One of the most serious and socially debilitating problems for HHT suffers are the
frequent and often severe nose bleeds which they experience. These can occur at any time with the slightest provocation making the simplest excursion outside the home fraught with difficulty, there are many treatments both medical and surgical available for the nose bleeds and the choice will depend upon the severity of the problem and factors relating to the patient and ENT surgeon involved.
a) First Aid Measures
The nose has an excellent blood supply and nose bleeds are common in the general population. Normally when blood vessels are damaged they are able to contract and this combined with pressure will usually stop the bleeding. In HHT the telangiectasia are easily traumatized and are not able to constrict in the usual way. Exerting pressure by pinching the front of the nose between the thumb and forefinger for approximately 10 minutes may help but some patients find it easier simply to wait until the bleeding stops. Under either circumstance it is better to bend the head forwards so that any blood coming into the mouth can be spat out and not run down into the stomach or airway where it will cause irritation. As the vessels bleed readily with trauma, any form of packing in the nose can potentially make the situation worse. Consequently, pushing bits of cotton wool or tissue paper in the nose may not help the situation and bits may get left behind to form a focus for infection later on.
b) Laser Treatment and Cautery
A number of methods are available to try and seal the telangiectasia within the nose. Hot wires and freezing have been used but most recently laser cautery has become available in some centres. The laser energy is absorbed by the blood vessel which effectively spot-welds the area and this can be done very accurately when combined with a fibre-optic illumination system. A variety of lasers are available of which the Argon and Neodinium Yag lasers are probably the most effective. However, the laser treatment is not successful in all patients, being most useful in those with mild to moderate disease. The treatment may be required every few months and this simple procedure can be done under local or general anaesthetic.
If the female hormone, oestrogen is taken by mouth it will often reduce the frequency and severity of nosebleeds. The way in which it does this is not certain but it may be due to a change in the lining of the nose which becomes more protective to the telangiectasia. Female hormones, however, do have side effects. In women who have not had a hysterectomy oestrogens may cause some changes to menstruation or restart periods in women who have already undergone the menopause. In men there may be feminizing effects such as enlargement of breast tissue. As an alternative, another hormone, progesterone has been used particularly in men as this has less of these feminizing effects.
The telangiectasia’s are most frequent at the front of the nose, particularly on the nasal septum which divides the nasal cavities in two. It is possible to remove a portion of the lining membrane of the nose and to replace this with a skin graft or lining from the mouth. Although both sides of the nose can receive a graft it is usual not to do both sides at the same time but to do the worse side first and reassess the situation after some months. The length of benefit again varies from patient to patient and over a period of time the bleeding may return as the graft shrinks and the telangiectasia recur.
e) Closure of the nostril
This has proved the most effective treatment in controlling nosebleeds. The very act of breathing through the nose can cause damage to the lining in patients with HHT due largely to the drying effect of the air. If air no longer enters the nose the bleeding will stop. This can be achieved by closing the inside of the nose with little skin flaps which are not visible from the outside. Patients are worried about the consequences of no longer breathing through the nose but they can be reassured that no harm can come to them if they breathe through the mouth either during the day or night. If a nose bleed did occur under these circumstances blood would simply run into the mouth but in all patients in whom the nose has been closed off, nose bleeds have stopped completely. This operation is quite a small procedure, though it is usually done under a short general anaesthetic.
Valerie J Lund
Consultant ENT Surgeon
Royal National Throat, Nose and
Tel: 0207-915-1497 Appts: 0203 456 5197 or 0203 4479393 FAX: 0207-833-9480
This Pamphlet was prepared and written by Valerie Lund on behalf of the
Telangiectasia Self Help Group, 39 Sunny Croft, Downley,
Tel: 01494 528047