CHAPTER 4 INFANTILE HEMANGIOMA
KEY POINTS
Resection of an infantile hemangioma during the proliferative phase is uncommon.
Surgical intervention for an infantile hemangioma is usually performed between 3 and 4 years of age after the lesion has completed regression.
Complex reconstruction after extirpation of an infantile hemangioma is rarely required; wounds typically can be closed with local tissues.
Round lesions located in visible areas can be removed with a circular excision and purse-string closure to limit the size of the scar.
Patients with an infantile hemangioma are best managed by physicians focused on this tumor.
Infantile hemangioma is the most common tumor of infancy, affecting 5% of infants. Eighty percent of lesions involve the integument and are red; 20% are located subcutaneously and may appear bluish or have normal overlying skin. An infantile hemangioma has a unique growth cycle; it is usually noted 2 weeks after birth and enlarges rapidly during the first 9 months of life (proliferating phase). Eighty percent of its size is achieved by 3.2 (± 1.7) months of age. Between 9 and 12 months of age, the lesion begins to slowly shrink (involuting phase); the appearance improves until 3½ years of age (involuted phase). After involution, 50% of infantile hemangiomas will leave behind a permanent deformity: residual fibrofatty tissue, anetoderma, scar, redundant skin, telangiectasias, and/or destroyed anatomic structures.
SURGICAL INDICATIONS
The primary morbidity of an infantile hemangioma is psychosocial, because the lesion can cause a deformity. Surgical procedures are generally carried out to improve a “cosmetic” problem. Because long-term memory and self-esteem do not begin to develop until 4 years of age, there is rarely an indication to remove a lesion before it has finished improving at 3 years of age. An involuted hemangioma can lower self-esteem if there is residual fibrofatty tissue, scarring from ulceration in infancy, anetoderma, redundant skin, altered anatomic structures, or telangiectasias. If only telangiectasias remain, the patient should be treated with pulsed-dye laser. This laser will erase the cutaneous blood vessels without scarring. The only way to improve redundant skin, anetoderma, or subcutaneous adipose tissue is resection. Altered anatomic structures (usually of the central face) are improved with the same principles used for other reconstructive problems.
During infancy an infantile hemangioma can cause functional problems by obstructing vision, the nose (infants younger than 3 months of age are primarily nasal breathers), or the oral airway. Resection of lesions in these areas should be the last option; removal of a proliferating infantile hemangioma is avoided when possible. Instead, problematic lesions are managed with topical or intralesional medications (if localized) or oral pharmacotherapy (if diffuse). These drugs will stop the growth of the tumor (and often decrease its size) until natural involution begins at 9 months of age. Sixteen percent of infantile hemangiomas ulcerate during the proliferating phase. The wounds will heal within 2 to 4 weeks after applying hydrated petroleum and a barrier dressing and eliminating the trauma that caused the wound. Ulcerated lesions do not require resection and are not at risk for infection, because the wound is open (even if urine and stool cover the area). Infantile hemangiomas involving the genitalia will not affect sexual or urinary function.
SURGICAL MANAGEMENT
TIMING OF INTERVENTION
Intervention will depend on the phase of the hemangioma: during the proliferating phase or the involuted phase (see Video 4-1, Infantile Hemangioma).
Proliferating Phase
Most infantile hemangiomas do not require treatment; they are small and nonproblematic. Lesions are allowed to proliferate and involute without intervention; after regression there is minimal evidence that the tumor was ever present. In my practice, which is biased toward referrals for problematic lesions, only 22% of infants are treated: 5% undergo corticosteroid injection, 8% receive oral pharmacotherapy, 2% undergo resection, and 7% receive topical timolol. The indications for treatment of a proliferating infantile hemangioma are:
The lesion is at risk for obstruction/destruction of an important anatomic structure.
It may leave behind a permanent deformity, requiring treatment later in childhood.
Almost all lesions that necessitate early intervention are located on the face or neck. My first-line treatment for localized lesions that are less than 3 cm in diameter is corticosteroid injection not to exceed 3 mg/kg (triamcinolone 40 mg/ml) per injection. If the infantile hemangioma is too large to inject, a patient is given systemic pharmacotherapy (prednisone or propranolol). Both drugs are effective and safe. A superficial hemangioma will respond to topical timolol if treatment is initiated early, usually before 10 weeks of age.
Resection of a proliferating infantile hemangioma is generally not recommended. The tumor is highly vascular during this period, and there is a risk of blood loss, iatrogenic injury, and an inferior outcome compared with excising residual tissue after the tumor has regressed. Indications for surgical intervention during this phase include:
There is a failure or contraindication to pharmacotherapy.
The patient has a well-localized tumor in an anatomically favorable area.
Resection will be necessary in the future and the scar would be the same.
Many parents want an infantile hemangioma removed as soon as possible, because it attracts the attention of others. I counsel families that it is better to wait until the lesion has completed involution (between 3 and 4 years of age) for the following reasons:
The tumor will be smaller, which facilitates the procedure and gives the child the shortest possible scar.
There is no urgency to remove the lesion, because the child’s long-term memory and self-esteem do not begin to form until 4 years of age.
In my practice only 2% of infantile hemangiomas are resected during the proliferating phase; the rest are excised after they have involuted. During the proliferating phase I have removed ulcerated lesions that the parents were unable to manage or were less likely to heal quickly (for example, back of the neck where the child sleeps on the area). Because the scalp has significant laxity during infancy, this is a favorable time to remove large ulcerated lesions in this location, because the area is more easily closed linearly. Retroauricular infantile hemangiomas can cause a prominent ear deformity; although the hemangioma will ultimately involute, the child may require an otoplasty. Consequently, lesions affecting the ear may benefit from removal during infancy.
Involuted Phase
There is rarely an indication to remove a hemangioma during the involuting phase (9 months through 3 years), because the tumor is either problematic while it is rapidly growing, or it leaves behind a permanent deformity. After involution, approximately 50% of infantile hemangiomas will have fibrofatty tissue or damaged skin, causing a deformity. It is best to wait until the tumor has completed involution between 3 and 4 years of age before deciding whether surgical intervention is indicated. Often a tumor in infancy that is predicted to leave behind a deformity regresses better than expected, and the child does not need surgery. If the child requires a procedure, it is safer after involution, because the tumor is much less vascular. In addition, the infantile hemangioma is significantly smaller, and thus the scar is much shorter.
If I believe a patient may require surgical intervention, they return for an evaluation when they turn 3 years of age. If the parents state that there has been minimal or no improvement over the previous few months, the surgery is scheduled. If the infantile hemangioma is continuing to improve or is in a very problematic location (philtrum, nasal tip, or eyelid), they will return in 6 months for another examination, and the procedure will be scheduled before their fourth birthday to allow the lesion to spontaneously improve for as long as possible. Occasionally an infantile hemangioma will stop improving between 18 and 36 months of age, and if so, I will intervene before 3 years of age. Some children will have a minor deformity, and it is unclear whether:
The area will be bothersome to the patient.
The scar from an operation would be more noticeable than the residual infantile hemangioma.
In this situation it is best to observe the child and intervene later if the child requests a procedure when he or she is older.
PRINCIPLES
An infantile hemangioma is benign and does not require resection, unless it causes lowered self-esteem. Patients will frequently have minor deformities, and the surgeon must determine if the resulting scar will be an improvement over the residual infantile hemangioma. Treatment of a benign vascular anomaly should never cause a worse deformity than the lesion. The goal of the surgery is to improve the appearance of the area and not complete extirpation of the infantile hemangioma. Consequently, most resections are subtotal, and it is preferable to leave some residual fibroadipose tissue rather than attempting to perform complete resection, which increases the risk of nerve injury and gives the patient a longer scar. An infantile hemangioma almost always involves the skin and subcutaneous tissue only; however, if deeper structures contain residual fibrofatty tissue, they do not need to be removed. Only a superficial lesion should be removed to improve the patient’s appearance; nerve dissections and deep resections are not performed.
Infantile hemangiomas in anatomically sensitive areas (for example, the face) should have minimal or no margins taken. If the overlying skin is normal and there is a greater subcutaneous component, a smaller incision can be made, skin flaps are elevated, and the underlying hemangioma is removed without performing a large skin resection. Residual adipose tissue at the base of the wound can be cauterized, which will cause atrophy and further improvement without resection. Often an infantile hemangioma has left residual fibrofatty tissue, with or without damaged skin, surrounded by an area of telangiectasias. Instead of removing the entire abnormal area, the lesion should be managed by two methods to reduce the length of scar:
Telangiectasias are treated with a pulsed-dye laser.
Damaged skin/fibrofatty tissue is resected.
Typically I will first treat the telangiectasias with a pulsed-dye laser and then perform the resection secondarily to give the smallest possible scar. Less commonly I will perform the resection initially and then treat peripheral telangiectasias later.
LENTICULAR EXCISION
Because an infantile hemangioma expands the skin, almost all lesions can be removed with lenticular excision and linear closure. If the entire area cannot be extirpated, serial resection can be performed. I have never had to use a skin graft or flap to reconstruct an area after I removed an infantile hemangioma. Although infantile hemangiomas bleed more than other types of skin lesions, blood loss is minimized by infusing a local anesthetic with epinephrine throughout the surgical site. If the volume of local anesthetic with epinephrine is limited by the weight of the child, I will infiltrate an epinephrine-only solution to ensure maximum vasoconstriction of the operative site (1 cc of 1:000 epinephrine in 200 cc normal saline solution = 1:200,000 solution). On the rare occasion that a proliferating lesion is removed, bleeding is more problematic, because the tumor is highly vascular, and an infant is less tolerant of blood loss compared with an older child. After the area is infiltrated with epinephrine, part of the area is incised, bleeding is controlled with cautery, and staged resection is continued rather than making the entire incision initially. With the use of epinephrine and cautery, I have never had significant blood loss or had a child undergo transfusion after I performed a resection of an infantile hemangioma.
Lenticular excisions are used for most infantile hemangiomas, including circular lesions in locations favorable for a one-stage excision and linear closure. Because infantile hemangiomas expand the skin, wide undermining is not usually necessary to obtain a tension-free closure. Incisions are closed with interrupted Vicryl sutures for the deep dermis and either interrupted chromic or subcuticular Monocryl suture to approximate the epidermis and superficial dermis, because most children cannot tolerate suture removal. The incisions are dressed with cyanoacrylate glue, Steri-Strips, and a gauze pressure dressing. I use 7-0 chromic sutures for facial lesions; suture marks are not visible because the diameter of the suture is similar to pore sizes. In older children and adolescents, I typically use interrupted nylon suture that is removed in the office. The incisions are then reinforced with Steri-Strips for 6 weeks to minimize widening of the scar. Patients are instructed to avoid sun exposure for 12 months.
If a hemangioma is too large to resect in one stage, serial excision is performed. I usually perform the next stage 6 to 12 weeks later. If a large hemangioma is resected from the upper extremity, a soft gauze and elastic dressing is applied to protect the area and limit the use of the arm. If significant wound tension exists, occasionally a splint is placed for 2 to 3 weeks. Extirpation of large lesions on the lower extremity is followed by placement of a knee immobilizer for 2 to 3 weeks. The child can remove the device when sleeping and showering. Previously I would place patients in a cast but found that the knee immobilizer is equally effective and easier for patients and parents.
CIRCULAR EXCISION
Many infantile hemangiomas have a circular morphologic structure and are amenable to circular excision and purse-string closure. Infantile hemangiomas are particularly favorable for purse-string closure, because they usually expand the surrounding skin. Lenticular excision of a circular lesion will result in a scar two to three times its diameter, because the area is removed in an ellipse to prevent dog-ears. Circular excision recruits the surrounding skin and gives a circular scar that is one third to one half the area of the infantile hemangioma. In my practice approximately 50% of patients and families are satisfied with the circular scar; it appears as a “pockmark” scar (for example, from acne or chicken pox). Other patients undergo a second stage to convert the circular scar into a line. Because the circle is smaller than the original lesion, the final scar is shorter. Ultimately, the two-stage approach leaves a linear scar approximately the same length as the diameter of the original infantile hemangioma. If the lesion is large, multiple purse-string excisions and circular closures can be performed to continually reduce the area of the scar until it is acceptable to convert into a line.
Another advantage of circular excision and purse-string closure is that it causes less deformation of surrounding structures compared with lenticular resection. Almost all infantile hemangiomas that undergo circular excision are located on the face, where it is most important to limit the length of the scar and accept the morbidity of more than one procedure. Lenticular excisions and linear scars on the trunk and extremities placed in relaxed skin-tension lines have favorable appearances. I will perform circular excisions outside of the face if the lesion is very large in a noticeable area (for example, a forearm), or if I would like to minimize distortion of adjacent structures. I will also use a circular skin excision over a large subcutaneous infantile hemangioma to allow the best access to the lesion with the smallest scar. After the subcutaneous tissue is removed, the skin undergoes purse-string closure, and the circular scar is later converted to a line. The only location where a circular excision is contraindicated is the scalp. The scalp is an unfavorable location for purse-string closure because:
It has poor elasticity.
Limiting the length of a linear scar is not important, because it is camouflaged by hair.
A circular scar in the scalp is much more likely than linear cicatrix to cause visible alopecia (especially in males).
When performing a circular excision, no margins should be included to maintain as much skin as possible to close the wound under minimal tension. Wide skin undermining is carried out in a 360-degree direction to recruit adjacent tissue. I typically use 4-0 or 5-0 Vicryl on the face and 2-0 or 3-0 Vicryl on the trunk and extremity for the purse-string suture. The less tension on the closure, the more likely the patient will have a smaller circular scar. The Vicryl suture is placed in the deep dermis, and the epidermis/superficial dermis is approximated with chromic suture (5-0 or 6-0 in the face; 3-0 or 4-0 in the trunk and extremity). Suture marks are difficult to appreciate along the circular scar. The area is then dressed with cyanoacrylate glue followed by Steri-Strips and a pressure dressing (gauze with Tegaderm or tape). Steri-Strips are applied to the scar for 6 weeks after surgery to prevent the circular scar from widening. The child is evaluated 6 to 12 months later to determine if he or she would benefit from another circular excision or conversion to a linear scar. In some locations the circular scar will mature in an orientation along a relaxed skin-tension line.
SUCTION-ASSISTED LIPECTOMY
Liposuction can be used to remove subcutaneous fibrofatty residuum after the hemangioma has involuted. The technique avoids a scar over the area, because the cannula can access the lesion from a remote 1 cm incision placed in an inconspicuous location. Suction-assisted lipectomy is rarely used, because almost all infantile hemangiomas have damaged skin that needs to be removed. However, if the integument is normal, liposuction should be considered. Suction-assisted lipectomy of an involuted hemangioma is more difficult compared with removal of normal adipose tissue, because the tissue is more fibrous and vascular. There is increased bleeding, and tissue removal is less predictable than open excision. Consequently, I generally do not use liposuction and instead prefer to make an incision and remove excess subcutaneous tissue sharply under direct vision. I believe in the principle of trading scar for contour. Patients are most satisfied with achieving the best symmetry, which is noticed beyond conversational distance at the expense of a scar that can only be appreciated on close examination (similar to having a longer scar instead of a shorter scar and dog-ears at each end).
Another option for removal of an infantile hemangioma of the subcutaneous cheek that has normal overlying skin is to perform an intraoral resection. Similar to liposuction, this technique is more difficult and less predictable than a direct cutaneous extirpation. In addition, this approach has increased risk of infection and injury to the facial nerve. As a result, I do not prefer intraoral removal of subcutaneous tissues.
ANATOMIC CONSIDERATIONS
Scalp
Proliferating infantile hemangiomas of the scalp have an increased risk of ulceration because of incidental trauma, particularly when the patient is sleeping. Indications to removal of an involuted infantile hemangioma of the scalp are alopecia and/or residual fibrofatty tissue that is causing a visible deformity. Anetoderma and telangiectasias are camouflaged by hair and usually do not require treatment. Infantile hemangiomas damage hair follicles, and thus lesions in the scalp will cause an area of decreased hair density. If an ulceration occurred in infancy, the resulting scar will not have any hair growing through it. The larger the size of the infantile hemangioma and area of ulceration, the more likely the patient will have visible alopecia (especially in males who have shorter hair).
An involuted infantile hemangioma causing a deformity should be removed by lenticular excision and linear closure. Limiting the length of the scar is not critical in the scalp, because it is hidden by hair; circular excision and purse-string closure should not be performed. When the surgeon performs a linear closure, the scar should be as narrow as possible so that the adjacent hair is able to cover it. Because Steri-Strips cannot be applied to an incision in the scalp to limit scar widening, I place a galeal/intradermal PDS suture followed by interrupted cutaneous sutures.
Forehead
Infantile hemangiomas located near the hairline can be removed with a lenticular excision and linear closure, because the scar is camouflaged by hair. If possible, lenticular excisions should be designed horizontally along the relaxed skin-tension lines. Lesions near the eyebrows and glabella are also usually excised with a lenticular excision and linear closure. Scars can be placed horizontally along the eyebrow or vertically extended toward the medial canthal area. Infantile hemangiomas located in the middle of the forehead are usually managed by circular excision and purse-string closure to limit the length of the scar and to minimize distortion of the eyebrow.
Nose
Cutaneous infantile hemangiomas are best removed by circular excision and purse-string closure to limit the scar burden on the nose. Subcutaneous nasal tip lesions require open rhinoplasty. After the skin is elevated, the underlying hemangioma is excised over the lower lateral cartilages. Because the tumor usually separates the cartilage, the dome/middle crus of each lower lateral cartilage is sutured together in the midline with 5-0 PDS suture. The hemangioma has usually expanded the overlying skin, and thus the skin is trimmed along the columella and rim incisions before the surgeon closes the wound. If overlying skin discoloration remains, it is treated with a pulsed-dye laser.
Lip
Infantile hemangiomas of the cutaneous lip may be removed with circular excision to limit distortion of structures in the area and length of the scar. However, some lesions are best removed along one of the philtral columns or mucocutaneous junction in a linear fashion. If the hemangioma has effaced the mucocutaneous junction and residual telangiectasias are present, I will first treat the area with a pulsed-dye laser to better define the cutaneous lip from the red vermilion.
Infantile hemangiomas affecting only the vermilion and mucosa are removed with a transverse mucosal incision. Usually the ellipse is centered along the junction of the keratinized and non-keratinized vermilion. If the hemangioma has preferentially expanded the keratinized vermilion or mucosa, the elliptical resection is adjusted toward the most overgrown area. After the surgeon marks the outline of the planned resection, the incision lines are tattooed, because marker is usually erased after application of povidone-iodine (Betadine). Next, local anesthetic with epinephrine is infused throughout the operative field; a minimal amount is used, because too much volume will distort the lip and increase the difficulty of the procedure.
After the expanded skin and residual fibroadipose tissue are removed, the area should be slightly overcorrected, because the postoperative swelling will create fibrosis that will contribute to lip fullness. To minimize swelling, patients are given dexamethasone (Decadron) intraoperatively. Before wound closure, the vermilion and mucosa are undermined and approximated with 4-0 or 5-0 Vicryl suture. Next, interrupted 6-0 chromic suture is used for the superficial closure and does not leave suture marks in the vermilion. In the upper lip the wound should be closed starting at the commissure and ending at the median tubercle, so that a dog-ear is in the midline where the lip is fullest. In the lower lip the incision should be closed starting medially and ending laterally, so that a dog-ear is hidden in the commissure. To protect the area from dehiscence, the patient is given a full-liquid diet for 2 weeks.
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