Lymphatic malformations and their treatment (Teenager)

What is a lymphatic malformation?

The lymphatic system is a system of very small tubes that drains fluid from various parts of the body. Lymphatic malformations occur when an area of the lymphatic system does not form properly; lymphatic malformations are often present at birth and tend to grow slowly in proportion with the body. However at certain times, when the body’s hormone levels increase, such as in puberty and pregnancy, they can grow more quickly, they can also increase in size when a patient is unwell. They are a type of vascular malformation.

What is a vascular malformation?

Vascular malformation is a general term used to describe an abnormal cluster of vessels. They can come in a number of forms.

Venous malformations

These often have a slow flow of blood running through them.

Arterio-venous malformations

These often have a higher flow of blood running through them.

Lymphatic malformations

These contain a clear fluid rather than blood; lymphatic malformations tend to be very slow flowing.

Sometimes there can be a combination of the above. In this information booklet we will be looking at lymphatic malformations.

Are they harmful?

Lymphatic malformations are benign. They are not a tumour or cancer, and whilst some people have extensive problems affecting several areas of their body, they cannot spread to other areas of the body.

Patients who have lymphatic malformations may experience the following symptoms:

• Swelling: This is often a soft lump that can appear on any part of the body; the swelling can increase and decrease at different times.
• Pain: This can be mild to severe, depending on the size and location of the malformation.
• Skin discolouration.
• Increased swelling and pain can occur in association with any viral infection.
• Rarely ulceration.

How do we treat lymphatic malformations?

We will go through several steps to allow us to make the best decision regarding your care.

Diagnosis

Once we have received a referral from the team looking after you we will, in the first instance, need to confirm the type of malformation you have and its extent as this will help us identify the correct course of treatment.

You will need to undergo a number of investigations; these may include an ultrasound scan and/or an MRI, sometimes we may request a biopsy.

MDT (Multidisciplinary Team)

We will often discuss your case with other professionals involved in treating patients with vascular malformations to make sure we are taking the best course of action in each case.

Treatment options

Once we have the results from your tests we will invite you to attend an outpatient appointment at which point the interventional radiologist (a doctor specialising in image guided operations and treatments) will discuss your treatment options with you. We may also discuss your case further with colleagues from other disciplines. After discussion we will normally offer the following options.

No treatment

If the malformation is causing you no real problems in terms of living a normal active life, it may be reasonable to leave the malformation alone. If you decide, after discussion, to take this option then we will normally discharge you from our care. This does not mean you cannot be re-referred if your symptoms increase.

Conservative treatment

We may offer conservative treatment in the form of compression garments. Some patients find relief from their symptoms by the use of a fitted medical grade, compression garment. We will refer you to your local Orthotics Department to be accurately measured and fitted.

We will often invite you back to clinic after three to six months to see if the garments are helping your symptoms.

Interventional radiology treatment

If your malformation is causing you significant problems and it is agreed that interventional radiology treatment would be your best option we will place you on our waiting list for treatment. It is important you understand that the treatments we offer are for symptom control, we cannot cure malformations at present.

Surgical treatment

If your malformation is causing you significant problems and it is agreed, after MDT discussion, that it would be best treated by surgery then we will arrange for you to be referred to the appropriate service. We will discuss this further with you in clinic.

Symptom diary

In the interim between referral and treatment we may give you a diary to allow you to log the severity and location of pain and swelling, as well as other problems you may have related to your malformation. If you are given a diary please remember to bring it with you to show to the doctor on your treatment date or when you attend clinic, as it will help to identify the areas of the malformation to target for treatment.

What does the treatment involve?

Normally lymphatic malformations are treated as a day case in Radiology Theatres; however if you need a general anaesthetic or your case is complex you will be admitted onto a ward.

On arrival you will be clerked in by one of our interventional radiology team. They will ask you to put on a hospital gown; they may also insert a cannula (small plastic tube) into a vein in your arm to administer drugs and fluids, if needed.

Finally, the consultant interventional radiologist performing the procedure will confirm your consent and answer any further questions you may have. When you are ready we will take you into theatre to have your procedure.

Sclerotherapy

This is the usual course of treatment for lymphatic malformations; the aim of this treatment is to cause the malformation to shrink in size and reduce symptoms. Sclerotherapy involves injecting a liquid agent, called a sclerosant, into the malformation by placing needles directly into it.

The needles are placed into the malformation under ultrasound guidance. Sclerotherapy normally takes between thirty minutes to an hour to complete. Adult patients will normally stay in our recovery area for a few hours to make sure they are safe to go home. You may have a compression dressing on the treated area; this can normally be removed after 24 to 48 hours. The area that has been treated may feel lumpy or harder to the touch, and is likely to be swollen; this is normal and should settle down after a couple of weeks.

Sclerotherapy is a safe and effective procedure – but, as with any medical procedure, complications can sometimes arise.

Increased swelling and pain will normally occur post procedure. The swelling and pain should generally settle down after a few weeks, during this time simple pain killers such as paracetamol or ibuprofen can be used to control the pain. We will contact you to make sure the pain and swelling post procedure is not excessive.

If the malformation is close to, or just under the skin, there is a small risk of skin blistering and ulceration. Most ulcers will heal of their own accord or may require simple dressings to keep them clean,this depends on their location. However, very rarely. a skin graft may be required to treat damaged areas of skin.

Occasionally, nerve damage can occur if the malformation is close to a nerve. This may be in the form of a feeling of numbness, tingling or pins and needles, and is usually temporary. However, rarely the nerve may be heavily bruised causing longer term symptoms or the damage could be permanent.

There is a very small risk, of bleeding and infection.

Risks are minimised by using image guidance to aim the needle into the correct area and by using a sterile technique.

Some sclerosants can cause discolouration of the skin, particularly if injected into areas of the malformation that are close to the skin surface. This will normally fade with time – but rarely, can leave some permanent discolouration.

Sclerotherapy with Doxycycline:

Doxycycline is an antibiotic and sclerosing agent that has been used in the treatment of lymphatic malformations. We will inject this drug in a similar way to other sclerosant agents; we may also inject it via a larger tube inserted directly into the area of abnormality. This will be discussed with you at clinic.

Doxycycline is not advised to be used on patients with liver or renal problems. Doxycycline can cause darkening of the colour of your skin, nails, eyes, teeth, gums, or scars.

Doxycycline may cause your skin to be more sensitive to sunlight than it is normally. Exposure to sunlight, even for brief periods of time, may cause a skin rash, itching, redness or other discoloration of the skin, or severe sunburn. It is advisable therefore that you:

• Stay out of direct sunlight, especially between the hours of 10:00am and 3:00pm, if possible, for five days post procedure.
• Wear clothing, to protect your skin including a hat. Also, wear sunglasses that protect against UVA and UVB rays.
• If you go outside, use a high quality sunblock lotion. You should continue to use this for three weeks after treatment.
• Apply a sunblock product that has a sun protection factor (SPF) number of at least 30. Some patients may require a product with a higher SPF number, especially if they have a fair complexion. Make sure you use a quality sunblock covering both UVA and UVB. If you have any questions about this, check with your doctor.
• Apply a sunblock lipstick that has an SPF of at least 15 to protect your lips.
• Do not use a sun lamp or tanning bed or booth.

Sclerotherapy with Bleomycin:

Bleomycin is a medicine that has been used for many years in the treatment of other illnesses, we may suggest this treatment if you have a lymphatic malformation in a region we would be keen to avoid any swelling, such as around the eye, for example, or if previous therapies with other sclerosants have failed. We have a separate information leaflet for this type of sclerotherapy.

Other risks

Radiation

Sometimes we use X-rays to visualise the blood vessels during the procedure. Radiation doses for the procedure are generally accepted to be low, though occasionally higher doses are required if the procedure is long or demanding.

Compartment syndrome

Sometimes if the malformation is located in a certain area of the body swelling post procedure may cause a condition called compartment syndrome. This can happen when swelling occurs in an area where there is not much room for the swelling to take place without impacting on other structures in the body, for example in the forearm or lower leg. We may arrange for you to take a short course of steroids before and after your treatment to combat this.

Follow up

Before you commence treatment we will ask you how is best for us to follow you up.

We have three methods

• By telephone: We will take the best contact number for yourself.
• By email: We will take the best contact email for yourself.
• By virtual clinic: We can talk to you via a private and secure video link. Again we will need your email to arrange a convenient time to talk.

We will contact you one week and then eight weeks after your procedure to see how you are recovering.

At one week, we will ask a number of questions which will enable us to establish how you have been after the treatment and make sure you are not having any problems post procedure or suffering from any complications.

At eight weeks, we will discuss if your symptoms have improved or not. We will use this information to determine the next best course of action for you; this is normally either further treatment or a clinic appointment.

If you have any questions or concerns in the meantime please contact

Please note if you are feeling unwell or unable to contact us regarding any urgent concerns or complications please attend your local GP Surgery, local walk in centre or your local Accident and Emergency Department if appropriate.