Tumors in the subglottis cause stridor and airway obstruction that can be life-threatening, said Dr. Bauman. Additionally, hemangiomas in the perineal region are prone to ulceration and infection, she said.
Explore this issue:June 2009
Ulceration can be painful enough to require hospitalization and narcotics, added Dr. Siegfried.
Physicians should also be aware that hemangiomas may occur as isolated lesions or in association with other findings, noted Dr. Siegfried. For example, PHACES (posterior fossa, hemangiomas, arterial, cardiac, eye, sternum) involves a large segmental hemangioma on the face or neck in addition to posterior fossa abnormalities, cerebrovascular anomalies of the neck and brain, cardiac defects, and eye and sternal abnormalities.
Physicians must be aware of this constellation of symptoms and evaluate patients appropriately, noted Dr. Bauman. Patients may not exhibit all signs of PHACES, so infants with large facial hemangiomas should undergo brain imaging to exclude intracranial anomalies, she added.
Another association is SACRAL (spinal dysraphism, anogenital, cutaneous, renal/ urologic anomalies, associated with an angioma of lumbosacral localization), said Dr. Bauman.
Imaging studies are useful to evaluate hemangiomas whose extent is not readily visible. Computed tomography (CT), ultrasound (US), and magnetic resonance imaging (MRI) are all valuable studies, but MRI is preferred because it clearly shows the relationship of the tumor with adjacent tissues without causing radiation exposure, said Dr. Bauman. Ultrasound is noninvasive, and can be useful in measuring and monitoring peak blood flow through a lesion, she said.
If the tumor is small, focal, and not at a site where it interferes with normal function, it may need anticipatory guidance rather than treatment, said Dr. Siegfried. However, because physicians can’t predict how fast hemangiomas will grow, they tend to treat those at high-risk sites.
First-line treatment options include topically applied or intralesionally injected corticosteroids, said Dr. Siegfried.
Systemic corticosteroids are effective in high doses but have side effects such as irritability, reflux, high blood pressure, glaucoma, and immune and adrenal suppression, noted Dr. Siegfried. They can also interfere with sleep and can increase appetite, she said.
Systemic steroids are likely effective in about 75% of cases of infantile hemangiomas, said Dr. Bauman. Steroids are most effective when given during the early phase of rapid tumor growth, she said, adding that she uses alcohol-free prednisolone. Steroids are typically administered for several months, and many babies develop a cushingnoid appearance from temporary redistribution of fat to their back and cheeks. Linear growth may also be temporarily delayed in those taking high-dose corticosteroids for prolonged periods, added Dr. Bauman.