Treating subglottic haemangiomas with propranolol

A haemangioma is a collection of small blood vessels that occur under the skin, sometimes called ‘strawberry marks’. Similar collections of blood vessels can occur in the air passage beneath the vocal cords. These are called "subglottic haemangiomas". Children with subglottic haemangioma will usually have noisy breathing but normal cry.

Nearly half of the patients with a subglottic haemangioma also have a skin haemangioma. In general, haemangiomas are not usually obvious at birth, but become apparent within a few weeks of birth. They grow rapidly in the first three months and can cause symptoms.

Subglottic haemangiomas are diagnosed using a microlaryngoscopy and bronchoscopy (MLB) test. An MLB allows the doctor to look into your child’s airway (larynx and bronchi) using a small telescope. This telescope is contained in a piece of equipment called an endoscope.

Usually haemangiomas grow up to six to ten months of age, then tend to have a rest period and then begin to shrink.

How are subglottic haemangiomas treated?

The treatment options for subglottic haemangiomas include medical treatment (steroids or propranolol) or surgery to remove the haemangioma, depending on its size and position.

The traditional medical treatment has been steroids. The steroid treatment usually needs to be given for five to six months and then gradually stopped. Propranolol is now being used as an alternative to steroid treatment or if steroids have not worked very well previously. Propranolol is not suitable for everyone and children started on propranolol treatment have to be monitored closely, before, during and after treatment.

What is propranolol?

Propranolol is a beta blocker. Nerve endings release a chemical called noradrenaline which stimulate beta adrenergic receptors. Beta blocker medications block the beta adrenergic receptors and stop them being stimulated.

How does propranolol help with subglottic haemangioma?

By blocking the beta adrenergic receptors, propranolol can tighten the blood vessels, reducing the amount of blood flowing through them. This is especially helpful in haemangiomas, as it reduces the blood flow through them. Also the cells that cause the growth of the haemangioma are also affected by propranolol so the subglottic haemangioma starts to reduce in size.

What are the side effects of propranolol treatment?

Propranolol is associated with some side effects which happen rarely. These include:

  • bradycardia (slow heart rate)

  • hypotension (low blood pressure)

  • bronchospasm (temporary narrowing of the airway, leading to wheezing and coughing)

  • peripheral vasoconstriction (reduced blood flow to fingers and toes, making them feel cold)

  • weakness and fatigue

  • sleep disturbance and sometimes nightmares

  • hypoglycemia (low blood sugar)

You should report any of these to your doctor as the dose of propranolol may need to be altered or maybe stopped.

What tests are needed before starting propranolol treatment?

Before your child is started on propranolol treatment, we need to do a range of tests including blood and urine tests, electrocardiogram (ECG) and an echocardiogram (ECHO). Other investigations may also need to be done.

What happens when my child starts treatment?

For the first few hours after starting treatment, your child will be closely monitored. We need to check your child’s pulse and blood pressure every half hour to ensure that they have not dropped too low. This close monitoring allows the doctors to be sure that your child can tolerate the dose prescribed. For the first two weeks after starting treatment, your child’s blood pressure needs to be checked twice a week. After that, they will need weekly blood pressure checks for the duration of the treatment.

What dose should my child take?

Your child’s dose is worked out depending on their weight. This will mean that the dose may change over time as your child grows. At GOSH, we supply propranolol as a 5mg/5ml liquid - that is 5ml of liquid contains 5mg of the active ingredient.

The British Society for Paediatric Dermatology has highlighted that there are three strength of propranolol oral solution: 5mg/5ml, 10mg/ml and 50mg/ml. Following incidents where propanolol 50mg/5ml has been dispensed by pharmacists instead of 5mg/5ml, parents are asked to check the dose with your pharmacist before giving it to your child. Ideally any other strength should be avoided to minimize any risk of the wrong dose being given to your child.

How long does treatment last?

In general, treatment with propranolol up to 12 months of age is usually sufficient to reduce the subglottic haemangioma. Propranolol treatment should not be stopped suddenly so your child’s medicine will be reduced gradually over a period of weeks.

What happens next?

Your child’s subglottic haemangioma will be monitored regularly, using MLB as before. The first MLB is carried out six weeks after starting propranolol to check whether it is shrinking. Follow-up MLB tests are carried out every three months until the subglottic haemangioma has resolved completely. 

Compiled by: 
The ENT department and Birthmark Unit in collaboration with the Child and Family Information Group.
Last review date: 
April 2016
Ref: 
2016F1094

Disclaimer

Please read this information sheet from GOSH alongside the patient information leaflet (PIL) provided by the manufacturer. If you do not have a copy of the manufacturer’s patient information leaflet please talk to your pharmacist. A few products do not have a marketing authorisation (licence) as a medicine and therefore there is no PIL.

For children in particular, there may be conflicts of information between the manufacturer’s patient information leaflet (PIL) and guidance provided by GOSH and other healthcare providers. For example, some manufacturers may recommend, in the patient information leaflet, that a medicine is not given to children aged under 12 years. In most cases, this is because the manufacturer will recruit adults to clinical trials in the first instance and therefore the initial marketing authorisation (licence) only covers adults and older children.  

For new medicines, the manufacturer then has to recruit children and newborns into trials (unless the medicine is not going to be used in children and newborns) and subsequently amend the PIL with the approved information. Older medicines may have been used effectively for many years in children without problems but the manufacturer has not been required to collect data and amend the licence. This does not mean that it is unsafe for children and young people to be prescribed such a medicine ‘off-licence/off-label’. However, if you are concerned about any conflicts of information, please discuss with your doctor, nurse or pharmacist.