Individualized approach for Coarctation of the Aorta in a Patient with PHACE syndrome and Extensive Hemangiomas

Individualized approach for Coarctation of the Aorta in a Patient with PHACE syndrome and Extensive Hemangiomas

Wejdan Ba-Atiyah, Riad Abou Zahr, Amjad Kouatli

Department of Pediatrics, Pediatric Cardiology section, King Faisal Specialist Hospital and Research Center, Jeddah, Saudi Arabia.

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PHACE syndrome is a neurocutaneous disorder with large facial segmental hemangiomas associated with anomalies of the brain, eye and heart.
We report a case of a 3 years old girl who presented at the age of 6 weeks with severe coarctation of the aorta and aortic arch aneurysm. In addition, she had extensive strawberry hemangiomas over the scalp, neck and chest which, after extensive investigation, led to the diagnosis of PHACE syndrome.
After complete evaluation we successfully initiated treatment with propranolol that was pursued without complications over three years with striking success and remarkable regression of her hemangiomas. Meanwhile the coarctation was managed conservatively.
This case highlights that propranolol treatment, while effective against hemangiomas, may contribute to slow the progression of aortic arch aneurysm and delay the need for high risk surgical intervention.

Keywords: PHACE syndrome, Propranolol, Coarctation of the aorta

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How to cite this article:

Wejdan Ba-Atiyah, Riad Abou Zahr, Amjad Kouatli. Individualized approach for Coarctation of the Aorta in a Patient with PHACE syndrome and Extensive Hemangiomas. International Journal of Case Reports, 2020 4:128. DOI: 10.28933/ijcr-2020-05-0105


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