Background
A drooping eyelid is called ptosis or blepharoptosis. In ptosis, the upper eyelid falls to a position that is lower than normal. In severe cases of ptosis, the drooping eyelid can cover part or all of the pupil and interfere with vision, resulting in amblyopia.[1, 2] Note the images below:
Chin-up posture due to congenital ptosis of the left eye. 
Congenital ptosis of the left eye partially obstructing the left pupillary axis. 
Congenital ptosis of the right eye. Ptosis can affect one eye or both eyes. Ptosis may be present at birth, or it may develop later in life. If a droopy eyelid is present at birth or within the first year of life, the condition is called congenital ptosis. In most cases of congenital ptosis, the problem is isolated and does not affect the vision. Any ptosis that develops over a period of days or weeks can signal a serious medical problem and needs further neurologic and physical evaluation.
Pathophysiology
The eyelids are elevated by the contraction of the levator palpebrae superioris.
In most cases of congenital ptosis, a droopy eyelid results from a localized myogenic dysgenesis. Rather than normal muscle fibers, fibrous and adipose tissues are present in the muscle belly, diminishing the ability of the levator to contract and relax. Therefore, the condition is commonly called congenital myogenic ptosis.
Congenital ptosis can also occur when the innervation to the levator is interrupted through neurologic or neuromuscular junction dysfunction.
Epidemiology
Frequency
United States
The frequency of congenital ptosis in the United States has not been officially reported. However, in approximately 70% of known cases, congenital ptosis affects only one eye.
International
The incidence rate of congenital ptosis worldwide is unknown.
Mortality/Morbidity
If congenital ptosis obscures any part of the pediatric patient's visual field, surgery must be performed to correct the problem early in life. Otherwise, a permanent loss of vision may occur as a result of amblyopia. Note the following:
- Occlusion amblyopia
- Astigmatism from the compression of the droopy eyelid
- Ocular torticollis
Race
Congenital ptosis occurs equally among the different races.
Sex
Congenital ptosis occurs equally between males and females.
Age
Congenital ptosis is usually present at birth but may manifest within the first year of life.
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