Orbital Hemangioma with Intracranial Vascular Anomalies and Hemangiomas: A New Presentation of PHACE Syndrome?
Pediatr Dermatol
; 32(6): e267-72, 2015.
Article
in En
| MEDLINE
| ID: mdl-26446288
ABSTRACT
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 IHs 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 IHs should undergo evaluation for anomalies associated with PHACE syndrome. Patients with orbital IHs and an additional major criterion for PHACE syndrome should be considered to have definite, and not just possible, PHACE syndrome.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Aortic Coarctation
/
Orbital Neoplasms
/
Eye Abnormalities
/
Neurocutaneous Syndromes
/
Hemangioma
Type of study:
Diagnostic_studies
Limits:
Female
/
Humans
/
Infant
Language:
En
Journal:
Pediatr Dermatol
Year:
2015
Document type:
Article