© 2015 Nevus Support Australia PO Box 262, Seacliff Park, South Australia 5049  
Every   CMN   is   unique.   There   is   no   specific   pattern   of   distribution   or   skin   texture   within   the nevus   itself   and   no   way   to   be   certain   just   how   having   a   CMN   will   affect   a   person’s   lifestyle. Below   are   some   of   the   most   common   questions   answered,   however   each   case   is   individual and will have its own particular areas of concern. Malignant Melanoma Melanoma   is   a   cancer   of   melanocytes,   the   skin   cells   which   produce   pigment   and   which make   up   congenital   melanocytic   nevus.   It   used   to   be   thought   that   melanoma   was   very common   in   people   with   CMN,   but   we   now   know   that   it   is   rare,   occurring   in   around   1-2%   of all   people   with   CMN   over   their   lifetime.   However,   the   risk   is   higher   in   people   with   very   large and    numerous    CMN,    perhaps    as    much    as    10%,    and    there    is    a    peak    of    risk    during childhood.   When   considering   these   risks,   you   need   to   bear   in   mind   that   a   person   without   a CMN   still   has   a   risk   of   about   40%   of   developing   some   kind   of   malignant   cancer   at   some time in his or her lifetime. Melanoma   in   people   with   congenital   melanocytic   nevus   can   occur   anywhere,   not   just   within the   CMN,   and   not   just   in   children.   It   can   present   as   a   lump   in   the   CMN,   in   another   area   of skin,   or   in   the   lymph   nodes   (which   are   throughout   the   body).   However,   melanoma   is   rarely if   ever   found   in   a   disseminated   “satellite”   nevus.   In   about   half   the   cases,   it   occurs   directly within    the    brain    or    spine,    when    it    can    present    with    persistent    headaches    with nausea/vomiting, or visual disturbances, balance problems or seizures, like other tumors. Unfortunately,   when   malignant   melanoma   occurs   in   a   person   with   a   congenital   melanocytic nevus,   it   is   often   aggressive   and   difficult   to   treat.   Our   recommendations   are   that   any   rapidly changing   area   should   be   reviewed   by   an   experienced   doctor,   who   can   decide   whether   a biopsy   is   required.   Do   remember   however,   that   nodules,   lumps   and   color   changes   appear frequently   in   congenital   melanocytic   nevi,   and   they   are   almost   as   frequently,   completely harmless.    Serial    photographs,    the    use    of    dermoscopy,    palpation    of    nevus    and    scars, examination   of   lymph   nodes   and   a   thorough   review   of   systems   may   aid   physicians   in   early detection   of   melanoma. Also,   only   experienced   pathologists   can   avoid   the   trap   of   finding   a growing   CMN   nodule   “melanoma-like”;   if   such   a   report   comes   to   you,   it   is   worth   asking   for a   second   opinion.   In   Australia   there   is   a   system   of   checks   that   occurs   during   pathological testing that reduces greatly any risk of false results. We    recommend    that    children    who    develop    any    new    neurological    problems    such    as recurrent   headaches,   visual   disturbances,   seizures,   or   developmental   changes   should   be seen     urgently     by     specialist     doctors,     such     as     a     paediatric     neurologist     and     a neuroradiologist, in a major paediatric hospital if at all possible. Skin Problems Fragility Many    congenital    melanocytic    nevi    are    more    delicate    than    normal    skin,    especially    in newborns.   They   can   therefore   tear   more   easily   if   they   are   knocked   or   scraped.   However, they   do   not   bleed   any   more   than   normal   skin,   and   they   tend   to   heal   well   with   minimal scarring.   Treat   as   any   superficial   scrape   or   wound   on   non-nevus   skin,   cleaning   with   soap and water, and use of an antiseptic ointment and a bandage. Dryness and itching The   lack   of   oil   glands   and   increased   hair   growth   along   with   the   CMN’s   naturally   volatile nature   also   has   the   potential   to   lead   to   a   problem   with   dryness   and   itching.   It   has   recently been   discovered   that   this   may   be   a   result   of   an   increase   in   mast   cells   in   the   nevus   skin   as well   as   the   ‘normal’   skin   of   the   CMN   patient.   This   is   the   same   extreme   itching   usually associated   with   eczema.   Steroid   and   non   steroid   creams   can   help   as   can   moisturisers   and emollients   particularly   oils.   Heat   or   cold   can   aggravate   this   condition.      Ice   packs   can sometimes   help.   This   extreme   itching   can   also   occur   in   scar   tissue.   It   is   possible   for   the child to break the skin while scratching. Decrease in subcutaneous fat This   is   usually   only   seen   with   larger   congenital   melanocytic   nevi.   For   some   reason,   the presence   of   the   CMN   interferes   with   setting   up   the   layer   of   fat   that   is   normally   present between   the   skin   and   underlying   muscle   and   bone.   This   can   result   in   the   nevus   appearing to   be   depressed   below   the   general   skin   surface,   the   limb,   buttock   or   side   of   the   face,   and appearing obviously thinner than normal. Overheating The   skin   of   a   CMN   is   often   lacking   in   subcutaneous   fat.   It   also   generally   has   no   sweat glands   or   oils   glands   within   the   pigmented   areas   plus   will   often   have   increased   hair   growth. This   makes   it   very   difficult   for   the   body   to   lose   heat   through   the   pigmented   areas   of   skin   or equally    through    any    scar    tissues    that    may    be    present    due    to    surgical    intervention. Overheating   can   be   a   major   problem   in   some   patients   with   CMN.   Modern   climate   controls make   this   easier   to   manage   however   be   aware   if   you   are   planning   sports   sessions   or   are   in a   non   air   conditioned   environment   that   this   is   taken   into   consideration.   It   is   not   necessary to   exclude   children   from   sports   sessions   nor   does   this   help   their   psychosocial   outcomes. Planning   outdoor   activities   for   the   mornings   or   evenings   during   warmer   months   helps.   Heat stress   and   heat   exhaustion   can   occur   very   quickly   and   are   potentially   life   threatening. Some   ways   to   manage   overheating   include   the   use   of   cooling   vests,   cold   face   washers, cool    foot    baths,    splashing    face    and    head    with    cool    water,    quiet    time    in    a    cool    spot. Occasionally   a   child   may   need   to   be   withheld   from   attending   activities   during   extreme heatwaves. Neurological Problems Problems   in   the   brain   or   spinal   cord   are   the   most   common   serious   complication   seen   in children   with   congenital   melanocytic   nevus.   Because   the   pigment   cells   found   in   the   skin and   (yes,   normally   in)   the   meninges   develop   from   the   same   cells   as   the   brain   in   the   early embryo,    people    with    CMN    can    also    have    differences    in    their    brain    or    spinal    cord. Approximately   one   in   fifteen   to   twenty   people   with   large   and   giant   forms   of   CMN   have excess   pigment   cells   in   the   brain   or   spinal   cord.   This   can   lead   to   a   complication   known   as neurocutaneous   melanocytosis   (NCM).   Earlier   studies   often   used   the   term   “melanosis”-the first means more pigment cells, and the second means more pigment. The difference is not easy to determine non-invasively. Other   much   rarer   problems   include   benign   brain   or   spinal   tumors   or   cysts,   too   much   fluid   in the   brain   (hydrocephalus),   or   abnormal   brain   structure.   All   of   these   neurological   problems are   more   common   with   larger   and   more   numerous   CMN,   but   there   is   no   connection   with the   site   of   the   CMN.   In   other   words,   having   a   congenital   melanocytic   nevus   overlying   the brain or spine does not increase the chance of having neurological problems. Should a Person with a CMN have Magnetic Resonance Imaging (MRI)? Current    recommendations    in    Australia    are    that    any    child    born    with    a    large    or    giant congenital   Nevus   and   multiple   satellite   lesions   (generally   20   or   more)      in   any   location should   have   a   routine   MRI   scan   of   the   brain   and   spine,   preferably   by   the   age   of   six months,   though   there   is   no   strict   cutoff   age.   It   is   not   possible   to   say   that   MRI   abnormalities could never occur in children with only one CMN at birth, but the likelihood is much less. The   overall   chance   of   finding   any   kind   of   abnormality   on   brain   and   spinal   MRI   scan   in children   with   large   and   multiple   CMN   may   be   as   high   as   25%,   but   only   around   half   of   these will    have    any    actual    neurological    problems.Things    to    look    out    for    include:    epileptic convulsions    (fits/seizures),    developmental    delay,    limb    coordination    issues,    frequent vomiting   or   headaches   not   linked   to   an   infection   or   fever,   and   tiptoeing   or   late   bed-wetting, which   can   be   signs   of   a   tethered   spinal   cord.   (And   which   are   also   quite   frequent   in   children and should not alarm you.) 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   tumors, cysts   and   extra   fluid   on   the   brain   that   require   an   operation,   and   to   allow   more   careful neurological   monitoring   of   those   children   with   MRI   findings.   The   pigment   cells   in   the   brain or   spine   cannot   currently   be   treated   themselves,   but   the   epilepsy   that   sometimes   occurs   in NCM   can   respond   to   conventional   medication   and   surgical   approaches,   as   can   a   tethered spinal cord or cysts. We   also   recommend   that   children   with   congenital   melanocytic   nevi   larger   than   20   cm   (8 inches)    projected    adult    diameter    be    followed    up    regularly    to    watch    their    neurological development,   even   if   they   do   not   have   more   than   one   nevus.   In   addition,   any   child   with   any type   of   CMN   who   has   neurological   symptoms   like   those   described   above   should   have   an MRI. These   neurological   problems   tend   not   to   afflict   adults   with   CMN   if   they   had   not   started during childhood or adolescence. Psychological Challenges  People   with   congenital   melanocytic   nevi   sometimes   attract   looks,   stares,   whispering   and finger-pointing. Children   who   grow   up   with   a   prominent   congenital   melanocytic   nevi   or   many   congenital melanocytic   nevi   may   well   have   problems   adjusting   to   the   disfigurement   that   they   perceive, particularly   during   the   teenage   years.   This,   however,   is   a   very   individual   thing,   and   varies depending   on   the   child’s   personality   and   on   the   support   from   family   and   friends.   The problems may manifest as anxiety, depression, or lowered self-esteem.     Family   members   sometimes   hope   that   a   nevus   will   ‘fade’   and   the   other   potential   medical challenges   will   also   disappear.   Some   large   congenital   melanocytic   nevi   can   lighten   over time,   but   the   birthmark   itself,   and   potential   challenges,   remain. This   reality   requires   not   only the person with the CMN, but also their loved ones, to take some time to adapt. Obviously   with   a   visible   physical   difference   the   potential   for   psychosocial   problems   arise. Different   children   manage   questions   and   stares   in   different   ways   and   we   recommend   that you   talk   with   your   child   regarding   how   they   feel   comfortable   having   questions   answered. A very   effective   response   is   that   it’s   a   birthmark,   it   doesn’t   hurt   and   you   can’t   catch   it.   Bullying is   an   issue   and   can   often   be   quite   subtle   but   hurtful   in   nature.   The   impact   of   this   will   also vary   from   child   to   child.   This   can   also   be   affected   by   the   gender   of   the   child   and   the location   of   the   pigmented   skin   and/or   scar   tissue.   Sometimes   areas   where   nevus   tissue has   been   removed   can   look   like   burns   scarring.   It   is   thought   that   over   30%   of   patients   with CMN   will   suffer   from   depression   and/or   anxiety   specifically   related   to   the   psychosocial pressures   related   to   their   CMN.   Depending   on   the   age   of   the   child   and   the   parent’s personal   preference,   some   will   be   aware   of   the   health   risks   associated   with   their   CMN others   will   not.   Becoming   aware   of   this   can   for   a   while   at   least,   place   extra   pressure   on   the wellbeing   of   the   child.   We   recommend   place   a   high   value   on   the   child’s   wellbeing   and emotional   health.   In   a   school   situation,   school   counsellors,   guidance   officers   and   pastoral care workers can play an important role in supporting the child. Bullying Teasing   and   bullying   can   be   a   problem.   There   is   much   written   on   the   subject   of   managing bullying   and   the   value   of   zero   tolerance   practices.   It   is   of   value   to   keep   strong   lines   of communication    open    between    home    and    the    care    environment    to    help    address    any problems as they arise. Will they grow out of it? No.   This   is   a   lifelong   condition.   The   nevus   may   change   over   time,   it   may   fade   slightly   (not disappear), get darker, get hairier, but it will not go away. So what is the general prognosis? Honestly   we   don’t   really   know.   CMN   patients   have   only   been   really   followed   for   about   the last   15   years   or   so   in   the   UK   and   USA   to   see   what   happens.   Nevus   Support Australia   has been   around   for   12   years.   We   have   no   national   birth   register   so   have   no   way   of   tracking outcomes   for   patients.   Thankfully   this   is   slowly   changing.   We   do   know   that   there   are   older CMN patients out there, some of them have married and had children of their own. So is it hereditary? We   don’t   think   so. There   are   a   few   cases   where   there   is   more   than   one   member   of   a   family with   a   large   nevus   but   not   with   a   giant   CMN   as   far   as   we   know   at   the   moment. As   far   as   we are   aware   no   one   with   a   CMN   has   had   a   child   with   a   CMN   nor   have   any   siblings. There   are cases   where   twins   and   triplets   have   been   born   with   one   affected   by   CMN   and   the   others not.   So   our   best   guess   at   the   moment   is   that   no   it   is   not   hereditary,   it   is   just   one   of   those things that we can’t explain. There is a lot of ongoing research to try to find the answers. So how does this child/person fit into normal life? These   are   normal   children/people   who   have   special   skin.   They   want   and   need   to   feel   just like any other child/person. Younger children will need your help to remind them to stay out of the sun and in the cool, even on warm not hot days. A day over about 25 degrees can get hot quickly. Other than being sun safe and aware of overheating, treat your CMN child as you would any other child.
ABN: 23543860400
This information on this website is for general information purposes only. It is not intended as a medical reference. Please talk with your doctor for medical advice.
There are a number of risks and considerations with CMN