Phaces syndrome with intraoral and perioral hemangiomas: a different approach with Diode Laser

INTRODUCTION: PHACES is an acronym which refers to a syndrome charachterized by Posterior cranial fossa malformation, Hemangiomas of the head, Arterial abnormalities, Cardiac abnormalities, Eye abnormalities, Sternal clefting. The syndrome shows a female to male predilection of 9:1. There is no definitive evidence of a familial tendency although an X chromosome linked dominant single gene etiology has been suggested with lethality in males. The diagnosis of PHACES syndrome requires the presence of a segmental hemangioma >5 cm in diameter of the head plus 1 major criterion or 2 minor criteria. There are many treatment options, one of the mainstay therapies is laser photocoagulation. The aim of this work is to show the differential diode laser treatment of intraoral hemangiomas (IH) and perioral hemangiomas (PH) in patients with PHACES syndrome. METHODS: We report the cases of 4 patients (age median 21 years) affected by the syndrome, showing 26 IH and 15 PH. Diagnostic protocol consist on: appropriate clinical exam (highlighting prominent reddish purple plaque-like lesions on lips, tongue, cheek, palate, mouth floor), echocardiogram, ophtalmologic and endocrinologic exams, MRI of the head and MRA of head and neck. Patients were treated with multiple laser sessions. A different approach depending on the site of the lesions was used: repeated diode laser impulses in pulsed mode at the power of 14-20W and the wavelength of 800 nm for IH and lower power for PH, preceded by treated site cooling to avoid the tissue damage. RESULTS and CONCLUSIONS: IH totally healed after 1 or 2 sessions. In each session only a limited area of the PH was treated, obtaining a progressive improvement of the lesions. Both in IH and PH slight post-operatory pain, bleeding and edema was proved, with a low incidence of complications such as ulceration and super-infection. Diode laser has been proved to be very effective as non-invasive treatment for IH and PH in PHACES syndrome patients.