Phakomatoses are a group of neurocutaneous disorders characterised by involvement of structures that arise from the embryonic ectoderm (thus central nervous system, skin and eyes). Other organs may also be involved



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Phakomatoses

Pathology

It is sporadic with no definite identifiable hereditary component 5.

The leptomeningeal haemangioma results in a vascular steal affecting the subjacent cortex and white matter producing localised ischaemia. The majority of cases (~ 80%) have unihemispherical involvement.

It is a type of craniofacial arteriovenous metameric syndrome (CAMS)



Associations

  • coarctation of aorta 10

  • paragangliomas 9 

Radiographic features

Plain film

Skull x-rays were historically useful and capable of identifying the gyriform calcification of the subcortical white matter although they no longer play a significant role in the diagnosis or management of this condition. The finding usually becomes evident between 2 and 7 years of age 2.



CT

  • detects subcortical calcification at an earlier age than plain film and can also demonstrate associated parenchymal volume loss

  • appearance liken to 'tram-track' in nature

  • calvarial and regional sinus enlargement may be evident

  • ipsilateral choroid plexus may be enlarged

  • in severe cases a Dyke-Davidoff-Masson appearence may be seen


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