Idiopathic mid-aortic syndrome in children

Area of Science:

• Vascular Surgery
• Pediatric Cardiology
• Rare Diseases

Background:

• Mid-aortic syndrome (MAS) is an uncommon condition involving abdominal aorta and major branch stenosis.
• Idiopathic MAS cases are rare, necessitating comprehensive reviews for understanding presentation and management.

Observation:

• This study reviewed six new idiopathic MAS cases alongside 96 literature cases, totaling 102 patients.
• The youngest patient presented at 19 days old, highlighting the condition's early onset potential.
• Hypertension was the most common clinical presentation (94%), followed by claudication (17%).

Findings:

• Angiography is the preferred diagnostic tool for MAS.
• Renal arteries were involved in 91% of cases, with superior mesenteric and celiac artery involvement at 35%.
• Medical management is feasible for initial blood pressure control, but surgical intervention is often required.

Implications:

• Early diagnosis and management of MAS are crucial to prevent complications like renal failure and intestinal ischemia.
• Conservative blood pressure management is effective unless unsatisfactory, or symptoms like claudication/ischemia arise.
• Surgical intervention timing should be carefully planned, potentially post-puberty, to accommodate patient growth.