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PEDIATRICS Vol. 113 No. 2 February 2004, pp. 412-415

EXPERIENCE AND REASON

Ipsilateral Hemangioma and Aortic Arch Anomalies in Posterior Fossa Malformations, Hemangiomas, Arterial Anomalies, Coarctation of the Aorta, and Cardiac Defects and Eye Abnormalities (PHACE) Anomaly: Report and Review

Gabriele Bronzetti, MD*, Alessandro Giardini, MD*, Annalisa Patrizi, MD{ddagger}, Daniela Prandstraller, MD*, Andrea Donti, MD*, Roberto Formigari, MD*, Marco Bonvicini, MD*, Fernando Maria Picchio, MD*

* Pediatric Cardiology
{ddagger} Institute of Dermatology, University of Bologna, Bologna, Italy

Posterior fossa malformations, hemangiomas, arterial anomalies, coarctation of the aorta, and cardiac defects and eye abnormalities (PHACE) is a rare congenital anomaly with a broad spectrum of clinical manifestations with a striking female predominance. We describe an infant with PHACE anomaly and aortic coarctation who underwent cardiac catheterization to clearly define the complex anatomy of the aortic lesion before surgical repair. Review of the literature documents a highly significant association between ipsilateral hemangiomas and cerebrovascular and aortic arch anomalies. We conclude that cardiac catheterization is required to define the complex anatomy of aortic lesions and should be performed in all patients with PHACE and evident aortic involvement to plan appropriate and safe surgical repair.

Key Words: PHACE • aortic arch • hemangioma • aortic coarctation • cardiac catheterization

Abbreviations: PHACE, posterior fossa malformations, hemangiomas, arterial anomalies, coarctation of the aorta, and cardiac defects and eye abnormalities

Received for publication May 29, 2003; Accepted Oct 29, 2003.


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