Abstract: TH-PO623

Hemihypertrophy versus Hemiatrophy: A Unique Presentation of Midaortic Syndrome

Session Information

Category: Nephrology Education

  • 1302 Fellows and Residents Case Reports

Authors

  • Herdes, Rachel, Louisiana State University Health Sciences Center, New Orleans, Louisiana, United States
  • Patel, Nikita R, Louisiana State University Health Sciences Center, New Orleans, Louisiana, United States
  • Clark, Cullen, Louisiana State University Health Sciences Center, New Orleans, Louisiana, United States
  • Ashoor, Isa, Louisiana State University Health Sciences Center, New Orleans, Louisiana, United States
Background

Midaortic syndrome is the acquired or congenital narrowing of the abdominal aorta and associated branches. Patients often present prior to adolescence with severe hypertension (HTN), headaches, claudication, or abdominal angina. Treatment consists of multiple antihypertensive medications and often requires surgical intervention for adequate control. Left untreated, patients usually die in their 40s from malignant HTN.

Methods

Case report

Results

A previously healthy 10-year-old girl was referred to genetics for right-lower extremity (RLE) hemihypertrophy. She was incidentally noted to have severe HTN (180/100 mmHg) in clinic and was admitted to the ICU for BP control. She denied headaches, vision changes, or abdominal discomfort. Exam was notable for coarse facies, enlarged RLE compared to left lower extremity (LLE), and a paraumbilical bruit. ECHO demonstrated mild LVH. CRP, serum aldosterone, plasma renin activity, creatinine, and metanephrines were normal. Renal ultrasound showed elevated bilateral peak systolic velocities. Abdominal CT angiogram revealed significant vasculopathy with abdominal aorta narrowing and multiple stenotic areas along the main branches, including complete left common iliac artery occlusion with collateral flow (Figure 1). She was diagnosed with idiopathic midaortic syndrome and treated with amlodipine, atenolol and chlorthalidone. Genetics evaluation of vascular anomalies is pending.

Conclusion

We present an unusual case of unilateral anatomic asymmetry. While the patient had been given a diagnosis of RLE hemihypertrophy, it is more likely that she suffered from LLE hemiatrophy due to ischemia from midaortic syndrome. This case demonstrates the importance of pediatric HTN evaluation including thorough examination to identify abdominal bruits, extremity size discrepancy, or end-organ damage from severe HTN.