Vascular lesion treatment options greatly improve

April 4, 2016

Advances in the diagnosis and treatment of vascular lesions are decreasing the morbidity and cosmetic disfigurement in some affected patients. Dermatologists should note that education and reassurance help parents understand the severity or non-severity of certain lesions and treatment protocols. Learn more

For parents, vascular lesions are among the most worrisome dermatologic conditions in children. Hemangiomas and related conditions raise concerns about a child’s appearance, and some vascular lesions pose serious health risks.

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The bad news: Infantile hemangiomas, the most common form of vascular lesion in kids, may be on the rise. A recent Journal of the American Academy of Dermatology article which tracked the incidence of infantile hemangiomas over a 10-year period in Minnesota found that the incidence was increasing in sync with an increase in prematurity in the overall population. The good news: Advances in treatment are making a huge difference, particularly on the hemangioma front.

“There are so many wonderful treatments now,” says pediatric dermatologist Sheila Fallon Friedlander, M.D., who spoke to colleagues during the recent Maui Derm 2016 meeting (Maui, Hawaii, January 2016). “The last decade has provided us with multiple new therapeutic options, which can decrease the morbidity and cosmetic disfigurement that may result in a subset of at-risk patients.” Dr. Friedlander is a professor of clinical dermatology and pediatrics at the University of California San Diego School of Medicine and pediatric dermatologist, Rady Children’s Hospital, San Diego.

Vascular lesions in children may seem like a topic of concern only for pediatric dermatologists. Indeed, that’s often the case in larger U.S. cities that are home to dermatologists specializing in kids. However, many parts of the country don’t have any easily accessible pediatric dermatologists, Dr. Friedlander says, and non-specialist dermatologists may find themselves frequently treating kids with these conditions.

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Not all vascular lesions require treatment. Spider angiomas are generally harmless. They are a common finding in kids, are “nothing to worry about”, and do not require treatment, Dr. Friedlander says. (While they’re often a sign of alcohol abuse and chronic liver disease in adults, a study found no link between spider angiomas and liver disease in kids.) 1

However, Dr. Friedlander says, some families choose to have them treated with laser.

Rarely, hereditary hemorrhagic telangectasia, a disorder associated with internal pulmonary and gastrointestinal (GI) vascular malformations, can present with small vascular lesions on the face, lips, tongue and fingertips. “I always look at the fingers, tongue and lips and ask about family history or personal history of nose or GI bleeds,” Dr. Friedlander says. “When I get a ‘no’ for those issues, I simply reassure the family about the ‘benign spiders.’”

NEXT: On the rise but mostly harmless

 

On rise but mostly harmless

Hemangiomas appear within the first three to six months of life. These bright red nodules, consisting of vascular vessels, will grow over time. Eventually their growth slows, and they regress, sometimes leaving a visible area of fibrofatty tissue or a parchment-like scar. While they’re usually harmless, it’s possible for hemangiomas to obstruct vital respiratory or visual function.

 Hemangiomas, which most commonly appear on the head and neck, are believed to occur in as many as one in 10 to 15 infants. A 2014 prospective study in the British Journal of Dermatology reports that hemangiomas appeared in about 5% of infants born to 578 women.2 Dr. Friedlander was one of the co-authors of the study, which tracked the women’s infants for nine months.

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Another study, this one published in 2016 in the Journal of the American Academy of Dermatology, suggests that hemangiomas are on the rise.3 Cases in a largely white county in Minnesota rose from 0.97 to 1.97 per 100 person-years between 1976 and 2010.

Prematurity seems to be a major factor.

“The average gestational age and weight of infants has decreased,” Dr. Friedlander says, “and we know that those two characteristics are linked to hemangiomas.”

Researchers have also linked hemangiomas to the female gender and to mothers with placental abnormalities.

NEXT: The power of touch in diagnosis

 

The power of touch in diagnosis

How can dermatologists differentiate between hemangiomas and other kinds of vascular lesions like port wine stains? Dr. Friedlander recommends using an extremely old-fashioned tool: your fingers.

“Facial hemangiomas may start out flat, but they will start to thicken over the subsequent four to eight weeks,” she says. “They’re more papular, and if you run your hand across them, you can feel them.”

By contrast, port wine stains stay flat, at least in the first few years of life. “If you run your hand across them,” she says, “you can’t feel them.”

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Since the form of lesions can change over time, Dr. Friedlander cautions dermatologists to defer a definitive diagnosis based on one visit in newborns if the lesion is flat. “We had a case of a two-week-old baby with a flat lesion. We assumed it was a port wine stain, and we told them to come back, but they never did. And it started to thicken over time. Appropriate therapy was thus delayed. You want to be able to watch these kids and never tell parents that ‘this is absolutely it, and you don’t need to return.’”

If you’re concerned about a hemangioma, she says, ultrasound evaluation and/or MRI can offer insight into whether it has characteristics suspicious for a vascular malformation rather than an infantile hemangioma, which is a generally benign proliferative tumor.

NEXT: Treating functional impairment

 

Treating functional impairment

If a hemangioma is causing functional impairment, the best treatment is the beta-blocker propranolol, says Dr. Friedlander. The drug appears to function in a variety of ways, including suppression of blood vessel growth by the induction of vascular cell death, she says. They typically cause significant change in coloration and shrinkage within a day or two.

“Propranolol has been used for decades in young children for cardiac disorders, which reassures us in terms of its relative safety,” she says. “But when it’s used, patients have to be monitored.”

Heart disease and respiratory problems like asthma in children should raise questions about whether use of propranolol is appropriate, she says. It’s also important to make sure children can eat on a regular basis throughout the day. “Propranolol can induce hypoglycemia,” she says. “We make sure the family is aware the young infant must eat every four to six hours to ensure that the blood sugar remains stable.”

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There’s interest in other beta blockers such as captropil or nadolol, which may have less side effects; because, they – unlike propranolol – don’t cross the blood brain barrier, she says.

For smaller lesions, a topical beta-blocker can be useful, she says, although it is not sufficient for deep, large and rapidly growing lesions.

“We frequently use twice-a-day timolol for smaller lesions; it is very useful particularly for peri-orbital lesions. As this drug has been used for treatment of glaucoma in infants for decades, there is not a significant concern if it smears into the eye. Although excessive application volumes could theoretically lead to systemic symptoms, that has not proven to occur with any frequency.”

NEXT: Treating vascular malformations

 

Treating vascular malformations

Vascular malformations are quite different than hemangiomas. They are malformations rather than proliferations, with aberrant growth of veins, arteries, or even lymphatic vessels. There can be a combination of vessel types present, Dr. Friedlander says. Unfortunately, unlike hemangiomas, these lesions do not resolve over time, and can enlarge, which may result in bleeding, interference with normal vital functions, and potentially life-threatening blood clots. she says. These lesions do not respond to propranolol.

Port wine stains-like the famous one on former Soviet Union leader Mikhail Gorbachev’s face - are perhaps the most commonly known form of vascular malformation, and more accurately referred to as capillary malformations. These represent a malformation of the most superficial capillary vessels and are occasionally associated with Sturge Weber Disease, particularly if the forehead and V1 area of the face are involved, she says. “If those areas are involved, the first job is to obtain an eye exam and refer to neurology concerning the possibility of imaging and neurological evaluation.”

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For the cutaneous lesion, she says, pulsed dye laser is first line of therapy. “For recalcitrant and recurrent lesions, some dermatologists are now utilizing other lasers and/or adding topical rapamycin to therapy to increase efficacy and decrease risk of recurrence.”

According to Dr. Friedlander, some of the most remarkable advances that have occurred in the last decade relate to our understanding of the pathogenesis of vascular malformations and associated syndromes. “It is now clear that cell molecular pathways involved in angiogenesis, vascular and cell proliferation are crucial in vascular homeostasis, and that activating or inhibiting mutations at any point in the pathway from cell membrane activation to nuclear transcription can lead to a variety of disorders,” she says. “We now have the ability to identify such mutations in patients who present with vascular or cellular proliferations of unclear etiology. This in turn has led to the utilization of new therapies, such as oral rapamycin, which is a potent inhibitor of the mTOR pathway.”

NEXT: Make sure to reassure parents

 

Make sure to reassure parents

Dr. Friedlander says it’s important to calm parents who worry about vascular lesions in their children.

“The internet has made everyone a partial expert and warned them of all the horrible possibilities, scaring them about mutations and rare disorders. One really important function of the dermatologist is to reassure when something is completely benign (an isolated spider angioma) and when nature is generally on our side (isolated infantile hemangiomas, which are not functionally or cosmetically deforming),” she says. “They need to know if they are looking at a short- or long-term problem, and they need to focus on long-term benefit for their child. It is reassuring to be able to explain the wide variety of therapies available for vascular lesions. Alternatively, one can really help a family out who has a child with a vascular malformation that can possible be identified with genotyping.”

Moving forward, she says, “we’re hoping for breakthroughs in treating malformations, including lymphatic and vascular malformations. Though they are the rarest, they are the most challenging to treat, and they can lead to signifiant morbidity and even mortality for some children.”

References

1. Finn SM, Rowland M, Lawlor F, et al. The significance of cutaneous spider naevi in children. Arch Dis Child 2006; 91(7):604-5.

2. Munden A, Butschek R, Tom WL, et al. Prospective study of infantile haemangiomas: incidence, clinical characteristics and association with placental anomalies. Br J Dermatol. 2014;170(4):907-13.

3. Anderson KR, Schoch JJ, Lohse CM, Hand JL, Davis DM, Tollefson MM. Increasing incidence of infantile hemangiomas (IH) over the past 35 years: Correlation with decreasing gestational age at birth and birth weight. J Am Acad Dermatol. 2016;74(1):120-6.