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Child Health Guideline Identifier

Infantile haemangioma (IH)

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Introduction

Infantile haemangiomas (IH) are benign skin tumours caused by proliferating vascular endothelial cells. They appear shortly after birth (not fully grown on day of birth), and grow rapidly to reach 80% of maximal size by 4 months. Most stop growing by 6-9 months, but some continue to grow until 18 months of age or more. Subsequently they involute over years, with the vascular component being largely resolved by 5 years of age, although some may leave permanent residua such as telangiectasia, scarring or excess fibro-fatty tissue.

Beta blockers have been used for treating IH since 2008. Their effectiveness is supported by data from randomised controlled trials and case series. Beta blockers have been used for both cutaneous and subglottic/tracheal IH and seem to be helpful in allowing ulcerated IH to heal. Beta blockers have now replaced oral corticosteroids as the gold standard treatment for complicated IH. The mechanism of action may include vasoconstriction, decreased expression of VEGF and bFGF genes, down regulation of the RAF-mitogen-activated protein kinase pathway, or triggering of apoptosis of endothelial cells.

Most IH do not require treatment as they will grow and involute without problem, however IH in some locations need treatment to prevent complications such as disruption to visual pathways, risk to the airway/feeding, ulceration, or poor cosmetic outcome. Generally the earlier that treatment is commenced the more effective it will be at preventing growth of the haemangioma and associated short and long term complications. Ideally propranolol should be commenced in the first few weeks or months of life, however IH may have some response even after the proliferative phase i.e. treatment has been effective in some children aged up to four years.

Rarely other tumours or vascular anomalies can mimic IH - if the diagnosis is not clear then a referral for an expert opinion should be made before starting treatment.

Potential Indications for Treatment

  • Life or function-threatening IH
    • Airway IH - subglottic or tracheal - these present with stridor and are at increased risk in association with IH in the "beard" distribution
    • Periorbital IH - risk of amblyopia from occlusion or pressure-induced astigmatism
    • Large IH associated with high flow and cardiac compromise eg hepatic lesions
    • Large IH interfering with physical development eg on the hand
    • Large segmental IH of the head and neck - may be associated with PHACES syndrome and abnormalities of the cerebral vasculature, great artieries and neurological abnormalities
    • Large segmental IH of the lumbosacral region - may be associated with LUMBAR or PELVIS syndromes and abnormalities of the spine or renal tract
    • Multifocal IH - may be associated with intrahepatic IH (and cardiac failure) and hypothyroidism
  • Ulcerated IH (or sites at high risk of ulceration)
    • Lip, perineum, flexures
  • IH at risk of long-term psychosocial impact or deformity
    • Eg located on lips, cheek, nose and ears
    • Exophytic IH with a 'step-edge' in a cosmetically sensitive site

IH on the scalp are at low risk of complication and do not result in areas of baldness unless associated with ulceration and scarring.

Children will be assessed by Paediatric Dermatology, ENT, Ophthalmology or Surgery depending on the location of the haemangioma. 

Propranolol

Risks and benefits of propranolol should be discussed with the family. Whilst it is still a relatively new therapy, there are a number of case series and randomised controlled trials in literature. Reviews comparing propranolol with corticosteroids have found propranolol to be more effective. Other beta blockers are also being studied. Treatment with oral propranolol solution 4mg/ml is funded under special authority for New Zealand children with complicated IH.

Benefits

  • Relatively low risk medication (compared with other treatment modalities)
  • Non-surgical approach
  • Current evidence has not shown adverse neurocognitive effects of propranolol

Risks

  • Sleep disturbance, cold extremities, diarrhoea - occasional but rarely limit treatment
  • Bradycardia and hypotension (rare)
  • Hypoglycaemia (rare but at risk if reduced feeding secondary to illness)
  • Bronchoconstriction during intercurrent respiratory illness

Topical Timolol

Topical treatment with Timolol may be considered for small (<1-2cm square) IH. Absorption of timolol is unpredictable and use on large, thick or ulcerated lesions is not advised

Assessment

Initial assessment

  1. Cardiovascular and respiratory history and examination (including heart rate and femoral pulses) by an experienced provider
  2. ECG as clinically indicated eg abnormal cardiovascular examination, bradycardia <80bpm, family history of congenital heart disease or arrhythmia, maternal connective tissue disease.
  3. Clinical photography - to include front and side-on views at discretion of team

Other investigations to consider

  • For segmental head and neck IH at risk of PHACES syndrome: echocardiogram, MRI/MRA head under GA, ophthalmology assessment
  • Beard distribution IH or symptoms of stridor: laryngoscopy
  • For segmental lumbar region IH at risk of PELVIS/SACRAL syndrome: MRI &/or USS spine, renal USS
  • For multfocal IH (>5 cutaneous IH): thyroid function, liver function tests, USS abdomen, USS/MR head, echocardiogram

Inpatient Management

  • Recommended for those <4 weeks corrected gestational age, small for gestational age, <2.5kg body weight, airway haemangioma, PHACES or other complications.
  • Baseline cardiovascular examination, observations (HR, BP) and investigations (ECG, bloods etc as above).

Give Propranolol at starting dose - 1mg/kg/day in two divided doses. Infants with PHACES syndrome may require lower doses and more gradual increases - liaison with paediatric dermatology/neurology is advised.

  • Hourly HR & BP observations for 3 hours then 4 hourly
  • Glucose to be checked after 3 hrs

Increase dose no more frequently than every 24 hours until on 2-3mg/kg/day in two divided doses

Outpatient management

In thriving healthy infants over 6 weeks of age with a normal cardiorespiratory assessment, treatment can be started in an outpatient setting with the first dose given at home.

Alternatively observation on the daystay unit for 3 hours after the first dose is a reasonable option if there are minor concerns.

Start at a dose of around 1mg/kg/day (0.5mg/kg bd) and increase after 1-2 weeks to 2-3mg/kg/day (1-1.5mg/kg bd). Dose should then be adjusted according to body weight and clinical response.

Complications

It is recommended that propranolol is administered during or after feeds and that infants are fed regularly. Parents should be aware of symptoms of hypoglycaemia (i.e. jitteriness, lethargy, sweatiness). They also need to be aware that the risk of hypoglycaemia is increased if the child is unwell, taking reduced feeds, and/or having vomiting/diarrhoea.

During intercurrent illness where oral intake is greatly reduced (eg gastroenteritis) it is recommended that propranolol is withheld until feeding improves.

Follow-up

Follow up will initially be every 1-4 weeks until there is evidence of improvement, then 3 monthly.

Treatment may be continued until the child is between 12 - 18 months or there has been adequate clinical response. Propranolol should be weaned and stopped over several weeks  to prevent rebound growth.

Information for Families

View or download the Starship Information Sheet for families 'Propranolol for treating haemangiomas'

References

DermNet NZ. Infantile Haemangioma.

Leaute-Labreze et al. Propranolol for severe hemangiomas of infancy. NEJM 2008; 358 (24): 2650-51.

Leaute-Labreze et al. A randomized controlled trial of propranolol in infantile haemangioma. N Engl J Med 2015; 372:735-746

Drolet BA et al. Initiation and use of propranolol for infantile hemangioma: report of a consensus conference. Pediatrics 2013;131:128-140

Denoyelle F. Role of Propranolol in the therapeutic strategy of infantile laryngotracheal hemangioma. Int J Ped Otorhino 2009; 73: 1168-72.

Hussain T, Greenhalgh K, McLeod K. Hypoglycaemic syncope in children secondary to beta-blockers. Arch Dis Child 2009; 94: 968-969.

Smithson SL et al Consensus statement for the treatment of infantile haemangiomas with propranolol. Australasian Journal of Dermatology May 2017; 58(2): 155-159.

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Document Control

  • Date last published: 13 June 2017
  • Document type: Clinical Guideline
  • Services responsible: Paediatric Dermatology, Paediatric ORL
  • Author(s): Diana Purvis, Murali Mahadevan, Karen Agnew
  • Owner: Diana Purvis
  • Editor: Greg Williams
  • Review frequency: 2 years

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