[Clinical results of primary and complex resection of levator palpebrae superioris musculus in treatment of blepharophimosis-ptosis-epicanthus inversus syndrome].

OBJECTIVE

To observe the clinical outcome of treating blepharophimosis-ptosis-epicanthus inversus syndrome (BPES) by means of primary and complex resection of levator palpebrae superioris musculus.

METHODS

From May 2001 to May 2007, 12 patients with BPES were treated, including 6 males and 6 females aged 4-15 years old (average 7 years old). All patients marked signs of BPES--typical ptosis of the upper eyelids, epicanthus inversus, palpebral fissure, and increased distance between inner canthus. The eye fissure width was (2.8 +/- 1.8) mm, the eye fissure length was (19.8 +/- 4.7) mm, and the inner canthi diameter was (41.6 +/- 6.5) mm. The muscular strength of levator palpebrae superioris was deficient in 4 cases, the muscular strength of levator palpebrae superioris was (2.0 +/- 0.6) mm in 8 cases. All patients were associated with visual function congenital defects of varying degrees. The surgical technique included shortening of the internal canthal ligaments, resection of the tarsus and levator muscle, and skin plasty.

RESULTS

All the incisions healed by first intension. Twelve patients were followed up for 12-48 months (average 30 months). Amelioration of ptosis and epicanthus was achieved. At 18 months after operation, the eye fissure width of 10 patients was (9.0 +/- 2.1) mm, the eye fissure length was (26.5 +/- 3.5) mm, and inner canthi diameter was (30.2 +/- 2.7) mm, the muscular strength of levator palpebrae superioris increased to (5.6 +/- 1.9) mm, showing significant difference when compared with preoperation (P < 0.05).

CONCLUSION

The primary and complex resection of levator palpebrae superioris musculus can provide relating good cosmetic and functional results for the correction of BPES. Patients with BPES should receive surgery as early as possible.