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Clinicoradiological Characteristics and Outcome of Three Patients with PHACES Syndrome Associated with Intracranial
Naema Al Shibli1, Sheikha Al-Badi2, Eiman Al-Ajmi3
1Child Health Department, Sultan Qaboos University Hospital, University Medical City, Muscat, Oman.
Abstract:
PHACES syndrome is characterized by segmental infantile hemangiomas (IHs) accompanied by various extra-cutaneous anomalies, including cerebral artery anomalies, cardiac anomalies, ocular anomalies, sternal deformities, and posterior fossa malformations. This report presents three case series of patients with PHACES syndrome, focusing on challenges encountered in managing stroke risk associated with propranolol therapy due to significant cerebrovascular anomalies. In each case, therapeutic strategies were tailored to individual patients, carefully considering their vascular risk, and the location and potential consequences of the IH. Successful management hinged upon collaborative efforts involving a multidisciplinary team, particularly in resource-limited settings. This collaborative approach allowed pediatricians to make well-informed decisions regarding the use of oral propranolol in cases of IH with prominent cranial arterial anomalies, effectively balancing potential therapeutic benefits against the risk of stroke. Through the development of individualized treatment plans, guided by this collaborative approach, pediatricians can address each patient's unique needs and challenges. This report emphasizes the importance of personalized and comprehensive care for patients with PHACES syndrome, offering valuable insights for clinicians faced with similar cases.