presents as a sunken chest, but these features might not be as pronounced or problematic as scoliosis in this demographic, which often