Relationship between site of infantile hemangioma in maxillofacial region and the site of intracranial deformity or arterial abnormalities in PHACE syndrome

Abstract

Objective To provide reference for the diagnosis and prognosis by retrospectively studying the relationship between the site of infantile hemangioma in maxillofacial region and the site of intracranial deformity or arterial abnormalities in PHACE syndrome. Methods A total of 104 children diagnosed as infantile hemangioma in maxillofacial region with diameter>5 cm underwent systematic examination. Twenty-three children diagnosed as PHACE syndrome were grouped according to the location of facial hemangioma and nuclear magnetic resonance imaging results. The difference between population rate of the location of tumor and the location of intracranial deformity and arterial abnormalities was examined by the McNemar method. The consistency of the position distribution was examined by the Kappa. Results In 23 children diagnosed with PHACE syndrome, there were 19 cases (82.61%) with infantile hemangioma in maxillofacial region bombining intracranial deformity or arterial abnormalities on the same side of middle line. The consistency of the positions was checked by Kappa (k=0.649, P=0.002), population rates of the two diseases in left and right sides were same tested by McNemar (P=0.625). Intracranial deformity and arterial abnormalities were divided into three groups according to the sources of embryo for comparison: the site (in left or right side) of infantile hemangioma in maxillofacial region were compared with the site of carotid artery intracranial or vertebral artery abnormal, both sites were moderately consistent (k=0.534, P=0.024), the overall positive rate of the left and right sides was same (P=0.625), correlation coefficient: r=0.481, P=0.024; the site (in left or right side) of facial hemangioma were compared with the site of cerebellar structure abnormalities or dysplasia, both sites were in consistency (k=0.814, P=0.006), the overall positive rate of the left and right sides was same (P=1.000), correlation coefficient: r=0.638, P=0.006; the site (in left or right side) of infantile hemangioma in maxillofacial region were compared with the site (in left and right side) of aortic arch abnormalities or subclavian artery abnormalities, both sites were in consistency (k=0.615, P=0.035), the overall positive rate of the left and right side was same (P=0.500), correlation coefficient: r=0.556, P=0.035. Conclusions In PHACE syndrome, the locations of infantile hemangioma in maxillofacial region and intracranial deformity or arterial abnormalities are in the same side of midline. There is a consistency of high degree in position distributions and the same overall positive rate of the left and right sides, that they are closely correlated with each other. Key words: PHACE syndrome; Infant hemangioma; Intracranial deformity; Arterial abnormalities