Connective Tissue Disease

6.18
Connective Tissue Disease

Marfan Syndrome

  • Etiology/Risk Factors
    • Autosomal dominant inheritance
  • Pathophysiology
    • Mutation of gene encoding connective tissue protein fibrillin‐1
    • Range of clinical severity
    • Excess growth of long bones and joint laxity
    • Cardiac dysfunction
      • Mitral valve prolapse
      • Aortic valve regurgitation
    • Aortic aneurysm
    • Aortic dissection
    • Emphysematous lung changes
    • Scoliosis
    • Retrognathia common
  • Treatment
    • Avoid vigorous physical activity
    • β‐blockers
    • ARBs
    • Elective surgical repair of aorta
  • Primary Concerns
    • Cardiac dysfunction
    • May be anticoagulated if mitral valve replaced
    • Aortic root disease
    • Spontaneous pneumothorax
  • Evaluation
    • Consider cardiology consultation
      • Monitoring of aortic disease should be done at least annually
    • Consider pulmonary consultation
    • PT/PTT/INR if anticoagulated
  • Anesthesia Management
    • Careful positioning
    • Antibiotic prophylaxis for prosthetic valve, if applicable
    • Potentially difficult airway due to facial dysmorphism
    • Avoid high airway pressures
    • Avoid extremes of blood pressure

Ehlers–Danlos Syndrome

  • Etiology/Risk Factors
    • Inherited genetic mutation affecting synthesis and processing of collagen
    • Autosomal dominant
  • Pathophysiology
    • Skin hyperextensibility
    • Joint hypermobility
    • Tissue fragility
    • Premature degenerative arthritis and joint pain
    • Symptoms of mast cell activation
      • Asthma
      • Urticaria
      • Flushing
    • Scoliosis
  • Treatment
    • Symptom management
    • Physical and occupational therapy
  • Primary Concerns
    • May experience easy bruising and poor wound healing
    • Chronic pain
  • Evaluation
    • Consider physician consultation
      • Periodic echocardiogram likely part of patient management
    • Pain medication regimen
    • Bleeding history
  • Anesthesia Management
    • Careful positioning
    • Resistance to local anesthesia has been reported [136]
    • High suspicion for cervical spine instability and temporomandibular dysfunction
    • Avoid extremes of blood pressure

References

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Oct 16, 2024 | Posted by in Oral and Maxillofacial Surgery | Comments Off on Connective Tissue Disease

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