title: Surgery in unilateral congenital cataract caused by persistent fetal vasculature or minimal fetal vascular remnants: age-related findings and management challenges.
abstract: OBJECTIVE
  To document in detail the surgical management challenges over the wide spectrum of persistent fetal vasculature syndrome (PFVS).
  
  
  METHODS
  Department of Ophthalmology, University of Vienna, Medical School, Vienna, Austria.
  
  
  METHODS
  As part of an ongoing prospective clinical trial of the treatment and etiology of pediatric cataract, a subgroup of 31 children with unilateral cataract was defined. Standard surgical techniques were used based on age. Group 1 comprised infants between 0 and 1.5 years; Group 2, preschool children between 1.6 and 5.9 years; and Group 3, school-aged children between 6 and 16 years. Additional surgical procedures were used based on the degree of PFVS.
  
  
  RESULTS
  All 31 eyes with unilateral cataract showed signs of PFVS. Characteristic features were found in 75% of eyes in Group 1, 8% of eyes in Group 2, and 67% of eyes in Group 3. Minimal fetal vascular remnants were found in 92%, 25%, and 33%, respectively. Correct diagnosis of PFVS was made preoperatively in 56% of eyes in Group 1, 8% in Group 2, and 67% in Group 3. Surgical procedures in addition to standard age-related techniques were necessary in all eyes with unilateral cataract.
  
  
  CONCLUSIONS
  Results indicate that varying degrees of PFVS are a frequent cause of unilateral congenital cataract. Most severe cases were in infants, and preschool children were usually mildly affected. Vitreoretinal complications may lead to challenges in the surgical management in infants. In preschool children, cataract surgery must be performed in a guarded fashion because of the high risk for preexisting posterior capsule breaks due to minimal fetal vascular remnants.
authors.affiliation: Department of Ophthalmology, University Hospital of Vienna, Vienna, Austria. andrea.mueller-eidenboeck@akh-wien.ac.at
authors.forename: Andrea
authors.initials: A
authors.lastname: Müllner-Eidenböck
authors.forename: Michael
authors.initials: M
authors.lastname: Amon
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Hauff
authors.forename: Nina
authors.initials: N
authors.lastname: Klebermass
authors.forename: Claudette
authors.initials: C
authors.lastname: Abela
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Moser
mesh_terms: D000293:Adolescent; D000367:Age Factors; D002386:Cataract / Q000151:congenital*; D002387:Cataract Extraction / Q000379:methods*; D002648:Child; D002675:Child, Preschool; D005124:Eye Abnormalities / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*; D005312:Fetal Blood; D006801:Humans; D006965:Hyperplasia; D007223:Infant; D007231:Infant, Newborn; D007908:Lens, Crystalline / Q000098:blood supply* / Q000473:pathology; D011446:Prospective Studies; D014822:Vitreous Body / Q000002:abnormalities* / Q000473:pathology