Bleomycin Sclerotherapy for Lymphatic Malformations

Bleomycin sclerotherapy is a procedure used treat some particular types of vascular malformations. Several medicines are used for sclerotherapy. Bleomycin is just one of those options.

It is usually suggested as a treatment for specific kinds of lymphatic malformations (microcystic lymphatic malformations), or when standard sclerotherapy has failed.

Bleomycin may also be useful in superficial malformations where the skin is directly involved as it is less likely to cause skin blistering or scarring. It is also considered for the treatment of malformations in areas where the doctors are keen to avoid swelling, such as behind the eye.

Sclerotherapy is carried out in the Radiology department by a paediatric Surgeon or by a doctor (radiologist) who specialises in using imaging.

Several different medicines can be used for sclerotherapy. Bleomycin is just one of those options. Most sclerotherapy medicines act by causing irritation or inflammation of a malformation, followed by scarring. Bleomycin is an antibiotic drug that can also cause gradual cell breakdown or destruction, so that malformations slowly become less bulky. As it causes no immediate irritation of the malformation, it is often less painful and it usually causes very little swelling. It causes less inflammation of the nearby tissues, so the risks of skin or nerve damage are much reduced. It takes longer to work than most other sclerotherapy medicines, and is not effective in every case. Bleomycin may be better at shrinking microcystic lymphatic malformations than the other medicines available. It may be useful for treating malformations in areas where swelling may be dangerous, such as near the eye or airway or for malformations that are in the skin surface or near important nerves.

Bleomycin is a medicine that has been used for many years to treat other conditions. In a very few cases, when it has been used in much higher doses and has been injected straight into the bloodstream, patients have developed some scarring of their lungs several months later, meaning that they cannot take in as big a breath as previously. Sometimes this is permanent. Doctors think this is extremely unlikely to happen in children where bleomycin is being used for sclerotherapy, as the doses are much, much lower and the medicine is not given in the same way. However, we will check your child’s lungs before the procedure, usually using a chest x-ray and some simple breathing tests. This gives a baseline picture of your child’s healthy lungs. If we need to test their lungs again later, we have the baseline picture against which to compare the new picture.

Six to 12 months after your child’s final bleomycin sclerotherapy procedure, they will be offered an appointment with a specialist lung doctor who will also repeat the lung tests to confirm that nothing has changed.

Very rarely, bleomycin can cause some discolouration of the skin or nails or make some hair fall out. This usually, but not always, gets better with time. We think that the skin is more likely to be affected if there are monitoring leads or plasters stuck to the skin at the time of treatment. During your child’s procedure, we will be very careful with how we attach monitoring leads, cannulas and bandages to your child’s skin. Scratching of the skin during the first 48 hours after treatment may cause skin marking. We recommend you buff your child’s fingernails very short before the procedure to minimise the risk of scratching.

You will already have received information about how to prepare your child for the procedure in your admission letter. You will need to come to hospital before the sclerotherapy so that your child can have a pre-admission assessment to check that they are well enough for the procedure and to confirm that their lungs are healthy. This may involve taking blood samples, photographs and other tests to measure lung function. This appointment may be booked for several weeks before sclerotherapy or for the day before the procedure.

You will also meet the paediatric surgeon, who will explain the procedure in more detail, discuss any questions you may have and ask you to sign a consent form giving permission for your child to have the sclerotherapy. If your child has any medical problems, please tell the doctors. An anaesthetist will visit to talk to you about your child’s anaesthetic.

As bleomycin could harm an unborn baby, we will ask all girls aged 12 years or older about their periods and any chance that they could be pregnant. We will also ask for a urine sample to carry out a pregnancy test.

Sclerotherapy is almost always carried out while your child is under a general anaesthetic. It is important that your child does not eat or drink anything for a few hours before the sedation or anaesthetic. This is called ‘fasting’ or ‘nil by mouth’. Fasting reduces the risk of stomach contents entering the lungs during and after the procedure. You will be informed the night before the procedure of the time that your child should be ‘nil by mouth’ – in other words, have nothing to eat or drink before the anaesthetic. Fasting times are provided in your admissions letter – in broad terms, this is six hours for food (including milk), four hours for breast feeding and two hours for clear fluids before the procedure.

It is equally important to keep giving your child food and drink until those times to ensure they remain well hydrated and get adequate nutrition. This may involve waking your child in the night to give them a drink which we recommend.

Once your child is under general anaesthetic, the radiologist/paediatric surgeon will check the malformation using an ultrasound scan and sometimes x-rays. Using the ultrasound scan as a guide, they will insert a small needle through the skin into the malformation. They will then inject the medicine through the needle. Finally, the radiologist removes the needle. No stitches or dressings are needed – you will only be able to see a few pinpricks in the skin.

Your child will be having sclerotherapy under general anaesthetic. Every anaesthetic carries a risk but this is extremely small. The area treated may look a little more swollen than usual after the procedure. The area may feel bruised and sore for a few days, but your child will have pain relief.

There is a slight risk of the malformation developing an infection after the treatment. Your child will need a short course of oral antibiotics if this happens. There is a slightly higher risk than usual of the malformation developing blood clots inside them. This causes sudden swelling and bruising, which may be uncomfortable. If this happens, the bruising will slowly settle. It will not affect how well the treatment works.

Very rarely, bleomycin can cause some discolouration of the skin or nails or make some hair fall out. This usually, but not always, gets better with time. Scratching of the skin should be avoided, if possible, for the first 48 hours after the procedure to reduce the risk of skin marking.

Sclerotherapy works for most children but not all of them. Experience helps us understand which malformations are more likely to respond. If the doctors feel that the sclerotherapy treatment has not been very successful, they might suggest surgery, perhaps in combination with further sclerotherapy.

Sclerotherapy seems to be the best treatment option of lymphatic malformations as it carries lower risks than open surgery. It does not cause any scarring and the malformation does not appear to grow back after treatment.

However, as every child with a lymphatic malformation is different, sclerotherapy may not be the best option. Sometimes a combination of sclerotherapy and surgery might be suggested. Your doctor will explain the options for treatment suitable for your child.

Your child will return to the ward after they have recovered from the anaesthetic. Some children feel sick and vomit after a general anaesthetic. Your child may have a headache or sore throat or feel dizzy, but these side effects are usually short-lived and not severe. Your child can start eating and drinking as normal once they feel like it.

Any bruising and swelling of the malformation will only last for a few days. Bleomycin usually causes less swelling than other sclerotherapy drugs. Children’s pain relief medicine is usually enough to deal with any pain.

You will get an appointment to come back to the hospital so the doctors can check on your child’s progress and decide whether the treatment has worked.

You will be able to go home once the doctors are happy that your child is recovering well. The swelling should start to go down after a few days. They should be able to go back to school after a few days.

It is recommended that any routine vaccinations that your child may need are postponed for three months after the bleomycin procedure as the bleomycin may temporarily make them less effective. Please discuss this with your family doctor (GP)/ Paediatrician and take their advice as to whether the benefit of the vaccination outweighs this risk.

The doctors will tell you if your child needs further sclerotherapy procedures and if so, when. Your child might need to have a routine outpatient appointment a few weeks afterwards before this decision is made.

Your child might need several sclerotherapy procedures to reduce the malformation. As sclerotherapy causes swelling and discomfort, it is safer to carry out several smaller treatments limiting the effects each time. Sclerotherapy will not ‘cure’ the malformation but it should shrink it significantly. Occasionally children need to have further sclerotherapy as they get older and some may need surgery to improve the final result.

At the end of your child’s treatment program, the total dose of bleomycin that they have received will be documented clearly and given to keep as a record of their treatment. This information may be important in later life.

You should call the hospital if:

• Your child is in a lot of pain and pain relief does not seem to help.
• The treated area is unusually hot, red and painful and your child is generally unwell with a high temperature or not eating or drinking as usual.