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

Arch Ophthalmol. 2011 Aug;129(8):1018-22. doi: 10.1001/archophthalmol.2011.218.


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.

MeSH terms

  • Adolescent
  • Blepharophimosis / physiopathology
  • Blepharophimosis / surgery*
  • Blepharoptosis / congenital
  • Blepharoptosis / physiopathology
  • Blepharoptosis / surgery*
  • Child
  • Child, Preschool
  • Eyelids / abnormalities*
  • Eyelids / physiopathology
  • Humans
  • Magnetic Resonance Imaging
  • Oculomotor Muscles / physiopathology*
  • Oculomotor Muscles / surgery*
  • Ophthalmologic Surgical Procedures*
  • Syndrome
  • Tendons / surgery
  • Treatment Outcome