Childhood tumours are rare, but amongst them, the most common benign tumour is a capillary haemangioma. It is seen around the orbit of the eye, and is 3 times more common in women than in men. It accounts for 10% of all forms of haemangioma.
In this article, we shall take a brief look at this clinical condition.
A capillary haemangioma is essentially overgrowth of normal blood vessel tissues where it should not be. If one were to look at these under a microscope, it would be evident that it consists of a mixture of cells (called endothelial cells) and blood vessels. Early lesions have more cells, while advanced lesions have a lot of capillaries in the form of lobules. As they get more advanced, the capillary walls become thickened and fibrosed, resulting in occlusion of the vessels.
The most common site where capillary haemangioma are found is the upper eyelid. It could just involve the surface of the skin, but can extend to the tissues beneath it all the way to the orbital cavity.
Clinical signs and symptoms
Capillary haemangioma are clinically evident within the initially 6 months after birth. A third of the cases are seen at birth, and over 9 out of 10 cases are seen by 6 months. Lesions that involve just the skin (also called cutaneous haemangioma) appear as a red, elevated nodule, while lesions that extend under the skin (subcutaneous lesions) are darker. In advanced cases involving the orbit, the eyeball could protrude out. This is called proptosis.
Haemangiomas on the upper eyelid tend to push the eyelid downwards, causing a droopy eyelid (called ptosis). Patients complain of blurred vision which occurs due to change in corneal shape, astigmatism and squinting (strabismus).
On pressing gently, the haemangioma can change colour and have a sponge-like texture. It is visibly evident on examination.
Diagnosis is primarily clinical, but imaging could be performed for further evaluation. Ultrasound can help ascertain the depth of involvement. CT scans can demonstrate blood flow into the lesion. MRI scans are also useful.
The natural course of capillary hemangiomas is an initial growth phase followed by a regression phase. Most lesions tend to undergo involution by the age of 8. Treatments are offered if there is visual impairment or infection.
Medical treatment involves the use of steroids, taken as tablets or injected directly into the lesion. However, the long term use of steroids has a number of side effects such as osteoporosis and weight gain. In addition steroid injections can affect blood flow to the retina and affect the local skin.
Treatments that alter body immunity have also been used, but with limited efficacy. Adverse effects are high and benefits can take a long time to show.
New treatment includes the use of beta blocker Propranolol. This drug is often used to manage high blood pressure, but it has found to help reverse visual problems with haemangiomas. It acts by reducing the amount of growth factors that stimulate haemangioma growth, reducing the blood supply and stimulating cell death within the lesion. Side effects are a few, and the drug must be avoided if a patient has asthma.
Surgical treatment includes laser photocoagulation for skin lesions, and entire removal with surgery for deeper lesions. Blocking off the blood supply to the lesion using a procedure called embolisation is also useful.
Finally, any associated medical conditions that form parts of different syndromes need to be managed as well.
Capillary haemangioma are the most common benign tumour of the orbit. Diagnosis is straightforward, but treatments seem to be limited. Surgery appears to be the better option when compared to medical therapy.