The parents of a white infant, aged 3 months, were concerned about a lesion on the child’s scalp. A very small bruise-like lesion was present at birth; the pediatrician thought it was caused by trauma from delivery.
At age 3 weeks, however, the “bruise” became a pink patch that grew into a firm, rubbery, red, and slightly blanchable nodule over the next several months. The lesion recently developed a painful ulceration that began crusting over. On physical examination, the baby appeared healthy. Complete physical and neurologic exam was significant only for the red, ulcerated nodule.
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Infantile hemangiomas are the most common benign tumor in children, affecting 10% to 12% of white infants at age 1 year. Female sex, prematurity, advanced maternal age, Caucasian race, placenta previa, pre-eclampsia, and multiple-gestation pregnancies are risk factors for the development of infantile hemangiomas. Hereditary factors may play a role since approximately 10% of patients have a positive family history.1,2
Infantile hemangiomas can be described as deep, superficial, or mixed. A deep hemangioma is a subcutaneous compressible tumor, often with an overlying blue tint and superficial telangectasias. A superficial infantile hemangioma appears as a bright-red rubbery nodule, papule, or plaque. A mixed infantile hemangioma combines features of both.
Infantile hemangiomas are not present at birth but become evident at age 2 to 3 weeks. However, as many as 30% to 50% of infantile hemangiomas develop from a precursor lesion that is present at birth. Precursor lesions have variable appearances and may appear as telangectasias, an area of pale skin, bruise-like, scratch-like, or ulceration. A fully formed infantile hemangioma present at birth is not an infantile hemangioma and is considered a congenital hemangioma instead.1,2
After becoming evident, the infantile hemangioma enters a proliferative growth phase. Approximately 80% of superficial infantile hemangiomas complete their growth by age 5 months. Deep hemangiomas typically proliferate for one month longer. However, large hemangiomas, particularly those with a deep component, may continue to proliferate for up to one to two years.
When the growth phase is completed, the infantile hemangioma enters the involution phase. During the involution phase, the infantile hemangioma flattens, shrinks in size, becomes less red, and ultimately disappears. Infantile hemangiomas involute at a much slower rate than they proliferate.
The rate of complete involution is estimated to be 10% per year. For example, 40% of infantile hemangiomas will be completely involuted by age 4 years, and 80% completely involute by age 8 years. Involution can result in completely normal skin, but some individuals may be left with atrophy, scar, fibrofatty masses, or telangectasias.1,2
Ulceration is the most common complication of infantile hemangiomas and occurs in 16% of patients. This complication may result in pain, bleeding, scarring, and/or infection.1,2
Hemangiomas involving large segmental portions of the face may be associated with the “PHACES” syndrome, which involves Posterior fossa malformations, Hemagioma, Arterial anomalies, Cardiac anomalies and aortic coarctation, Eye abnormalities, and Sternal clefting and supraumbilical abdominal raphe. When the beard area of the face is involved, there is risk of hemangiomas involving the upper airway, which can lead to airway obstruction. PHACES syndrome may be the most common neurocutaneous syndrome.1,2
Other particularly problematic infantile hemangiomas are those involving the nasal tip, as this can lead to permanent disfigurement, and those that involve the periorbital skin and eyelids, which can obstruct the visual axis. There is a risk of associated spinal dysraphism when infantile hemangiomas are located perianally and extend towards the gluteal cleft or are located over the lumbosacral spine.1,2
Visceral involvement is another complication of infantile hemangiomas, with the liver being the most commonly involved organ. It is currently recommended that infants with five or more cutaneous hemangiomas undergo a liver ultrasound. Hepatic hemangiomas are usually asymptomatic but occasionally lead to high-output heart failure, hepatomegaly, or thrombocytopenia.1,2
The diagnosis of infantile hemangioma is made by classic physical-examination findings and patient history. Biopsy is rarely indicated. Histologically, infantile hemangiomas stain for GLUT-1 (erythrocyte-type glucose transporter); all other forms of hemangioma are negative for GLUT-1.1, 2
The differential diagnosis includes other vascular tumors. Congenital hemangiomas are fully formed at birth and may or may not involute. Other vascular tumors in the differential include kaposiform hemangioendothelioma, tufted angioma, and pyogenic granuloma. Each of these tumors has distinctive clinical and histopathologic characteristics that distinguish it from an infantile hemangioma.1,2
Most infantile hemangiomas do not require active treatment, as the lesions will involute spontaneously at a rate of approximately 10% per year. Parental education and guidance is critical during this period. No therapy is typically recommended until the individual is school-aged.
By the time the patient enters school, the bulk of the infantile hemangioma will have regressed spontaneously, at which point vascular laser therapy can be offered to improve the appearance of any residual red color. Occasionally, patients are referred to a plastic surgeon for excision of residual fibrofatty tissue.1,2
Wound-care measures, antibiotics, and as-needed analgesics are used to manage ulcerated infantile hemangiomas. Pulse-dye laser may also be useful.1,2
When infantile hemangiomas involve the nasal tip, interfere with visual axis, involve the airway, obstruct the ear canal, or have the potential to cause any morbidity or mortality, options include intralesional or oral corticosteroids, oral propranolol (Inderal, InnoPran, Pronol), vincristine (Oncovin, Vincasar), or surgical excision.1,2
Oral propranolol has proven to be a very safe and effective medication for the management of problematic hemangiomas. This observation is supported by recent studies demonstrating that oral propranolol is more effective, requires fewer surgical referrals after treatment, is better tolerated, is more cost-effective, and has fewer adverse effects that systemic corticosteroids, which have historically been considered the gold standard for medical management of infantile hemangiomas. Oral propranolol can be considered a first-line agent.3
The ulcerated hemangioma in this case was managed conservatively with topical white petrolatum. The ulceration was completely healed within one month. By age 3.5 years, the hemangioma had completely regressed with only a small scar at the site of prior ulceration. The patient’s hair covered the scar completely, and no further management was required.
Adam Rees, MD, is a first-year dermatology resident at Baylor College of Medicine in Houston.
1. Hurwitz S. Vascular disorders of infancy and childhood. In: Paller AS, Mancini AJ. Clinical Pediatric Dermatology: A Textbook of Skin Disorders of Childhood and Adolescence. 4th edition. Philadelphia, Pa.: Elsevier Saunders; 2011:268-275.
2. Maguiness SM, Frieden IJ. Vascular birthmarks. In: Schachner LA, Hansen RC. Pediatric Dermatology. Philadelphia, Pa.: Elsevier Saunders; 2011:1135-1141.