Hemihypertrophy Associated with Hemimegalencephaly in a Newborn

A 2-day-old female patient was seen shortly after birth for seizures involving her left face and left side of her body. Notable findings on examination included macrocephaly, enlarged right lower face, and hypertrophy of the right side of her tongue. The right lower extremity was also larger compared with the left (Figure 1). Magnetic resonance imaging (MRI) done shortly thereafter demonstrated an asymmetric increase in the size of the right cerebral hemisphere, right cerebellum, and right side of the brain stem (Figure 2).

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• Figure 1 

  Photograph of the child with hypertrophy of the right lower extremity.

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• Figure 2 

  A, Asymmetry in the cerebellum demonstrated in axial fluid attenuated inversion recovery (FLAIR) MRI. B, MRI of the brain demonstrating poorly differentiated white and grey matter with larger ventricle on the affected side. The sulci are not as clearly demonstrated on the right compared with the left hemisphere. Hypertrophied brain stem on the affected side noted and at the level of the midbrain. Inversion recovery (FLAIR) MRI in axial section.