I am excited to start the “PHACE/LUMBAR research” blog! The purpose of this blog is to periodically share interesting things with you all about PHACE and LUMBAR research that I’ve learned from meetings I’ve attended or come across in the medical literature. In full transparency, this is my very first blog EVER, I can't wait to read your comments and learn about other topics you are interested in learning about...here we go!
Earlier this summer there was a publication from the dental department at the University of California-San Francisco that reminded me about the association between PHACE and tooth problems. The authors described a 2-year-old boy with PHACE and a “beard” distribution hemangioma of his face and neck, who also had regional odontodysplasia or “ghost teeth”, a very rare condition that has not previously been reported in association with PHACE. Ghost teeth are soft, discolored, poorly formed and fragile, often leading to infections and pain. Other dental issues in association with PHACE have been reported before. From a survey done at the PHACE family meeting in 2012, Drs. Siegel and Drolet recognized that children with PHACE and intraoral hemangiomas often have abnormal tooth enamel, which makes their teeth more prone to cavities. Then in 2019 they reported 4 children with PHACE and malformed or missing roots in their permanent first molars, which can lead to problems like early tooth loss.
It has been suggested that the presence of a regional hemangioma, located in the mouth, maxillary segment (“S2”) or mandibular segment (“beard or S3”) may cause poor blood supply to the teeth, explaining these problems. The studies above are a good reminder that children with PHACE, especially those with an intraoral, S2 or S3 hemangioma, should be evaluated by a pediatric dentist by age 1 year. In addition, such children should have panoramic dental X-rays at the eruption of their first permanent tooth, which usually happens around age 6-7 years, to check for root abnormalities. These evaluations help in early detection and management of dental problems, preventing complications and ensuring better oral health outcomes for PHACE children.
Thanks for reading, will post again soon 😊
Dr. Metry
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