PHACE syndrome is a neurocutaneous disorder characterized by large cervicofacial infantile hemangiomas and associated\nanomalies: posterior fossa brain malformation, hemangioma, arterial cerebrovascular anomalies, coarctation of the aorta\nand cardiac defects, and eye/endocrine abnormalities of the brain. When ventral developmental defects (sternal clefting or\nsupraumbilical raphe) are present the condition is termed PHACE. In this report, we describe three PHACE cases that presented\nunique features (affecting one of the organ systems described for this syndrome) that have not been described previously. In the\nfirst case, a definitive PHACE association, the patient presented with an ipsilateral mesenteric lymphatic malformation, at the age\nof 14 years. In the second case, an anomaly of the posterior segment of the eye, not mentioned before in PHACE literature, a\nretinoblastoma, has been described. Specific chemotherapy avoided enucleation. And, in the third case, the child presented with\nan unusual midline frontal bone cleft, corresponding to Tessier 14 cleft. Two patients� hemangiomas responded well to propranolol\ntherapy.The first one was followed and treated in the pre-propranolol era and had amoderate response to corticoids and interferon.
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