Congenital Melanocytic Naevi

Transcription

Congenital Melanocytic Naevi
Congenital
Melanocytic
Naevi
Information for families
Great Ormond Street Hospital for Children, London,
and St John’s Institute of Dermatology, St Thomas’ Hospital, London
Congenital
Melanocytic
Naevi
What are they?
A congenital melanocytic naevus
(usually abbreviated to CMN: plural CMNs) is one of a wide variety of different
types of birthmark that may occur in newborn babies:
M congenital means present at birth.
M a melanocyte is a pigment cell which is present
in normal skin - its function is to produce
melanin – the brown pigment responsible for
skin colour in all races.
M naevus is a (Greek) word used to describe any
type of birthmark on the skin; the plural is
naevi.
In a CMN, this development of pigment cells does
not happen in a normal way. Instead of flowing
out smoothly and evenly into the skin, too many
immature cells develop and persist in a large
group. It is not yet known why this happens or
exactly what goes wrong.
What causes them?
These birthmarks are probably caused by changes
A CMN is an abnormally large collection of pigment
in the body’s instructions about how to make
cells in the skin and can be regarded as a type of
normal skin.
benign tumour.
These instructions are called genes. A change in a
In the embryo, pigment cells originate in the region
gene is called a “mutation”. This mutation would
of the developing spine from where they migrate
have happened during the early growth of the
to the skin. As they arrive in the skin, the pigment
child during pregnancy. It might be more likely to
cells normally spread out and become evenly
happen in some families.
distributed among the other skin cells. Their
numbers are small. Their function is to produce
melanin pigment which protects the skin from
damage by ultraviolet rays. The amount of pigment
they produce depends on the degree of natural
skin colour and the degree of recent exposure to
sunlight (ie. degree of tanning).
Congenital
Melanocytic
Naevi
Size of and
frequency of CMNs
CMNs usually grow in proportion to the child.
They will therefore usually always cover the same area of skin as at birth. CMNs
are classified according to the size they will be in adulthood. This is a difficult
definition to understand because different parts of the body grow at different
rates, but it is used to give doctors a way of comparing different CMNs.
Strictly speaking, a ‘small’ CMN is one that will
Characteristics of CMNs
measure less than 2.5cm (1 inch) across in
CMNs show a number of characteristics which
adulthood; a ‘medium’-sized CMN measures
vary considerably and which may change
between 2.5-10cm across; a ‘large’ CMN is between
somewhat in any CMN over a period of time.
10-20cm and a giant CMN will measure more than
These include:
20 cm across.
Size
Using these definitions, a small CMN will be found
CMNs vary greatly in size, from a few millimetres
in about 1 in 100 newborn babies which makes it
to many centimetres across. The very largest may
one of the most
cover most of a limb or much of the trunk.
common types of
However, as the figures above indicate, smaller
birthmark. Giant
ones are far more common. As the child grows, a
CMNs only occur in
CMN will enlarge in proportion to the child’s
1 in about 20,000
general growth. Occasionally, during the first year
newborns. Multiple
or two, there may be some true extension of the
CMNs does not just
pigmentation at the outside edge, but this does
mean having more
not usually lead to a substantial increase in the
than one CMN – it
overall size of the CMN.
is a particular
Site
classification used by
doctors when there is
no one CMN larger
than all the others.
CMNs can occur at any site on the skin, including
CMN of the neck and back
which will be more than
20cm diameter in adulthood.
the scalp. Some of the very large CMNs cover parts
of the body in such a way as to resemble items of
clothing, most frequently boxer shorts or oldfashioned bathing trunks; therefore , these are often
termed ‘garment naevi’, or ‘bathing trunk naevi’.
Colour and lightening
The colour of a CMN depends to a great extent on
the background skin colour of the child. CMNs
therefore tend to be lighter brown in blond-haired
children, darker in Asians and in Orientals, and
almost black in Afro-Caribbeans.
Immediately after birth, they often have a rather
purplish hue. They are almost always much darker
at birth, particularly in those with white skin, and
often become progressively lighter over the next
year or two. Very occasionally they may seem to
disappear altogether, though this is very rare.
Nevertheless, a CMN that is regarded as disfiguring
at birth may become quite tolerable after a year or
two because of this lightening effect.
The colour may vary a good deal in different parts
of a CMN, particularly in larger ones. Over the years,
there may be considerable change. Darker areas or
lumps may appear, but these changes rarely need
cause medical anxiety. This issue is dealt with in
greater detail later.
Texture
The texture of large CMNs tends to be different
from that of normal skin, being softer, looser and
more wrinkled. The skin tends to be more fragile
than normal, tearing rather easily if traumatised.
They do not bleed any more than normal skin and
tend to heal well.
This CMN on the back is hairy in some places and
relatively hairless in others.
Hairiness
CMNs are usually hairier than normal areas of skin,
but this is very variable. In some cases, the increased
hair growth is barely perceptible, whereas in others,
hair may grow profusely from the surface of a CMN.
This increased hair growth may not be apparent in
the first weeks or months and tends to increase
over the first few years of life. It is not known why
the hair grows more on CMNs.
The colour of the hair over a CMN is generally darker
than the child’s scalp hair, although it can be the
same colour and occasionally it is lighter. If a CMN
is on the scalp, this will usually result in an area of
darker, more luxuriant hair growth over the lesion,
which may itself be invisible under the hair.
Paradoxically, some CMNs are completely hairless,
even ones that occur on the scalp.
Lumpiness
Lumpiness in a CMN on the hand.
Smaller CMNs are generally more or less flat, flush
with the surrounding normal skin. However, when
a CMN is bigger, there are often raised or lumpy
areas. This does not imply any special medical
problem, just that there are larger and deeper
collections of melanocytes in these areas. Some
lumps are paler and softer than the rest of the CMN,
and some are firmer and darker.
Hardness
Very rarely, a CMN may be really quite hard from the
beginning, or may become harder over a period of
time. This hardness is usually due to fibrosis, a kind
of scarring process, the exact cause of which is
unknown. This process is generally accompanied by
loss of hair and often also by decreasing
pigmentation.
Eczema
The skin overlying a CMN is often rather dry and
itchy and may sometimes develop frank eczema.
This can largely be avoided by regular application
of a moisturiser as soon as any dryness becomes
apparent. Avoid the use of soap as it tends to dry
the skin further. If eczema becomes a problem, you
may need to see your GP or speak to a
dermatologist.
Multiple satellite lesions of different sizes, colours and
textures.
Underlying absence of fat
‘Satellites’
This is something that
is generally only seen
in the case of larger
CMNs, particularly
those on the buttocks
or limbs. For some
reason, the presence
of the CMN interferes
with the development
of the layer of fat that
is normally present
between the skin and
underlying muscle
and bone. This can
result in the CMN
The right leg is noticeably
appearing to be
thinner than the left, due to
depressed below the
less fat under the CMN in
general skin surface.
that area.
An affected limb,
buttock or side of the
face may be obviously thinner than normal. The
thinner area functions just as normal – for example a
thinner leg will be perfectly strong, as the thinness is
only due to less fat and not less muscle.
Many children who have a CMN, particularly a
larger one, will have smaller CMNs elsewhere on
the skin. These are known as ‘satellites’. While these
can look quite similar to ordinary ‘moles’, they are
often bigger. Some may already be present at
birth. These will tend to be bigger than those that
develop later, which are usually not more than ½ 1 cm across. Larger satellites can be hairy and
raised.
Satellites may or may not increase in number over
the years, and it is impossible to predict how many
are likely to appear in any individual. Their final
number may be as many as a few hundred.
Congenital
Melanocytic
Naevi
What are the medical
implications of a CMN?
There are 3 principal health implications of which parents
should be aware:
Risk of involvement of the brain or
spinal cord
Problems in the brain or spinal cord are the
commonest complication seen in children with
CMNs. The skin and the brain develop from the
same cells in an embryo. As a result, children with
CMNs can have differences in their brain or spinal
cord with the most common being pigment cells
appearing in the brain or spinal cord. This is called
“neuromelanosis”, or “neurocutaneous melanosis”.
Other much rarer problems include benign brain
or spinal tumours, too much fluid in the brain, or
abnormal brain structure.
Problems in the brain or spinal cord can be detected
by an MRI scan. Analysis of all the MRI scans done
on children with CMNs at Great Ormond Street
Hospital between 1991 and 2007 showed that
there is more chance of finding problems on the
MRI scan in children who have satellite lesions
(small CMNs) at birth. Problems on MRI scans are
also more common with larger CMNs, although
they can occur with smaller CMNs with satellite
lesions as well. The overall chance of finding an
abnormality on an MRI scan in children with
satellite lesions is 15-20%, but not all these children
will have any actual problems. If they do have
problems, these can be fits (convulsions),
developmental delay, or problems with their limbs.
It is possible to have problems in development even
when the scan is normal, but these tend to be
milder. The reason for doing the scan is to pick up
the rare cases of tumours and extra fluid on the brain
which require an operation. The pigment cells in
the brain or spine cannot currently be treated.
Our current recommendations are that only children
with satellite lesions at birth need a routine MRI
scan, preferably by the age of 6 months. It is not
possible to say that abnormalities in the MRI scans
can never be detected in children without satellite
lesions at birth, but the risk is much less. We also
recommend that children with giant CMNs (more
than 20cm in adulthood) should be followed up
regularly to watch their development, even if they
do not have satellites. In addition any child with
any type of CMN who has a problem with
development or fits or limbs should have an MRI.
Risk of malignancy
Is there a risk of malignant tumours in those with a
CMN? The medical literature on CMNs has been
preoccupied with this issue for many years. There
has been a general assumption that large CMNs
carry a risk of 5-10% of becoming malignant at
some time in the individual’s life. It has also been
assumed that the larger the CMN, the greater will
be the risk.
These assumptions are unreliable because they
are based on information that was inadequate,
both in quality and in quantity. The risk of
malignancy has been calculated by counting up
the total number of patients with CMNs who have
been seen in a particular hospital, and the number
in whom malignant melanoma was thought to
have occurred. This method can be expected to
produce a distorted picture of the true situation
because patients are more likely to attend a hospital
if they have a serious complication, particularly a
suspected malignancy. This effect would inflate
the apparent frequency of malignancy in CMNs, as
there is likely to have been substantial number of
patients with uncomplicated CMNs who did not
turn up at the hospital. In countries where patients
have to pay to go to a hospital (most countries in
the world), they are unlikely to do so unless they
can afford to, or are anxious that they may have a
serious medical problem that makes seeking
medical advice seem a priority.
Another major problem has been over-diagnosis
of malignant melanoma in specimens (biopsies)
taken from CMNs, especially from nodules or areas
of changing pigmentation. This has also added
greatly to the overestimating of the risk of malignant
melanoma in the past, and is almost certainly still
occasionally a cause of misdiagnosis and
unnecessary anxiety today. The correct interpretation
of this kind of biopsy is notoriously tricky, and
should be undertaken only by those who have the
necessary skill and experience.
At Great Ormond Street Hospital, we have been
collecting data on people and families with CMNs
since 1988 and we are still doing so. This involves
sending a questionnaire once a year to each family
and one of the questions is whether they have
had malignant melanoma in that year. In 2007, this
data was analysed and out of 350 children with
CMNs, there had been 5 cases of malignant
melanoma. This is a very low rate overall and it is
very similar to that found in other recent studies
from other countries. However, all 5 cases were
found in the very largest CMNs (those expected to
be more than 40cm in adulthood). It may be,
therefore, that the risk of melanoma is very low in
CMNs less than this size (less than 1%), but relatively
high in the CMNs greater than this size (perhaps
up to 10-15%). Unfortunately, most often it seems
to be the case that when malignant melanoma
occurs in a person with a CMN, it has often spread
widely internally by the time there is any outward
sign of a problem. Unfortunately, once malignant
melanoma has spread internally, it is generally
impossible to treat effectively.
When considering these risks, you need to bear in
mind that every one of us has a risk of about 40%
of developing some kind of malignant tumor at
some time in our lives.
Risk of involvement of the eye
The best hope of curing malignant melanoma is
when it takes the form of a well-defined nodule
appearing within an established CMN, which can
be removed surgically before it spreads to internal
organs. When this does happen, a nodule appears
within a CMN and will be noticeable by its rapid
growth, and after a few weeks, by ulceration and
bleeding. The problem is that new nodules appear
in CMNs quite frequently, and the great majority is
completely harmless. While it is therefore important
to keep an eye on a large CMN for possible
malignant change, in practice this may lead to
anxiety about every slight change that occurs. In
some cases, such anxieties have led to many
biopsies being taken for analysis over the years, a
process that can be distressing for children and
parents alike. Our recommendations are that any
rapidly changing area should be reviewed in our
clinic, and we can decide whether a biopsy is
required.
Out of all this difficulty emerge certain reasonable
recommendations. Firstly, you should keep an eye
on a large CMN and its satellites. If a new nodule
arises within them, you should seek medical advice.
You need to remember that the risk of a nodule
being malignant melanoma is very low. Secondly,
if malignant melanoma is diagnosed, it is worth
asking whether a second opinion on the biopsy
has been sought from an expert. You will generally
find that this is the case, but be aware that even
the experts find it difficult to be certain.
Very rarely, CMNs close to the eye can be associated
with increased pressure in the eye, known as
glaucoma, or with pigment cells in the eye.
Pigment cells in the eye can be seen as darker,
often bluish patches over the white areas, or they
can be invisible inside the eye. Children with a
CMN very close to the eye should have their eyes
checked by an ophthalmologist (eye doctor) at
least once, and the pressure should be checked if
there are ever any problems with the eye.
Congenital
Melanocytic
Naevi
What treatments
are available?
Depending on the size, site and colour of CMNs, they can be unsightly. Some are
highly disfiguring, and therefore a potential cause of great distress. If CMNs
could be completely removed, easily and without trace, there would be little for
us to discuss here. Currently this is not the case.
Choosing not to have surgery for a CMN
This can be a very difficult thing to do, both for
families and for doctors. However it is a very valid
option and should be considered very carefully.
In the past, the natural reaction of doctors when
confronted with a CMN was to suggest ways to
remove it, often as soon as possible. This was
based on the data that was available at the time,
in particular on the fear that there was a high risk
of melanoma in CMNs. As you will have read
above, we now know that the risk for most CMNs
is low, and we also think that there is no evidence
that surgery to remove a CMN takes away this risk.
We now feel that it is important to weigh up the
pros and cons of surgery more carefully.
Points to consider in this decision
M Many CMNs will lighten spontaneously over a
period of years, sometimes dramatically. This
can be monitored with repeat photographs.
M Surgery has not been shown to reduce the risk
of melanoma in the child.
M Early surgery has not been shown scientifically
to be advantageous in any way.
M The site of the CMN is very important – for
example the child may get more benefit if a
CMN on the face is removed, compared to one
hidden on the scalp
M The size of the CMN is very important - we have
found that children with larger CMNs were less
pleased with the cosmetic result than those with
small lesions which could be completely removed.
M The number of satellites is important, in
particular if the child has a tendency to
develop lots of new ones.
M Whether you want your child to take part in
the decision, in which case it is better to
decide later.
Obviously, if a CMN can be removed easily, for
example by excision or serial excision (more than
one operation but relatively straight-forward) the
cosmetic benefits may easily outweigh the small
risks associated with any operation. However, if a
CMN is in a difficult place for removal, or if it is too
large ever to be removed completely, then that
balance changes. It is very important in these cases
to take time to decide about surgery, particularly to
see if the CMN is lightening over time.
Choosing the right surgery
This decision would always be taken in conjunction
with a plastic surgeon, but this section gives you
information on each type.
Before talking in more detail about treatments, it
might be helpful for readers to understand more
about the skin, and about the way it heals after
injury. This requires a basic knowledge of the way
the skin is constructed.
The skin has 3 principal layers, the epidermis on
the outside surface, the dermis immediately below
it and a layer of fat below that, known as the
subcutaneous fat.
Epidermis
The main functions of the skin are to stop the
body from losing fluid, and to keep out infections
and noxious substances. These functions are
provided by the outer layer of the skin, known as
the epidermis.
Dermis
The dermis is a physically strong and resilient
foundation for the epidermis, containing all the
services that the epidermis requires to function.
The dermis contains collagen which provides the
skin with its great flexibility. Elastic fibres are also
present and allow some stretching. Between these
fibres run the blood vessels that nourish the
epidermis, as well as its supply of nerves.
Subcutaneous fat
Under the dermis is a layer of fat. This fat has a
number of functions other than its principal one
of storing nourishment. It provides protection
against heat loss from the muscles, which have a
high blood supply and is important aesthetically
in conferring a smooth contour to the skin.
Under the fat are the muscles, separated from it by
a thin layer called fascia.
Surgical excision:
basic principles
Having considered the basic construction of the
skin, this is a good point to consider the basic
principles of surgical removal (excision) of skin
lesions. The pigment cells in a CMN may extend
though the full thickness of the dermis and even
through the subcutaneous fat, to the level of the
fascia. They do not enter the underlying muscle.
Full thickness excision
To remove a large CMN completely will usually
require full thickness removal of the skin, including
the subcutaneous fat. Full-thickness excision of
skin requires the wound to be closed, either by
sewing the edges together in some way if
possible, or by moving in skin from some other
site (grafting) to cover the area.
For small CMNs, this is usually straightforward as
the wounds can be stitched up directly. If the
CMN is a little bit too large to be removed at one
go, it can often be removed in two stages. This is
called serial excision.
If a CMN is even larger its removal would leave a
hole which cannot simply be repaired by stitching it
up. Such holes can be closed in one of three ways
(or by a combination of these), by grafting, by
rotation flaps, or by tissue expansion. Whatever
method is used to close the defect left when a CMN
is removed, there will be a risk of pigmentation
appearing at the edges of the site of the excised
CMN. This is known as the ‘tide-mark’ effect.
Grafting
The simplest way of repairing a large hole in the
skin is with a skin graft. Most skin grafts are of
partial thickness and are taken from a ‘donor’ site
somewhere else in the body, usually (for
convenience) the thighs. After the donor site has
healed, it may be left with some change in
pigmentation and – occasionally – scarring. When
the graft is placed in the gap left by the removed
CMN, it will heal but there will be a net loss of bulk
as the thick CMN is being replaced by a very thin
skin graft. In addition, the surface will tend to look
scarred, like a burn. It will be hairless and rather
shiny, and experience has shown that appearance
of spotty repigmentation (brown pigment coming
through the graft) is almost invariable. A graft will
not grow in proportion to a child’s own growth.
This can cause problems if the graft crosses a joint,
as the mobility of the joint will be compromised.
This can usually be corrected by a further
operation to insert an extra graft, but it is usually
best to avoid grafting over joints if this is possible.
Skin grafts can be used to remove very large areas
of CMN, but there is usually too little donor skin
available to do more than a relatively small area at a
time, using the same donor site on each occasion.
One can use full-thickness skin grafts in some
situations. These can give a very good result, but
they can only be small; otherwise the donor site
will not heal. They will be reserved for important
sites, usually on the face. Small full-thickness grafts
are usually taken from behind the ear or in the
groin, where the skin can be stitched up to give a
straight line scar. Sometimes larger areas are used,
in which case the donor site will have to be closed
with a partial thickness graft from elsewhere.
The main problem with grafts is that they do not
always ‘take’ as well as one would wish, and the
final aesthetic appearance will then be less
satisfactory. Even when they take well, skin grafted
with partial thickness grafts will tend to look
unsightly. For this reason, grafting is generally best
avoided in cases where excision is undertaken
primarily on aesthetic grounds.
Tissue expansion is a technique in which the
area of skin available to close a defect is increased
by prior stretching of the normal skin around the
site at which an excision is planned. The stretched
skin is (technically) of full thickness, and will have
some of its underlying fat, and it will have a colour
and texture appropriate to the site where it will be
used. Usually this stretching is achieved over a period
of weeks. The first stage is the insertion (which requires
a general anaesthetic) of a silicone balloon (tissue
expander) just beneath the skin. The balloon is
attached, via a small tube, to a filler port. This port
is also under the skin, and the balloon can be filled
progressively by injecting it with small volumes of
saline (salt water) every few days. The injections
can be undertaken by a nurse or by parents. It
generally takes 6-8 weeks to fill the balloon adequately.
Removal of the CMN can now be scheduled, and
the balloon will be removed during the operation.
This technique is only suitable for certain sites. The
skill is to expand the right area of skin, and then to
move it in to replace the CMN with the minimum
of scarring. Sometimes, if the CMN is large, two or
three tissue expanders may be used at the same
time. It may also be necessary to repeat the whole
process on more than one occasion.
The swelling that is visible while the balloon is in
place can prove an aesthetic problem in adults and
in adolescents, but rarely bothers younger children.
There is a small but real risk that the tissues around
the balloon will become infected, which will usually
mean that the balloon will have to be removed.
Occasionally, the stretching is too great for the
skin to cope with, and it breaks down.
Where the amount of extra skin required is not
great, the expansion can sometimes be done
quickly during the operation itself (‘intra-operative’
tissue expansion).
CMN on the face before surgery (left), while the tissue expanders are in place (middle), and after surgery (right).
Rotation and transposition flap
Partial thickness removal
A rotation flap is an area of full-thickness skin that
is lifted off the underlying tissues, and moved with its blood vessels still connected - to fill a
defect in an adjacent area. It is rather difficult to
describe exactly how this is done, but whether it is
an option depends very much on the site. The
scalp and the face are good sites for this technique.
This includes dermabrasion, curettage and laser
techniques. We do not currently recommend this
type of approach because the benefits are usually
only temporary, the end result may not be any
better than if the CMN undergoes spontaneous
lightening, the process is painful and can produce
scarring. However in certain cases it may be
considered to be worth pursuing and we therefore
provide the following information about it.
A transposition flap is similar, but in this case, an
area of full thickness skin, often also including fat,
is moved to a distant site where the blood vessels
are re-connected.
The main advantage of these techniques over
grafting is the excellent final quality of the relocated skin, and the advantage over tissue
expansion is that no additional operations are
required, nor the long period of balloon-filling.
Because the pigment-producing cells are those
immediately below the epidermis, one can
remove all or most of them by removing the top
surface of the skin, without fully removing the
whole CMN. Healing generally takes about 10
days, without requiring any of the techniques
described above, but the skin is very painful
during that time, rather like having an open graze.
Several different techniques may be used to
achieve this partial thickness (‘superficial’) removal.
The most traditional is dermabrasion, in which
abrasion is used to scour away the surface of the
skin. As you can imagine, this is a bit messy. In
another technique, called shaving, a thin layer of
skin is removed with a special blade called a
dermatome - the same type of blade that is used
to take skin grafts. Another technique is to use a
curette, an instrument that resembles a very small
spoon with a sharpened edge, which can be used
to scoop out bits of skin. Chemicals such as
trichloro-acetic acid have also been used to burn
off the surface of the skin (chemical peeling).
‘Resurfacing’ lasers can be used, particularly the
carbon dioxide laser. Lasers have the advantage of
causing less bleeding, however, the treated site
will generally be painful and oozy for a few days
afterwards whichever technique is used.
Laser treatments
The results of any kind of superficial removal are
very variable and difficult to predict before the
operation is performed. The treated area may look
good initially, but later on new pigmentation often
appears around the edges and spotty
pigmentation frequently re-appears in the treated
area. This re-pigmentation may increase for many
years and can be substantial. A further
disadvantage is that superficial removal will not
deal with hairiness, because the hair follicles are
not removed. Another problem that can occur is
scarring. The occurrence of scars will indicate that
the removal of tissue has been too deep in those
areas, but often this has happened because the
surgeon has simply removed pigment as deeply
as it can be seen to extend in a genuine attempt
to achieve the best possible aesthetic effect. The
scarring that frequently occurs after superficial
removal of CMNs tends to improve a great deal
over a period of many years, and in fact, the best
long term benefit from this type of surgery is often
seen in areas that were initially scarred.
There is a widespread belief that the success of
this type of procedure depends on the surgery
being undertaken very early. There is no scientific
evidence that it has to be done in the first few
days of life, as is often advocated, whereas there
are many reasons for it being more hazardous at
this age.
Earlier, I considered the use of ‘resurfacing’ lasers
which remove layers of skin, such as the carbon
dioxide laser, for the superficial removal of CMNs.
Other types of lasers are being developed all the
time, including ones that target pigment in
particular. However, to date none of the types of
laser that are currently available have been able to
provide consistent results in terms of improving
the appearance of CMNs, and I do not feel able to
recommend any of them. It is possible that new
and more effective lasers will be developed. For
the time being, perhaps the most immediately
promising area for the use of lasers in CMNs is for
semi-permanent hair removal, ideally left until the
multiple treatments required can be tolerated
under local rather than general anaesthesia.
Congenital
Melanocytic
Naevi
At what age should
CMNs be treated?
We recommend that any type of routine surgery for CMNs should be delayed until
after the age of 1 year at least. This is to reduce the risk of having an anaesthetic.
In practice, we often delay surgery for longer so that we can see if the CMN is
lightening. Families are often worried about children starting school with a CMN,
and if the CMN is on the face we would tend to suggest operating before school.
In considering these matters, it is worth being aware
that children rarely experience troublesome
prejudice in terms of birthmarks until they are about
10 years old. In practice, it turns out that children
may be subjected to dreadful teasing during
adolescence, even when they have no real problem
at all with their appearance. I have read articles in
which some of the best-looking women in the
world tell of their merciless persecution as teenagers,
because they were considered too beautiful by
other girls. What probably matters most is, firstly,
one’s personality – whether one is vulnerable to
teasing or not – and secondly, one’s upbringing –
whether one was made to feel normal or
imperfect in the family situation. We can do little
about a child’s personality, but we can, as parents
and family friends, do a lot about the second. It
would be good if more professional psychological
support were available through the NHS to
support children with significant CMNs through
later childhood and adolescence, but currently
there is not anywhere near what is needed. Both
the Birthmark Support Group and Caring Matters
Now Support Group are working towards providing
help by young adults who have CMNs, and this is
in my view an exciting development.
What treatment is available for
‘satellites’?
It sometimes makes a big difference to cosmetic
appearance to remove large satellites. This may be
worthwhile for a small number of these lesions,
but if it is envisaged that large numbers will be
removed, the appearance of the scarring that will
result will almost certainly be unacceptable.
What can we do about hairiness?
rules in mind:
Dealing with the hairiness of CMNs is difficult as
hair follicles are situated very deeply in the skin.
Superficial removal techniques have little effect on
this aspect of CMNs and the only absolutely
reliable way to remove the hair is to excise the skin
and the naevus completely.
M Children should be kept out of direct sun as
much as possible during the hottest hours of
the day in the hottest months of the year.
Electrolysis is a slow and laborious process and is
therefore not suitable for large areas. It is also
usually too uncomfortable for children to tolerate.
Parents have the option of shaving the hair regularly.
An electric shaver is probably best, and the models
designed for use on women’s legs are probably
best of all. Shaving does not affect the amount or
thickness of hairs that grow subsequently. Hair
regrowth after shaving is generally slow, and the
new hairs will have exactly the same appearance
and feel as the original ones. Most parents find
that they do not need to shave an area more often
than once every couple of weeks in order to
maintain a satisfactory appearance. Depilatory
creams should not be used as they can irritate the
skin. I have already mentioned laser hair removal.
Regular treatment over a relatively long period
might prove able to provide semi-permanent hair
removal in many cases. However, this should
probably not be considered until a person is able
to have the procedure undertaken with local
anaesthesia alone, and this is not likely to be the
case until adolescence at the earliest.
Are there any special precautions my
child should take?
Children with large CMNs can and should enjoy a
normal life. They should have very good sun
protection the same as all children.
We know that the greatest risk factor for
malignant melanoma is sunburn, and this
therefore has to be avoided especially. The
presence of the sun is a fact of life, and complete
avoidance of exposure of any part of the skin at
any time is an impossibility. Therefore one has to
achieve a compromise. When considering this
subject, you should bear the following general
M The hottest hours of the day are 10am-4pm,
inclusive.
M The hottest months of the year are April to
October, inclusive.
M Exposure is increased greatly by reflection when
beside water or snow, so special care should be
taken when swimming outside or skiing.
M The sun is more harmful at higher altitudes.
M The sun remains almost as harmful in cloudy
conditions, so even if the day is cloudy the child
should wear appropriate clothing such as sunhat.
M The best protection is clothing, which is most
protective when darkly coloured.
M Shade (including hats) provides some
protection, but less when near water or snow
or when the weather is cloudy.
M Sunscreens are merely a last and at best, partial
barrier, and are not a substitute for other forms
of protection.
M The best sunscreens contain a reflectant barrier
such as titanium dioxide; select a high
protection factor (SPF), ideally 25 or greater.
M Sunscreen needs to be refreshed every 2 hours
or so, more often when swimming or sweating.
M Children with CMNs probably have an
increased risk of skin cancer for their whole
skin, not just on their CMN.
Skin care of CMNs
The skin over a CMN tends to be rather fragile and
therefore, easily torn if traumatised. It is usually
also dry and very prone to develop eczema. This
dryness and predisposition to eczema will tend to
be worse during the winter months. It follows that
good care needs to be taken of the surface of a
CMN to maintain optimal skin condition and
minimise problems.
Soap should be avoided at all times and a cleanser
such as Aqueous Cream, Cetraben® cream or
Diprobase® cream used to wash with instead. These
can be bought over the counter but can also be
prescribed by a doctor. If the surface remains dry
despite this, the same type of cream should be
applied as a moisturiser at other times, as often as is
necessary. Itchiness of the surface can mean that
eczema is developing, in which case a mild topical
steroid preparation may be required to bring the
situation under control. Dealing with this type of
eczema is not normally difficult once the problem
is recognised.
Psychological issues
Children who grow up with a prominent CMN
and/or satellites may well have problems adjusting
to the disfigurement that they perceive, particularly
during the years of adolescence. This will vary
greatly for different children, depending largely on
their own personality and on the support they
have enjoyed from family and friends.
As we considered earlier, it would be ideal if we
were able to offer all children at risk some degree
of automatic counselling during their childhood,
but currently the resources are simply not available
for this. Additional help can now be obtained
through Caring Matters Now, and/or the Birthmark
Support Group. In addition, there are some
organisations specifically dedicated to disfigurement
issues, and they are well worth approaching on
this subject. Changing Faces is an example of a
support group specialising in disfigurement; its
website is www.changingfaces.org.uk.
Will our next child be at risk
of having a CMN?
No – it would be very unusual to have another child
with a CMN. A tendency to have a CMN probably
does run in some families but the condition is still
very rare. The risk to any further children of a couple
who already have a child with a CMN is very small.
Similarly, the risk of a person with a CMN themselves
having a child with one is very small.
Is there a support group?
Caring Matters Now is a support group specifically
for people with CMNs. This means that the members
have an understanding of the birthmark and what
it means to have one. They organise family days for
the children to enjoy themselves and the parents
to get to know each other, as well as to present
information about the latest research results from
Great Ormond Street. They also raise enormous
amounts of money to fund the research at Great
Ormond Street, with the aim of finding out more
about CMNs and potential treatments.
Further information is available on the website:
www.caringmattersnow.co.uk.
Or at:
Bridge Chapel Centre, Heath Road,
Liverpool, L19 4XR
Telephone: 0845 458 1023
Email:
info@caringmattersnow.co.uk
The Birthmark Support Group supports children,
teenagers and adults with birthmarks (including
CMNs) and their families, through telephone
helplines, information packs, newsletters and
regular ‘Fun Days’ held in different parts of the UK.
Its ‘Teentalk’ group puts older children and teenagers
with birthmarks in touch with one other for mutual
support, and also provides a mentoring service.
‘FaceItTogether’ provides a similar service for adults.
Further information is available from the website:
www.birthmarksupportgroup.org.uk
or via:
PO Box 327, West Malling,
Kent, ME19 6WW
Telephone: 0845 045 4700
Email:
info@birthmarksupportgroup.org.uk.
For specific CMN enquiries
Email:
cmn@birthmarksupportgroup.org.uk
There are two support groups in the USA each of
which has a slightly different emphasis. The original
one is called the Nevus Network
(www.nevusnetwork.org). This website provides
access to some fascinating statistics gathered from
its over 1000 members. It also provides details of
CMN support groups in other countries. The more
recently formed group is called Nevus Outreach;
their website is www.nevus.org.
Congenital
Melanocytic
Naevi
Research
Is any research being done and can we help?
Yes. Caring Matters Now support group has been funding new research at Great
Ormond Street Hospital (GOSH) since June 2006.
There is a dedicated weekly NHS clinic for CMNs,
Disability Living Allowance
and now also a weekly research clinic for families
Parents of children with CMNs are eligible for this
who have not been referred to GOSH or whose
benefit if they are spending more time on their child’s
child is now more than 16 years old. In these two
care than would be the case for a normal child. The
clinics, families are given the opportunity to take
calculation includes time spent on skin care and
part in the research projects. Anyone who would
hospital visits. If you would like further advice,
like to take part in these projects should ask their
contact your Citizen’s Advice Bureau or the Social
GP/Dermatologist/Paediatrician for a referral to Dr.
Services Department in any hospital your child
Kinsler in the Paediatric Dermatology Dept. at GOSH.
visits.
The Registry of CMNs has been running since
1988. This is a large database of information about
children and families with CMN. Taking part in the
Registry involves filling in a yearly questionnaire
and sending it back by Freepost. To try to
maximise the quality of the data collected, only
families seen at GOSH (either in the NHS clinic or
in the research clinic) can take part. If you are not
seen at GOSH and wish to be part of the registry,
you will need to be referred by your GP to:
Dr. Kinsler,
Paediatric Dermatology Department,
Great Ormond Street Hospital,
London WC1N 3JH.
All information held by the Register is completely
confidential.
Great Ormond Street Hospital for Children, London
Revised version David Atherton March 2007
Revised version Veronica Kinsler March 2009
Caring Matters Now
Miss Jodi Unsworth
Caring Matters Now Support Group
Bridge Chapel Centre
Heath Road
Liverpool
L19 4XR
Tel: 0845 458 1023
Email: info@caringmattersnow.co.uk
Charity Number: 1120988
Research programme undertaken by Dr Veronica Kinsler
Congenital Melanocytic Naevus Research
Great Ormond Street Hospital
London
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