Making sense of Vascular Anomalies

A few days ago, an anxious young couple came to see me with their two month old baby. The baby girl was absolutely fine at birth. A few days later they noticed a pink “rash” on the baby’s face on the cheek extending to the eyelid. Gradually they noticed that it was getting more red and puffier in appearance, and now looked like a strawberry. I explained to them that the baby had an infantile haemangioma, and reassured them that this was treatable. This is the most common vascular anomaly, which is seen in about 1-10 percent of the population.

Vascular anomalies, previously referred to as vascular birthmarks, are a group of about 50 different blood vessel disorders. These differ significantly in the way they look, how they change over time, and how they are treated. Broadly, they can be divided into vascular tumours and vascular malformations. Vascular tumours are true tumours which arise from proliferation of the endothelium, the inner lining of blood vessels. On the other hand, vascular malformations arise from abnormal development of vascular channels in the foetus. They are further divided into categories depending on the type of vessel from which they originate, and can be mixed.

Infantile haemangioma typically present shortly after birth and rapidly grow in size (Proliferate) over the next year. After infancy, they slowly start shrinking. This involution takes about 7 to 9 years to complete. Vascular malformations are present at birth, but can manifest much later. They continue to grow with the child and can show sudden spurts associated with trauma or puberty.

Since the treatment of these lesions differs greatly, an accurate diagnosis is important. Interestingly a study from a major referral centre in the US indicated that the term haemangioma was wrongly used in 73 percent of manuscripts studied. Not surprisingly, patients who were wrongly diagnosed were more likely to receive the wrong treatment. Many lesions can be accurately diagnosed with a careful history and examination. Sometimes further testing such as Ultrasound, CT, or MRI scans is required. Very rarely such lesions are associated with other problems such as limb overgrowth, or involvement of internal organs, requiring further testing.

About 40 percent of haemangioma and almost all malformations will require treatment. Haemangioma in the initial phase can be treated with medications which can stop proliferation and promote involution. Surgery is required in some children after full involution, to excise the residual lesion, or to treat complications. Vascular malformations do not respond to medication. Some of them can be treated by intervention radiologists who inject various substances under radiological guidance. The rest of the children will require surgery. In lesions with high blood flow they may require procedures before surgery to reduce the blood flow.

In short, vascular anomalies require proper evaluation in order to formulate a correct diagnosis and plan of treatment. Complicated cases should ideally be treated at centres with multidisciplinary teams.