Surgical Management of Blepharophimosis-Ptosis-Epicanthus Inversus Syndrome (BPES): A Comprehensive Review

Blepharophimosis-ptosis-epicanthus inversus syndrome (BPES) is a rare congenital eyelid disorder that leads to drooping eyelids, narrowing of the palpebral fissures, and a characteristic facial appearance. It is a genetic condition, typically inherited in an autosomal dominant pattern, and is primarily caused by mutations in the FOXL2 gene, which is essential for eyelid development. Two types of BPES have been described: type II presents eyelid malformations and facial dysmorphism, while type I is additionally associated with premature ovarian insufficiency. If untreated, these features can lead to visual impairment and psychosocial consequences. A pronounced epicanthal fold may lead to a reduction in the visual field and contribute to visual disturbances, including strabismus. BPES is associated with underdeveloped or dysplastic levator palpebrae muscles, leading to four clinical features-telecanthus, ptosis, epicanthus inversus, and blepharophimosis-with reduced horizontal palpebral fissure length.

A systematic review of the literature was performed to summarize current surgical management strategies for BPES. Surgical indications, outcomes, and complications were extracted and analyzed.

Surgical treatment remains the standard of care for BPES. In recent years, numerous techniques have been developed to correct deformities including both classic and modern methods of epicanthoplasty, canthoplasty, and forehead muscle suspension using autologous and synthetic materials. Complication rates vary depending on technique and patient factors. This systematic review summarizes current surgical strategies, indications, outcomes and complications, highlighting modern approaches that optimize visual function and aesthetic outcomes.

Surgical management of BPES is essential to restore eyelid function, improve the visual field, and achieve favorable cosmetic outcomes. A range of classical and contemporary techniques are available, and selection should be individualized. Modern approaches optimize both functional and aesthetic results, though further prospective studies are needed to standardize procedures and minimize complications.