About Vascular Birthmarks

Vascular birthmarks are caused by abnormal growth or formation of blood vessels and vessel cells.

There are two main types of vascular birthmarks: hemangiomas (strawberry birthmarks) or vascular malformations.

Haemangiomas are caused by overgrowth of the cells providing the lining for vessels in the affected area. Vascular malformations are caused when vessels are formed with an abnormal or weak wall structure. Both are birth-associated lesions, with lesions appearing at or after birth in most cases.

When do hemangiomas (strawberry birthmarks) occur?

Haemangiomas (strawberry nevi), appear when the endothelial cells (vessel lining cells) within vessels in a certain area of the body are stimulated to increase in number. This often occurs in the head and neck areas, but can occur anywhere on the body. The exact trigger is unknown.

Stimulation of the cells usually occurs in the first month after birth. There may be a small red patch present at birth. The endothelial cells grow rapidly once stimulated and lead to a visible enlargement of the lesion in the weeks following birth.

The rapid growth usually stabilizes by one year. This is followed by a slow resolution over the following years. Half of haemangiomas disappear by five years of age and 70% by seven years. Some hemangiomas are visible at birth and some can disappear earlier than a couple of years.

What do hemangiomas look like?

Typical superficial haemangioma

Haemangiomas appear as bright red fleshy tumours if they are within the top skin layers. Deeper hemangiomas can appear as a bruise or swelling under the skin. On examination they feel firm and fleshy. The image shows a typical superficial hemangioma.

When do vascular malformations occur?

Vascular malformations are caused by abnormal formation in the structure of blood vessels in the affected area. This occurs during foetal development in the uterus. The lesion is often present at birth, but can appear later during the teenage years, following pregnancy, or after trauma to the lesion.

Depending on the type of vessel that is abnormally formed, they are classified as follows:

  • Capillary malformation / port wine stain: caused by abnormal hair vessels in the skin.
  • Venous malformation: caused by abnormal veins.
  • Lymphatic malformation: caused by abnormal lymphatic vessels. Lymphatic malformation lesions consisting of a large cyst (sac) in the neck are known as cystic hygromas.
  • Arterio-venous malformation: an abnormal direct connection between arteries and veins.
  • Combined vascular malformation: Any combination of the above is possible.

A vascular malformation usually grows with the patient and does not have a rapid growth phase as seen in haemangiomas. They usually do not disappear over time like haemangiomas.

What does a vascular malformation look like?

Venous malformation lower lip

Vascular malformations are usually soft and compressible apart from multi-centric (small cyst) lymphatic malformations.

They are filled with blood or lymphatic fluid. Arterio-venous malformations have a pulse that can be felt or heard with a stethoscope.

Vascular malformations may be more filled or swollen after activity or due to the effect of gravity, especially in the arms or legs.

Cystic hygroma of the neck

Swelling of the lesion can cause it to be painful. These images show a venous malformation lower lip and cystic hygroma of the neck.

Are these lesions dangerous?

Vascular malformations and hemangiomas are benign, (good natured) lesions. Most lesions do not cause symptoms although the lesion is often markedly different to normal tissues which can make them obvious.

Hemangiomas can outgrow their blood supply causing ulceration and spontaneous bleeding. A growing lesion around a body opening such as the nostrils or ear can cause obstruction or prevent normal hearing development. Eyelid haemangiomas can prevent normal development of sight and cause pressure on the eyeball. Neck haemangiomas can sometimes lead to airway obstruction. Very large malformations can cause trapping of platelets with clotting (bleeding) abnormalities.

Vascular malformations can cause pain, swelling and discomfort due to engorgement and swelling of the abnormal vessels. Underlying bone and soft tissues may be stimulated to grow abnormally fast in relation to surrounding tissues in some types of malformations. Lymphatic malformations affecting the skin can cause recurrent skin infections and scabbing and weeping from blisters. Arterio-venous malformations can “steal” blood supply from tissues lower down leading to poor oxygen supply.

How are these lesions diagnosed?

95% of vascular anomalies can be diagnosed without the need for special investigations. Determining when the lesion appeared (at birth, or after birth), asking about the growth pattern (slow, with the patient, or exponential), and examining the lesion clinically will allow distinguishing between a hemangioma and a vascular malformation.

When patients are considered for treatment, an MRI scan (scan taking multiple images using a magnet), is usually the first choice in investigation although ultrasound, angiography and MRA (MRI with the specific evaluation of blood flow), are sometimes needed.

Should all hemangiomas be treated?

Hemangiomas are often not treated actively as a large percentage will disappear over time. This is appropriate in non-complicated lesions hidden from view. Not all haemangiomas disappear, and it takes a number of years for that to happen. Haemangioma lesions that affect the lips, nose and eyelids areas may have a lower disappearance rate.

All complicating haemangiomas should be considered for active treatment. Patients should have the benefit of discussing treatment options with a knowledgeable consultant with a specific interest in vascular birthmarks.

Should all vascular malformations be treated?

Vascular malformations are usually permanent. Lesions without symptoms or complications can be left untreated. Some lesions can change characteristics during puberty or following pregnancy. Patients should have the benefit of discussing the options in treatment of their/their child’s vascular malformation with a knowledgeable consultant with a specific interest in vascular birthmarks.

How can hemangiomas be treated?

Timoptol skin ointment

For selected small, but growing hemangiomas timolol gel would be suggested as a treatment option. Alternative treatment options include systemic propranolol treatment, bleomcyin injection and surgery.

What is timolol maleate?

Timolol maleate is a beta-blocker medication, which means it blocks receptors, or points on the cells of vessels. By blocking these, timolol can make blood vessels tighten, reducing the amount of blood flowing through them.

This affects blood flow through the hemangioma and also in a yet unknown way blocks further growth of the hemangioma cells.
The gel ointment used is called Timoptol®-LA 0.5% originally used for treating raised eye pressure.

How is the gel applied?

The dose of topical timolol is one drop once to twice a day. You should apply the drop directly to the hemangioma and carefully spread it with your finger to cover the surface of haemangioma.

Please wash your hands with soap and water before and after putting timolol on your child’s skin.

Are there any side effects with topical timolol treatment?

Skin application of timolol is safe and side effects are extremely rare.

However, you should report any of the following to your doctor as the dose of timolol may need to be altered or on very rare occasions, stopped. We will explain the side effects to you at the first consultation.

Very rare side effects, which have not occurred in any patients to date, but may be possible includes:

  • bradycardia (slow heart rate)
  • hypotension (low blood pressure)
  • bronchspasm (temporary narrowing of the airway, leading to wheezing and coughing)
  • peripheral vasoconstriction (reduced blood flow to the extremities, such as fingers and toes, making them feel cold and turn a blue colour)
  • weakness and fatigue, showing as floppiness and disinterest in surroundings
  • sleep disturbance
  • hypoglycemia (low blood sugar)

If you have any concerns about these side effects, please discuss them with us.

How long does the treatment last?

We expect treatment to continue for up to six months or one year.

Your child will be reviewed 4-6 weekly after starting the treatment so the doctors can decide whether the treatment is working.

Who do I contact if I have any concern or questions?

Arrangements for follow up appointments and further care will be made when your child is seen in the James Cook Birthmark Clinic. If you have any queries or concerns, contact Mr Muir’s secretary on 01642-854216

Oral propranolol treatment

Rapidly growing or problematic hemangiomas need treatment with propranolol treatment and/or bleomcyin injection or rarely surgery.

All these options would be discussed in the birthmark clinic appointment.

What is propranolol?

Propranolol is a beta-blocker medication, which means it blocks receptors, or points on the cells of vessels. It is normally used to lower high blood pressure.

How is it given?

You will get a prescription for Syprol® propranolol solution in a liquid form. The amount of millilitres to be given will be calculated and prescribed. The medication is given two or three times daily. You should give a feed shortly after giving each dose and not give a dose if your baby is not feeding well or vomiting.

Are there any side effects with topical propranolol treatment?

Propranolol treatment has been given to a large number of children without serious complications. Although the drug lowers blood pressure and can slow the heart rate, this has only been seen in less than 1% of cases.

Your child will be admitted for about 3 hours on the Paediatric Day Unit to check their blood pressure before, and after every 30 minutes from giving the first dose of medicine. It is unlikely to have blood pressure changes after the first two hours. In a week’s time, a higher dose will be given and the same process will be followed.

If your child becomes sweaty, shaky, or unwell relating to the medicine given, you should go to A&E directly.

Other possible side effects and the overall frequency in 1175 patients include:

  • bronchospasm (temporary narrowing of the airway, leading to wheezing and coughing) 1.4%
  • peripheral vasoconstriction (reduced blood flow to the extremities, such as fingers and toes, making them feel cold and turn a blue colour) 1.7%
  • sleep disturbance 3.7%
  • hypoglycemia (low blood sugar) 0.9%
  • Nausea 0.7%

If you have any concerns about these side effects, please discuss them with us.

How long does the treatment last?

We expect treatment to continue for up to 9 months. Your child will be reviewed 4-6 weekly after starting the treatment and less frequently thereafter.

Who do I contact if I have any concern or questions?

Arrangements for follow-up appointments and further care will be made when your child is seen in the James Cook Birthmark Clinic. If you have any queries or concerns, contact Mr Muir’s secretary on 01642-854216

Bleomycin injection treatment

This treatment has been successfully used in treatment of hemangiomas. Bleomycin treatment is especially effective in bleeding and ulcerated and rapidly growing hemangiomas.

Unlike some other treatments, bleomycin often blocks further growth and development of a hemangioma after one to two treatments and long courses of treatment are not needed to control the growth. Bleomycin is administered in a number of day case treatment sessions, and in children a short general anaesthetic is necessary to perform the procedure.

Surgery

Complicated hemangiomas can be surgically removed. The benefit is that it often is a one-off, single procedure. It always leaves a permanent surgical scar. The scar can sometimes be worse than if the hemangioma has been left to disappear over time. Large hemangiomas in young children cannot be removed without a potential risk of blood loss.

How can vascular malformations be treated?

Surgery

Surgery is possible in small vascular malformations.. The benefit is that it often is a one-off, single procedure. It always leaves a permanent surgical scar. In larger or extensive malformations, the results are often disappointing. Vascular malformations can re-appear after surgery and is the biggest drawback of this form of treatment. Due to the vessel rich nature of these lesions, bleeding during and after surgery is a risk. Some types of vascular malformations surround and envelop important structures such as nerves and normal vessels, which makes it impossible to remove without causing damage to the normal tissues.

Alcohol injection

Alcohol injection is often the first line treatment used in many vascular malformations. The response rate varies between 58% and 75%. Alcohol is extremely toxic, and causes damage to blood red cells. Due to this, patients need to be catheterized, and receive large amounts of fluid to wash the drug out of the system. It is toxic to nerves and can cause nerve damage. It has a high risk of skin ulceration. Some rare instances of lung damage and death have been reported after alcohol injection treatment.

Bleomycin injection treatment

This new treatment has been very successfully used in treatment of vascular malformations and is the first choice in our unit. The response rate in venous malformations in our unit is 100%. 71% of patients presented with a complete disappearance of the lesion after treatment. The response rate in lymphatic malformations in our unit is 100%. 40% of patients presented with a complete disappearance of the lesion after treatment. Bleomycin is administered in a number of treatment sessions. A day case short general anaesthetic is necessary to perform the procedure in children. In adults the treatment is performed with day case sedation only.

Laser treatment

Laser treatment is the best option for treatment of port wine stains (capillary malformations). It is effective in other vascular malformations that are shallow without a deep component.

Who runs the South Tees specialist birthmark clinic?

The vascular birthmark clinic is a multi-disciplinary clinic which is supported by a group of specialists with specific expertise in the treatment of vascular birthmarks or monitoring of safety in patients undergoing bleomycin treatment.

Together these specialists form the Cleveland Vascular Malformation group, consisting of a plastic surgeon (Mr Tobian Muir), a paediatric lung specialist, an adult lung specialist, and a psychologist, providing valuable support to adults and children where necessary. General anaesthesia is provided by two dedicated anaesthetists,(Dr Gareth Kessell and Dr Malik) with specific expertise in the anaesthesia of babies and young children. Further support is provided from colleagues in radiology and ENT (ear, nose and throat)) for specific cases.

What types of treatments are offered by the South Tees vascular birthmark clinic?

All the standard treatment options are provided by the vascular birthmark clinic, including bleomycin injection treatment. Alcohol injection treatment is not routinely offered as the complication rate of this treatment is high. Laser treatment is provided by referral to a dermatologist in our trust.

How can I get further information or arrange for a consultation?

There is a weekly vascular birthmark clinic and a dedicated paediatric vascular birthmark clinic at The James Cook University Hospital. Your GP can book an appointment directly to this clinic through the current choose and book system, or can send a referral letter to the reconstructive plastic surgery and burns department.

The plastic surgeon responsible for the birthmark clinic is Mr Tobian Muir. If you have any difficulty in arranging a consultation, you can contact Mr Muir’s secretary on 01642 854216 or your GP can request further information about treatment in the vascular birthmark clinic.