title: Ultrabiomicroscopic-histopathologic correlations in individuals with autosomal dominant congenital microcoria: three-generation family report.
abstract: BACKGROUND
  Congenital microcoria (CMC) is due to a maldevelopment of the dilator pupillae muscle of the iris, with a pupil diameter of less than 2 mm. It is associated with juvenile open angle glaucoma and myopia. We report on a three-generation Mexican-Mestizo family with CMC. The eldest member's iris biopsy proved muscle anomalies. Further, we analyzed novel ultrasound biomicroscopy findings in the family members who did not require surgery.
  
  
  METHODS
  A 62-year-old woman, her 41-year-old son and her 9-year-old grandson affected with microcoria since birth, documented by clinical examination and ultrasound biomicroscopy. The eldest member underwent phacoemulsification, and a biopsy of the iris and the anterior capsule of the lens was taken.
  
  
  RESULTS
  Ultrasound biomicroscopy confirmed the CMC diagnosis showing iris thinning and a pupil diameter of less than 2 mm. Histopathology of the iris showed a significant reduction of smooth muscle cells, but no alterations of the anterior lens capsule.
  
  
  CONCLUSIONS
  Although CMC is a rare disorder, which is due to a maldevelopment of the dilator pupillae muscle of the iris, it could be associated with juvenile open angle glaucoma and myopia; therefore, precise diagnosis is required. Ultrasound biomicroscopy could be a great option to confirm the disorder.
authors.affiliation: Anterior Segment Department, Instituto de Oftalmología Fundación Conde de Valenciana, Mexico City, Mexico.
authors.forename: Arturo
authors.initials: A
authors.lastname: Ramirez-Miranda
authors.forename: Juan M
authors.initials: JM
authors.lastname: Paulin-Huerta
authors.forename: Eduardo
authors.initials: E
authors.lastname: Chavez-Mondragón
authors.forename: Gilberto
authors.initials: G
authors.lastname: Islas-de la Vega
authors.forename: Abelardo
authors.initials: A
authors.lastname: Rodriguez-Reyes