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Midaortic syndrome in a premature infant
Prakash M Kabbur1, Victor Herson, Leslie Wolkoff
1Department of Pediatrics, Connecticut Children's Medical Center, University of Connecticut School of Medicine, Hartford, USA.
Connecticut Medicine
|June 8, 2012
Summary
Midaortic syndrome (MAS) is a rare cause of intractable hypertension in premature infants. This case report details the youngest infant diagnosed with MAS, highlighting diagnostic challenges in neonates.
Area of Science:
- Pediatric Cardiology
- Neonatology
- Medical Genetics
Background:
- Hypertension in premature infants is uncommon and requires thorough investigation.
- Known causes include endocrine, renal, and cardiac anomalies.
- Coarctation of the thoracic aorta is a recognized cardiac cause of hypertension.
Observation:
- Midaortic syndrome (MAS), or midaortic dysplastic syndrome, is a rare condition characterized by abdominal aorta hypoplasia and narrowing of visceral branches.
- MAS often presents as intractable hypertension, posing diagnostic difficulties in neonates, particularly preterm, low-birth-weight infants.
- This report describes a premature infant with in utero congestive heart failure and postnatal intractable hypertension.
Findings:
- The case involves the youngest reported infant with MAS.
- Autopsy confirmation was obtained, providing definitive diagnosis.
- This case highlights the extreme difficulty in diagnosing MAS in neonates.
Implications:
- Early diagnosis and management of MAS are crucial for improving outcomes in affected infants.
- This case underscores the need for heightened awareness of rare vascular anomalies in neonatal hypertension.
- Further research into the etiology and optimal treatment strategies for MAS in neonates is warranted.
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