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Bilateral Fist Lid-Lift: A Novel Compensatory Behavior in an Infant with Blepharophimosis Syndrome
Biljana Kuzmanović Elabjer1,2,3, Daliborka Miletić1,2, Mirjana Bjeloš1,2,3
1Department of Ophthalmology, Sveti Duh University Hospital, 10000 Zagreb, Croatia.
Insights
An infant with blepharophimosis syndrome (BPES) used a unique compensatory behavior, lifting eyelids with fists, to improve vision. This novel bilateral fist lid-lift strategy resolved after surgery.
Area of Science:
- Ophthalmology
- Pediatric Medicine
- Developmental Neuroscience
Background:
- Severe bilateral congenital ptosis can significantly impair visual development in infants.
- Blepharophimosis syndrome (BPES) is a genetic disorder often associated with congenital ptosis.
- Compensatory mechanisms are crucial for infants to maintain visual function despite eyelid obstruction.
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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