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Updated: Dec 28, 2025
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State of the Art Cranial Ultrasound Imaging in Neonates
Published on: February 2, 2015
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Macrocephaly Secondary to Superior Vena Cava Syndrome
Pouya Entezami1, Amanda Custozzo1, Matthew Adamo2
1Department of Neurosurgery, Albany Medical Center, Albany, New York, USA.
Pediatric Neurosurgery
|February 19, 2020
Summary
Congenital heart defect repair can lead to superior vena cava syndrome and macrocephaly in neonates. Conservative management and cardiovascular intervention stabilized head growth, avoiding neurosurgical procedures.
Area of Science:
- Pediatric Cardiology
- Pediatric Neurosurgery
- Neonatal Neurology
Background:
- Neonatal cardiac defects frequently coexist with neurological or neuroanatomical anomalies.
- Superior vena cava (SVC) syndrome is a potential complication following cardiac surgery.
Observation:
- A neonate with congenital tetralogy of Fallot developed macrocephaly secondary to SVC syndrome post-repair.
- The patient presented with macrocephaly but no overt signs of increased intracranial pressure.
Findings:
- Conservative neurosurgical management, including serial imaging and close observation, was employed.
- Successful stent placement in the superior vena cava resolved the SVC syndrome.
- Head growth stabilized without the need for cerebrospinal fluid diversion, such as ventriculoperitoneal shunting.
Implications:
- This case highlights a conservative approach to neonatal macrocephaly secondary to cardiac issues.
- Effective management of the underlying cardiovascular problem can resolve SVC syndrome-induced macrocephaly.
- It suggests that cerebrospinal fluid diversion may not always be necessary for such cases.
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