eMedicine Specialties > Ophthalmology > Lid

Ptosis, Congenital: Treatment & Medication

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
Contributor Information and Disclosures

Updated: Nov 6, 2007

Treatment

Medical Care

Observation is only required in mild cases of congenital ptosis if no signs of amblyopia, strabismus, and abnormal head posture are present.

  • Depending on the severity, patients should be monitored every 3-12 months for signs of amblyopia due to congenital ptosis. External photographs can be helpful in monitoring patients.
  • Head posture should be carefully examined. If the patient acquires a chin-up posture due to the worsening of ptosis, surgery may be indicated.
  • The patient should be checked for astigmatism due to the compression of the droopy eyelid.

Surgical Care

Congenital ptosis has physical, functional, and psychological consequences. The method of repair depends on treatment goals, the underlying diagnosis, and the degree of levator function. Although the primary reason for the repair is functional, the surgeon has an opportunity through this procedure to produce symmetry in lid height, contour, and eyelid crease for better cosmesis.

Surgical correction of congenital ptosis can be undertaken at any age depending on the severity of the disease. Earlier intervention may be required if significant amblyopia or ocular torticollis is present. Severe cases of ocular torticollis may delay mobility in infants and toddlers because of the balance problems from extreme chin-up head posture. If intervention is not urgent, surgery is often delayed until age 3-4 years. Waiting until this age allows for more accurate measurements preoperatively.

Surgery for ptosis in patients with a history of dry eyes, seventh cranial nerve palsy, or significant extraocular muscle abnormalities, such as severe Graves ophthalmopathy, double elevator palsy, or progressive external ophthalmoplegia, should be approached with great caution to avoid exposure keratopathy following the surgery.

  • Levator muscle resection
    • This procedure is the shortening of the levator-aponeurosis complex through a lid-crease incision. The skin incision is hidden either in the existing lid fold or in a new lid fold created to match that of the contralateral eyelid.
    • Indications: Moderate levator function must be present to offer a chance for correction with a levator resection. If the levator function is greater than 4 mm but less than 6 mm, a levator resection of greater than or equal to 22 mm is recommended. If the levator function is 6-8 mm, a levator resection of 16-18 mm is indicated. If the levator function is greater than 8 mm, a levator resection of 10-13 mm is indicated.
    • Contraindications: An external levator resection is not indicated when the levator function is less than 4 mm. In such cases, a long-term surgical outcome may result in undercorrection. Poor Bell phenomenon (limited elevation of the eye), reduced corneal sensitivity, or poor tear production can produce exposure keratopathy.
  • Frontalis suspension procedure
    • This procedure is designed to augment the patient's lid elevation through brow elevation. Frontalis suspension procedures produce lagophthalmos in most cases. Some surgeons prefer to perform a bilateral suspension procedure for severe unilateral congenital ptosis to obtain symmetry.
    • Indications: The procedure is indicated when the levator function is less than 4 mm.
    • Contraindications: Poor Bell phenomenon (limited elevation of the eye), reduced corneal sensitivity, or poor tear production can produce exposure keratopathy. If surgery is still indicated, these patients need close postoperative follow-up care.
    • Surgical technique: Several materials are available to secure the lids to the frontalis muscles. These materials include autogenous fascia lata, preserved (tissue bank) fascia, nonabsorbable suture material (eg, 2-0 Prolene or Mersilene), silicone bands, silicone rods, and Gore-Tex. Autogenous materials used less frequently include palmaris longus tendon and temporalis fascia. Autogenous fascia lata can be obtained from the leg of patients older than 3 years.
    • Surgical outcome: Patients may not be able to close their eyelids during sleep from a few weeks to several months following surgery. Families must be warned of this outcome before the operation. The problem of open lids during sleep improves with time; however, aggressive lubrication is needed to avoid exposure keratopathy.
  • Fasanella-Servat procedure
    • The upper lid is elevated by removing a block of tissue from the underside of the lid. This tissue includes the tarsus, conjunctiva, and Müller muscle.
    • This procedure is not commonly performed for cases of congenital ptosis.
  • Müller muscle–conjunctival resection
    • This surgery is chosen if the eyelid has had a good response to phenylephrine.
    • The conjunctiva and the Müller muscle are marked off, clamped, and sutured. The tissues are resected. Then, the conjunctival layer is closed.
    • This procedure is not commonly performed for cases of congenital ptosis.

Consultations

Patients with congenital ptosis may have other conditions that need to be addressed. These conditions include amblyopia, strabismus, craniofacial abnormalities, and other neurologic findings. Appropriate consultation may be needed depending on the associated findings.

  • Pediatric ophthalmologist
  • Pediatric oculoplastic service
  • Pediatric neurologist
  • Cardiologist (if mitochondrial disorder suspected)

Diet

Normal

Activity

As tolerated

More on Ptosis, Congenital

Overview: Ptosis, Congenital
Differential Diagnoses & Workup: Ptosis, Congenital
Treatment & Medication: Ptosis, Congenital
Follow-up: Ptosis, Congenital
Multimedia: Ptosis, Congenital
References

References

  1. Bagheri A, Aletaha M, Saloor H, Yazdani S. A randomized clinical trial of two methods of fascia lata suspension in congenital ptosis. Ophthal Plast Reconstr Surg. May-Jun 2007;23(3):217-21. [Medline].

  2. Bergin DJ. Management and surgery of congenital and acquired ptosis. Continuing Ophthalmic Video Education. 1990.

  3. Bernardini FP, Devoto MH, Priolo E. Treatment of unilateral congenital ptosis. Ophthalmology. Mar 2007;114(3):622-3. [Medline].

  4. Clark BJ, Kemp EG, Behan WM, Lee WR. Abnormal extracellular material in the levator palpebrae superioris complex in congenital ptosis. Arch Ophthalmol. Nov 1995;113(11):1414-9. [Medline].

  5. Guercio JR, Martyn LJ. Congenital malformations of the eye and orbit. Otolaryngol Clin North Am. Feb 2007;40(1):113-40, vii. [Medline].

  6. Malone TJ, Nerad JA. The surgical treatment of blepharoptosis in oculomotor nerve palsy. Am J Ophthalmol. Jan 15 1988;105(1):57-64. [Medline].

  7. Meyer DR, Rheeman CH. Downgaze eyelid position in patients with blepharoptosis. Ophthalmology. Oct 1995;102(10):1517-23. [Medline].

  8. Wabbels B, Schroeder JA, Voll B, Siegmund H, Lorenz B. Electron microscopic findings in levator muscle biopsies of patients with isolated congenital or acquired ptosis. Graefes Arch Clin Exp Ophthalmol. Oct 2007;245(10):1533-41. [Medline].

  9. Weinberg DA, Lesser RL, Vollmer TL. Ocular myasthenia: a protean disorder. Surv Ophthalmol. Nov-Dec 1994;39(3):169-210. [Medline].

  10. Yilmaz N, Hosal BM, Zilelioglu G. Congenital ptosis and associated congenital malformations. J AAPOS. Jun 2004;8(3):293-5. [Medline].

Further Reading

Keywords

congenital ptosis, drooping of the upper eyelid, droopy eyelid, droopy lid, droopy eye, congenital myogenic ptosis, congenital aponeurotic ptosis, congenital neurogenic ptosis, localized myogenic dysgenesis, amblyopia, strabismus, blepharoptosis

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, American Medical Association, and Iowa Medical Society
Disclosure: Nothing to disclose.

Medical Editor

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, American College of Surgeons, and North Carolina Medical Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Simon K Law, MD, PharmD, Assistant Professor of Ophthalmology, Jules Stein Eye Institute; Chief of Section of Ophthalmology Surgical Services, Department of Veterans Affairs Healthcare Center, West Los Angeles
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.

Managing Editor

Mark T Duffy, MD, PhD, Consulting Staff, Division of Oculoplastic, Orbito-facial, Lacrimal, and Reconstructive Surgery, Green Bay Eye Clinic, 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 Consulting fee Consulting; Quest medical - lacrimal balloons Honoraria Speaking and teaching

CME Editor

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.

 
 
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