PHACE syndrome in a child with segmental IH of face

Description

An 8-month-old female infant was referred for paediatric and dermatology consultation for evaluation of a facial haemangioma with progressive growth since the first days of life. The observation showed a large segmental infantile haemangioma (IH) over the right face (figure 1), a small vulvar haemangioma and exotropia of the right eye.

Figure 1

Right facial segmental infantile haemangioma at the level of the mandible, with extension to the lower lip.

Due to the segmental appearance of the facial IH, we prompted the investigation of PHACE syndrome, an acronym corresponding to a set of alterations: Posterior fossa anomalies, Haemangioma, Arterial lesions, Cardiac abnormalities/coarctation of the aorta alterations and Eye anomalies.1

The MR angiography showed an absence of the right internal carotid artery, with circulation assured through the circle of Willis (figure 2). Ophthalmology assessment revealed microphthalmia and...

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