June 2013
Volume 54, Issue 15
Free
ARVO Annual Meeting Abstract  |   June 2013
A modified staged surgical intervention for blepharophimosis-ptosis-epicanthus inversus syndrome: 125 cases with encouraging results
Author Affiliations & Notes
  • Xin Song
    Ophthalmology, Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China
  • Xianqun Fan
    Ophthalmology, Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China
  • Footnotes
    Commercial Relationships Xin Song, None; Xianqun Fan, None
  • Footnotes
    Support None
Investigative Ophthalmology & Visual Science June 2013, Vol.54, 6374. doi:
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      Xin Song, Xianqun Fan; A modified staged surgical intervention for blepharophimosis-ptosis-epicanthus inversus syndrome: 125 cases with encouraging results. Invest. Ophthalmol. Vis. Sci. 2013;54(15):6374.

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Abstract
 
Purpose
 

Blepharophimosis-ptosis-epicanthus inversus syndrome (BPES) is a rare autosomal dominant condition characterized by a typical eyelid malformation that includes blepharophimosis, ptosis, epicanthus inversus, and telecanthus. This study aimed to objectively assess the effects of a modified staged surgical intervention for BPES, especially frontalis function.

 
Methods
 

Retrospective review of 125 consecutive patients with BPES who underwent staged surgical intervention from July 2003 to December 2011. All patients received a medial canthoplasty and lateral canthoplasty at first, followed by blephroptosis correction 6 months to 12 months later. The parameters that were studied included horizontal palpebral fissure length (PFL), vertical interpalpebral fissure height (PFH), inner intercanthal distance (IICD), the ratio of IICD to PFL and frontalis function. Facial photographs were taken before and after the operations. A paired t-test and group t-test were used as the statistical analysis methods to evaluate the surgical outcomes.

 
Results
 

With these consecutive operations, the mean PFL was increased from 19.5 mm to 25.7 mm, with a mean difference of 6.2 mm (P<.01). The mean PFH was increased from 3.4 mm to 8.5 mm, with a difference of 5.1 mm (P<.01). The mean IICD was decreased from 38.0 mm to 30.9 mm with a mean difference of 7.1 mm (P<.01). The mean frontalis function of BPES patients was 7.3 mm for patients about 5 years old and was 10.4 mm for patients about 7 years old. There was no difference between children who underwent muscle flap suspension operations and healthy children of the same age (P>.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 frontalis function was not weakened by surgery.

 
 
Frontalis muscle flap suspension
 
Frontalis muscle flap suspension
   
Keywords: 466 clinical (human) or epidemiologic studies: treatment/prevention assessment/controlled clinical trials • 526 eyelid • 650 plasticity  
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