Hemangiomas, medical and surgical treatment

Hemangiomas

1-hemangioma-regression

Hemangiomas are the most common benign growth in infancy, occurring in 10% of full term babies and up to 25% of premature babies.  They represent excessive blood vessel growth, most obviously in the dermis layer of the skin, but they can occur in the fatty layer of the skin, and also deeper within the body such as in the liver.  For unclear reasons they occur 3 times more frequently in girls. 60% of hemangiomas occur in the head and neck area, but they can occur anywhere on or inside the body.

Diagnosis and work up

Most hemangiomas can be diagnosed by history and physical examination alone. In most cases the hemangioma is not present at birth but appears as a small red spot in the first couple weeks of life.  It then begins to grow faster than the child grows, often protruding above the surface of the skin.  Most hemangiomas will stop growing somewhere between 6-12 months of age and then begin to regress.  The bright red color becomes more pale, with grayish-white 2-compound-hemangiomapatches, and the hemangioma loses its engorged appearance (Fig 1).  Hemangiomas shrink very slowly, over an average of 5 years. Parents should realize that this means some hemangiomas will take longer than 5 years (even into the early teenage years) to fully shrink.

Hemangiomas in the fatty layer tend to appear more blue in pigmentation than red, and some hemangiomas will have both a central superficial red portion and a deeper bluer subcutaneous portion (Fig 2).

Hemangiomas that involve the eye region risk blockage of vision and possible permanent visual impairment, therefore these babies should be evaluated by a pediatric ophthalmologist. (Fig 3) Hemangiomas that occur below the vocal cords are potentially life-threatening because of airway blockage, and these should be immediately evaluated by a pediatric 3-periorbital-hemangiomaotolaryngologist.  Large facial hemangiomas warrant an MRI scan to rule possible related abnormalities of the brain, as well as a possible cardiac evaluation to rule out heart and great vessels abnormalities. Rarely, a sternal cleft can also occur. This constellation of findings as been given the name PHACES syndrome – Posterior cranial fossa abnormalities, Hemangioma, Arterial abnormalities, Cardiac abnormalities and coarctation of the aorta, Eye abnormalities, and Sternal cleft, but patients do not need to have every abnormality to be diagnosed with this syndrome.

Hemangiomas involving the central back and buttocks warrant an MRI scan to rule a possible spinal cord abnormality (lipomyelomeningocele with tethered cord), and other genital and urologic abnormalities should be checked.  There is another constellation of findings termed PELVIS syndrome – Perineal hemangioma, External genitalia deformities, Lipomyelomeningocele, Vesicorenal defects, Imperforate anus, and Skin tag, and again, patients need not have every abnormality to fulfill the diagnosis, but they should be ruled out.  (Fig 4)

Babies who have 6 or more external hemangiomas should 4-PELVIS-syndromehave an ultrasound to rule out possible liver or spleen hemangiomas.  Large liver hemangiomas may inactivate thyroid hormone, resulting in symptomatic hypothyroidism. Additionally large liver hemangiomas and any very large hemangioma elsewhere may put additional stress on the heart resulting in high output heart failure.

Lastly, there are always some exceptions to the rule – some hemangiomas shrink extremely rapidly within a year (RICH – rapidly involuting congenital hemangioma) (Fig 5) and some seem to never shrink, maintaining a bluish pigmentation with small superficial red vessels and a very warm temperature due to the increased blood flow . (NICH – non-involuting congenital hemangioma) (fig 6)

Actual biopsy is rarely indicated unless the diagnosis remains unclear.  On rare occasions other tumors can mimic hemangiomas, in which case a biopsy may be warranted. Classic hemangiomas of infancy will stain positive for Glucose transporter protein 1 (Glut-1).  Interestingly, neither RICH nor NICH hemangiomas stain positive for Glut-1.


5-RICH-hemangioma

6-NICH-hemangiomaMedical treatment of hemangiomas

The vast majority of hemangiomas can be treated conservatively with serial observation and parental reassurance. Hemangiomas will typically grow for 6-12 months, then reach a plateau and shrink slowly over an average of 5 years.  It has been estimated that up to 70% of hemangiomas will adequately regress and require no additional treatment.

However, that still leaves 30% of hemangiomas that can sometimes leave significant deformity, and all treatment methods should be discussed with the parents to avoid medicolegal issues whereby parents may feel a treatment option was withheld from them that might have prevented a permanent deformity.

Hemangiomas that threaten the airway or vision of the child cannot be followed conservatively, and medical intervention is indicated, usually in the form of steroid therapy.  In the majority of cases, this is given orally in the form of prednisolone 2-4 mg/kg/day mixed with the morning feeding.  Liquid Zantac is often prescribed simultaneously.  Once a response is detected, the steroids can be tapered slowly.  Some practitioners will cycle on and off steroids while others will start at a high dose, then slowly taper down, occasionally bumping the dose slightly and tapering more slowly if rebound growth is detected –  the dosage graph resembling a roller coaster ride. Babies on steroid therapy cannot receive live-attentuated virus vaccinations (rotavirus, measles, mumps, rubella, chicken pox) until the steroids have been discontinued for at least a month.

Steroids can also be injected into hemangiomas every 4-6 weeks using slowly absorbed preparations such as triamcinolone (Kenalog), however in the eye region, this should only be done by an ophthalmologist because of the potential risk of steroid particles travelling through the blood stream to the eye, causing possible blindness.

Topical steroid cream ( clobetasol propionate – Temovate) can be applied to relatively indolent hemangiomas, but the amount of steroid absorption is unpredictable, and therefore the oral route is preferred for hemangiomas that threaten vision, breathing, or significant permanent deformity.  The topical cream can be applied once a day in cyclical fashion, two weeks on, two weeks off for as long as is deemed necessary.

Laser therapy

7-laser-treatmentThe most commonly used laser for hemangiomas is the pulsed yellow dye laser. The laser light is absorbed by the blood inside the vessels, causing them to heat up, swell, and burst.  The light penetrates only about 1-2 mm into the skin, therefore the laser works best on small flat hemangiomas.  The effect is unpredictable.  Some hemangiomas seem to shrink and disappear with a single treatment.  Most hemangiomas will exhibit some initial shrinkage but then show rebound growth within 2-3 weeks, warranting a series of laser treatments until no further growth is noticeable. Some hemangiomas will show little or no benefit to laser treatment.  Large thick hemangiomas do not respond well as the light cannot penetrate very deeply into the tissues. Parents should be aware that the laser treatments are painful, although pre-treatment with a topical anesthetic cream and a coolant spray attached to the laser can reduce the discomfort of the laser sessions.

The pulsed yellow dye laser has two other uses in hemangiomas.  Hemangiomas that have ulcerated (the overlying skin breaks open) can be exquisitely sensitive, especially in the genital and buttock region.  The laser may help to decrease the pain significantly within 24-48 hours (probably by cauterizing sensitive nerve endings) and also speed the rate of healing (perhaps by causing temporary hemangioma suppression) in many patients.  Older hemangiomas that have shrunk but left residual spider vessels in the skin can be treated with the laser to reduce the size and number of vessels.  Often more than one treatment session is necessary for best results (Fig 7) .

Interferon and beta blockers

In the 1990’s interferon alfa-2a showed promising results in hemangioma suppression, however significant side effects have limited its use to life or vision-threatening hemangiomas that appear resistant to steroid therapy.

Very recently beta-blockers have been shown to significantly suppress large hemangiomas, although the exact mechanism remains unclear. The use of beta blockers currently requires initial ICU monitoring, and is generally restricted to significant steroid-resistant hemangiomas that are life or vision threatening, or likely to otherwise cause severe facial deformity. As more experience is gained with this therapy, therapeutic indications and protocols will evolve appropriately.

Surgery for hemangiomas

The role of surgery in the treatment of hemangiomas has evolved significantly.  Initially pediatricians were taught to follow the majority of hemangiomas conservatively, advise parents to wait at least 5 years, and then refer patients for surgical excision or revision if the hemangiomas failed to adequately involute. This strategy worked very well for the majority of families, as an estimated 70% of patients required no surgical treatment.

Unfortunately, 30% of hemangiomas will not shrink satisfactorily, leaving either thin, crệpey redundant skin, bulky fatty scar tissue, dermal scarring (which always occurs if a hemangiomas has ulcerated at some point), or distortion of facial features, including the nose, lips, eyelids, and ears, or distortion of the trunk and extremities.  Surgery is often indicated in these situations, and each patient must be individually evaluated to determine the appropriate timing and extent of surgery.

With the popularity of the internet, parents are well aware of the variety of treatment options, and the pendulum may have swung too far to the surgical side.  While early surgery is often a good option, surgical excision always leaves a scar, and impatient parents and overly aggressive surgeons may be doing some patients a disservice in the long run if they accept a permanent surgical scar for something nature might have completely erased.  Surgery is appropriate if one can be reasonably certain that natural involution will leave a worse result.

Timing of surgery

8-bleeding-hemangiomaHemangiomas can be excised during the early growth phase or after many years when there is residual deformity and no further perceived improvement, or anywhere in between.  It is a combination of clinical judgment and parental input that will help to determine when it is appropriate to proceed with surgery.

Early excision

Excision during the growth phase of a hemangioma can be considered for problematic hemangiomas such as those that have ulcerated and bled significantly.  These are fairly rare, but occasionally an ulcerated hemangioma erodes into an arterial vessel, causing spurting bleeding, or occasionally an ulcerated hemangiomas may ooze enough to cause anemia.  In such cases surgery may consists of suture placement around the bleeding area, or if the hemangioma is small enough, complete excision and suture closure of the wound.  In this situation, the creation of a permanent surgical scar is a worthwhile exchange for control of bleeding and parental peace of mind (Fig 8).

Excision during the regression phase

9-complete-excisionThe majority of hemangiomas can be excised at some point during the shrinkage phase, prior to complete involution.  At this point there is no further growth, but there is still active circulation through the residual hemangioma vessels.  If it is determined that unsatisfactory shrinkage is likely to occur, and if the operation can be done safely with acceptable blood loss, then surgical excision can be considered.  In many cases, the boundaries of the hemangioma are well-defined, there is adequate adjacent skin laxity for closure, and the hemangioma can be excised with minimal blood loss by staying peripheral to it (Fig 9).  Pedunculated hemangiomas can often be excised with a reasonably short scar.  Hemangiomas with both a central dermal component and a peripheral subcutaneous component can usually be incised just peripheral to the central part, and then the entire hemangioma removed through the central opening.

Hemangiomas that infiltrate normal adjacent skin and are too big to be excised in their entirety are more challenging. Not only is there more intraoperative bleeding because one must cut through the hemangioma, but the surgeon must also decide how much hemangioma to intentionally leave behind to avoid a deficiency of soft tissue in the future.  This commonly occurs with lip and nasal tip hemangiomas, Aggressive debulking or complete excision may result in inadequate long term lip or nasal tip volume, especially if the residual hemangioma goes on to shrink more than anticipated.

It may be advisable to intentionally stage the procedure into at least 2 separate operations.  The first procedure removes the majority of the bulk, and then the second procedure can fine tune any residual asymmetries that persist after at least a year of follow up. Parents should be advised of what can reasonably be expected, that symmetry may be an impossible goal, and that multiple revisions may be necessary for best results.

Excision after complete regression

Many hemangiomas will leave behind a combination of redundant stretched skin and bulky subcutaneous fibrofatty tissue after the vessels have involuted. These patients are the safest candidates for surgical debulking because there are few if any residual vessels, and it is much easier to judge how much soft tissue to leave behind.  A smooth surgical scar is generally superior to a protruding mound or loose redundant skin.  In most cases, however, patients and their parents will be reluctant to wait until complete shrinkage because the waiting period will extend into the early school years.

Surgical treatment of hemangiomas in special cases

Some hemangiomas seem to have a great propensity for leaving residual deformity, particularly those in the facial area.  Suppression with steroids is a judgment call, but the temporary side effects of steroids are often well worth it if one can prevent or at least minimize the potential lifetime deformity caused by an enlarging or ulcerated hemangioma

10-nasal-tip-hemangiomaNasal tip hemangiomas

Nasal tip hemangiomas often create a bulbous tip (Cyrano deformity, named after Cyrano de Bergerac) as they leave behind bulky fibrofatty tissue, redundant skin, and splayed nasal tip cartilages. Surgical correction often requires excision of redundant skin, centralization of the two pieces of cartilage that make up the nasal tip that have been separated by the hemangioma, and debulking of the excess soft tissue (Fig 10).  Overly aggressive soft tissue removal, however, can leave very thin skin over the tip cartilage, therefore it is advisable to intentionally leave residual hemangioma or fatty tissue to soften the nasal tip and improve the long term result.   Steroid suppression during the growth phase of the hemangioma may avoid the need for any surgery, or at least prevent over-expansion of the skin envelope. In such cases, debulking can be done through an intranasal approach, avoiding any external scars.

11-upper-lip-hemangioma

Lip hemangiomas

The vast majority of lip hemangiomas will leave excess soft tissue despite years of slow regression, and commonly hemangiomas can ulcerate because of feeding trauma to the thin fragile vermilion. Large hemangiomas may also affect the ability to suck.  Steroid suppression is often advisable to limit the degree of distortion.  Early first-stage debulking may assist with feeding issues and also help parents cope with the stress of dealing with a child with an oral hemangioma deformity.  Depending upon the location of the hemangioma, the scars can often be at least partially hidden just inside the lip vermilion.  However hemangiomas that extend onto the skin portion of the upper or lower lip usually require partial skin excision and therefore leave permanent external lip scars.  Upper lip hemangioma debulking can often take advantage of techniques learned through cleft lip reconstruction, placing the scars along natural boundaries to make them as subtle as possible (Fig 11).  Scar revisions are often desirable to optimize the final outcome, and therefore parents should understand that more than one operation is often necessary to improve the final result.

Eyelid (periorbital) hemangiomas

The thin eyelid skin is subject to significant distortion and expansion by the growing hemangioma.  If the eyebrow is involved, this can result in excess skin, low hair density, and distortion of the eyebrow shape. Steroids are usually indicated to avoid obstruction of vision, and this will also help to decrease the amount of skin distortion.   In cases where visual impairment is still a concern, ophthalmologists and pediatric oculoplastic surgeons may institute a patching regiment for the opposite eye, to encourage the child to use the obstructed eye, or they may elect early debulking to facilitate eyelid opening.

12-lower-lid-hemangioma

Late debulking of excess skin and subcutaneous tissue is often desirable, at the expense of a permanent surgical scar (Fig 12). Reconstruction of a missing or distorted eyebrow can be very difficult. Micrografting from the scalp results in hairs that require frequent trimming because they will continue to grow like scalp hair.  Micropigmentation (tattoo) is sometimes a good option, particularly if the patient can achieve good results initially with an eyebrow pencil.

Cheek and parotid hemangiomas

The salivary gland in front of the ear is the parotid gland, and sometimes intraparotid hemangioma growth can cause massive cheek enlargement. This can also cause partial obstruction of the external auditory canal, and if bilateral involvement is noted, steroid suppression is indicated to maintain auditory stimulation. In addition to parotid gland involvement, often the skin around the ear and cheek is involved.  This will usually regress very well over time, although there may be some residual cheek skin redundancy that may benefit from a facelift type reduction.  Overly aggressive debulking, however, risks injury to the facial nerve that runs through the parotid gland.  Early debulking when vessels are still active can increase the risk of inadvertent nerve injury because of the increased risk of blood in the field that may obscure anatomic landmarks.

13-surgical-scar-hemangioma

Hemangiomas involving the skin of the cheek and often the underlying subcutaneous tissue can be problematic.  The surgeon and parents must decide whether a linear surgical scar is preferable to a circular or oval patch of scarred dermis or redundant skin left by the hemangioma.  Some surgeons have advocated excision of the hemangioma using a purse-string type closure, but the vast majority of hemangiomas are excised in a football shaped pattern, which results in a long linear scar because of the need to taper the corners (Fig 13). Sometimes if the skin is in relatively good shape but there is underlying subcutaneous bulk, liposuction may provide a significant contour improvement using an intraoral puncture site to avoid any external scars.

Subcutaneous hemangiomas

14-hemangioma-nippleSubcutaneous hemangiomas often grow without causing overlying skin expansion and loss of skin tone.  In these cases, conservative observation is the best initial course to see if the hemangioma will spontaneously shrink completely, leaving totally intact normal skin.  Aggressive early surgical removal is contraindicated, as this will leave an unnecessary surgical scar.  If the hemangioma regresses but leaves behind bulky fatty tissue, at this point liposuction can provide excellent contour reduction through 1 or 2 small puncture sites.

Breast mound hemangiomas

Particularly in female infants, breast mound hemangiomas (Fig 14) should be left alone to avoid potential injury to the breast bud that can result in failure of breast development during puberty.  In many cases, as the hemangioma regresses and the breast develops, surgical correction will become unnecessary.  Nipple distortion or redundant skin can be addressed after normal breast development, and fortunately the deformity can usually be easily camouflaged with clothing until that time. 

Since breast bud preservation is not necessary in male patients, any nipple or chest wall deformity can be addressed at a much earlier age, usually at some point during the shrinkage phase, or after complete regression.

15-scalp-hemangioma

Scalp hemangiomas

Many scalp hemangiomas will obliterate the hair roots, leaving patches of redundant, non-hairbearing scalp (Fig 15).  These can be easily excised either during the shrinkage phase or later, with minimal blood loss since one can compress the surround scalp during the excision to control bleeding.  A surgical scar always results, but usually there is adequate adjacent hair density to help camouflage this.  With short hair, the scar may be more obvious. With advancing age and a history of male-pattern baldness, some scalp scars may become evident later in life.

16-buttock-hemangioma

Buttock and perineal/genital hemangiomas

While these often cause significant problems during the growth period because of their propensity to ulcerate, causing

significant pain with urination or stooling, these rarely need any surgical revision later on (Fig 16).  Sometimes a hemangioma in the labial area can result in some excessive skin, but this is a

rarity.  Laser can be useful during the ulcerated phase, as described in the laser treatment section above.  Topical lidocaine jelly (2%) followed by antibiotic ointment is a common temporizing strategy until the skin has healed.

David W. Low, MD
Associate Professor of Surgery, Division of Plastic Surgery
University of Pennsylvania School of Medicine
Clinical Associate, The Children’s Hospital of Philadelphia

Figures

Fig 1 – typical bright red dermal hemangioma with signs of early regression

Fig 2 – hemangioma with both a superficial dermal and a deeper subcutaneous component

Fig 3 – periorbital hemangioma that is causing partial visual obstruction, which can cause permanent amblyopia

Fig 4 – PELVIS syndrome patient with extensive ulcerated hemangioma.  MRI scan confirmed the presence of a lipomyelomeningocele and a tethered cord

Fig 5 – Rapidly involuting congenital hemangioma (RICH). The photos are taken 3 months apart

Fig 6 – Non-involuting congential hemangioma (NICH) which was later surgically excised

Fig 7 – laser treatment for residual vessels after hemangioma involution

Fig 8 – early excision of an ulcerated, bleeding hemangioma

Fig 9 – typical complete hemangioma excision using a football-shaped pattern

Fig 10 – nasal tip hemangioma debulking utilizing a cosmetically favorable incision that permits skin excision, cartilage adjustment, and soft tissue debulking.

Fig 11 – lip hemangioma debulking, utilizing cleft lip techniques to maximize symmetry

Fig 12 – periorbital hemangioma debulking of the lower eyelid. Complete excision would have resulted in lower lid retraction (ectropion)

Fig 13 – cheek hemangioma excision with subsequent hypertrophic scar.  The contour improvement is an acceptable trade off for the less than optimal scar

Fig 14 – chest hemangioma involving the nipple and underlying breast bud

Fig 15 – scalp hemangioma, with destruction of involved hair follicles

Fig 16 – ulcerated buttock hemangioma, with complete healing within 2 months