Pearls:

Obstruction of function (vital or otherwise), disfigurement or ulceration are indications for treatment. Treatment is typically with beta blockers either topical or systemic.

Five or more hemangiomas anywhere on the body, a large facial segmental hemangioma, a large lumbosacral segmental hemangioma or a beard distribution segmental hemangioma require further workup.

The typical course for a superficial infantile hemangioma is for appearance in the first few weeks of life, proliferation to three to four months of age, a plateau phase and then involution starting in the first year of life. Involution does not lead to complete resolution and there may be residual findings.

How can we correctly identify vascular birthmarks in children? Many traditional terms such as nevus flammeus, stork bite, angel kiss are not precise and can lead to confusion.

Vascular anomalies are broadly separated into two categories tumors and malformations by the International Society for the Study of Vascular Anomalies(ISSVA). These two categories are malformations and tumors.

What is that a nevus flammeus, the birthmark we can see in Sturge–Weber syndrome(SWS)? This is a capillary malformation that is also called a port wine stain. This can be located anywhere on the body but unlike nevus simplex (below) they are often unilateral and are sharply demarcated. Certain facial distributions can be associated with Sturge-Weber syndrome. These lesions will grow in proportion to the patient and may become thicker and darker in color with time.

Traditionally and trigeminal distribution was associated with SWS but more recent data suggests a forehead distribution is more predictive. This aligns with cells developing from the same part of the neural crest that develops into the forebrain and eye.

Waelchli R et al. New vascular classification of port-wine stains: improving prediction of Sturge-Weber risk. Br J Dermatol. 2014 Oct;171(4):861-7. doi: 10.1111/bjd.13203. Epub 2014 Oct1. PMID 24976116

What about angel kisses, salmon patch and stork bites? These are called nevus simplex. These are not true malformations but are persistance of fetal circulation. They can be differentiated from true capillary malformations based on appearance and location. They are very light red in appearance, and poorly defined. The are typically distributed on the glabella, the medial eyelids, the dorsal nose, philtrum and occipital scalp. They can also be found midline on the lower back. These typically resolve with time though they can persist when located on the occiput or glabella.

What about infantile hemangiomas? These are vascular tumors. Unlike capillary malformations that are present at birth and remain for life these proliferate. These may not be present at birth or may have a premonitory mark such as a small red areas, a bruise like area, or a pale or vaso-constricted appearing spot. Usually within the first few weeks of life they will start to grow and become deeper in color.

Who gets infantile hemangiomas? Anybody can get them but risk factors for hemangiomas include being female, being white, and a low birth weight.

Once we make that diagnosis of an infantile hemangioma, what do we expect to happen now through the first year of life? How do we counsel parents on the new infantile hemangioma? Hemangiomas have a well-defined life course.

Usually within the first few weeks of life, that hemangioma becomes more apparent and begins to proliferate. That we call the proliferative phase. The length of that proliferative phase depends on the exact type of hemangioma.

For a superficial type hemangioma, what was once called a "Strawberry hemangioma," that proliferative phase usually lasts until three to four months. By three months, that hemangioma is nearly done growing. Recent studies have shown that the period of most rapid growth actually is prior to seven weeks of age.



Mixed lesions or deep lesions may not appear up until a little bit later, sometimes not until one or two months of age. They may have a little bit of a longer growth phase and may grow for six or more months.

After the proliferative phase they plateau for some time and sometime before the end of the first year of life they go into involution. Involution does not mean complete resolution as there is often residua such as fibro-fatty tissue, stretched skin or scars.

If I am calling a dermatologist about a hemangioma what information do they need?

1) what is the age of the patient?

2) is it a superficial type hemangioma, a mixed type, or a deep type? Those two pieces help determine more or less what phase in its lifecycle this particular hemangioma is.

3) where is that hemangioma located? That helps risk stratify that hemangioma in terms of its potential complications..

4) what is the distribution of that hemangioma? Is it a focal hemangioma? The one that looks like a coin shape, or that looks like just a one nodule or bump on the skin. Or is it segmental? This is a hemangioma that has a distribution that is almost like a territory. These are more plaque like.

5) is it multifocal? Meaning, are there multiple of these hemangiomas?

Which of these hemangiomas need further evaluation?

Five or more cutaneous hemangiomas is a risk factor for having a visceral hemangiomas, of which the most common is in the liver. The current recommendations are to screen within an abdominal ultrasound looking for hepatic hemangiomas in those who have five or more cutaneous hemangiomas of any size.

A large head and neck segmental hemangioma will need further workup. That is an indication for a workup for a syndrome called PHACE or PHACES syndrome. PHACE syndrome is a syndrome consisting of posterior fossa malformations, a segmental hemangioma that is five centimeters or more anywhere on the head and neck, arterial anomalies in the brain or neck, coarctation of the aorta, eye anomalies and sternal clefting or supraumbilical raphe. These are worked up with brain and neck MRI/MRA, ophthalmologic examination and an echocardiogram.

Sometimes patients who have PHACE syndrome are misdiagnosed as having a capillary malformation and worked up for Sturge-Weber. It is important to go back to that natural history of capillary malformation being stable in appearance and rapid growth of infantile hemangiomas.



Garzon MC, et al. PHACE Syndrome: Consensus-Derived Diagnosis and Care Recommendations. J Pediatr. 2016 Nov;178:24-33.e2. PMID: 27659028

A large segmental hemangioma on the midline lumbosacral area can be associated with a syndrome that is analogous to PHACE syndrome in a different area. This is called LUMBAR (Lower body infantile hemangioma, Urogenital anomalies and ulceration, Myelopathy, Bony deformities, Anorectal malformations and arterial anomalies, and Renal anomalies). These hemangiomas can be seen in association with spinal dysraphism, as well as anogenital malformations, as well as anomalies of the urogenital tract.

The final concerning distribution is a segmental facial hemangioma in a “beard” or mandibular distribution. These can be either unilateral or bilateral and is a cutaneous marker for airway involvement for which they need to be evaluated.

Does every hemangioma need treatment? While very effective treatments now exist for hemangiomas, it's still very important to keep in mind that the majority do not need treatment. Treatment is indicated for three broad categories.

One is impairment of function, whether that be vital or nonvital. For example, risk for impairment of vision in a periorbital hemangioma, risk for impairment of feeding in a perioral hemangioma and airway involvement. Even some, for example, large, bulky hemangiomas on the neck may place a patient at risk for positional torticollis.

The second major indication is risk for disfigurement. Hemangiomas regress but may not fully resolve. That is significant in an area like the face, where a permanent residua may leave a patient at risk for permanent disfigurement.

The final category is ulceration. Ulceration is the most common complication of hemangiomas and occurs in 15% of cases.

What is the typical treatment? For those hemangiomas that require treatment beta-blockers are most effective class of medicine. That is either in a form of timolol, which is an ophthalmic solution or oral propranolol.

Recent studies show that timolol is probably best reserved for extremely thin hemangiomas a millimeter or less. The prescription is for 0.5% gel-forming solution one drop twice a day to the affected area with a maximum recommended at the moment is two drops twice a day.

Systemic therapy is then reserved for lesions that require treatment, but are not good candidates for timolol. Most deep and mixed hemangiomas will require propranolol if they need to be treated.

Do we need to admit patients for monitoring when we initiate propranolol? Protocols for initiation and escalation of propranolol differs from center to center and from region to region. In most academic hospitals outpatient initiation of propranolol is considered for those who are somewhere between four to six weeks of age or older. There may be other mitigating factors such as prematurity, or a low weight.

For outpatient initiation or titration baseline, one hour and two hour vital signs are taken with the dose given in the office. The main focus is on heart rate.

Under what circumstances do we have to hold propranolol? The risk of hypoglycemia occurs generally in those who have been fasting. If a child for whatever reason is not tolerating feeds they should hold propranolol.

It should also be held for wheezing or bronchospasm. We instruct the parents that if the patient has an upper respiratory infection and appears to be having respiratory symptoms, we ask them to hold the propranolol until they've been checked by their pediatrician.

What should I tell the parents when they ask me about bleeding? Bleeding and ulceration are relatively common. Parents are very afraid of touching the hemangioma. They think it's going to be life-threatening bleeding. Bleeding occurs relatively frequently but that bleeding is rarely life-threatening. If there is bleeding the parents should hold pressure with one finger for 10 minutes without letting go.