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Review
. 2018 Jun;93(3):405-411.
doi: 10.1590/abd1806-4841.20187693.

PHACE syndrome: clinical manifestations, diagnostic criteria, and management

Anita Rotter  1 , Luciana Paula Samorano  1 , Maria Cecília Rivitti-Machado  1 , Zilda Najjar Prado Oliveira  1 , Bernardo Gontijo  2
Affiliations
Review

PHACE syndrome: clinical manifestations, diagnostic criteria, and management

Anita Rotter et al. An Bras Dermatol. 2018 Jun.

Abstract

Infantile hemangioma can be linked to other organ malformations. In 1996, PHACE syndrome was first defined as the association of large and segmental infantile hemangioma, usually on the face, head, or cervical region, with malformations of the posterior fossa of the brain, arterial anomalies of the central nervous system, coarctation of the aorta, cardiac defects, and ocular abnormalities. Over 300 cases of PHACE syndrome have been reported, and it is cconsidered one of the most common neurocutaneous vascular disorders in childhood. Knowledge of the features and locations of lesions that imply a greater risk of systemic involvement is crucial for the diagnosis and proper management of PHACE syndrome patients. This review highlights the diagnostic criteria for PHACE syndrome, the imaging workup for extracutaneous involvement, the treatment of infantile hemangioma, and the importance of a multidisciplinary approach in the management of these patients.

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Conflict of interest statement

Conflict of interest: None.

Figures

Figure 1
Figure 1
Infantile hemangioma on the frontotemporal segment
Figure 4
Figure 4
Infantile hemangioma on the mandibular segment
Figure 5
Figure 5
Brain MRI with a hemangioma on the right periorbital region
Figure 6
Figure 6
MRA showing stenosis and tortuous 1. right internal carotid artery and 2. right posterior cerebral artery
Figure 7
Figure 7
Brain MRI showing hypoplasia of the right cerebellum
Figure 2
Figure 2
Infantile hemangioma on the frontonasal and frontotemporal segments
Figure 3
Figure 3
Infantile hemangioma on the maxillary segment
See this image and copyright information in PMC

References

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MeSH terms

Supplementary concepts