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Researchers concluded that patients with long-term PHACE face persistent, varying morbidities like infantile hemangioma (IH) remnants, headaches, learning challenges, and progressive arterial issues, necessitating continued primary and specialty care into adulthood.

The following is a summary of “Multicenter Study of Long-term Outcomes and Quality of Life in PHACE Syndrome after Age 10,” published in the January 2024 issue of Pediatrics by Braun et al.

Researchers conducted a retrospective study to evaluate the long-term outcomes of individuals with PHACE Syndrome, employing a multicenter approach involving cross-sectional interviews and chart reviews for those aged ≥10 years.

They encompassed data from various PHACE-related aspects, both medical charts and direct patient interactions. Utilizing likert scales, specific findings were assessed for their impact, while the Patient-Reported Outcomes Measurement Information System (PROMIS) scales were employed to evaluate quality-of-life domains. 

The results showed 153 individuals contacted, 104 (68%) participated, with a median age of 14 years (range 10-77 years). Notably, 94.1% exhibited infantile hemangioma (IH) residua, and nearly half had received laser treatment for residual IH, with the majority expressing satisfaction (89.5%) with the cosmetic outcome. Neurocognitive manifestations, such as headaches/migraines (72.1%), participant-reported learning differences (45.1%), and the need for individualized education plans (39.4%), were prevalent. Cerebrovascular arteriopathy was identified in 91.3% of participants, with 29.4% showing progression in follow-up imaging reports. Among these cases, 8.8% developed moyamoya vasculopathy or progressive stent occlusion, leading to isolated circulation at or above the level of the circle of Willis. Despite the high prevalence of cerebrovascular arteriopathy, the occurrence of ischemic stroke was relatively low (1.9% of 104). PROMIS global health scores indicated a deviation below population norms by at least 1 standard deviation. 

They concluded that long-term PHACE patients faced persistent, varying morbidities like IH remnants, headaches, learning challenges, and progressive arterial issues, necessitating continued primary and specialty care into adulthood.

Source: sciencedirect.com/science/article/abs/pii/S0022347624000106