Conditions and Treatments
Our dedicated team of physicians specialize in the non-surgical and surgical treatment of hemangiomas and vascular anomalies. Combination therapy of medical treatment, surgical excision and laser therapy may be required depending on the severity of the anomaly.
WHAT ARE HEMANGIOMAS?
A hemangioma is a benign vascular tumor (growth) that may be present at birth (congenital) or appear shortly after birth (infantile). It may grow rapidly from a small pin sized red dot to a large mass (tumor) during the first few months of life. The tumors are composed of small blood vessels (capillaries) that may ulcerate causing pain or may even bleed. Most fade away to a fleshy colored fatty mass in later childhood (5-7yrs old) but often require surgical excision to improve the contour of the affected area.
The incidence of hemangioma is estimated between 1-2.5%, affecting girls over 2x more then boys. It is more common in premature infants and twins, with a direct correlation with low birth weight (<4 lbs). Former names given to hemangiomas were based on the foods they resembled: strawberry, cherry.
Congenital hemangiomas grow in utero and present at birth. Congenital hemangiomas are not succeptible to treatment with propanolol and often require surgical treatment. Congenital hemangiomas can often disrupt the normal anatomy do to their rapid growth. They must be differentiated from other vascular tumors such as Kaposiform hemangioendothelioma (KHE) and tufted angioma. Congential hemangiomas are often isolated (solitary) and located on the head or near joints. Workup typically includes an ultrasound examination and possibly a soft tissue biopsy. Congenital hemangiomas histologically are GLUT-1 negative, differentiating them from infantile hemangiomas.
NICH – Non Involuting Congenital Hemangioma
Non-involuting congenital hemangiomas are present at birth and may continue to grow or simply persist. These are unique in that they will not fade away without surgical intervention.
RICH – Rapidly Involuting Congenital Hemangioma
RICH grow in utero and then start fading away after birth (post-natal). The involution (fading) can occur up to one year of age. Surgical intervention may still be required if the involution leaves a tell-tale fibro-fat deposit in an anatomically sensitive area.
Infantile hemangiomas are noted after birth and begin as a small red mark on the body, face or head. A rapid growth phase then occurs where the hemangiomas can enlarge and cause obstruction or distortion of the surrounding areas and structures. The majority of the growth phase occurs during the first 3 months of life. The hemangioma growth then plateaus (up to 1-1.5yrs) and eventually enters an involution phase. During this involution phase, the hemangioma blood vessels slowly become replaced with a fibro-fatty tissue. The color will change from a bright red to a dull or gray color.
Infantile hemangiomas are receptive to treatment with B-blocker agents (such as propanolol). A biopsy of an infantile hemangioma shows the presence of a positive stain for the GLUT1 marker (a contradistinction from a congenital hemangioma which is GLUT1 negative).
Infantile hemangiomas can be superficial (on the top layer of skin) or deep (buried under the skin). Superifical hemangiomas have the characteristic red color and are what we classically consider a “typical” hemangioma. Deep hemangiomas are under the skin and produce a bump that is often dark or blue in color. An ultrasound exam can help differentiate this from other masses, such as cysts.
Infantile hemangioma of the scalp – these may grow to large sizes when left untreated.
An involuted hemangiomas is a hemangioma that has regressed. A common rule is 50% of hemangioma regress by age 5, and 70% by age 7, An involuted hemangioma often leaves a tell-tale sign of having had a previous hemangiomas present. The skin overlying it thins, and becomes crepe-like. There may discoloration of the skin. A soft, fleshy bump can remain in the area also causing disfigurement.
Many patients often present during this involution phase for corrective surgery to remove the “stigmata”, typically when present in an anatomically sensitive area (such as the face). The surgery involve removal of involved skin and fibrofat, and is conducted as an outpatient under local sedation or general anesthesia.
Before and after of surgical excision of an involuted hemangioma
A painful wound from hemangioma can occur when the tumor outgrows its blood supply and ulcerates. This can cause bleeding and is very painful for your child. Treatment can consist of topical lidocaine patches as well as laser or an earlier surgical intervention, in an attempt to reduce the size of the hemangioma and treat the symptoms.
Example of Ulcerated Hemangioma
Hemangiomas of the scalp are unique given the tension of the tissue overlying the scalp. If you feel the top of your own scalp, there is not much laxity of the skin. Younger babies however (<3mos) have significant laxity, facilitating the possibility of a direct excision and closure of a scalp defect. If your baby has a large scalp hemangioma, we often suggest surgical excision at an earlier age.
The galea aponeurosis (layer between the skin and the bone) acts a natural barrier to how deep the hemangioma can progress. The sub-galeal plane is a perfect surgical plane because the dissection is blood-less. Scalp hemangiomas can therefore safely be removed at a young age without fear of significant blood loss. Large scalp hemangiomas are therefore removed surgically around 3 months of age.
Hemangioma of the scalp removed in the office under local anesthesia
Hemangiomas on the face can be unsightly and potentially ulcerate and bleed. When hemangiomas involve the eyelid they can cause obstruction and potential deformations of the cornea. Larger hemangiomas may require excision in stages to prevent non-optimal scarring.
Eyelid hemangiomas can potentially cause significant visual impairment. They can deform the cornea (amylopia) leading to decreased visual acuity. If the eye is completely obstructed it can lead to cortical blindness (the brain does not develop the visual center due to decreased stimulation of that area during development). Eyelid hemangiomas therefore must be addressed very early to prevent these complications.
Hemangiomas on the neck can be troublesome particularly due to positioning. They can ulcerate and bleed, sometimes from frequent motion of the neck. In addition, an evaluation of the airway is important, as sometimes there may be hemangiomas deeper inside the neck as well.
Hemangiomas on the windpipe (trachea) or vocal cords (glottis) can present with noisy breathing (stridor) or snoring. If they grow or bleed they can cause significant airway obstruction and an emergency situation. A pediatric otolaryngology (ENT) consult is paramount for proper workup and surveillance of tracheal/glottal hemangioma. Medical treatment with propanolol has replaced our previous gold standard of steroids and laser therapy for treatment of tracheal and glottal hemangiomas. Symptom improvement can be appreciated in as little as two days in some reports.
Hemangiomas can present anywhere along the body and are often amenable to surgical treatment without significant scarring. Often these may be simply monitored and allowed to regress (involute) since they are often not visible while wearing clothing.
Body hemangiomas often can be easily surgically removed in the office or under sedation.
Hemangiomas of the arms and legs may be painful while wearing clothes and particularly when they ulcerate. Hemangiomas in areas such as the foot, may require earlier excision to prevent problems related to dependent positioning and walking.
Medical treatment may involve a medication called propanolol to induce regression in areas where surgical treatment is not optimal.
Laser treatment can also be used but since it only penetrates about 1mm, it is not optimal for thicker tumors.
Removal of all, or part of the hemangioma is the goal of surgery. Surgery is indicated for hemangiomas that may bleed or are painful, are unsightly, or that are causing deformation of neighboring normal anatomic structures. Surgery is typically performed in an ambulatory (outpatient setting). Surgery is covered by most insurances.
MEDICAL TREAMENT: PROPANOLOL
Initially used to treat cardiac problems (pulmonary hypertension) an amazing side effect was noted, the shrinking of hemangiomas. Propanolol was developed 50 years ago as a non-selective B-blocker. It is now considered the gold standard for the 1st line treatment of complicated hemangiomas. The effect of the treatment can be seen soon after drug initiation (less vascular appearing, decrease in size or swelling). Propanolol treatment for hemangioma is typically maintained at a dose of 3mg/kg (after dose titration)
Previously, a hospital admission for administering the drug (2-3 days) in newborns was required for monitoring, but we have slowly moved towards outpatient therapy with excellent results.
PROPANOLOL SIDE EFFECTS
Propanolol is safe drug, but during the titration phase (first week) potential side effects must be monitored. Propanolol can cause bradycardia (slow heart rate), hypotension (low blood pressure), hypoglycemia (low blood sugar), bronchospasm (can exacerbate asthma). Prior and during initiation of propanolol, serial EKG exams are performed. In addition, blood sugar may be checked using a finger stick.
Early studies of propanolol use in treatment for hemangioma do not show any risk of growth or impairment or developmental risk.
PROPANOLOL TEAM PROTOCOL
<3 mos of age
Consider inpatient admission for serial EKG, blood sugar test
Pediatric cardiology consult, check blood sugar, EKG
Initiate Propanolol 1mg / kg /day bid
Increase 0.5mg /kg /every other day up to 3 mg/kg/day
Initiate Propanolol 1mg / kg /day bid
Increase 0.5mg /kg /every other day up to 3 mg/kg/day
Treatment time – 10-11 months
WHEN TO OPERATE?
One of the most common questions parents have, after a decision for surgery is made, is the timing of surgery. The decision on when to operate on hemangiomas is based on several factors. The following questions must be taken into account?
1) Is there a danger to waiting?
When present around in or around areas that can cause significant functional impairment or restriction, earlier surgical intervention must be strongly considered. As mentioned previously, eyelid and airway hemangiomas can produce significant impairment in vision and in breathing. These two locations are of paramount importance in both monitoring treatment and may often require early surgical intervention.
Is your child experiencing significant pain or discomfort from the hemangioma? Typically hemangiomas do not cause significant pain unless they ulcerate (form open wounds). Bleeding can also be a significant problem and may require frequent trips to the emergency room. Surgery may offer symptom reduction by shrinking the size of the hemangioma and by inducing scarring/regression.
3) What is the Risk vs the Reward?
Surgical excision is an exchange of tumor for scar. The resulting scar is often linear, pale and thin. The hemangioma is vascular, can be raised, and wide. If the hemangioma can be removed without significant scar burden or bleeding, the benefits likely outweigh any risks of surgical intervention. The surgery itself is usually short, safe, may require general anesthesia, and is performed as an outpatient.
4) What are the psychological implications in waiting?
Pediatricians often instruct patients to wait for the hemangioma to disappear. When located on an antomically sensitive or visible area this can cause significant psychological distress to the young developing mind. Body self image develops around the time children start entering school. Teasing and low self esteem may result and should be factored in to our decision making process on when to surgically intervene.
Newborn hemangiomas can often be simply excised under straight local anesthesia in the office safely
When the hemangioma is too large to be removed in a single surgery without creating distortion, the operations may be staged to allow gradual removal of the hemangioma. The resulting scar is often placed within the hemangioma and the thickness of the tumor can be reduced to improve the overall aesthetic quality of the affected area.
Involves reducing the hemangioma to better camouflage it within the anatomic area, ie. the lip. Incisions are typically made within the hemangoma itself and the tumor is thinned out to provide better contour. Interestingly, in situations where not all the hemangioma can be surgically removed, we have appreciated that surgery causes residual hemangioma to regress.
Lasers targeting the hemoglobin molecule (585nm) allow for selective destruction of the hemangioma. These are known as pulsed dye light lasers (PDLs). The laser can only penetrate 1mm deep and so lasers are typically used as a secondary procedure to surgery. Laser may also aid in ulcerated hemangiomas, by causing the outer layer of the hemangioma to regress and thus heal/scar.
COMBINED MEDICAL AND SURGICAL
Complex hemangiomas are often treated with a combination of medical and surgical therapy. Propanolol is initiated as a first line treatment to reduce the size and growth of the hemangioma . Surgery is then performed to remove the hemangioma or improve the physical appearance after propanolol therapy is exhausted (at least 1 year).