PHACES syndrome, first described in 1978 and named in 1996, is a rare disorder involving multiple malformations, including abnormalities of the posterior fossa, cervicofacial segmental hemangiomas, cerebrovascular arterial

anomalies, cardiac or large vessel defects, ocular abnormalities and sternal defects such as the sternal cleft. It predominantly affects females, with a ratio of 9:1 and is seen in 2–3% of infantile hemangioma cases. Some patients may also present with thyroid disorders. The sternal cleft, one of the anomalies associated with PHACES syndrome, is a rare congenital malformation caused by a failure in the fusion of the sternal midline during embryonic development. It can be classified as partial or complete, with the superior partial cleft being the most common form. This defect although benign, can compromise the protection of mediastinal organs and can result in varying degrees of respiratory distress. Diagnosis is typically straightforward through inspection and palpation at birth. We present the case of a 10-month-old male patient with PHACES Syndrome diagnosed at 7 months after birth through genetic testing. Patient showed signs of hemangiomas in lower lip, right zygomatic region, and left nasal border at birth as well as evident malformation defect in anterior superior thorax characterized by a sternal cleft. Patient underwent adequate surgical alignment and approximation of sternum borders and closed with 2-0 wire points. Patient was discharged from pediatric intensive care unit 5 days after surgery and presented no complications throughout follow- up outpatient care. Congenital sternal cleft repair at an early age as in this patient is simple and with good functional and aesthetic results. When repair of the defect is deferred to an older age, more invasive procedures are required.

Keywords: Congenital malformations, PHACES Syndrome, Surgical repair, Sternal cleft