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Aortic arch repair in children with PHACE syndrome. J Thorac Cardiovasc Surg 2016 09;152(3):709-17 PMID: 27160940

Pubmed ID





BACKGROUND: PHACE syndrome is characterized by infantile hemangioma and developmental abnormalities of the brain, arteries of head and neck, and aortic arch.

METHODS: We retrospectively reviewed The PHACE Syndrome International Clinical Registry to identify children with PHACE who had operative repair of aortic arch obstruction at Children's Hospital of Wisconsin.

RESULTS: Seven patients (median 11 months, range 1 week-6 years) with PHACE required aortic arch reconstruction from 1996 to 2015. All needed complex surgical approaches (4 conduit grafts, 2 patch aortoplasties, 1 subclavian flap) to relieve the obstruction because of long-segment transverse and proximal descending aortic arch dysplasia that included multiple areas of stricture with adjacent aneurysmal dilatation. Aberrant origin of a subclavian artery was found in 6 of 7. The 3 children who had surgery after age 1 showed significant progression of the arch obstruction and/or adjacent aneurysmal segment dilatation after their initial infant evaluation. No deaths or perioperative complications occurred despite associated cerebrovascular arterial dysplasia in 5 of 7. Recurrent arch obstruction developed in 3 of 7 at an intermediate follow-up interval of 6.2 years (2 had interposition graft replacement at 8 and 11 years due to somatic growth; 1 had repeat patch aortoplasty 11 months after initial repair secondary to recurrent stenosis).

CONCLUSIONS: Extensive aortic arch reconstruction is commonly required in children with PHACE syndrome and coarctation due to the bizarre nature of the obstruction. Complete preoperative imaging is needed to fully characterize the aortic and cerebrovascular arterial anomalies. Recurrent obstruction is common given the non-native tissue techniques needed to relieve the arch anomaly.

Author List

Caragher SP, Scott JP, Siegel DH, Mitchell ME, Frommelt PC, Drolet BA


Peter C. Frommelt MD Professor in the Pediatrics department at Medical College of Wisconsin
Michael Edward Mitchell MD Professor in the Surgery department at Medical College of Wisconsin
John P. Scott MD Associate Professor in the Anesthesiology department at Medical College of Wisconsin
Dawn Siegel MD Associate Professor in the Dermatology department at Medical College of Wisconsin


2-s2.0-84975709026   6 Citations

MESH terms used to index this publication - Major topics in bold

Aorta, Thoracic
Aortic Coarctation
Child, Preschool
Eye Abnormalities
Infant, Newborn
Neurocutaneous Syndromes
Retrospective Studies
Treatment Outcome
Vascular Surgical Procedures
jenkins-FCD Prod-332 f92a19b0ec5e8e1eff783fac390ec127e367c2b5