| Organ systems | Major criteria | Minor criteria |
| Arterial anomalies | - Anomaly of major cerebral or cervical arteries*
- Dysplasia¶ of the large cerebral arteries
- Arterial stenosis or occlusion with or without moyamoya collaterals
- Absence or moderate-severe hypoplasia of the large cerebral and cervical arteries
- Aberrant origin or course of the large cerebral or cervical arteries except common arch variants such as bovine arch
- Persistent carotid-vertebrobasilar anastomosis (proatlantal segmental, hypoglossal, otic, and/or trigeminal arteries)
| - Aneurysm of any of the cerebral arteries
|
| Structural brain | - Posterior fossa brain anomalies
- Dandy-Walker complex
- Other hypoplasia/dysplasia of the mid and/or hind brain
| - Midline brain anomalies
- Malformation of cortical development
|
| Cardiovascular | - Aortic arch anomalies
- Coarctation of the aorta
- Dysplasia*
- Aneurysm
- Aberrant origin of the subclavian artery with or without a vascular ring
| - Ventricular septal defect
- Right aortic arch/double aortic arch
- Systemic venous anomalies
|
| Ocular | - Posterior segment abnormalities
- Persistent hyperplastic primary vitreous
- Persistent fetal vasculature
- Retinal vascular anomalies
- Morning glory disc anomaly
- Optic nerve hypoplasia
- Peripapillary staphyloma
| - Anterior segment abnormalities
- Microphthalmia
- Sclerocornea
- Coloboma
- Cataracts
|
| Ventral/midline | - Anomaly of the midline chest and abdomen
- Sternal defect
- Sternal pit
- Sternal cleft
- Supraumbilical raphe
| - Ectopic thyroid hypopituitarism
- Midline sternal papule/hamartoma
|
| |
| Definite PHACE | Possible PHACE |
- Hemangioma >5 cm in diameter of the head, including scalp
PLUS one major criteria or two minor criteria - Hemangioma of the neck, upper trunk or trunk, and proximal upper extremity
PLUS two major criteria | - Hemangioma >5 cm in diameter of the head, including scalp
PLUS one minor criteria - Hemangioma of the neck, upper trunk or trunk, and proximal upper extremity
PLUS one major criteria or two minor criteria - No hemangioma
PLUS two major criteria |
