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

TL;DR

A 4.5-month-old infant with a rare eyelid condition developed a unique behavior using her fists to lift her eyelids, which stopped after surgery.

Contribution

The paper reports a novel compensatory behavior, the bilateral fist lid-lift, in an infant with blepharophimosis syndrome.

Findings

The infant used both fists to elevate her eyelids when conventional compensatory mechanisms failed.

This behavior ceased after successful surgical intervention with frontalis suspension.

The behavior is proposed as a sensorimotor strategy to maintain visual axis clearance.

Abstract

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.

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