PHACES syndrome with cataract and Horner’s syndrome: a case report

Phaces syndrome (posterior fossa malformations, hemangioma,arterial anomalies, cardiac defects and coarctation of theaorta, eye abnormalities, and sternal abnormalities or ventraldevelopmental defects) is a rare neurocutaneous syndrome,which characteristic feature is large segmental hemangioma.extracutaneous involvement is an important cause of morbidityin this syndrome.described below is an infant with large hemangiomatous lesionson the right side of the face, suprasternal notch, midline sternaldefect and supraumbilical raphe. based on the new consensus onthe diagnostic criteria of phaces syndrome, a definitive diagnosisof phaces syndrome has been corroborated. accordingly, ourpatient was analyzed with regards to other clinical features throughmagnetic resonance imaging of the head, neck and abdomen, allof which were normal. in echocardiography, the patient showedatrial septal defect, in addition to ipsilateral conductive hearingloss, contralateral cataract and horner’s syndrome, which wasreported only in very few cases. the clinical presentation of thepresent case was different from most previous reported ones, assegmental hemangioma in phaces syndrome was, for the mostpart, located on the left side of the face and ocular involvementwas predominantly reported ipsilateral to hemangiomatous lesion.