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Epidermal nevus syndrome in a 16-year-old boy (MRI)

(A) Axial MRI at level of orbits reveals a left ocular hamartoma and also shows a mass in the occipital horn of the left lateral ventricle that does not obstruct the flow of CSF. (B) Coronal view of MRI at the level of the parietal lobes shows abnormal cerebral convolutions in the left hemisphere. The occipital horn of the left lateral ventricle shows the amorphous cyst of density less than brain but more than ventricular fluid. (Contributed by Dr. Laura Flores-Sarnat.)

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Associated Disorders

  • Cardiac anomalies
  • Hemimegalencephaly
  • Infantile spasms
  • Ocular anomalies
  • Vitamin D-resistant rickets