Congenital Ptosis

Updated: Mar 03, 2017
  • Author: Donny W Suh, MD, FAAP; Chief Editor: Edsel Ing, MD, FRCSC  more...
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Overview

Background

A drooping eyelid is called ptosis or blepharoptosis. In ptosis, the upper eyelid falls to a position that is lower than normal. Severe ptosis may 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 le Chin-up posture due to congenital ptosis of the left eye.
Congenital ptosis of the left eye partially obstru Congenital ptosis of the left eye partially obstructing the left pupillary axis.
Congenital ptosis of the right eye. Congenital ptosis of the right eye.

Ptosis can affect one eye or both eyes and can present at birth or be more apparent later in childhood. Ptosis that is present at birth or within the first year of life is called congenital ptosis. Most cases of congenital ptosis are isolated and do not affect vision, but refractive errors and amblyopia should always be excluded. Any ptosis that develops over a period of days or weeks can signal a serious medical problem and needs further neurologic and physical evaluation.

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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. Some forms of congenital ptosis may represent a form of congenital cranial dysinnervation disorder. [3]

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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:

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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