A novel “Reflexive Fist Lid-Lift” to compensate for severe upper lid congenital ptosis in Blepharophimosis Syndrome
Author: Biljana Kuzmanović Elabjer
Base Hospital / Institution: University Eye Department, University Hospital „Sveti Duh”, Zagreb, Medical Faculty Osijek, Faculty of dental medicine and health Osijek, J.J. Strossmayer University of Osijek
ePoster presentation
Abstract ID: 25-118
Purpose
Describe a novel compensatory mechanism for severe bilateral congenital upper eyelid ptosis in Blepharophimosis Syndrome (BPES).
Methods
A 4.5-month-old female infant with hereditary BPES was referred for evaluation of severe bilateral congenital upper eyelid ptosis.
On examination, the infant exhibited blepharophimosis with a horizontal palpebral aperture measuring only 15 mm, severe upper eyelid ptosis with a vertical palpebral aperture of less than 2 mm, and an epicanthus inversus.
Frontalis muscle overaction and chin elevation were insufficient to compensate for the profound ptosis. Notably, the infant employed an unusual compensatory strategy: she used the dorsal side of her fists to manually lift both upper eyelids and maintained this position while fixating on the examiner’s face. This adaptive maneuver, which we have termed the “Reflexive Fist Lid-Lift,” has not been previously described in the literature.
Results
At five months of age, she underwent bilateral upper eyelid ptosis correction with frontalis suspension using a silicone rod. The procedure was uneventful, and in the postoperative period, the use of the “Reflexive Fist Lid-Lift” ceased entirely, confirming its role as a transient compensatory mechanism.
Conclusion
To the best of our knowledge, this is the first report documenting a novel compensatory mechanism in severe bilateral congenital upper eyelid ptosis associated with BPES. The “Reflexive Fist Lid-Lift” highlights the adaptive responses in infants with extreme visual deprivation due to ptosis and underscores the importance of early surgical intervention to restore functional vision.
Additional Authors
| First name | Last name | Base Hospital / Institution |
|---|---|---|
| Daliborka | Miletić | University Eye Department, University Hospital „Sveti Duh”, Zagreb, Medical Faculty Osijek, Faculty of dental medicine and health Osijek, J.J. Strossmayer University of Osijek |
| Mirjana | Bjeloš | University Eye Department, University Hospital „Sveti Duh”, Zagreb, Medical Faculty Osijek, Faculty of dental medicine and health Osijek, J.J. Strossmayer University of Osijek |
| Mladen | Bušić | University Eye Department, University Hospital „Sveti Duh”, Zagreb, Medical Faculty Osijek, Faculty of dental medicine and health Osijek, J.J. Strossmayer University of Osijek |
| Iva | Bulat | University Eye Department, University Hospital „Sveti Duh”, Zagreb, Medical Faculty Osijek, Faculty of dental medicine and health Osijek, J.J. Strossmayer University of Osijek |