Congenital cutaneous capillary hemangioma (infantile hemangioma, benign hemangioendothelioma, strawberry nevus) is a benign vascular tumor that is either apparent at birth or develops a few weeks after birth. It is one of the most common tumors of infancy (1,2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30,31,32,33,34,35,36). It can be located superficially, deep, or both. There is a tendency for this lesion to occur in siblings and affected triplets have been described (7). Rarely, cutaneous capillary hemangioma can be associated with extensive hemangiomatosis that can involve the viscera and other organs. These large tumors trap platelets within their vascular channels, leading to thrombocytopenia and secondary coagulopathy. This condition, called the Kasabach-Merritt syndrome, is sometimes fatal (3).

The superficial eyelid and periocular capillary hemangioma (strawberry hemangioma) appears initially as a red vascular macule that progressively enlarges and becomes more elevated for 3 to 6 months after diagnosis. It usually becomes stable by age 12 to 18 months and then slowly involutes. It has been estimated that about 30% of cutaneous capillary hemangiomas regress completely by age 3 years and 75% to 90% by age 7 years (4). Almost complete regression usually occurs by age 5 years. Although it may have a very bothersome cosmetic appearance early in its course, the eventual regression is often dramatic, leaving little cosmetic defect.

In contrast to the superficial form, the deep capillary hemangioma lies in the subcutaneous tissues with little or no involvement of the epidermis. It is blue-gray in color, soft to palpation, and becomes more prominent with crying or straining. A tumor that extends deeper in the orbit can produce proptosis and displacement of the globe, and is important in the differential diagnosis of infantile orbital tumors. A typical overlying hemangioma in the skin should suggest that the deeper orbital tumor is also a capillary hemangioma. Its natural course is similar to the superficial variant, with fairly rapid growth followed by regression. This deep capillary hemangioma is covered in the atlas of orbital tumors.

The main complications of periocular capillary hemangioma are strabismus and amblyopia. The strabismus is either secondary to tumor impingement on the rectus muscles or secondary to amblyopia. The amblyopia can be a result of the tumor obstructing the pupil or a result of the anisometropia induced by the compression of the globe by the tumor. The refractive error often persists even after the hemangioma has regressed (5).

When the eyelid skin is involved by the typical red lesion, the diagnosis is generally quite evident. The differential diagnosis of deeper capillary hemangioma includes several other childhood anterior orbital neoplasms, including lymphangioma, rhabdomyosarcoma, and myeloid sarcoma (leukemia). These are discussed further in the Atlas of orbital Tumors.

Histopathologically, capillary hemangioma consists of lobules of capillaries that are separated by fibrous tissue septa. The proliferating endothelial cells may obliterate the capillaries (6). As a capillary hemangioma undergoes regression, it becomes less cellular and less vascular, and is replaced by fibrous tissue.

The pathogenesis of cutaneous capillary hemangioma, previously unknown, has been the subject of recent interest. It has been recognized that placenta and cutaneous capillary hemangiomas share unique immunohistochemical similarities. This has led to speculation that infantile hemangiomas could be of placental origin. Two theories have been proposed to explain this hemangioma-placental connection. First, angioblasts may proliferate toward placental tissue in the site where the hemangioma develops. A second intriguing theory is that cells of placental origin could embolize to the target areas and proliferate into the tumor (“metastatic placenta”) (8,9).

Because most infantile capillary hemangiomas regress spontaneously, it is generally appropriate to follow the tumor with observation rather than surgical resection. However, it is important to check visual acuity and do a refraction. Any amblyopia or potential amblyopia should be treated with patching the opposite eye. Most authorities also use local or systemic corticosteroids to hasten resolution.

There has been considerable enthusiasm for the use of systemic corticosteroids or intralesional injection of cortico-steroids to hasten regression of capillary hemangioma of the ocular region that have amblyopic potential (10,11,12,13,14,15,16,17,18,19,20,21,22,23,24). Most patients treated in that manner have had a good response without complications. However, occasional complications of intralesional corticosteroids include central retinal artery obstruction (16,17), linear perilymphatic subcutaneous fat atrophy (18,19), eyelid depigmentation (20), eyelid necrosis (21), and adrenal gland suppression (22,23,24). As an alternative, topical corticosteroids have been occasionally used (25,26). Intralesional interferon a-2a and -2b have also been used for capillary hemangioma that is unresponsive to corticosteroids (27,28,29). Laser therapy has been advocated for some cutaneous hemangiomas (30,31,32).

Finally, although surgical removal has historically been discouraged, there is a valid argument to manage selected periorbital capillary hemangiomas by surgical resection (33,34,35,36). An indication for surgical resection may be a circumscribed subcutaneous hemangioma that is enlarging in a very young infant. Such a tumor is destined to become very large
before it regresses and early complete surgical removal can avoid more difficult management of a larger tumor and worse amblyopia. An eyelid crease incision, with removal of the mass intact if possible, and closure of the skin with fine absorbable sutures can achieve a very satisfactory outcome (35). However, surgical resection should generally be avoided for larger superficial lesions that have extensive involvement of the epidermis. Such tumors may show satisfactory spontaneous regression.

The superficial type of capillary hemangioma (strawberry hemangioma) has typical clinical features and can show dramatic spontaneous regression.

The deep type of capillary hemangioma does not affect the epidermis and appears as a soft blue or pink subcutaneous mass.

Sometimes a capillary hemangioma can be resected entirely with a good result without waiting for it to grow to a large size.