The majority of IHs are small, harmless lesions that can be monitored under the watchful eye of a pediatrician. Ten percent of proliferating IHs, however, cause significant deformity or complications, usually when located on the head or neck. Ulcerated lesions may destroy the eyelid, ear, nose, or lip. IH of the scalp or eyebrow can result in alopecia. Periorbital hemangioma can block the visual axis or distort the cornea, causing amblyopia. Subglottic hemangioma may obstruct the airway.

Approximately 20% of infants have more than one IH. The term hemangiomatosis designates five or more small (<5 mm) tumors. These children are at increased risk for IH of internal organs, although the risk is low. The liver is most commonly affected; the brain, gut, or lung are rarely involved. Ultrasonogram should be considered to rule out hepatic IH.

The liver is the most common extracutaneous site for IH. Ninety percent of fast-flow hepatic lesions are IH. The differential diagnosis includes AVM, hepatoblastoma, and metastatic neuroblastoma, which do not demonstrate significant shunting on imaging. There are three subtypes of hepatic hemangioma: focal, multifocal, and diffuse.
Although most hepatic IHs are nonproblematic and discovered incidentally, large tumors can cause heart failure, hepatomegaly, anemia, or hypothyroidism. Focal hepatic hemangioma usually are asymptomatic and not associated with cutaneous lesions; they are often identified prenatally. There is evidence that solitary hepatic hemangioma is a RICH. Occasionally this tumor can cause cardiac overload and thrombocytopenia; however, these symptoms resolve as the tumor regresses. Multifocal hepatic IHs are often accompanied by cutaneous lesions. Although usually asymptomatic, multifocal lesions can cause high-output cardiac failure, which is managed by corticosteroid or embolization. Diffuse hepatic IH can cause massive hepatomegaly, respiratory compromise, or abdominal compartment syndrome. Infants are also at risk for hypothyroidism and irreversible brain injury because the large tumor volume expresses enough deiodinase to inactivate thyroid hormone. Patients require thyroid stimulating hormone monitoring and, if abnormal, intravenous thyroid hormone replacement until the IH begins to regress.

There are uncommon presentations of IH with malformations, either in the head/neck or in the lumbosacral regions. PHACE association affects 2.3% of patients with IH, and consists of a plaque-like IH in a regional distribution of the face with at least one of the following anomalies: Posterior fossa brain malformation; Hemangioma; Arterial cerebrovascular anomalies; Coarctation of the aorta and cardiac defects; Eye/Endocrine abnormalities. When ventral developmental defects (Sternal clefting or Supraumbilical raphe) are present, an “S” is added (PHACES). Ninety percent of infants are female and cerebrovascular anomalies are the most common associated finding (72%). Because 8% of children with PHACE have a stroke in infancy, patients should have magnetic resonance imaging (MRI) to evaluate the brain and cerebrovasculature. Infants are referred for ophthalmologic, endocrine, and cardiac evaluation to rule out these associated anomalies.

Reticular hemangioma is an uncommon variant of IH that most commonly affects the lumbosacral area and lower extremity. Females (83%) are usually affected. Unlike typical IH, reticular tumors are likely to ulcerate and rarely cause cardiac overload. Reticular hemangioma is often associated with ventral–caudal malformations (omphalocele, recto-vaginal fistula, vaginal/uterine duplication, solitary/duplex kidney, imperforate anus, tethered cord lipomyelomeningocele). After involution small veins often remain, which may be treated by sclerotherapy. Ultrasonography (US) is obtained to rule out associated anomalies in infants less than 4 months of age. MRI is indicated in older infants or when US is equivocal.

Topical corticosteroid has minimal efficacy; especially against IH involving the deep dermis and subcutis. Ultrapotent agents may be effective for a very superficial IH. Although lightening may occur, if there is deep component it will not be affected. Adverse effects include hypopigmentation, skin atrophy, and even adrenal suppression.

Small, well-localized IHs that obstruct the visual axis or nasal airway or those at risk for damaging important structures (e.g., eyelid, lip, nose) are best managed by intralesional corticosteroid. Triamcinolone (3 mg/kg) stabilizes the growth of the lesion in at least 95% of patients; 75% of tumors will decrease in size. The corticosteroid lasts 4 to 6 weeks and thus infants may require one to two more injections during the proliferative phase. Intralesional corticosteroid may cause subcutaneous fat atrophy. Blindness has been reported following injection of deep periorbital hemangioma due to embolic occlusion of the retinal artery.

Any problematic IH that is larger than 3 to 4 cm in diameter is managed by daily oral prednisolone. The patient is started on 3 mg/kg/d for 1 month, which then is tapered by 0.5 cc every 2 to 4 weeks until it is discontinued between 10 and 12 months of age when the tumor is no longer proliferating. Recently, propranolol has been described for the treatment of IH, but its efficacy and safety, compared with corticosteroid, has not been studied. Corticosteroid, in contrast, has been used to treat IH for over 40 years and has proven to be very safe and effective. Meta-analysis has shown that 84% of patients treated with different doses of corticosteroid will have (a) stabilization of growth or (b) accelerated regression. Almost all patients, however, will respond to 3 mg/kg. Treatment response is usually evident within 1 week of therapy by signs of involution: decreased growth rate, fading color, and softening of the lesion. The location of the hemangioma does not influence response rate. For the rare lesion that fails to stabilize with corticosteroid, the dose may be increased up to 5 mg/kg, which may improve treatment response. Alternatively, the child may be switched to vincristine. Interferon is no longer recommended in children less than 12 months of age because it can cause neurologic sequela, particularly spastic diplegia.

Complications of systemic corticosteroid for the management of IH have been studied; there are no adverse effects on neurodevelopment. Short-term morbidity may include cushingoid face, personality change, gastric irritation, fungal (oral or perineal) infection, myopathy, decreased gain in height, and decreased gain in weight. These findings resolve after the completion of therapy. Over 90% of children return to their pretreatment growth curve for height by 24 months of age.

Large IHs, most commonly multifocal hepatic lesions, can cause high-output congestive heart failure. Embolization may be indicated for the initial control of cardiac overload while systemic corticosteroid therapy takes effect. Cardiac failure often recurs even after initial improvement, and drug therapy should be continued after embolization until the child is approximately 12 months of age when natural involution begins.

There is little, if any, role for pulsed-dye laser treatment for proliferating IH. The laser penetrates only 0.75 to 1.2 mm into the dermis, and thus only affects the superficial portion of the tumor. Although lightening may occur, the mass of IH is not affected. Instead, patients have an increased risk of skin atrophy and hypopigmentation. The thermal injury delivered by the laser to the ischemic dermis increases the risk of ulceration, pain, bleeding, and scarring. Pulsed-dye laser is indicated during the involuted phase to fade residual telangiectasias.

Operative treatment in infancy is generally not recommended. The tumor is highly vascular during this period and there is a risk for blood loss, iatrogenic injury, and an inferior aesthetic outcome, compared with excising residual tissue after the tumor has regressed. Nevertheless, in experienced surgical hands, there are indications for operative intervention during the proliferating phase: (a) failure or contraindication to corticosteroid; (b) well-localized tumor in an anatomically favorable area; (c) resection will be necessary in the future and the scar would be the same. Circular lesions located in visible areas, particularly the face, are best removed by circular excision and purse-string closure. This technique minimizes the length of the scar as well as distortion of surrounding structures. Lenticular excision of a circular hemangioma results in a scar as long as three times the diameter of the lesion (Fig. 3). In comparison, a two-stage circular resection followed by lenticular excision/linear closure 6 to 12 months later will leave a scar approximately the same length as the diameter of the original hemangioma (Fig. 4).

While operative management of IH is generally not indicated during the proliferative phase, resection during involution is much safer because the lesion is less vascular and smaller. Because the extent of the excision is reduced, the outcome is superior. Approximately 50% of IHs leave behind fibrofatty tissue or damaged skin after the tumor regresses, causing a deformity. Sometimes a child requires reconstruction of damaged structures (e.g., nose, ear, lip). Staged or total excision should be considered during this period, rather than waiting for complete involution if (a) it is clear that the lesion will require resection (e.g., postulceration scarring, destroyed structures, expanded skin, significant fibrofatty residuum); (b) the length of the scar would be similar if the procedure was postponed to the involuted phase; (c) the scar is in a favorable location. Advantages of operative intervention during this period, compared with late childhood, is that reconstruction is under way prior to the child’s development of memory or awareness of a facial difference.

Waiting until IH has fully involuted prior to resection ensures that the least amount of fibrofatty residuum and excess skin is resected, resulting in the smallest possible scar. Postponing intervention until complete involution has occurred must be weighed against the possible psychosocial implications of maintaining a deformity until late childhood. Allowing for full involution is recommended for lesions when it is unclear if a surgical scar would leave a worse deformity than the appearance of the residual hemangioma.