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Increased levator muscle function by supramaximal resection in patients with blepharophimosis-ptosis-epicanthus inversus syndrome

CHRISTIAN DECOCK UGent, Akash D Shah, Christophe Delaey UGent, Ramses Forsyth UGent, WOUTER BAUTERS UGent, Philippe Kestelyn UGent, Elfride De Baere UGent and Ilse Claerhout UGent (2011) ARCHIVES OF OPHTHALMOLOGY. 129(8). p.1018-1022
abstract
Objective: To study the efficacy and clinical and anatomical results of supramaximal levator resection in patients with blepharophimosis-ptosis-epicanthus inversus syndrome (BPES) with severe congenital ptosis with poor levator function (LF). Methods: Eleven patients with molecularly proven BPES underwent supramaximal levator resection. Palpebral fissure height and LF were measured preoperatively and postoperatively. Results: All patients showed an excellent reduction in ptosis with a single intervention resulting in a clear visual axis. Palpebral fissure height improved from mean (SD) 3.3 (0.7) mm preoperatively to 7.1 (0.9) mm postoperatively (P value < .001). Four patients underwent additional surgery because of cosmetic issues with eyelid height asymmetry. All patients showed a marked, consistent, and lasting improvement in LF, going from mean (SD) 1.9 (0.9) mm preoperatively to 7.4 (1.1) mm postoperatively (P value < .001). This improvement could be attributed to the presence of a very long and thin tendon, as well as a striated muscle belly. This elongated aponeurosis inhibits the levator muscle from having sufficient impact on the vertical eyelid excursion. Conclusions: We demonstrated that supramaximal levator resection performed in patients with BPES not only results in good cosmetic appearance in terms of ptosis reduction in the majority of cases but also in a significant increase of the levator palpebrae superioris function. An anatomical substrate was found to explain these findings. To our knowledge, this is the first study to provide evidence of a marked increase in LF in BPES due to resection of the elongated tendon with reinsertion of the muscle belly.
Please use this url to cite or link to this publication:
author
organization
year
type
journalArticle (original)
publication status
published
subject
keyword
Fascia lata, suspension, Severe blepharoptosis, syndrome BPES, surgery
journal title
ARCHIVES OF OPHTHALMOLOGY
Arch. Ophthalmol.
volume
129
issue
8
pages
1018 - 1022
Web of Science type
Article
Web of Science id
000293648800007
JCR category
OPHTHALMOLOGY
JCR impact factor
3.711 (2011)
JCR rank
5/56 (2011)
JCR quartile
1 (2011)
ISSN
0003-9950
DOI
10.1001/archophthalmol.2011.75
language
English
UGent publication?
yes
classification
A1
copyright statement
I have transferred the copyright for this publication to the publisher
id
2015647
handle
http://hdl.handle.net/1854/LU-2015647
date created
2012-02-02 10:34:41
date last changed
2012-03-01 16:02:01
@article{2015647,
  abstract     = {Objective: To study the efficacy and clinical and anatomical results of supramaximal levator resection in patients with blepharophimosis-ptosis-epicanthus inversus syndrome (BPES) with severe congenital ptosis with poor levator function (LF). 
Methods: Eleven patients with molecularly proven BPES underwent supramaximal levator resection. Palpebral fissure height and LF were measured preoperatively and postoperatively. 
Results: All patients showed an excellent reduction in ptosis with a single intervention resulting in a clear visual axis. Palpebral fissure height improved from mean (SD) 3.3 (0.7) mm preoperatively to 7.1 (0.9) mm postoperatively (P value {\textlangle} .001). Four patients underwent additional surgery because of cosmetic issues with eyelid height asymmetry. All patients showed a marked, consistent, and lasting improvement in LF, going from mean (SD) 1.9 (0.9) mm preoperatively to 7.4 (1.1) mm postoperatively (P value {\textlangle} .001). This improvement could be attributed to the presence of a very long and thin tendon, as well as a striated muscle belly. This elongated aponeurosis inhibits the levator muscle from having sufficient impact on the vertical eyelid excursion. 
Conclusions: We demonstrated that supramaximal levator resection performed in patients with BPES not only results in good cosmetic appearance in terms of ptosis reduction in the majority of cases but also in a significant increase of the levator palpebrae superioris function. An anatomical substrate was found to explain these findings. To our knowledge, this is the first study to provide evidence of a marked increase in LF in BPES due to resection of the elongated tendon with reinsertion of the muscle belly.},
  author       = {DECOCK, CHRISTIAN and Shah, Akash D and Delaey, Christophe and Forsyth, Ramses and BAUTERS, WOUTER and Kestelyn, Philippe and De Baere, Elfride and Claerhout, Ilse},
  issn         = {0003-9950},
  journal      = {ARCHIVES OF OPHTHALMOLOGY},
  keyword      = {Fascia lata,suspension,Severe blepharoptosis,syndrome BPES,surgery},
  language     = {eng},
  number       = {8},
  pages        = {1018--1022},
  title        = {Increased levator muscle function by supramaximal resection in patients with blepharophimosis-ptosis-epicanthus inversus syndrome},
  url          = {http://dx.doi.org/10.1001/archophthalmol.2011.75},
  volume       = {129},
  year         = {2011},
}

Chicago
DECOCK, CHRISTIAN, Akash D Shah, Christophe Delaey, Ramses Forsyth, WOUTER BAUTERS, Philippe Kestelyn, Elfride De Baere, and Ilse Claerhout. 2011. “Increased Levator Muscle Function by Supramaximal Resection in Patients with Blepharophimosis-ptosis-epicanthus Inversus Syndrome.” Archives of Ophthalmology 129 (8): 1018–1022.
APA
DECOCK, C., Shah, A. D., Delaey, C., Forsyth, R., BAUTERS, W., Kestelyn, P., De Baere, E., et al. (2011). Increased levator muscle function by supramaximal resection in patients with blepharophimosis-ptosis-epicanthus inversus syndrome. ARCHIVES OF OPHTHALMOLOGY, 129(8), 1018–1022.
Vancouver
1.
DECOCK C, Shah AD, Delaey C, Forsyth R, BAUTERS W, Kestelyn P, et al. Increased levator muscle function by supramaximal resection in patients with blepharophimosis-ptosis-epicanthus inversus syndrome. ARCHIVES OF OPHTHALMOLOGY. 2011;129(8):1018–22.
MLA
DECOCK, CHRISTIAN, Akash D Shah, Christophe Delaey, et al. “Increased Levator Muscle Function by Supramaximal Resection in Patients with Blepharophimosis-ptosis-epicanthus Inversus Syndrome.” ARCHIVES OF OPHTHALMOLOGY 129.8 (2011): 1018–1022. Print.