Hypomelanosis of Ito presenting with unilateral dilation of Virchow-Robin spaces: a case report


Hypomelanosis of Ito is a rare heterogeneous neurocutaneous disorder often associated with central nervous and musculoskeletal system involvement. Herein, we report the first case of hypomelanosis of Ito in the literature presenting with unilateral dilation of Virchow-Robin spaces (VRS). A girl aged 16 years old presented with a 1-year history of headache. Her physical and neurological examinations were normal, except for the presence of unilateral cutaneous macular hypopigmented whorls and streaks on lower side of the right trunk and lower limb, termed as Blaschko’s lines. She had mild deficits in cognitive and adaptive functioning. Hearing, renal, dental, ophthalmologic, metabolic, and cardiac assessments were normal. Brain magnetic resonance imaging (MRI) showed markedly unilateral hemispheric enlarged VRS without contrast enhancement and diffusion restriction. To the best of our knowledge, our case is the first report describing the unilateral hemispheric enlarged VRS in a patient with hypomelanosis of Ito. Our report suggested that hypomelanosis of Ito may have unilateral dilation of VRS in brain MRI.

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Correspondence to Miraç Yıldırım.

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Uçar, Ç.İ., Yıldırım, M., Sayar, Y. et al. Hypomelanosis of Ito presenting with unilateral dilation of Virchow-Robin spaces: a case report. Childs Nerv Syst (2021). https://doi.org/10.1007/s00381-021-05083-1

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  • Hypomelanosis of Ito
  • Virchow-Robin spaces
  • Magnetic resonance imaging
  • Unilateral
  • Blaschko’s lines