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Orbital Hemangioma with Intracranial Vascular Anomalies and Hemangiomas: A New Presentation of PHACE Syndrome?
Author(s) -
Antonov Nina K.,
SpenceShishido Allyson,
Marathe Kalyani S.,
Tlougan Brook,
Kazim Michael,
Sultan Sally,
Hess Christopher P.,
Morel Kimberly D.,
Frieden Ilona J.,
Garzon Maria C.
Publication year - 2015
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1111/pde.12695
Subject(s) - medicine , hemangioma , orbit (dynamics) , internal carotid artery , presentation (obstetrics) , radiology , engineering , aerospace engineering
We present two cases of infants with a similar constellation of clinical findings: retro‐orbital infantile hemangioma ( IH ), internal carotid artery ( ICA ) arteriopathy, and intracranial IH . In both cases, intracranial vascular anomalies and hemangiomas were found incidentally during evaluation of unilateral proptosis. Neither infant had evidence of cutaneous segmental IH of the face or neck, which might have provided a clue to the diagnosis of PHACE syndrome or of intracranial hemangiomas. In one case, intracranial involvement was particularly extensive and function threatening, with mass effect on the brain parenchyma. These cases serve to highlight the fact that clinical findings of proptosis, globe deviation, and strabismus should prompt immediate imaging to confirm the presence of orbital IH s and to exclude other diagnoses. Moreover, based on our cases and the embryologic origin of the orbit as a unique developmental unit, patients with confirmed retro‐orbital IH s should undergo evaluation for anomalies associated with PHACE syndrome. Patients with orbital IH s and an additional major criterion for PHACE syndrome should be considered to have definite, and not just possible, PHACE syndrome.