title: Blepharophimosis: a recommendation for early surgery in patients with severe ptosis.
abstract: OBJECTIVE
  To determine the optimal age for surgical correction of blepharophimosis. Associated features and their effects on incidence of amblyopia were also investigated.
  
  
  METHODS
  The study was a retrospective case series of 28 patients with blepharophimosis, ptosis and epicanthus inversus syndrome presenting to a tertiary referral eyelid, lacrimal and orbital clinic.
  
  
  RESULTS
  Amblyopia was present in 39% of patients. Patients with coexistent strabismus had a 64% incidence of amblyopia compared to 24% for those without strabismus. Hypermetropia was present in 43% of patients and 7% were myopic. Significant astigmatism was found in 40% of patients, but these factors did not increase the risk of amblyopia. Patients with severe ptosis had lower rates of amblyopia than those with moderate ptosis but had their ptosis corrected at a median age of 2 years compared to 5 years for those with moderate ptosis. There was an 18% incidence of nasolacrimal drainage problems. A good to excellent cosmetic outcome was achieved in 86% of patients. A positive family history was noted in 75% of patients, usually with paternal inheritance.
  
  
  CONCLUSIONS
  Patients with blepharophimosis have a high rate of amblyopia. Co-existent strabismus doubles the risk of amblyopia. Ptosis alone causes mild to moderate amblyopia only. Patients with severe ptosis should have their ptosis corrected before 3 years of age, and all other patients should undergo surgery before 5 years of age.
authors.affiliation: Eyelid, Lacrimal and Orbital Clinic, Royal Children's Hospital, Brisbane, Queensland, Australia.
authors.forename: Peter S
authors.initials: PS
authors.lastname: Beckingsale
authors.forename: Timothy J
authors.initials: TJ
authors.lastname: Sullivan
authors.forename: Vincent A
authors.initials: VA
authors.lastname: Wong
authors.forename: Christine
authors.initials: C
authors.lastname: Oley
mesh_terms: D000367:Age Factors; D000368:Aged; D000550:Amblyopia / Q000209:etiology; D016569:Blepharophimosis / Q000150:complications* / Q000175:diagnosis / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D005143:Eyelids / Q000601:surgery; D005260:Female; D006801:Humans; D015994:Incidence; D007223:Infant; D008297:Male; D008875:Middle Aged; D013508:Ophthalmologic Surgical Procedures; D012189:Retrospective Studies; D012307:Risk Factors; D013285:Strabismus / Q000150:complications; D013577:Syndrome