Hemangiomas have varied presentations, treatments

Miami - Hemangiomas and vascular malformations are relatively common in children and sometimes prove difficult to manage.

Elizabeth Alvarez Connelly, M.D., assistant professor of dermatology and pediatrics at the University of Miami School of Medicine Department of Dermatology, shared her experiences in diagnosis and management of these sometimes unsightly childhood vascular tumors and malformations at the Masters of Pediatrics Conference in January.

Hemangiomas of infancy or HOI (infantile, juvenile, strawberry, capillary and cavernous hemangiomas) are benign vascular lesions with a classic clinical history consisting of three phases - a rapid growth phase, a plateau phase and a slow involution phase. Clinically they are described as being superficial, deep or mixed lesions. In addition, they can present as focal or segmental lesions. The most common location for focal lesions is on the head and neck. Therapy is recommended for endangering or cosmetically disfiguring lesions.

Dr. Connelly tells Dermatology Times, "Statistically, hemangiomas of infancy are the most common tumors of childhood affecting 10 to 12 percent of white infants and 22 to 30 percent of pre-term infants weighing less than 1 kg at birth, with females seeming to be affected four times more than males."

Dr. Connelly says in the growth phase, one can treat superficial HOI with off-label alternatives, including superpotent topical steroids or imiquimod cream and/or pulsed dye laser, if necessary. Treatment of hemangiomas is most effective early during the proliferative phase. Dr. Connelly achieves positive results in management of the plateau phase with monthly IL Kenalog (Bristol-Myers Squibb) 10 to 20 mg/ml, but maintains, however, that results are better if lesions are treated during the proliferative phase.

Segmental HOI of the face have been associated with PHACES syndrome. PHACES syndrome consists of:

Appropriate work-up of these patients is recommended, including MRI/MRA of the head, ophthalmologic exam and echocardiogram.

The first-line treatment of segmental HOI includes oral prednisone at starting doses of 3 mg/kg/day. The treatment course varies per patient, but generally ranges from four to six months.

Ulcerations are a common complication of proliferating HOI. Standard treatment options include use of prednisone in topical, intralesional or oral forms or the use of pulsed dye laser.

Growth in treatment modalities

More recently, platelet-derived growth factor has been shown to be effective in the treatment of ulcerated HOIs. A study by Denise Metry, M.D., showed results that Dr. Connelly has been able to reproduce in her own practice. Dr. Connelly also cites a prospective study for the treatment of ulcerated HOI with a pulsed dye laser in 78 children, where the patients were treated every three to four weeks until involution or resolution of the ulcer was achieved. Ninety-one percent of the patients responded to laser treatment alone (with a mean of two treatments), and six patients with large HOI required oral steroids in combination with the laser treatment.

"Diffuse neonatal hemangiomatosis is a rare condition, has its onset at birth or shortly thereafter, and consists of multiple hemangiomas of the skin and visceral organs. Work-up should be done when more than five to six lesions are present. The liver is the most common location, but other sites include the brain, GI tract, lungs and mouth/tongue. If systemic involvement is present, there is an up to 60 percent mortality rate, due to high output cardiac failure, hemorrhage or dysfunction of vital organs," Dr. Connelly says.

Dr. Connelly suggests use of a pulsed dye laser for the treatment of capillary malformations.

"The pulsed dye laser treatment can lighten the stain, prevent proliferation after puberty, bleb formation and possible varicosities as well as prevent the darkening of lesions with age," she says.