We noted enlargement of the internal auditory canal in several of our patients with posterior fossa malformations, hemangiomas, arterial anomalies, cardiac defects, eye abnormalities, and sternal or supraumbilical defects (PHACES) association and hence evaluated children with PHACES for the presence of an enlarged internal auditory canal and potential associated findings, including infantile he...

PHACES Syndrome: From the Brain to the Face via the Neural Crest Cells As the neural tube closes, a set of specialized cells develops along its dorsal crests. Under the influence of specific proteins (RhoB, Slug, and N-cadherin), these cells detach from the neural crests just before the closure of the neural tube and begin to migrate extensively and populate 3 main regions: trunk, heart, and cr...

BACKGROUND Sclerocornea, a congenital corneal pathology characterized by bilateral scleralization of the cornea, which can be found in few cases with posterior fossa malformationshemangiomas-arterial anomalies-cardiac defects-eye abnormalities-sternal cleft and supraumbilical raphe (PHACES) syndrome. Presence of vascularization in peripheral cornea and smaller diameter of recipient cornea corre...

Abstract Infantile hemangiomas (IHs) are common vascular tumors. In most cases, a benign course with favorable outcome can be anticipated. IH typically present as cutaneous lesions either localized or diffuse segmental distribution. Segmental in the face may associated brain and cardiac anomalies (PHACES syndrome), whereas airway involvement has been reported to “beard” area. Multiple visceral ...

BACKGROUND AND PURPOSE We present neuroradiologic findings in 17 patients with posterior fossa malformations, hemangiomas, arterial anomalies, cardiac defects, eye abnormalities, and sternal or ventral defects (PHACES) association and identify those at highest risk of central nervous system (CNS) structural, cerebrovascular, and neurodevelopmental abnormalities. MATERIALS AND METHODS Patients...

This article was accepted: 15 February 2013 Corresponding Author: Su Yuen Ng, Institut Pediatrik, Paediatrics, Hospital Kuala Lumpur, Jalan Pahang, Kuala Lumpur, Wilayah Persekutuan 50586, Malaysia Email: [email protected] We report a case of a 29 day old infant who was referred for evaluation of a vision threatening extensive segmental facial haemangioma. She developed a small reddish patch o...