title: Hallermann-Streiff syndrome with severe bilateral enophthalmos and radiological evidence of silent brain syndrome: a new congenital silent brain syndrome?
abstract: BACKGROUND
  We present the first case of a congenital form of silent brain syndrome (SBS) in a young patient affected by Hallermann-Streiff syndrome (HSS) and the surgical management of the associated eyelid anomalies.
  
  
  METHODS
  HSS signs were evaluated according to the Francois criteria. Orbital computed tomography (CT) and genetic analysis were performed. An upper eyelid retractor-free recession was performed. Follow-up visits were performed at day 1, weeks 1 and 3, and months 3, 6, 9 (for both eyes), and 12 (for left eye) after surgery.
  
  
  RESULTS
  The patient exhibited six of the seven signs of HSS. Orbital CT showed bilateral enophthalmos and upward bowing of the orbital roof with air entrapment under the upper eyelid as previously described for SBS. Genetic analysis showed a 2q polymorphism. During follow-up, the cornea showed absence of epithelial damage and the upper eyelids were lowered symmetrically, with a regular contour.
  
  
  CONCLUSIONS
  Our HSS patient shares features with SBS. We postulate that SBS could include more than one pattern, ie, an acquired form following ventriculoperitoneal shunting and this newly reported congenital form in our HSS patient in whom typical syndromic skull anomalies led to this condition. The surgical treatment has been effective in restoring an appropriate lid level, with good globe apposition and a good cosmetic result.
authors.affiliation: Eye Clinic, San Giuseppe Hospital, University of Milan, Italy.
authors.forename: Paolo
authors.initials: P
authors.lastname: Nucci
authors.forename: Carlo
authors.initials: C
authors.lastname: de Conciliis
authors.forename: Matteo
authors.initials: M
authors.lastname: Sacchi
authors.forename: Massimiliano
authors.initials: M
authors.lastname: Serafino