Bilateral macrostomia associated with aqueductal stenosis and glial heterotopias

  • Pepe E
  • Petricig P
  • Peretta P
 et al. 
  • 11


    Mendeley users who have this article in their library.
  • 3


    Citations of this article.


We report on an Italian boy, born to normal and nonconsanguineous parents with a prenatal diagnosis of ventriculomegaly and subependymal glial heterotopias. At birth bilateral macrostomia was diagnosed without other evident facial anomalies. Magnetic resonance imaging (MRI) showed triventricular hydrocephalus and aqueductal stenosis and confirmed the nodules of glial heterotopia. The bilateral macrostomia was surgically corrected with the vermilion square flap method and W-plasty technique and follow up MRI at 6 months showed mild increase of ventricular dilatation without signs of active hydrocephalus. The association between macrostomia and hydrocephalus has been reported only in rare cases of complex malformative syndromes but never with isolated macrostomia.

Author-supplied keywords

  • Aqueductal stenosis
  • Brain malformation
  • Glial heterotopias
  • Hydrocephalus
  • Macrostomia
  • Vermilion flap

Get free article suggestions today

Mendeley saves you time finding and organizing research

Sign up here
Already have an account ?Sign in

Find this document


  • Ernesto Pepe

  • Paola Petricig

  • Paola Peretta

  • Giuseppe Cinalli

Cite this document

Choose a citation style from the tabs below

Save time finding and organizing research with Mendeley

Sign up for free