Congenital Ptosis in a Young Boy

The parents of a 3-year-old boy wereconcerned about their son's "droopy"right eyelid; the abnormality hadbeen present since birth. All other aspectsof the child's eyes and his generaldevelopment had been normal.

Congenital ptosis was diagnosed.This complex condition resultsfrom a dystrophic levator musclethat is fibrotic and deficient instriated muscle fibers.¹ This disorderis unilateral in up to 70% of patients²and may be associated with strabismus,amblyopia, or anisometropic refractiveerror. Other related anomaliesinclude ipsilateral superior rectuspalsy, double elevator palsy,jaw-winking phenomenon, or blepharophimosis(ptosis, epicanthus inversus,and telecanthus). This patienthad none of these problems.

When you suspect congenitalptosis, ask the child to stare straightahead; determine whether the lid coversthe pupil. If it does, the child willnot use the eye and occlusion amblyopiacan develop. The ptotic eyelid remainselevated on downward gaze only.

Children with congenital ptosisoften exhibit a poorly developedupper eyelid crease, which indicatesan extremely weak levator muscle. Toassess levator function, have the childlook up and down; the ptotic eyelidwill remain slightly higher than thenormal eyelid because of structuralchanges that prevent the lid from relaxingon downward gaze.²

Surgery to correct congenitalptosis is usually performed betweenages 4 and 5 years, before the childstarts school.³Corrective surgery ata younger age is appropriate for childrenwho:

• Are at risk for amblyopia becausethe lid covers the center of the pupil.
• Constantly compensate with a "chinup"head position. Children with bilateralptosis usually do this.
• Have significant disfigurement.This patient's mildly ptotic rightupper eyelid is being monitored; surgicalrepair will be done before heenters school.

References:

REFERENCES:1. Simons BD, Tse DT. Congenital ptosis. In: ParrishRK, ed. The University of Miami Bascom PalmerEye Institute’s Atlas of Ophthalmology. Philadelphia:Butterworth-Heinemann; 2000:612-620.
2. Walsh TJ. Ptosis. In: Walsh TJ, ed. Neuro-Ophthalmology:Clinical Signs and Symptoms. 3rd ed. Philadelphia:Lea & Febiger; 1992:108-123.
3. Hecht SD. Blepharoptosis: etiology and diagnosticevaluation. In: Stewart WB, ed. Ophthalmic Plasticand Reconstructive Surgery. San Francisco: AmericanAcademy of Ophthalmology; 1984:160-163.