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Orthopedics & Traumatology

Marfan Syndrome with Skeletal Manifestations

ICD-10 Code
Q87.40_1

Genetic disorder affecting connective tissue, leading to skeletal abnormalities like tall stature, long limbs, and scoliosis.

Clinical Presentation & Protocol

Patient Usually Complains Of

Patient presents for evaluation of known Marfan syndrome. Reports progressive skeletal changes including increased height, joint hypermobility, and recent onset of back pain. No current complaints of chest pain, palpitations, or visual disturbances. Family history positive for connective tissue disorders.

Clinical Examination Findings

General: Tall, thin habitus, dolichostenomelia. Skeletal: Positive wrist sign (Walker-Murdoch) and thumb sign (Steinberg). Spine: Evidence of thoracic scoliosis with mild kyphosis. Pectus excavatum/carinatum noted. Joints: Generalized ligamentous laxity. Feet: Pes planus.

Treatment Protocol

Orthopedic referral for scoliosis monitoring and bracing if indicated. Physical therapy for core strengthening and joint stabilization. Annual monitoring of skeletal progression. Referral to cardiology for echocardiographic screening of aortic root diameter.

Detailed clinical guide coming soon.