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Congenital Nevocellular Naevi - Do we need to screen with neuroimaging?

Three Part Question

In [a patient with no obvious neurology but a large congenital nevocellular naevus] is [screening with neuroimaging] in [identifying subclinical lesions]important?

Clinical Scenario

A 3 day old neonate female was seen by the neonatologist and noted to have a large congenital melanocytic/nevocellular naevus. The neonate had no focal neurology and was otherwise well in herself following a normal vaginal delivery. There was no family history of note.

Search Strategy

Medline interface on the world wide web. 1966 – February 2011
[({congenital nevocellular or melanocytic} AND {imaging} AND {screening}]
LIMIT to English

Search Outcome

3 papers were found which addressed the question.

Relevant Paper(s)

Author, date and country Patient group Study type (level of evidence) Outcomes Key results Study Weaknesses
V A Kinsler, S E Aylett, S C Coley,W K Chong, D J Atherton
13 July 2000
43 children (20 boys and 23 girls) with a median age of 2.6 years, with congenital melanocytic naevi over 2 cm on th head or over spine were offered MRI of the brain and/or spine.Prospective case control studyLesion found on MRI prior to abnormal neurologyAbnormal neurology could have been present prior to the screening but not reported
Frieden IJ, Williams ML, Barkovich AJ.
20 neurologically asymptomatic children with large or multiple congenital melanocytic naevi. Prospective case control studyAbnormal MRI in 45%33% of abnormal MRIs were due to intracranial melanosis
Ruiz-Maldonado R, del Rosario Barona-Mazuera M, Hidalgo-Galvan LR, et al.
13 children with congenital melanocytic naevi and normal neurology who had a screening MRI.Prospective case control None of the patients had intrcranial melanosis on MRIHowever 6 patients of 13 had anatomical abnormalities of the CNSAll were thought to be neurologically asymptomatic, but 11 were subsequently found to have neurological signs on examination.


There is no evidence to determine if early detection of intracranial melanosis improves prognosis.

Clinical Bottom Line

Recommendation: All patients with large lesions should have MRIs of their CNS at an early stage and routine follow up with neurological history and examination.


  1. V A Kinsler, S E Aylett, S C Coley,W K Chong, D J Atherton Central nervous system imaging and congenital Arch Dis Child
  2. Frieden IJ, Williams ML, Barkovich AJ. Giant congenital melanocytic nevi: brain magnetic resonance findings in neurologically asyptomatic children. J Am Acad Dermatol 1994;31:423–9.
  3. Ruiz-Maldonado R Giant congenital melanocytic nevi, neurocutaneous melanosis and neurological alterations. Dermatology 1997;195:125–8.