Bilateral Fist Lid-Lift: A Novel Compensatory Behavior in an Infant with Blepharophimosis Syndrome.

Background/

Objectives

To describe a previously unreported compensatory behavior used by an infant with severe bilateral congenital ptosis associated with blepharophimosis syndrome (BPES).

Methods

Observational case report of a 4.5-month-old infant with severe bilateral congenital upper eyelid ptosis due to BPES.

Results

The infant demonstrated classic compensatory mechanisms, including frontalis overaction and chin elevation, which were insufficient to clear the visual axis. Notably, she repeatedly used the dorsal surfaces of both fists to elevate the upper eyelids simultaneously and maintain fixation on faces. This behavior ceased following bilateral frontalis suspension surgery with silicone rods.

Conclusions

In early infancy, severe bilateral ptosis may prompt the emergence of alternative, developmentally constrained compensatory behaviors. The bilateral fist lid-lift appears to represent a visually driven, sensorimotor strategy to clear the visual axis when conventional mechanisms are ineffective. Recognition of this behavior expands understanding of early compensatory responses in congenital ptosis and BPES.