Infantile Hemangiomas: Accurate Diagnosis Is Crucial

Randy Dotinga

March 15, 2021

The first rule about infantile hemangiomas: Make sure they're actually infantile hemangiomas, a pediatric dermatologist urged colleagues. Then watch patients closely, refer to specialists when appropriate, and consider propranolol in complicated or high-risk cases, Andrea L. Zaenglein, MD, said at MedscapeLive's Women's & Pediatric Dermatology Seminar.

"In my career as a pediatric dermatologist, propranolol has been a life changer for us more than any other medicine," said Zaenglein, professor of dermatology and pediatric dermatology, Penn State University, Hershey.

Before the point where propranolol is prescribed, confirm the diagnosis and use the correct terminology, she advised. It's still appropriate to use the International Society for the Study of Vascular Anomalies (ISSVA) vascular lesion classification system released in 1982. "For most people, it serves the purpose well," she said. Another option is an updated and more complex classification system from 2015.

Zaenglein highlighted two studies – one published in 2011 and the other published in 2020 – that revealed high levels of misclassification of vascular malformations in research reports. The earlier study found that 21% of patients with misclassified lesions were mistreated, compared with none of those who were classified using ISSVA terminology.

"I cannot stress [proper classification] enough when you're dealing with babies and children with vascular lesions. If not sure, be vague. Say 'a vascular tumor' or a 'vascular malformation.' But only reserve 'infantile hemangioma' for that very diagnosis," she said.

As Zaenglein noted, infantile hemangiomas affect 5%-10% of 1-year-olds, of whom 20% have multiple lesions. They're more common in females by a 3-to-1 margin, and also seen more in premature infants, and in cases of multiple births, higher maternal age, and low birth weight.

The pathogenesis of these lesions is unclear, she said, although there are hints about genetic components and tissue hypoxia, among other possible causes. "Importantly, you get 80% of the growth by 3-4 months of age. Then it'll slow in its growth and kind of slowly go away over time, but it's not linear regression. It's more that you get more improvement upfront, usually until about 5, and then you can get some continued gradual evolution up until about 7 or 10 years of age."

Complications can include ulceration, infection and – in rare cases – hemorrhage and high-output cardiac failure, she said. "Knowing which ones are at high risk for complications is important, and also there are systemic associations that we have to be mindful of. We also want to think about aesthetic outcomes as well when we talk about management of infantile hemangiomas."

High-risk infantile hemangiomas include those with the following features:

  • Extensive facial involvement. Zaenglein highlighted a case of a 2-year-old baby with a large, bulky hemangioma that distorted facial features around the eye. "This would be a medical emergency" requiring immediate evaluation and treatment, she said.

  • Periocular involvement. Refer to ophthalmology, she recommended. "Even smaller hemangiomas can cause refractive errors or amblyopia, and oftentimes need to be treated with either systemic or topical therapy depending on the size and extent," she said.

  • PHACE syndrome (Posterior fossa malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, eye abnormalities). "Propranolol has been safely used in PHACE, but every patient is different," she said. "You need to make sure to do a good risk assessment before starting because if they have narrowed blood flow or limited blood flow, there is a question of whether there is potential risk for stroke if you drop a baby's blood pressure. Make sure that the vasculature is evaluated before started on propranolol. Also, there are recent reports of risk of long-term risk of stroke with PHACE syndrome as patients are getting into their adulthood."

  • Beard distribution. Be aware of possible airway involvement that can be revealed by biphasic stridor. In those cases, immediate treatment – perhaps even with tracheostomy – is needed to avoid mortality, she said.

  • Multiple sites: Patients with five or more hemangiomas may have liver involvement, she said, and should undergo hepatic evaluation. Consider evaluating if this is suspected, even if the number of hemangiomas is under five, she said.

  • Perineal/lumbosacral involvement: A third of these cases are associated with spinal dysraphism. Refer to neurosurgery, she recommended.

Zaenglein highlighted a report on the use of propranolol published in 2008 and noted that clinical practice guidelines for managing infantile hemangiomas published in 2019 are also helpful.

Flat hemangiomas, meanwhile, can benefit from timolol maleate 0.5% solution or gel-forming solution – 1 drop twice daily or 2 drops once daily, she said. This treatment should be avoided in thick hemangiomas, she said.

This article originally appeared on MDedge.com, part of the Medscape Professional Network.

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