Infantile (Capillary) Hemangioma
Infantile (capillary) hemangiomas are common primary benign tumors of the orbit in children (Fig 5-1). These lesions present at birth or within the first few weeks of life. They enlarge dramatically over the first 6–12 months and begin to involute after the first year of life; 75% of lesions resolve by the age of 3–7 years. Female sex, low birth weight, prematurity, and maternal chorionic villus sampling are associated with infantile hemangiomas.
Figure 5-1 Infantile (capillary) hemangioma. A, Infantile hemangioma involving the right upper eyelid. B, Marked regression of lesion 6 weeks after initiation of propranolol therapy.
(Courtesy of William R. Katowitz, MD.)
Congenital infantile hemangiomas may be superficial—in which case they involve the skin and appear as a bright red, soft mass with a dimpled texture—or they may be subcutaneous and bluish. Hemangiomas located deeper within the orbit may present as a progressively enlarging mass without any overlying skin change. Magnetic resonance imaging (MRI) may help to distinguish infantile hemangiomas from other vascular malformations by demonstrating characteristic fine intralesional vascular channels and high blood flow. Color Doppler ultrasound imaging is a reliable and inexpensive imaging tool for diagnosing these lesions, often showing numerous blood vessels within the mass and abundant blood flow.
In the periocular area, infantile hemangiomas occur most commonly in the superonasal quadrant of the orbit and the medial upper eyelid (see also the section Congenital Eyelid Lesions in Chapter 10). They may be associated with hemangiomas on other parts of the body; lesions that involve the neck can compromise the airway and lead to respiratory obstruction, and multiple large visceral lesions can produce thrombocytopenia (Kasabach-Merritt syndrome).
Most lesions regress spontaneously, requiring only observation with refractive correction and amblyopia therapy. The main ocular complications of infantile hemangiomas are amblyopia, strabismus, and anisometropia. Although disruption of vision or severe disfigurement may require therapy, treatment can be deferred until it is clear that such complications may develop.
For infantile hemangiomas that require therapy, β-blockers are the first-line treatment. Topical timolol gel treats superficial tumors and has limited systemic adverse effects. Oral propranolol treats deeper lesions and offers fewer adverse effects than systemic steroids, although life-threatening hypotension, bradycardia, and hypoglycemia can occur in rare instances. Oral β-blocker treatment should be initiated under the guidance of a pediatrician, so the patient can be monitored for systemic adverse effects. Lesions that do not respond adequately to β-blockers may require treatment with steroids, administered topically, by local injection, or orally (see the section Congenital Eyelid Lesions in Chapter 10). Adverse effects of steroid injection include skin necrosis, subcutaneous fat atrophy, and retinal embolic vision loss. Steroid treatment by any route in infants may produce hypothalamic-pituitary-adrenal axis suppression, systemic growth retardation, and a variety of other metabolic adverse effects.
Surgical excision with meticulous hemostasis may be considered for active-phase lesions refractory to steroids or for some smaller or subcutaneous nodular lesions. Pulseddye laser therapy can improve superficial components of the hemangioma. Radiation therapy has also been used, but it can lead to cataract formation, bony hypoplasia, and future malignancy. Sclerosing solutions are not recommended, as they can cause severe scarring. Residual lesions that remain after involution or treatment can be removed surgically.
Chambers CB, Katowitz WR, Katowitz JA, Binenbaum G. A controlled study of topical 0.25% timolol maleate gel for the treatment of cutaneous infantile capillary hemangiomas. Ophthalmic Plast Reconstr Surg. 2012;28(2):103–106.
Fridman G, Grieser E, Hill R, Khuddus N, Bersani T, Slonim C. Propranolol for the treatment of orbital infantile hemangiomas. Ophthalmic Plast Reconstr Surg. 2011;27(3):190–194.
Excerpted from BCSC 2020-2021 series: Section 10 - Glaucoma. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.