What is the best approach for treating pectus excavatum in a child with Marfan's syndrome?

Treatment of Pectus Excavatum in a Child with Marfan's Syndrome

The treatment of pectus excavatum in a child with Marfan's syndrome requires a multidisciplinary approach with primary focus on cardiovascular monitoring and management, with surgical correction of the pectus deformity considered when clinically significant or causing psychological distress.

Cardiovascular Assessment and Management (Priority)

In children with Marfan's syndrome, cardiovascular monitoring takes precedence over cosmetic concerns:

• Echocardiogram evaluation is essential before any consideration of pectus repair 1

  • If aortic root diameter is normal: Repeat echocardiogram every 2-3 years until adult height is reached
  • If aortic root dilation is present: More frequent monitoring (every 6-12 months depending on severity)

• Medical therapy for cardiovascular protection:

  • Beta-blockers or angiotensin receptor blockers (ARBs) in maximally tolerated doses to reduce aortic dilation rate 1
  • Combination therapy with both beta-blockers and ARBs may be reasonable for better protection 1

Pectus Excavatum Management Options

Conservative Management

• Vacuum Bell therapy should be considered as first-line treatment for mild to moderate pectus excavatum in younger patients 2
  • Requires consistent use for multiple hours daily over several months
  • Most effective in prepubertal children with flexible chest walls

Surgical Intervention

Surgical correction should be considered when:

1. Cardiopulmonary compromise is present:

   • Compression of cardiac structures causing hemodynamic effects
   • Respiratory limitations affecting quality of life

2. Significant psychological impact affecting the child's quality of life

3. Surgical options:

   • Nuss procedure (minimally invasive repair) - preferred in children and adolescents 2
   • Ravitch procedure (open surgical repair) - may be necessary in more complex cases

Important Considerations and Potential Complications

• Timing of intervention is critical:

  • Surgical repair of pectus excavatum should be delayed if aortic root dilation is present and approaching surgical thresholds (≥4.5 cm) 1
  • Aortic root surgery takes precedence over pectus repair

• Risk of hemodynamic instability:

  • Cases have been reported where pectus excavatum required emergent repair after aortic root surgery due to hemodynamic compromise 3
  • Careful preoperative planning is essential to avoid complications

• Surgical approach considerations:

  • Standard median sternotomy may be difficult in severe pectus excavatum
  • Alternative approaches (partial sternotomy with thoracotomy) may be required for cardiac procedures in patients with severe pectus 4

Follow-up Protocol

• Regular cardiovascular monitoring regardless of pectus treatment decision
• Annual evaluation for scoliosis until adult height is reached 1
• Continued medical therapy for cardiovascular protection even after any surgical intervention

Caution

Failure to recognize cardiovascular manifestations of Marfan's syndrome before pectus repair can lead to serious complications. There have been cases where annuloaortic ectasia was discovered only after pectus repair complications 5, highlighting the importance of thorough cardiovascular assessment before any chest wall surgery.