Congenital Ptosis 

  • Author: Donny W Suh, MD, FAAP; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Mar 9, 2012
 

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 leChin-up posture due to congenital ptosis of the left eye. Congenital ptosis of the left eye partially obstruCongenital 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. 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.

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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.

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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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Contributor Information and Disclosures
Author

Donny W Suh, MD, FAAP  Clinical Assistant Professor, Pediatric Ophthalmology Service, University of Nebraska Medical Center; Pediatric Ophthalmologist, Adult Strabismus Specialist, Wolfe Eye Clinic, PC; Consulting Staff, Blank Children's Hospital, Mercy Medical Center of Des Moines, Iowa Methodist Hospital of Des Moines, and Marshalltown Medical Center

Donny W Suh, MD, FAAP is a member of the following medical societies: American Academy of Ophthalmology, American Academy of Pediatrics, and American Medical Association

Disclosure: Alcon Honoraria Speaking and teaching

Specialty Editor Board

Michael J Bartiss, OD, MD  Medical Director, Ophthalmology, Family Eye Care of the Carolinas

Michael J Bartiss, OD, MD is a member of the following medical societies: American Academy of Ophthalmology, American Academy of Pediatrics, American Association for Pediatric Ophthalmology and Strabismus, and North Carolina Medical Society

Disclosure: Nothing to disclose.

Simon K Law, MD, PharmD  Associate Professor of Ophthalmology, Jules Stein Eye Institute, University of California, Los Angeles, David Geffen School of Medicine

Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, and Association for Research in Vision and Ophthalmology

Disclosure: Nothing to disclose.

Mark T Duffy, MD, PhD  Consulting Staff, Division of Oculoplastic, Orbito-facial, Lacrimal and Reconstructive Surgery, Green Bay Eye Clinic, BayCare Clinic; Medical Director, Advanced Cosmetic Solutions, A BayCare Clinic

Mark T Duffy, MD, PhD is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, American Society of Ophthalmic Plastic and Reconstructive Surgery, Sigma Xi, and Society for Neuroscience

Disclosure: Allergan - Botox Cosmetic Honoraria Speaking and teaching

Lance L Brown, OD, MD  Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri

Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD  Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Additional Contributors

I would like to thank my family for their patience and unremitting support.

References
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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.
 
 
 
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