Marfan syndrome

Overview

Board takeaway: Review aortic and valve disease, cardiac medicines, and anticoagulation before invasive care; support the jaw to prevent dislocation.

Tall stature and long fingers are recognition clues; aortic disease and cardiac medications drive treatment planning.

How does Marfan syndrome affect dental management?

• Cardiology consult: aortic size, valve disease, prior surgery, medications (beta blockers, anticoagulation after valve replacement)

• Prophylaxis: Prophylaxis only for a qualifying indication (such as a prosthetic valve), not for MVP or aortic dilation alone

• Cardiovascular safety: Reduce pain and stress and monitor cardiovascular status.

• Local anesthesia: Individualize local-anesthetic/vasoconstrictor use to the actual aortic and cardiac disease and medications; Marfan syndrome alone does not define a universal epinephrine limit.

• Jaw support: Support the jaw and use short visits to avoid TMJ dislocation; plan orthodontics for crowding

• Antithrombotic changes: do not stop or reduce prescribed anticoagulants or antiplatelets independently. Most routine dental procedures can use local hemostasis; higher bleeding risk or extensive surgery needs a prescriber-directed plan. ADA: medication management.

Exam pearl

What is Marfan syndrome?

An FBN1-related connective-tissue disorder affecting the skeleton, eyes, and cardiovascular system, particularly the aorta.

What are the main systemic features of Marfan syndrome?

• Genetics: FBN1 (fibrillin-1) mutation, autosomal dominant (~25% de novo); ~1 in 5,000
• Skeleton: Tall stature, arachnodactyly, long limbs (arm span over height), pectus deformity, scoliosis, joint laxity
• Eyes: Ectopia lentis (upward lens dislocation), myopia
• Heart and aorta: Aortic root dilation and dissection risk; mitral valve prolapse
• Treatment: Beta blockers or ARBs slow aortic dilation; prophylactic aortic root surgery

What oral findings occur in Marfan syndrome?

• Palate: High-arched, narrow palate with crowding; long narrow face
• Occlusion and differential: Class II with retrognathia; a bifid uvula or cleft palate should broaden the differential, including Loeys–Dietz syndrome, rather than being treated as a defining Marfan feature
• Joints: TMJ hypermobility, recurrent dislocation and TMD

For Marfan syndrome, does the syndrome require endocarditis prophylaxis?

• MVP/aortic dilation alone do not qualify
• Prosthetic valve/prior endocarditis qualify

A tall 14-year-old with long fingers, lens dislocation and a high palate needs extractions for orthodontics. What do you clarify?

Clarify the cardiovascular history and obtain a cardiology consult:

• Aorta and valves: aortic root dilation, prior aortic surgery or valve replacement, and mitral valve prolapse
• Medications: beta blockers or ARBs, and anticoagulants after valve replacement

Sources

• AAPD dental guidance: AAPD: Antibiotic prophylaxis (2026 revision)
• Supplemental systemic features: MedlinePlus Genetics: marfan syndrome
• Supplemental study guide: Pediatric Dental Study Guide: Marfan Syndrome
• Management of Dental Patients with Special Health Care Needs
• Temporomandibular Disorders in Children and Adolescents, Including Those with Special Health Care Needs
• Cardiology Consultation for Pediatric Dental Patients