title: Ocular anomalies in incontinentia pigmenti: literature review and meta-analysis.
abstract: BACKGROUND
  Incontinentia pigmenti (IP) is an X-linked genodermatosis in which skin changes are combined with dental, eye and central nervous system anomalies.
  
  
  OBJECTIVE
  The goal of the study was to analyze ocular findings, IP minor criteria in available literature concerning IP cases published until now.
  
  
  METHODS
  We have done meta-analysis of 1931 IP patients found in 302 references published until 2010. Comparison of data published for the 1906-1976 and 1976-2010 periods was made. The collected data were mainly frequencies of ocular anomalies. Chi-square test was used to compare observed frequencies with their expectations.
  
  
  RESULTS
  Of total number of IP patients, 1227 were ophthalmologically investigated. In 449 such patients 972 eye anomalies were registered, 2.16 anomalies per patient. Proportion of ophthalmologically investigated IP patients in the period 1906-1975 (70%) was higher than corresponding proportion (60%) for the period 1976-2010. For 1906-2010 period 36.5% IP patients with eye anomalies were diagnosed. The number of amaurotic eyes per patient did not significantly differ for the two periods (p = 0.50; > 0.05). The total number of eye anomalies per patient significantly differed for the same periods (p = 0.00005; < 0.05). Retinal anomalies were most frequent in both periods.
  
  
  CONCLUSIONS
  This study suggests that IP is far more frequent than anyone could estimate. We believe that this study, covering 1906-2010 period, gives more reliable information about ophthalmological findings in IP; considering them as severe anomalies. Early detection and treatment of ophthalmological, neurological etc. findings may prevent severe consequences that IP may cause.
authors.affiliation: 'Institute of Dermatology, Clinical Centre of Serbia, School of Medicine, University of Belgrade, Belgrade, Serbia.
authors.forename: Snezana
authors.initials: S
authors.lastname: Minić
authors.forename: Miljana
authors.initials: M
authors.lastname: Obradović
authors.forename: Igor
authors.initials: I
authors.lastname: Kovacević
authors.forename: Dusan
authors.initials: D
authors.lastname: Trpinac
mesh_terms: D005124:Eye Abnormalities / Q000150:complications*; D005128:Eye Diseases / Q000150:complications; D006801:Humans; D007184:Incontinentia Pigmenti / Q000150:complications*