title: A modified staged surgical intervention for blepharophimosis-ptosis-epicanthus inversus syndrome: 125 cases with encouraging results.
abstract: BACKGROUND
  Blepharophimosis-ptosis-epicanthus inversus syndrome (BPES) is a rare autosomal dominant condition characterized by typical eyelid malformations that include blepharophimosis, ptosis, epicanthus inversus, and telecanthus.
  
  
  METHODS
  We retrospectively reviewed 125 consecutive BPES patients who underwent staged surgical intervention from July 2003 to December 2011. All patients underwent initial medial and lateral canthoplasties, followed by blephroptosis correction 6 to 12 months afterward. The parameters that were studied included horizontal palpebral fissure length (PFL), vertical interpalpebral fissure height, inner intercanthal distance (IICD), the ratio of IICD to PFL, and frontalis function (FF). Facial photographs were taken preoperatively and postoperatively. Paired and group t tests were used for statistical analysis to evaluate surgical outcomes.
  
  
  RESULTS
  After consecutive operations, the mean PFL increased from 19.5 to 25.7 mm (mean difference, 6.2 mm; P < 0.01). The mean interpalpebral fissure height increased from 3.4 to 8.5 mm (mean difference, 5.1 mm; P < 0.01). The mean IICD decreased from 38.0 to 30.9 mm (mean difference, 7.1 mm; P < 0.01). The mean FF was 7.3 mm for BPES patients approximately 5 years old and 10.4 mm for patients approximately 7 years old. There was no difference between children who underwent muscle flap suspension and healthy children of the same age (P > 0.05).
  
  
  CONCLUSIONS
  The modified staged surgical intervention, including Y-V flap, von Ammon, and frontalis muscle flap suspension, provided effective results both in function and cosmesis for BPES. The FF was not weakened by surgery.
authors.affiliation: From the *From the Department of Ophthalmology, Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai; and †Taipei Medical University-Shuang Ho Hospital, Taipei, China.
authors.forename: Xin
authors.initials: X
authors.lastname: Song
authors.forename: Renbing
authors.initials: R
authors.lastname: Jia
authors.forename: Huimin
authors.initials: H
authors.lastname: Zhu
authors.forename: Yixiong
authors.initials: Y
authors.lastname: Zhou
authors.forename: Ying
authors.initials: Y
authors.lastname: Sun
authors.forename: Ming
authors.initials: M
authors.lastname: Lin
authors.forename: Yao
authors.initials: Y
authors.lastname: Fu
authors.forename: Jin
authors.initials: J
authors.lastname: Li
authors.forename: Zhengkang
authors.initials: Z
authors.lastname: Li
authors.forename: Linna
authors.initials: L
authors.lastname: Lu
authors.forename: Yundun
authors.initials: Y
authors.lastname: Shen
authors.forename: Shengfang
authors.initials: S
authors.lastname: Ge
authors.forename: Xianqun
authors.initials: X
authors.lastname: Fan
mesh_terms: D016569:Blepharophimosis / Q000601:surgery*; D019882:Blepharoplasty / Q000379:methods*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007765:Lacrimal Apparatus / Q000601:surgery; D008297:Male; D012189:Retrospective Studies; D012868:Skin Abnormalities / Q000601:surgery*; D013524:Surgical Flaps; D016896:Treatment Outcome; D014564:Urogenital Abnormalities / Q000601:surgery*