Pediatric Clinics of North America - CIPERJ

346 ADAMS & WENTZEL
Unfortunately, there are limited prospective randomized controlled studies
that have looked at steroid dosing or efficacy. Therefore, current data
and treatment recommendations are based on retrospective studies and clinical
experience. Steroids can be given orally or topically or injected into the
lesion. The generally recommended starting dose for systemic steroids is 2 to
5 mg/kg per day of prednisolone as a single morning dose. Typically response
is assessed after 2 weeks. If there is good response, the initial dose
is maintained for 4 to 6 weeks and then tapered over 4 to 6 months. The
use of ranitidine hydrochloride, a histamine H 2 -receptor antagonist, or
one of the proton pump inhibitors to decrease gastrointestinal irritation is
recommended during steroid treatment. In addition, some centers recommend
the use of trimethoprim/sulfamethoxazole as prophylactic treatment
for Pneumocytis carinii, particularly if steroids are used at higher doses or
for an excessive period of time [35]. Intralesional corticosteroids are used
best for smaller, localized, problematic lesions rather than larger segmental
hemangiomas. Topical steroids also are used to treat localized lesions not
causing significant impairment, such as on the forehead or cheek.
Short-term side effects of systemic steroids include personality changes,
gastric irritation, diminished gain of height and weight, nonsystemic fungal
infections, and a cushingoid appearance. Boon and colleagues evaluated 62
patients receiving systemic corticosteroid therapy for problematic infantile
hemangiomas and found cushingoid facies in 71% of patients, personality
changes in 21%, gastric irritation in 21%, fungal infections in 6%, and reversible
myopathy in one patient. Diminished longitudinal growth was seen in
35% and diminished weight in 42%; however, catch-up growth occurred in
most patients [36]. Potential long-term side effects of steroid use include immunosuppression,
hypertension, significant suppression of the hypothalamic
pituitary adrenal function, hyperglycemia, ophthalmologic changes, myositis,
osteoporosis, cardiomyopathy, and neurologic changes. Therefore, patients
on systemic glucocorticoid therapy should be monitored for the development
of potential side effects. Height and weight, blood pressure checks, developmental
milestones, and adrenal suppression should be monitored closely.
Live vaccines should not be administered while on systemic glucocorticoids.
If exposure to varicella occurs, a physician should be called immediately
and varicella zoster immune globulin should be administered promptly.
Patients should be evaluated for significant febrile events (O38.5 C) and
further work-up, including blood cultures and antibiotics, may be needed.
Currently, VCR is the preferred second systemic therapeutic agent for
patients who have failed other local medical therapies or cannot tolerate steroids
[37–41]. VCR interferes with mitotic spindle microtubules and induces
apoptosis in tumor cells in vitro. Its known side-effect profile includes
peripheral neuropathy, constipation, jaw pain and irritability, electrolyte
disturbances, and neurologic problems. The insertion of a central line
is desirable because VCR is a vesicant. The experience with VCR for hemangiomas
is described in several retrospective studies with limited numbers