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

(2011) ARCHIVES OF OPHTHALMOLOGY. 129(8). p.1018-1022
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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.
Keywords
Fascia lata, suspension, Severe blepharoptosis, syndrome BPES, surgery

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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.
@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},
}

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