Infantile midline facial hemangioma with agenesis of the corpus callosum and sinus pericranii: Another face of the PHACE syndrome

In the majority of cases, infantile hemangiomas are not associated with any other abnormalities. Occasionally, they may indicate the presence of systemic malformations. PHACE syndrome includes the coexistence of hemangioma, posterior fossa brain abnormalities, arterial anomalies, coarctation of the...

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Bibliographic Details
Published in:Journal of the American Academy of Dermatology 2006-02, Vol.54 (2), p.348-352
Main Authors: Drosou, Anna, Benjamin, Latanya, Linfante, Italo, Mallin, Karen, Trowers, Andrea, Wakhloo, Ajay K., Thaller, Seth R., Schachner, Lawrence A.
Format: Article
Language:English
Subjects:
Abnormalities, Multiple
Agenesis of Corpus Callosum
Biological and medical sciences
Dermatology
Embolization, Therapeutic
Facial Neoplasms - therapy
Female
Glucocorticoids - therapeutic use
Hemangioma - therapy
Humans
Infant
Medical sciences
Prednisolone - therapeutic use
Sinus Pericranii
Syndrome
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Summary:In the majority of cases, infantile hemangiomas are not associated with any other abnormalities. Occasionally, they may indicate the presence of systemic malformations. PHACE syndrome includes the coexistence of hemangioma, posterior fossa brain abnormalities, arterial anomalies, coarctation of the aorta, cardiac defects, and eye abnormalities. We report a case of a 2-month-old female with PHACE syndrome who also had sinus pericranii. A 2-month-old girl was seen for a plaque-like, segmental, midfacial hemangioma, with recurrent hemorrhages, noted at birth. As part of the PHACE syndrome, she had a midline facial hemangioma, absent corpus callosum, hypoplastic internal carotid artery, and an abnormal tortuous dysplastic basilar artery. Digital subtraction angiography showed sinus pericranii. The patient underwent successful endovascular embolization of the hemangioma that prevented further bleeding. Sinus pericranii is a rare finding that has not, to our knowledge, been previously associated with PHACE syndrome. Central nervous system, not only posterior fossa, abnormalities are frequently encountered in PHACE syndrome. Endovascular embolization of the facial hemangioma in our patient was a useful therapy.
ISSN:0190-9622
1097-6787
DOI:10.1016/j.jaad.2005.05.037