PHACE association

ORPHA: 42775

Overview

cutaneous condition characterized by multiple congenital abnormalities. ("PHACES"=Posterior fossa malformations–hemangiomas–arterial anomalies–cardiac defects–eye abnormalities–sternal cleft and supraumbilical raphe syndrome)

Available Treatments (0)

No treatments linked yet

Clinical trials or compassionate use may be available — consult a specialist.

Clinical Presentation

Signs and symptoms associated with PHACE association, sourced from HPO and Orphanet clinical annotations.

Abnormal cerebral artery morphologyGlaucomaMicrophthalmiaOptic nerve hypoplasiaDandy-Walker malformationAbnormal heart morphologyAbnormal cardiac septum morphologyCerebral arteriovenous malformationMicrocephalyAbnormality of the orbital regionStrabismusPtosisCataractIris colobomaAmblyopiaSclerocorneaAbnormal sternum morphologyHypothyroidismHeterochromia iridisSeizureHypotoniaGlobal developmental delayAgenesis of corpus callosumCerebellar hypoplasiaTetralogy of FallotCoarctation of aortaAortic root aneurysmHemiplegia/hemiparesisCapillary hemangiomaAbnormal carotid artery morphologyDevelopmental retinal vascular malformationEctopic thyroidLens colobomaVisceral angiomatosis

Classification & Codes

Orphanet Code

ORPHA:42775
PHACE association
OrphanetORPHA:42775
Treatments0 drug(s)
Symptoms on record34 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO