In a new clinical practice guideline on the management of infantile hemangioma (IH), the American Academy of Pediatrics (AAP) recommends that infants at high risk for complications receive a referral to a specialist for evaluation and possible treatment by age 4 weeks.
"The traditional approach to infantile hemangiomas was very hands-off, since most begin to go away on their own without causing problems," Ilona Frieden, MD, FAAP, vice-chairperson of the multidisciplinary AAP subcommittee that spearheaded the new guidelines, said in a news release.
"But for some hemangiomas, waiting until they cause problems misses a critical window of opportunity for treatments that can prevent significant complications, such as permanent scarring, skin breakdown, or medical problems," Frieden added.
The guideline, by Daniel P. Krowchuk, MD, from the Departments of Pediatrics and Dermatology, Wake Forest School of Medicine, Winston-Salem, North Carolina, Frieden, and colleagues on the AAP Subcommittee on the Management of Infantile Hemangiomas, was published online December 24 in Pediatrics.
Caused by a rapid proliferation of endothelial cells, IHs — classified as superficial, deep, or mixed lesions — are the most common vascular tumors of infancy. The majority of these benign growths, which may be localized to a small area or involve one or more segments of the face and body, resolve without treatment or consequence. A small percentage cause functional impairment or are ulcerated or potentially disfiguring, the authors explain.
Early Treatment of High-Risk Lesions Is Important
IHs usually grow most quickly from ages 1 to 3 months and stop growing by approximately 5 months of age. This age range for growth was determined on the basis of an extensive evidence review and is earlier than previously believed, Krowchuk, chairperson of the clinical practice guideline subcommittee, said in the news release.
"This is why the AAP recommends treating problematic hemangiomas ideally by 1 month of age," Krowchuk said. "The goal is to keep them from getting any bigger during their period of rapid growth, or to make them shrink more quickly."
The AAP's first-ever Clinical Practice Guideline for the Management of Infantile Hemangioma outlines five indications for early treatment of potentially problematic IHs, including lesions that are
associated with life-threatening complications, such as airway obstruction, high-output congestive heart failure (associated with IHs on the liver), or profuse bleeding from ulceration;
potentially associated with functional impairment, such as vision disturbance resulting from the location of the lesion near the eyes, or feeding problems when they involve the lips or mouth;
ulcerated or at risk for ulceration, which can cause pain, bleeding, infection, and scarring. Ulceration occurs most commonly in infants younger than 4 months during the period of rapid IH proliferation. Ulceration is especially common in IHs that are of the superficial or mixed type, segmenta, and those located on the scalp, neck, perioral, perineal, perianal, or intertriginous sites;
potentially associated with underlying structural abnormalities, such as with PHACE syndrome, in which large IHs of the face, head, and/or neck are associated with one or more developmental defects of the eyes, heart, major arteries, and brain; and
disfiguring and could lead to permanent scarring, such as superficial strawberry-appearing lesions that can cause skin changes, and other lesions in visible locations on the head and neck that can distort anatomic landmarks, such as cartilage of the nose or ear or shape of the lip.
For hemangiomas that warrant evaluation and treatment, timing is important, the authors stress. "IH is a disease with a window of opportunity in which to intervene and prevent poorer outcomes, and this critical time frame for optimizing outcomes can be missed if there are delays in referral or treatment," they write.
"Even for the most experienced clinicians, it can be difficult to predict the degree of IH growth until several weeks to months after the lesion is first noticed. By that time, damage to the dermis and subcutaneous tissues as well as permanent distortion of important anatomic landmarks, such as the nose or lips, may already have occurred," the authors add.
Given this window of opportunity and because proliferation may occur early and be unpredictable, the consensus recommendation of the subcommittee is that consultation and/or referral to a hemangioma specialist should occur by 1 month of age in infants with high-risk lesions.
"Because the time to appointment with a hemangioma specialist may exceed the window of opportunity during which evaluation and possible treatment would be of maximum benefit, those who care for infants with IHs should have mechanisms in place to expedite such appointments, including the education of office staff to give young infants with high-risk IHs priority appointments," the authors explain. When timely in-person consultation is not feasible, telemedicine or other remote options should be employed to streamline triage, evaluation, and management.
Management of IHs should begin with risk stratification to identify high-risk lesions that require intervention. Imaging (ultrasound and possible MRI) should be undertaken only when the diagnosis is uncertain, when five or more cutaneous tumors are present, or when associated anatomic abnormalities are suspected.
When systemic therapy is indicated, oral propranolol 2 - 3 mg/kg/day is the recommended first-line agent. If there are contraindications or if the response to propranolol is inadequate, prednisone and prednisolone are second-line agents.
In some instances, intralesional injection of triamcinolone and/or betamethasone can be used to treat focal, "bulky" lesions in certain critical anatomic locations or during proliferation. Topical timolol may be prescribed for thin or superficial lesions.
Although surgery for hemangiomas is typically not performed in infants because of the associated risks, it may be considered in certain clinical situations, such as when lesions are ulcerated or obstruct or deform vital structures (eg, the airway or orbit). In such cases, and in situations in which the tumor involves "aesthetically sensitive areas," surgery may be indicated when drug treatment has not been optimally effective and when the lesion is well localized, the authors note.
A key component of IH management is parent and caregiver education about the natural history of tumors, as well as potential complications, the authors stress. When specialty consultation is warranted, clinicians should advise parents and caregivers of the potential difficulty of obtaining specialty consultation in a timely manner, so they can be prepared to advocate on behalf of the infant.
Implementing Guidelines May Be Difficult in Some Cases, Authors Say
The authors acknowledge some of the difficulties in implementing the new guidelines in practice — in particular, the variable nature of IHs and the challenge of predicting the clinical course in the absence of surrogate markers or imaging technologies that can reliably anticipate the growth course. "Hence, frequent in-person visits or a review of parental photos may be needed, especially in infants younger than 3 to 4 months," they write.
Another challenge is in dispelling the myth that these hemangiomas are benign and that they will always self-resolve and disappear. Although this is true in most cases, "there is ample evidence that false reassurance can be given even in high-risk cases; indeed, all hemangioma specialists have seen examples of lost opportunities to intervene and prevent poor outcomes because of lack of or delayed referral," the authors explain.
In addition, lack of access to specialty care can delay referrals or treatment. "Possible solutions could include establishing resources for the photographic triage of cases in which risk stratification is uncertain or in which triage to hasten referral can be augmented by this methodology," the authors note.
Dr Frieden reports an advisory relationship with Venthera/Bridge Bio. Coauthor Anthony M. Mancini, MD, has advisory board relationships with Verrica, Valeant, and Pfizer. The remaining authors have disclosed no relevant financial relationships.
Pediatrics. Published online December 24, 2018. Full text
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