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Haemangioma

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Introduction

Haemangiomas are benign skin tumours caused by proliferating vascular endothelial cells. They appear shortly after birth, and grow rapidly to reach 80% of maximal size by 3 months. Most stop growing by 6 months, but some continue to grow until 18 months of age or more. Subsequently they involute over years, 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 infantile haemangiomas since this effect of propranolol was first reported in 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 haemangiomas and seem to be helpful in allowing ulcerated infantile haemangiomas to heal. Beta blockers have now replaced oral corticosteroids as the gold standard treatment for complicated infantile haemangiomas. 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.

For many haemangiomas treatment is not required as they will grow and involute without problem, however haemangiomas 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. There does seem to be response from haemangiomas even after the proliferative phase i.e. treatment has been effective in some children aged up to four years.

Potential Indications for Treatment

  1. Subglottic and/or tracheal haemangioma
  2. Periorbital or retrobulbar haemangioma
  3. Perioral haemangioma
  4. Large facial segmental haemangiomas (can be associated with PHACES syndrome)
  5. Nasal tip, ear, lip, central cheek, large facial haemangioma
  6. Nappy area and flexural haemangiomas - risk of ulceration
  7. Lumbrosacral haemangioma (can be associated with underlying spinal and urogenital anomalies)
  8. Multiple haemangiomas (ie visceral)
  9. Ulcerated haemangiomas

Children will be assessed by Paediatric Dermatology, ENT or Ophthalmology depending on the location of the haemangioma. Most children can commence treatment in Daystay, however for very young babies (i.e. <6 weeks) or those with PHACES or airway involvement admission overnight should be considered. This would be at the discretion of the managing team. Children will be admitted under ENT or Dermatology/General Paediatrics.

Propranolol

Risks and benefits of propranolol should be discussed with the family. Whilst it is still a 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 infantile haemangiomas.

Benefits

  • Relatively low risk medication (compared with other treatment modalities)
  • Non-surgical approach

Risks

  • New treatment option. Long term neurodevelopmental and cardiovascular effects are uncertain, but studies are reassuring to date.
  • Bradycardia
  • Hypotension
  • Hypoglycaemia (esp if reduced feeding secondary to illness)
  • Sleep disturbance
  • Bronchoconstriction

Investigations

Initial Investigations

  1. Cardiovascular and respiratory history and examination (including femoral pulses)
  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 at the discretion of the managing team:

  • For segmental head and neck haemangiomas at risk of PHACES syndrome: echocardiogram, laryngoscopy & bronchoscopy, MRI/MRA head under GA, ophthalmology assessment
  • For segmental lumbar region haemangiomas at risk of PELVIS/SACRAL syndrome: x-ray &/or USS spine, renal USS
  • For multiple haemangiomas: thyroid function, liver function tests, USS abdomen, USS/MR head, echocardiogram

Inpatient Management

Consider for those <6 weeks, 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 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 when stable until on 2-3mg/kg/day in two divided doses

Daystay Management

Admit to Daystay in the morning (0830hrs).

Baseline cardiovascular examination, observations (HR, BP) ECG if required.

Give Propranolol at starting dose - 1-1.5mg/kg/day in two divided doses

  • Hourly HR & BP observations for 3 - 4 hours.
  • If observations stable, then discharge home. 
  • Blood glucose measurement if clinical concern re hypoglycaemia eg pale, jittery.

Patient to return in 4-7 days for increase in Propranolol dose to 2-3mg/kg/day in two divided doses

Complications

Parents to 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.

It is recommended that propranolol is administered during or after feeds and that infants are fed regularly. During intercurrent illness where oral intake is greatly reduced (eg gastroenteritis) it is recommended that propranolol is withheld until feeding improves.

Follow-up

Dermatology patients
Outpatient review with dermatology to be arranged within 4-6 weeks to assess response.

ENT patients
Repeat L&B to be scheduled at 1 week, 4 weeks, and then every 6 months until age 18 months. If no response then consider introduction of corticosteroids or other treatments.

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

Parent info  Click on the image to view or download a copy of the document

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.

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Document Control

  • Date last published: 13 August 2015
  • 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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