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Review
. 2016 Nov:178:24-33.e2.
doi: 10.1016/j.jpeds.2016.07.054. Epub 2016 Sep 19.

PHACE Syndrome: Consensus-Derived Diagnosis and Care Recommendations

Maria C Garzon  1 , Leon G Epstein  2 , Geoffrey L Heyer  3 , Peter C Frommelt  4 , Darren B Orbach  5 , Adriane L Baylis  6 , Francine Blei  7 , Patricia E Burrows  8 , Sarah L Chamlin  9 , Robert H Chun  10 , Christopher P Hess  11 , Shawna Joachim  12 , Katherine Johnson  13 , Wendy Kim  14 , Marilyn G Liang  15 , Mohit Maheshwari  8 , Garrett N McCoy  12 , Denise W Metry  16 , Priya A Monrad  17 , Elena Pope  18 , Julie Powell  19 , Tor A Shwayder  13 , Dawn H Siegel  12 , Megha M Tollefson  20 , Sudhakar Vadivelu  21 , Sean M Lew  22 , Ilona J Frieden  23 , Beth A Drolet  24
Affiliations
Review

PHACE Syndrome: Consensus-Derived Diagnosis and Care Recommendations

Maria C Garzon et al. J Pediatr. 2016 Nov.
No abstract available

Keywords: PHACE; PHACES syndrome; aortic coarctation; infantile hemangioma.

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Figures

Figure 1.
Figure 1.
Large segmental infantile hemangioma of the face in a 2-month-old girl with definite PHACE.
Figure 2.
Figure 2.
MRA of the brain (maximum intensity projection and three dimensional reformatted images obtained by time of flight Multiple Overlapping Thin Slab Acquisition technique) in a female child with definite PHACE demonstrating absence of the right internal carotid artery with reformation of the right middle and anterior cerebral arteries via circle of Willis collaterals (anterior and posterior communicating arteries).
Figure 3.
Figure 3.
Cardiac MRI of the aortic arch and brachiocephalic vessels in a patient with PHACE with coarctation of the aorta and long segment bizarrely twisted areas of narrowing in the transverse arch (large arrows). The right subclavian artery also has a “corkscrew” twist in the proximal segment (small arrow). The left ventricle (LV) and ascending aorta (AAo) are normal.
Figure 4.
Figure 4.
Example of 3-month-old female with midline defect of sternal cleft and abdominal raphe and definite PHACE.
Figure 5.
Figure 5.
Recommended health surveillance of patients with PHACE based on results of initial screening examinations.
See this image and copyright information in PMC

References

    1. Smart Tots. San Francisco: c2012–2015. http://www.smarttots.org. Accessed December 2015.
    1. Haggstrom AN, Garzon MC, Baselga E, Chamlin SL, Frieden IJ, Holland K, et al. Risk for PHACE syndrome in infants with large facial hemangiomas. Pediatrics 2010;126:e418–26. - PubMed
    1. Brandon K, Burrows P, Hess C, Metry D. Arteriovenous malformation: a rare manifestation of PHACE syndrome. Pediatr Dermatol 2011;28:180–4. - PubMed
    1. Chan YC, Eichenfield LF, Malchiodi J, Friedlander SF. Small facial haemangioma and supraumbilical raphe—a forme fruste of PHACES syndrome? Br J Dermatol 2005;153:1053–7. - PubMed
    1. Nabatian AS, Milgraum SS, Hess CP, Mancini AJ, Krol A, Frieden IJ. PHACE without face? Infantile hemangiomas of the upper body region with minimal or absent facial hemangiomas and associated structural malformations. Pediatr Dermatol 2011;28:235–41. - PubMed

Publication types

Supplementary concepts