Organ systems | Major criteria | Minor criteria |
Arterial anomalies | Anomaly of major cerebral or cervical arteries *† Dysplasia ‡ of the large cerebral arteries
| Aneurysm of any of the cerebral arteries |
Structural brain | Posterior fossa brain anomalies
| Midline brain anomalies Malformation of cortical development |
Cardiovascular | Aortic arch anomalies
| Ventricular septal defect Right aortic arch/double aortic arch Systemic venous anomalies |
Ocular | Posterior segment abnormalities
| Anterior segment abnormalities
|
Ventral/midline | Anomaly of the midline chest and abdomen
| Ectopic thyroid hypopituitarism Midline sternal papule/hamartoma |
Definite PHACE | ||
Haemangioma >5 cm in diameter of the head, including scalp plus one major criteria or two minor criteria† | Haemangioma of the neck, upper trunk or trunk and proximal upper extremity Plus two major criteria | |
Possible PHACE | ||
Haemangioma > 5 cm in diameter of the head including scalp plus one minor criteria | Haemangioma of the neck, upper trunk or trunk and proximal upper extremity Plus one major or two minor | No haemangioma Plus two major criteria |
*Internal carotid artery, middle cerebral artery, anterior cerebral artery, posterior cerebral artery or vertebrobasilar system
†Diagnostic criteria present in this patient
‡Includes kinking, looping, tortuosity and/or dolichoectasia
PHACE, posterior fossa malformations, hemangioma, arterial anomalies, coarctation of the aorta/cardiac defects and eye abnormalities.