Recognize & Refer: Hemangiomas in pediatrics

Sheila Fallon Friedlander, MD

This month, Contemporary Pediatrics focuses on Dermatology-to be specific, pediatric hemangiomas-and talks with Sheila Fallon Friedlander, MD, professor of Dermatology and Pediatrics at the University of California-San Diego School of Medicine (UCSD) and former Director of the Dermatology Fellowship Training Program at Rady Children’s Hospital-San Diego. Dr. Friedlander trained at the University of Chicago and the University of California, Los Angeles (UCLA) Medical Center and is board certified in Pediatrics and Dermatology, with subspecialty training in Infectious Diseases. She is a member of the Academy of Clinician Scholars at UCSD, past-president of the Society for Pediatric Dermatology, and an elected member of the American Academy of Dermatology Board of Directors. Dr Friedlander says that some older views about pediatric hemangiomas are outdated in the light of just-published guidelines that will help pediatricians quickly identify these lesions in children and begin what could possibly be life-saving intervention.

Q. Dr. Friedlander, what is the one key dermatologic condition or disease state that you believe community pediatricians should be especially vigilant about, either because it’s trending upward in severity or frequency or is being missed or underdiagnosed?

A. I think that infantile hemangiomas pose a challenge for clinicians. They are sometimes very subtle and oftentimes don’t need to be treated, but a significant minority can be functionally and/or life-threatening for patients. They occur in about 5% of newborns, so this is something that pediatric clinicians see a lot and therefore can be challenged by what’s the way to approach them and how important are they. I think that we have an increased amount of knowledge in the last few years in terms of how to diagnose them, when they’re a problem, and what is the best plan in terms of treating them.

Q. Are there some key misconceptions or data gaps that you believe some pediatricians may hold about pediatric hemangiomas?

A. Yes, and having been a pediatrician before I was a dermatologist I remember clearly that we were incorrectly taught that hemangiomas generally are nothing to worry about-they’ll grow and then they’ll go away without any sequelae. However, we now know that even the smallest hemangiomas can leave parchment-like or sometimes disfiguring scars, so that even though they didn’t obstruct vision, they didn’t risk this baby’s life, they may leave this child with a permanent mark. We now have treatments that can help decrease the chance of leaving a significant mark for these kids. More important, new therapies can prevent functional or life-threatening complications.

Q. What are some of the subtle signs or diagnostic tip-offs and clues community pediatricians need to know and at what point should they refer a patient to a specialist?

A. That is such a good question. In the last few months, actually in January of this year, a “Clinical Practice Guideline for the Management of Infantile Hemangiomas” by Daniel Krowchuk and colleagues¹ was published in Pediatrics, which goes over that very issue. What are the signs we look for? Which are the ones that we have to worry about? We know that some are known to be higher risk, long term, and we need to look for those.

Which ones are high risk? Sometimes they’re pretty subtle. Sometimes just more than 5 little hemangiomas anywhere on the body can be a concern because those can be associated with liver hemangiomas, internal hemangiomas, and can be a problem long term for the baby. So, any child who has 5 or more hemangiomas, even if they look tiny-and often they are very tiny in this particular subset disorder-those children need to be evaluated, and often we get an ultrasound of the liver.

The other thing that presents subtly is the child has had some little hemangiomas on the face in what we call the beard area around the chin, the neck, right in front of the ears, and sometimes those hemangiomas are quite small and seem inconsequential, while other times they are more apparent. They don’t look that big or worrisome, but they can be associated with airway hemangiomas that can be a big problem for those kids. Often the mom will come in and say: “Well, my son has had these little spots. I wasn’t worried about them, but he gets croup and he’s had croup 3 or 4 times in the last 6 months, but when he gets steroids he gets better.” Well, the astute clinician will know that the croup that baby is getting is actually an internal hemangioma blocking his airway-1 or more growing in the airway. Every time that child gets steroids, it shrinks those lesions and therefore he does better, but the hemangiomas will keep growing every time the steroids are stopped.

How would I know that looking at that baby? Those sometimes inconspicuous hemangiomas that are in the beard distribution, in front of the ears, along the neck, along the chin line are other subtle lesions that often portend a more significant problem. Any lesion on the face we need to take seriously, no matter how small it is, because it can leave a mark that may be worrisome to the family, even if they never caused a medical issue.

Also concerning are hemangiomas around the eyes or nose or mouth. A small hemangioma around the eyes can enlarge significantly over a 3- to 6-month period and can obstruct vision, and sometimes, unfortunately, leave children with permanent visual disabilities because the hemangiomas are obstructing vision at a very important time of visual development.

Again, this Clinical Practice Guideline actually lays out the higher-risk areas and the higher-risk lesions, as I’ve talked about: Lesions on the beard area are perhaps related to underlying airway problems; multiple lesions on the body might be related to liver hemangiomas; any hemangiomas that could obstruct vision or could cause a problem with eating or distorting the nose or lips are a potential issue, as are those in the genital areas. If you have a hemangioma in the genital area or the perianal area, it often will erode from friction and cause a significant degree of pain for the baby.

One of the main goals for the guideline is to recognize these high-risk lesions and get them in for appropriate evaluation and treatment. There’s a window, in the first 1 to 3 months, that if you get in there and intervene you can prevent these lesions from getting bigger and becoming more of a problem.

Q. When hemangiomas do need to be treated, what are some of the treatment guidelines for which pediatricians should be aware?

A. Again, this guideline lists the most effective treatment that we’re now clear about, and it shows a chart delineating that propranolol is the treatment of choice for worrisome hemangiomas. That doesn’t mean that every child who comes in to your office with a hemangioma gets propranolol, but for those lesions where you are significantly concerned about an untoward outcome, propranolol is the drug of choice at a dose of 2 mg to 3 mg per kilogram.

Now there are some concerns with propranolol. Kids who are on this drug need to be fed. If they do not eat or absorb food for a prolonged period, they can become hypoglycemic. In addition, because propranolol is a beta-blocker, you don’t want to put a child with bad asthma or reactive airway disease on the drug because it may make that worse. Certainly you want to know that the patient doesn’t have cardiac problems or any significant central nervous system (CNS) or cranial vascular problems, but often if the cardiac, blood pressure, pulmonary, and general exam are within normal limits, those kids will do very well on propranolol.

We do, however, always warn the families that if your child stops eating, has diarrhea and vomiting, is sick, or if is there anything untoward going on with his health, then we want you to withhold the treatment. However, other than that, most kids will do absolutely fine, even though one would think propranolol is going to drop their blood pressure. The effect propranolol has on blood pressure in a healthy child is minimal, and in a healthy child on the right dose it doesn’t usually lead to any significant problems.

One serious issue, though, is that as soon as this baby starts to get better and his hemangioma shrinks, the families want to discontinue the drug. They’re so happy he started it and they’re so excited about stopping it. Yet we know it’s extremely important that the family understands that if we want to prevent recurrence or rebound growth of the hemangioma, that child needs to be on medication for a minimum of 6 months and most often up to 10 to 12 months. So I tell my patients, your child is going to be on this drug probably for at least 10 months. If you tell them out of the gate that that’s the case, they’re much better able to deal with the long duration of treatment. Pediatricians can handle much of this follow-up, and we dermatologists can work with our primary care docs in following up on these kids so that it’s a shared responsibility during their treatment.

Q. Any other aspects of aftercare that community pediatricians should know about?

A. I was just thinking that one other treatment we use a lot is topical timolol. The 0.5% gel-forming solution is a beta-blocker as well and works very similarly to propranolol, but it has the advantage of being a topical formulation. You don’t have to give it by mouth, and therefore, when used appropriately, the systemic exposure is less than that of oral propranolol. We have used this for hemangiomas that aren’t really that deep or problematic, and it’s very useful. It appears to help short-circuit the growth cycle so that it will often lead to lightening of the lesion and sort of an abrupt arrest in growth. Most experts don’t want to use more than 1 to 2 drops twice a day, but it appears to be a very safe drug when used in the right amount on children. It is important, though, to monitor response, because if it is ineffective, propranolol may need to be initiated.

Timolol is something that pediatricians can feel comfortable with. I do think that if a child comes in to the office and has a small lesion on the forehead, for example, and the family’s concerned, you know that the lesion is not going to interfere with vision but it’s a cosmetic issue for them. For a thinner lesion, in the first month or 2 of life, start the topical timolol-1 or 2 drops twice a day-and it really can hold things at bay. Primary care providers can feel comfortable with this plan if they monitor response to the treatment. If there is an issue or if the lesion starts to grow, they can involve the dermatologist at that point.

The other thing is, with any lesion we need to observe and have vigilance about it because these lesions can start out and look a little smaller but then get more of a life of their own in the first 3 to 4 months of life. Follow-up’s really important, and the pediatrician, dermatologist, or chronic care doc can all work together to take care of these families.

Q. Is there anything else that community pediatricians need to know?

A. I think that they need to remember that although the vast majority of these lesions will not cause a medical harm to the children who have them, they often can leave a scar that is a psychological issue, so for any hemangioma that’s in a cosmetically important area, and that includes the face and the hands and the neck, those kids are worthy of monitoring and therapeutic intervention. Many of them will respond to topical timolol treatment. If they have large, rapidly enlarging lesions, or for those that pose a significant risk in a problematic site, then they may need systemic propranolol. We now can stop the cycle and make a big difference in the eventual outcome medically and often cosmetically for these kids.

References:

1. Krowchuk DP, Frieden IJ, Mancini AJ, et al; Subcommittee on the Management of infantile hemangiomas. Clinical Practice Guideline for the Management of Infantile hemangiomas. Pediatrics. 2019; 143(1):e20183475. Available at: https://pediatrics.aappublications.org/content/143/1/e20183475. Accessed June 25, 2019.