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Congenital Ptosis


The parents of a 3-year-old boy were concerned about their son's "droopy" right eyelid; the abnormality had been present since birth.

The parents of a 3-year-old boy were concerned about their son's "droopy" right eyelid; the abnormality had been present since birth. All other aspects of the child's eyes and his general development had been normal.

Congenital ptosis was diagnosed. This complex condition results from a dystrophic levator muscle that is fibrotic and deficient in striated muscle fibers.1 This disorder is unilateral in up to 70% of patients2 and may be associated with strabismus, amblyopia, or anisometropic refractive error. Other related anomalies include ipsilateral superior rectus palsy, double elevator palsy, jaw-winking phenomenon, or blepharophimosis (ptosis, epicanthus inversus, and telecanthus). This patient had none of these problems.

When you suspect congenital ptosis, ask the child to stare straight ahead; determine whether the lid covers the pupil. If it does, the child will not use the eye and occlusion amblyopia can develop. The ptotic eyelid remains elevated on downward gaze only.

Children with congenital ptosis often exhibit a poorly developed upper eyelid crease, which indicates an extremely weak levator muscle. To assess levator function, have the child look up and down; the ptotic eyelid will remain slightly higher than the normal eyelid because of structural changes that prevent the lid from relaxing on downward gaze.2

Surgery to correct congenital ptosis is usually performed between ages 4 and 5 years, before the child starts school.3 Corrective surgery at a younger age is appropriate for children who:

  • Are at risk for amblyopia because the lid covers the center of the pupil.
  • Constantly compensate with a "chin- up" head position. Children with bilateral ptosis usually do this.
  • Have significant disfigurement.

This patient's mildly ptotic right upper eyelid is being monitored; surgical repair will be done before he enters school.

REFERENCES:1. Simons BD, Tse DT. Congenital ptosis. In: Parrish RK, ed. The University of Miami Bascom Palmer Eye 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 diagnostic evaluation. In: Stewart WB, ed. Ophthalmic Plastic and Reconstructive Surgery. San Francisco: American Academy of Ophthalmology; 1984:160-163.

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