Approach
Treatment for asymptomatic haemangiomas in low-risk areas is generally not required as they pose no threat to function and carry minimal risk of significant cosmetic deformity. A useful social defence for patients who are particularly sensitive to comment about visible haemangiomas is to turn the conversation to the term 'birthmark' or 'beauty mark'. Early treatment or further evaluation by 1 month of age should occur for haemangiomas that are associated with:
Life-threatening complications
Functional impairment, or risk thereof
Ulceration, or risk thereof
Anomalies of other organ systems
Risk of permanent scar, disfigurement, or anatomical landmark distortion.[1][14][40] AAP Clinical practice guideline for the management of infantile haemangiomas: table 3 Opens in new window
Conventional therapy
Oral beta-blockers
While for many years the mainstay of treatment for haemangiomas was systemic corticosteroids, propranolol has emerged as the systemic treatment of choice.[14][41][42][43] The efficacy of propranolol for the treatment of infantile haemangiomas was a chance discovery first described in 2008 in two children who received the drug for cardiopulmonary conditions.[44] Since that time, many studies showing the safety and efficacy of propranolol have been completed and it is approved for infantile haemangioma. Efficacy has been demonstrated in haemangiomas in functionally or cosmetically important locations, in airway haemangiomas, in ulcerated haemangiomas, and in visceral haemangiomas.[45][46][47][48][49][50] Rebound growth has been noted after cessation of therapy, so treatment is often continued through to the time of theoretical involution or around 12 months.[51] While propranolol is considered a relatively safe drug, adverse effects have been reported, including hypoglycaemia, bronchospasm, hypotension, and hypothermia. For this reason, most academic medical centres have a protocol in place for the initiation of the medication. Because paediatric cardiologists are the most familiar with the use of propranolol in infants, they often work collaboratively with the prescribing physician. Protocols vary by institution but usually include a thorough medical history, physical examination, and post-medication monitoring of heart rate, blood pressure, and glucose in an inpatient or outpatient setting. Obtaining an electrocardiogram prior to initiation of treatment is controversial. Consensus recommendations advise a screening electrocardiogram for infants with baseline bradycardia, family history of congenital heart conditions or arrhythmias, maternal history of connective tissue disease, and patient history of arrhythmia or one auscultated during exam.[42] In general, most experts initiate propranolol in infants >5 weeks adjusted gestational age in an outpatient setting, with intermittent heart rate and blood pressure monitoring. Younger patients, or those with comorbidities, may have treatment initiated during a brief inpatient stay with closer monitoring.[1][42]
Absolute contraindications to propranolol include certain conduction defects such as sick sinus syndrome or 2nd or 3rd degree atrioventricular (AV) block. Relative contraindications include impaired cardiac function, sinus bradycardia, hypotension, 1st degree AV block, asthma or bronchial hyper-reactivity, diabetes mellitus, and chronic renal insufficiency.[1][52] The risk of hypoglycaemia is lessened if the medication is always given with food and if patients avoid prolonged fasts. For this reason many clinicians advise that the medication is not given during times of illness or other situations when oral intake is inadequate.[1][53] Because most patients receiving treatment for haemangiomas are infants, it is rare that such patients carry the diagnosis of asthma. Therefore, parents need to be counselled to stop the medication if the infants develop wheezing of any kind, including in the setting of a viral illness. Propranolol should be used cautiously in the setting of PHACES syndrome because propranolol-induced hypotension could theoretically compromise already tenuous cerebral perfusion.[1][54]
Topical beta-blockers
The success of oral propranolol led physicians to investigate the usefulness of topical beta-blocker formulations for the treatment of superficial haemangiomas. Many case series report successful treatment with topical timolol maleate 0.5% gel, especially when used in the treatment of superficial infantile haemangiomas.[55][56][57] This treatment can be considered when systemic treatment is not warranted or is contraindicated.
Systemic corticosteroids
Systemic corticosteroids are still occasionally used instead of beta-blockers for infantile haemangiomas and can be used as an adjunct to other treatments including oral propranolol.[58] Depending on the response and the age of the patient, anticipated duration of therapy may continue from 6 to 12 months. Rebound growth of haemangioma has been well documented while tapering oral corticosteroids, so close clinical follow-up is required.[2][16][59][60]
Common adverse effects of prolonged, high-dose oral corticosteroids include cushingoid facies, irritability, gastric upset, thrush or candidal nappy dermatitis, and diminished height and weight gain. About 90% of children with diminished growth return to their pre-treatment growth curve for height by 2 years of age. Additional concerns, although rare, include an increased risk of serious infections due to systemic immunosuppression. For this reason, live vaccines are contraindicated. Other worrisome adverse effects include hypertension and hypothalamic-pituitary-adrenal (HPA) axis suppression. HPA axis suppression results in difficulty in weaning off corticosteroids.[61][62]
Intralesional corticosteroids
If the haemangioma is well localised without deep extension, intralesional corticosteroids represent an additional treatment option. In general, intralesional treatments are spaced about 1 month apart. A range of corticosteroids has been used, although triamcinolone is a typical agent.[63] The systemic adverse effects of oral corticosteroids are avoided in most cases.[64][65]
There has been a single, small clinical trial with 75 participants comparing the efficacy of no treatment, systemic corticosteroids, and intralesional corticosteroids in the treatment of problematic infantile haemangioma. The authors concluded that both treatment arms using systemic and intralesional corticosteroids, compared with the no treatment arm, showed significant efficacy with regard to reduction in lesion size. Complications were few.[66]
Intralesional injection of corticosteroids to periorbital infantile haemangioma has resulted in retinal and ophthalmic artery occlusion leading to vision loss. Presumably, injection pressures may result in retrograde flow of particulate matter into these vessels. There has also been a report of eyelid necrosis. Some of these problematic locations for even small infantile haemangioma include the upper eyelid, ear, lip, and nose. In these circumstances, referral to an ophthalmologist or other specialist is recommended.[1]
Advanced and adjunct therapy
Surgical intervention
Whether and when to excise an infantile haemangioma is decided after weighing the risk of waiting versus the benefit of immediate excision. If conservative treatment has been inadequate, a child with a proliferative infantile haemangioma that threatens functional and cosmetic integrity is a good candidate. Excision may also be necessary in the setting of ulceration or bleeding. The nature of some infantile haemangiomas causes them to lend themselves to easy surgical intervention: for example, small pedunculated lesions with a narrow base that would leave minimal scar after excision.[2] Surgery is more often used to improve cosmetic appearance after involution is complete. For example, a child entering school with redundant, unsightly fibro-fatty tissue is a good candidate for surgical intervention.
Inappropriate surgical intervention can increase the morbidity of infantile haemangiomas. In one review, children with a complicated parotid gland haemangioma (one with rapid proliferation leading to facial distortion and obstruction) who underwent observation and conservative medical management with corticosteroids and interferon had the best outcomes with no major medical complications. In contrast, severe complications were seen following surgical interventions, including temporary or permanent facial nerve paralysis, Frey's syndrome, salivary and arteriovenous fistula, haematomas, scarring, facial asymmetry, and one death.[67]
Pulsed dye laser
The pulsed dye laser (PDL) works via selective destruction of blood vessels. However, it has limited depth of penetration, so it is not effective for deep lesions. One randomised controlled trial assessed early PDL therapy versus no treatment and showed that at 1 year the PDL was more likely to result in clearance of uncomplicated infantile haemangiomas. However, side effects such as atrophy and hyperpigmentation were noted.[68] Many experts agree that pulsed dye laser should only be used for ulcerated haemangiomas and to treat residual telangiectasias after involution.[1][13][69]
Care for ulcerated haemangiomas
Burow's solution compresses may be used as adjunct for gentle debridement. Petrolatum-impregnated gauze is helpful for discomfort from lesions in the perineal area. Becaplermin, a recombinant human platelet-derived growth factor, promotes wound repair cell proliferation and granulation tissue formation. Topical antibiotics, including mupirocin and metronidazole, may be applied under a thin hydrocolloid dressing to prevent colonisation of abrasions. Oral antibiotics should be given if secondary infection is present. Mild analgesics such as paracetamol and lidocaine may be applied to the affected area for pain management. The pulsed dye laser is also effective for treatment of ulcerated haemangioma.[70] Many clinicians will institute treatment with either a topical or systemic beta-blocker at the time of ulceration if the lesion has not been previously treated.
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